UNIV ERSIT Y O F IB ADAN L IB RARY ACKNOWLEDGEMENTS The first edition of the Nigerian Standard Treatment Guidelines is a product of the support, recommendations and contributions of the following: Prof. Eyitayo Lambo Prof. Adenike Grange Mr. R.K. Omotayo mni Mr. J.E. B. Adagadzu Dr Peter Eriki Dr Mohammed Belhocine Dr. Olaokun Soyinka Dr Ogori Taylor Federal Ministry Of Health World Health Organization Expert Committee Members Prof. Abdu-Aguye, I. Prof. Adelowo, F. Prof. Bayeroju-Agbeja, A. Prof. Borodo, M. Prof. Danbauchi, S.S Prof. Gureje, O. Prof. Idoko, J. Prof. Isah, A.O. Prof. Mabadeje, A.F.B. Prof. Mbonu, O. Prof. Ogisi, F. Prof. Ohwovoriole, A. Prof. Olumide, M. Prof. Ojogwu, L.I. Prof. Okonofua, F. Prof. Oruamabo, R. Prof. Oviawe, O Dr. Ameh, E. Dr. Abosede, Y. Dr. Akoria, O. Dr. Irhibhogbe, P. Dr. Kehinde, M. Dr. Mero-Asagba, G. Dr. Okpapi, J.U. Dr. Savage, S. Dr. Taylor, O. Mr. Obiaga, G.O. International Network for Rational Use of Drugs (INRUD) - Nigerian Core Group Editorial Team Prof. Abdu-Aguye, I. Prof. Isah, A.O. Prof. Mabadeje, A.F.B. Dr. Abosede, O.A. Dr. Aina, B.A. Dr. Akinyede, A. Dr. Akoria, O.A. Dr. Asalu, A.F. Dr. Akinlekan, J. Mr. Obiaga, G. Dr. Obodo, J.O. Mr. Okoko, O.O. Dr. Oparah, A. Dr. Olayemi, S.O. Dr. Oreagba, I. Mr. Otahabru, B. Dr. Mero-Asagba, G. Dr. Taylor, O. Prof. Abdu-Aguye, I. Prof. Isah, A.O. Dr. Akoria, O.A. Dr. Mero-Asagba, G. Mr. Obiaga, G.O. Mrs. Okpeseyi, M.I. Mr. Fagbemiro, L.O. Mr. Otahabru, B. Dr. Olaokun Soyinka Dr. Taylor, O. European Commission For funding the programme © 2008 Federal Ministry of Health, Nigeria All rights reserved. No part of this publication may be reproduced, stored in retrieval system or transmitted in any form or by any means, electronic, mechanical, photocopying, recording and/or otherwise, without prior written permission of the Federal Ministry of Health, Nigeria. For all enquiries or comments, write to the publishers: The Honourable Minister, Federal Ministry of Health, Federal Secretariat Complex, Shehu Shagari Way, P.M.B. 080 Garki, Abuja. Nigeria Printed in Nigeria. UNIV ERSIT Y O F IB ADAN L IB RARY FOREWORD I am indeed very pleased to write the foreword to this maiden edition of the Standard Treatment Guidelines (STG) for the Nigerian health care system. I am aware that the process of its production began in 2005 involving contributions and recommendations of various experts and stakeholders in the health care sector. The STG is an important tool for the attainment of comprehensive and effective health care delivery services thereby achieving the goals of the National Drug Policy, which inter alia are: the availability of safe, efficacious and affordable medicines to satisfy the healthcare needs of the majority of the population and ensure the rational use of drugs. The fulfillment of the above mentioned goals is part of the strategic thrust of the Health Sector Reform Programme aimed at the reduction of disease burden and the improvement of access to quality health services. It is expected that the STG will become a major reference document for all health workers both in the public and private sectors. It is instructive to note that the development of the STG followed due process with wide consultations and meetings involving various stakeholders and interest groups. The document that has come out of this process is a reflection of the quality of the inputs that went into its development. In my opinion, this maiden edition of the STG has been produced and serialized in such a way as to assist health care providers especially doctors in the effective discharge of their duties as prescribers. It will also ensure discipline as only those medicines recommended will be prescribed for patients within a given health facility. I commend all those who worked tirelessly towards the completion of this maiden edition STG. Special mention and gratitude must go to the World Health Organization (WHO) for sponsoring and providing sustained technical support to the committee. Without this support, this STG would not have seen the light of the day. Finally, let me quickly add that this STG must be widely circulated and disseminated. Everything possible must be done to ensure that practitioners maximize the benefit of such a useful document. If it has worked in other parts of the world, it should also work in Nigeria. It must also be subjected to regular reviews in view of the dynamic nature of health care management. Dr. Hassan Muhammed Lawal, CON Supervising Minister of Health UNIV ERSIT Y O F IB ADAN L IB RARY UNIV ERSIT Y O F IB ADAN L IB RARY SECTION A Chapter 1: Alimentary Tract Chapter 2: Blood And Blood-forming Organs Chapter 3: Cardiovascular System Gastrointestinal Disorders ...................................1 Amoebiasis ..........................................................1 Bacillary Dysentry ................................................1 Cholera ................................................................2 Constipation .........................................................2 Diarrhoea (acute) .................................................3 Gastritis ................................................................4 Giardiasis .............................................................4 Haemorrhoids ......................................................5 Pancreatitis ..........................................................5 Peptic Ulcer Disease ...........................................6 Upper Gastrointestinal Bleedin ............................6 Hepatic And Biliary Disorders ............................. 7 Hepatitis.. .............................................................7 Hepatic Encephalopathy ......................................8 Jaundice ..............................................................9 Liver Cirrhosis ......................................................9 Nutritional Disorders ..........................................10 Kwashiokor And Marasmus ...............................10 Micronutrient Deficiencies ..................................10 Obesity................................................................11 Anaemias ...........................................................13 Blood Transfusion ..............................................14 Haemostasis And Bleeding Disorders ...............16 Leukaemias .......................................................16 Lymphomas .......................................................19 Sickle Cell Disease.............................................20 Angina Pectoris..................................................23 Cardiac Arrhythmias ..........................................23 Congenital Heart Disease .................................24 Deep Venous Thrombosis .................................24 Heart Failure .....................................................25 Hyperlipidaemia ................................................26 Hypertension .....................................................26 Infective Endocarditis ........................................27 Myocardial Infarction..........................................29 Myocarditis ........................................................30 Paediatric Cardiac Disorders.............................30 Pericarditis ........................................................30 Pulmonary Embolism ........................................31 Pulmonary Oedema ..........................................32 Rheumatic Fever ..............................................32 Rheumatic Heart Disease .................................33 Standard Treatment Guidelines for Nigeria 2008 i PREFACE This first edition of Standard Treatment Guidelines (STG) for the Nigerian health practitioner is coming relatively later than those of many other countries. It is indeed a welcome development. The standard of medical practice and the wage bill of health services are usually remarkably improved by health personnel putting to use STG. This among other benefits can only lead to improved health of the community. In Nigeria our health indices are among the worst in the world. Our country Nigeria does not lack the manpower or the necessary infrastructure to turn things around. What appears to be lacking is the organization of health services required to put both to optimal use. Efforts such as the actualization of our own national STG and the various health reforms currently in progress will definitely improve our situation. It is therefore my pleasure and privilege to write the preface to this maiden edition of the STG. This is the outcome of a long journey that started several years ago. The previous chairmen of the National Formulary and Essential Drugs Review Committees made efforts to start the project but were unsuccessful due to lack of funds. The current committee had the luck of being assisted by the country office of the World Health Organization (WHO) in not only this endeavor but in the preparation and printing of the last edition of the Nigerian Essential Medicines List. The desk officer, Dr Ogori Taylor showed great commitment to the project and the country owes a debt of gratitude to WHO. In preparing this document every effort was made to ensure that the stakeholders own the project so that it is not seen as an imposition. Accordingly, the major contributions came from various practitioners and their associations as well as from many practitioners whose input were judged crucial to the success of the project. We also adopted the acceptable practices in the field that were in use by special health projects such as HIV/AIDS, Malaria, TB/Leprosy programmes etc. The academia was also involved. There were several fora where the contributions were discussed openly with the stakeholders and consensus arrived at. It is my hope therefore that this document will be widely used by Nigerian health practitioners. I salute the contributors and those that helped in one way or the other. The committee of course accepts responsibility for any lapses but also hopes that these would be brought to our attention for correction in subsequent editions. Professor Ibrahim Abdu-Aguye,MBBS; FMCP;SFIAM; FIICA;D. Sc (Hon) Chairman, National Formulary and Essential Drugs Review Committee. Chapter 4: Central Nervous System Chapter 5: Dental And Oral Disorders Chapter 6: Dermatology Non-psychiatric Disorders ..................................34 Dizziness ............................................................34 Headaches..........................................................35 Meningitis ...........................................................36 Migraine ..............................................................37 Parkinsonism.......................................................38 Seizures/epilepsies .............................................39 Stroke .................................................................41 Syncope ............................................................42 The Unconscious Patient ....................................42 Psychiatric Disorders ..........................................43 Alcoholism (alcohol Dependence) ......................43 Anxiety Disorder .................................................44 Bipolar Disorders ................................................44 Delirium...............................................................45 Depression .........................................................46 Insomnia..............................................................47 Panic Disorder.....................................................47 Schizophrenia......................................................48 Acute Necrotizing Ulcerative Gingivitis................49 Acute Periapical Abscess ...................................49 Alveolar Osteitis ..................................................50 Cellulitis...............................................................50 Dental Caries ......................................................50 Gingivitis .............................................................51 Neoplasms Of The Oral Cavity ...........................51 Oral Thrush (candidiasis) ...................................51 Pericoronitis ........................................................52 Periodontitis.........................................................52 Pulpitis ................................................................53 Salivary Gland Diseases ....................................54 Temporo-mandibular Joint Disorders...................54 Bacterial Infections .............................................56 Cellulitis ..............................................................56 Furunculosis (boils) ............................................56 Impetigo Contagiosa ..........................................57 Dermatitis And Eczema ......................................58 Atopic Dermatitis (atopic Eczema)......................58 Contact Dermatitis ..............................................59 Exfoliative Dermatitis (erythroderma)..................59 Parasitic Dermatoses .........................................60 Cutaneous Larva Migrans (creeping Eruption).............................................................61 TABLE OF CONTENTS UNIV ERSIT Y O F IB ADAN L IB RARY Guinea Worm Disease (dracunculiasis)..............61 Myiasis ...............................................................62 Onchocerciasis (river Blindness) .......................62 Pediculosis (lice) ................................................63 Scabies ..............................................................64 Papulosquamous Disorders ..............................65 Lichen Planus ....................................................65 Pityriasis Rosea .................................................66 Psoriasis ............................................................67 Superficial Fungal Infections ..............................69 Dermatophyte Infections (tinea) .........................69 Pityriasis Versicolor (tinea Versicolor ) ...............70 Viral Infections ...................................................71 Herpes Zoster ....................................................71 Molluscum Contagiosum ...................................72 Varicella (chickenpox) ........................................72 Viral Warts (verrucae) ........................................73 Miscellaneous Disorders ....................................74 Acne Vulgaris (pimples) .....................................74 Pruritus ..............................................................76 Urticaria And Angioedema .................................78 Vitiligo ................................................................80 Acute Otitis Media...............................................81 Adenoid Disease ................................................82 Chronic Otitis Media ...........................................82 Epistaxis .............................................................83 Foreign Bodies In The Airways ..........................83 Foreign Bodies In The Ear .................................84 Foreign Bodies In The Nose And Rhinoliths.......84 Mastoiditis...........................................................84 Nasal Allergy ......................................................85 Otitis Externa ......................................................86 Peritonsillar Abscess (quinsy) ............................86 Pharyngitis (sore Throat) ...................................86 Sinusitis ..............................................................87 Tonsillitis .............................................................88 Tracheostomy ....................................................89 Wax In The Ear ..................................................89 Diabetes Mellitus ................................................90 Hyperthyroidism (thyrotoxicosis) ........................97 Hypothyroidism (myxoedema) ...........................99 Acute Anterior Uveitis (iritis) .............................100 Acute Keratitis ..................................................100 Allergic Conjunctivitis .......................................101 Eye Injuries ......................................................101 Foreign Bodies In The Eye ...............................102 Infective Conjunctivitis ......................................103 Ophthalmia Neonatorum ..................................103 Scleritis / Episcelitis .........................................104 Stye (hordeolum) .............................................104 Chapter .7: Ear, Nose And Throat Chapter 8: Endocrine System Chapter 9: Eye Disorders Chapter 13: Obstetrics And Gynaecology Chapter 14: Respiratory System Chapter 15: Injuries And Acute Trauma Chapter 16: Surgical Care And Associated Disorders Chapter 17: Paediatric Perspectives Abortion ..........................................................149 Antenatal Care ................................................150 Anaemia In Pregnancy ...................................152 Cancer Of The Cervix .....................................153 Cardiac Disease In Pregnancy .......................154 Eclampsia .......................................................156 Ectopic Pregnancy ..........................................158 Hyperemesis Gravidarum ...............................159 Immunization Schedules .................................160 Jaundice In Pregnancy ...................................160 Pelvic Inflammatory Disease ..........................162 Rape ................................................................163 Acute Epiglottitis ..............................................165 Acute Laryngo-tracheo-bronchitis (croup) .......165 Acute Rhinitis (common Cold) ........................166 Bronchial Asthma ...........................................166 Bronchiectasis ................................................167 Chest Pain ......................................................168 Chronic Obstructive Airways Disease(coad) ....168 Cough .............................................................169 Dyspnoea .......................................................170 Lung Abscess .................................................170 Pneumonia .....................................................171 Pulmonary Embolism .....................................172 Bites And Stings ..............................................173 Burns ..............................................................175 Disaster Plan ..................................................176 Head Injury .....................................................177 Multiple Injuries ...............................................180 Acute Abdomen ..............................................182 Antimicrobial Prophylaxis In Surgery ...............184 Intestinal Obstruction ......................................184 Preoperative Evaluation and Postoperative Care ..........................................186 Use Of Blood Transfusion In Surgery ..............189 Measles (rubeola) ............................................190 Poliomyelitis .....................................................191 Vitamin A Deficiency .......................................193 Section B Standard Treatment Guidelines for Nigeria 2008 Standard Treatment Guidelines for Nigeria 2008 ii iii The Red Eye ....................................................104 Trachoma..........................................................105 Xerophthalmia ..................................................105 Nephrology ......................................................106 Acute Renal Failure..........................................106 Chronic Kidney Disease ..................................106 Nephrotic Syndrome ........................................107 Sexually Transmitted Infections ......................108 Bacterial Vaginosis ..........................................108 Chancroid (ulcus Molle, Soft Chancre) ............109 Chlamydial Infection ........................................110 Gonorrhoea .....................................................111 Granuloma Inguinale (donovanosis; Granuloma Venereum)......................................113 Lymphogranuloma Venereum ..........................114 Syphilis ............................................................115 Trichomoniasis ................................................116 Vulvo-vaginal Candidiasis ...............................117 Urology ............................................................118 Benign Prostatic Hyperplasia ..........................118 Carcinoma Of The Prostate ............................118 Erectile Dysfunction (impotence) ....................119 Male Infertility ..................................................120 Posterior Urethral Valves ................................120 Priapism ........................................................121 Prostatitis ........................................................121 Scrotal Masses ...............................................122 Torsion Of The Testis ......................................122 Urethral Stricture ............................................123 Urinary Schistosomiasis .................................123 Urinary Tract Calculi .......................................124 Fevers: Management Approach ......................125 Food Poisoning ...............................................125 Helminthiasis ..................................................127 Human Immunodeficiency Virus Infection ......129 Malaria ............................................................135 Rabies...............................................................137 Tetanus ...........................................................138 Trypanosomiasis (sleeping Sickness) .............140 Tuberculosis ....................................................140 Typhoid Fever .................................................142 Back Pain ........................................................143 Gout ................................................................144 Osteoarthritis ..................................................145 Rheumatoid Arthritis .......................................146 Septic Arthritis .................................................147 Systemic Lupus Erythematosus .....................148 Chapter 10: Genito-urinary System Chapter 11: Infectious Diseases / infestations Chapter 12: Musculoskeletal System Section C Chapter 18: Emergencies Chapter 19: Therapeutics Chapter 20: Notifiable Diseases ....................209 Acute Left Ventricular Failure ...........................195 Cardiac Arrest ..................................................196 Drowning And Near-drowning ..........................197 Electrolyte Abnormalities .................................198 Hypertensive Emergencies ...............................200 Hypoglycemia ...................................................200 Myxoedema Coma ..........................................201 Thyroid Storm (thyrotoxic Crisis) .....................201 Poisoning .........................................................202 Prescription Writing .........................................206 Adverse Drug Reactions ..................................208 APPENDICES Appendix I Appendix II: Appendix III Appendix IV: Appendix V: Appendix VI: WHO clinical staging of HIV for infants and children with established HIV infection............211 WHO new antenatal care model classifying form 2001.........................................................212 Calculation of dosage requirements in children............................................................214 Medicines with teratogenic potential................215 Medicines that could cause harm when administered to breastfeeding mothers.............215 NAFDAC Adverse Drug Reaction Reporting form ................................................................217 UNIV ERSIT Y O F IB ADAN L IB RARY CHAPTER 1: ALIMENTARY TRACT Amoebic dysentery Amoebic abscess Chronic Carriers Amoebic dysentery Amoebic liver abscess GASTROINTESTINAL DISORDERS A common parasitic infection of the gastrointestinal system caused by the protozoan It may present as: Fever/chills Liver abscess: swelling, pain in the right sub-costal area Intracranial space-occupying lesion Lungs: cough and blood stained sputum Amoeboma: swelling anywhere in the abdomen Anal ulceration: may occur by direct extension from the intestinal infection Symptom-free Bacillary dysentery Any other cause of bloody diarrhoea Cancer of the liver Other causes of liver enlargement Rupture of abscess into the lungs, peritoneum Space-occupying lesion in the brain Right inguinal mass Stool: microscopy for cysts and motile organisms (amoebic dysentery) Full Blood Count Chest radiograph (in amoebic liver abscess) Abdominal Ultrasound Scan Rehydrate adequately Eradicate the protozoa Correct dehydration (see section on rehydration) Metronidazole 800 mg 8 hourly for 5 days 30 mg/kg/day in 3 divided doses for 5 days Metronidazole 800 mg 8 hourly for 10 days 50 mg/kg/day in 3 divided doses for 7-10 days Aspiration is indicated to prevent spontaneous rupture of abscesses. AMOEBIASIS Introduction Clinical features Differential diagnoses Complications Investigations Treatment objectives Drug treatment Non-drug treatment Entamoebahistolytica Adult: Child: Adult: Child: Acquired through faeco-oral transmission. Persistent mucoid / bloody diarrhoea Abdominal pain This can occur in any of the following forms as a result of spread via the blood stream: diarrhoeas resulting from bacterial infection are usually self-limiting. Appropriate systemic antibiotics are however required when systemic infections occur. - Amoxicillin 500 mg 8 hourly for 5 days Or: - Cotrimoxazole 960 mg 12 hourly for 3-5 days Or: - Ciprofloxacin 500 mg - 1 g orally 12 hourly for 5 days - Azithromycin 500 mg daily for 3 days for resistant strains Ciprofloxacin may induce tendinitis especially in children . Ciprofloxacin is not recommended for use in children less than 18 years. Antidiarrhoeal medicines are not advised. Safe drinking water Sanitary disposal of human waste material An acute severe diarrhoeal illness of worldwide importance; endemic in many developing countries. Caused by bacilli (classical and species) Excessive secretion of fluid is mediated by the release of enterotoxin (released by the bacilli), which acts on the enterocytes of the small intestine via cyclicAMP. Highly infectious; spread by faeco-oral route. Mild watery diarrhoea Severe life-threatening diarrhoea leading to hypovolaemic shock if untreated Occasionally, vomiting Hypovolaemic shock with multiple end organ failure leading to death Hypoglycaemia Paralytic ileus Stool microscopy, culture and sensitivity Full Blood Count Urea, Electrolytes and Creatinine Rehydrate adequately and rapidly Eradicate the infective organism Prevent spread of the infection Intravenous Ringer's lactate/Darrow's solutions Oral Rehydration Therapy Antibiotic therapy Tetracycline: 500 mg orally every 6 hours for 5 days Notable adverse drug reactions Precaution Prevention CHOLERA Introduction Clinical features Complications Investigations Treatment objectives Drug treatment Vibrio cholerae El Tor . Adult: Consult a surgeon. Treat cyst carrier if patient is a food handler: Diloxanide furoate 500 mg every 8 hours for 10 days 20 mg/kg orally every 8 hours for 10 days Metronidazole is contraindicated in pregnancy. Avoid alcohol during treatment and at least 48 hours after treatment. Provision of safe drinking water Sanitary disposal of faeces Regular examination of food handlers and appropriate treatment where necessary. An important cause of colonic diarrhoea in developing countries. Caused by pathogenic species of Shigella A-D ( ) Transmitted via the faeco-oral route. Mucoid bloody diarrhoea associated with severe central and lower abdominal pain Tenesmus Moderate-grade pyrexia Sometimes only a mild, self-limiting diarrhoea lasting 2- 3 days Articular features occasionally Septicaemic spread with multi-system involvement occasionally. Amoebic dysentery Idiopathic enterocolits (ulcerative) infection Colorectal cancer Septicaemia/bacteraemia Severe rectal bleeding Intestinal perforation Reiter's syndrome Stool microscopy, culture and sensitivity Full Blood Count Urea, Electrolytes and Creatinine Adequate rehydration Eradicate bacterial pathogens Oral Rehydration Therapy (see rehydration under diarrhoea) Parenteral hydration therapy (see rehydratrion under diarrhoea) Antibacterial drugs are not usually necessary: even Asymptomatic cyst carriers Adult: Child over 25 kg: dysenteri, flexneri, boydii and sonnei . Campylobacter jejuni Notable adverse drug reactions, caution Prevention BACILLARY DYSENTRY Introduction Clinical features Differential diagnoses Complications Investigations Treatment objectives Drug treatment Or: Doxycycline: : 200 mg orally once daily for 5 days 12 - 18 years, 200 mg on first day, then 100 mg daily - Severe infections, 200 mg orally daily Erythromycin: : 250 - 500 mg orally every 6 hours for 5 days or 500 mg -1 g every 12 hours 125 mg every 6 hours; 2 - 8 years: 250 mg every 6 hours - Doses doubled in severe infection Or: Sulfamethoxazole-trimethroprim (Co-trimoxazole) 960 mg orally every 12 hours for 5 days 6 weeks - 6 months 120 mg 12 hourly; 6 months - 6 years 240 mg; 6 - 12 years 480 mg; 12 - 18 years 960 mg orally every 12 hours for 5 days Monitor fluid intake and output (vomitus, urine and stool) Provide access to safe drinking water Food hygiene Safe disposal of human waste Cholera vaccine A clinical condition characterized by infrequent bowel opening and/or passage of hard stools. Inadequate fibre in diet (simple constipation) Drugs e.g. antidepressants, narcotic analgesics, etc Diseases of the anus, rectum and colon e.g. fissures, haemorrhoids, cancer Functional: irritable bowel syndrome Metabolic diseases e.g. hypothyroidism, hypercalcaemia Stools are often hard Abdominal bloating Excessive flatulence Relevant associated history to determine aetiology should be vigorously pursued Physical examination should be thorough, and must include a rectal examination Megacolon Anal fissures/tears Haemorrhoids Rectal bleeding Stool examination including microscopy Proctoscopy/sigmoidoscopy Adult Child: Adult and child over 8 years Child up to 2 years: Adult: Child: Supportive measures Prevention CONSTIPATION Introduction Aetiology Clinical features Complications Investigations 1 2 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 SECTION A UNIV ERSIT Y O F IB ADAN L IB RARY Barium enema Serum hormonal levels e.g. thyroxine, triiodotyronine, thyroid stimulating hormone to exclude hypothyroidism Identify and eliminate cause(s) Evacuate hard faecal matter Situations where straining will exacerbate pre-existing medical/surgical conditions - Angina - Risk of rectal bleeding - Increased risk of anal tear Other indications - Drug-induced constipation - To clear the alimentary tract before surgery or radiological procedures Avoid precipitants High fibre diet (including fruits and vegetables) Adequate fluid intake Stimulant laxatives - Senna 7.5 mg tablet (as sennoside B) 2 - 4 tablets at night 6 - 12 years 1 - 2 tablets at night (or in the morning if preferred) 12 - 18 years: 2 - 4 tablets at night Or: Bisacodyl tablets 10 mg orally at night; suppositories 10 mg per rectum at night Laxatives should generally be avoided. Most times these drugs are needed for only a few days A very common clinical problem the world over, particularly in developing countries. Accounts for significant morbidity and mortality, especially in children. Infective agents are recognized in about 70% of cases and are transmitted by the faeco-oral route. Viruses (particularly Rotavirus) are responsible for over 70% of diarrhoeas in children below 2 years. Many bacteria and some parasites are also important aetiologic agents, particularly in adolescents and adults. Endemic and epidemic presentations can occur. Contamination of food and water by bacterial toxins can also lead to acute diarrhoea, sometimes with associated vomiting (i.e. food poisoning). This is usually self- limiting. Treatment objectives Non-drug treatment Drug treatment Caution DIARRHOEA(Acute) Introduction Indications for use of laxatives Megacolon: Saline enema Surgical: resection of large bowel Adult: Child : Personal hygiene: hand-washing, care in food-handling Inflammation of the gastric mucosa. Can be acute or chronic. The most important risk factors for acute gastritis include use of drugs (NSAIDs in particular) and alcohol. H. infection is the most important risk factor for chronic gastritis All agents of gastritis work through the common path of disrupting the protective mucosal barrier of the stomach. Acute gastritis may evoke pain that mimics peptic ulcer disease; chronic gastritis is a precursor of peptic ulcer disease (type B gastritis) and gastric cancer (type A gastritis). Chronic gastritis is essentially asymptomatic Acute gastritis evokes acute abdominal pain that mimics peptic ulcer disease (see peptic ulcer disease) Occasionally acute gastritis may be haemorrhagic with melaenal stools or rarely haematemesis Acute gastritis: haemorrhage Chronic gastritis: peptic ulcer disease; gastric cancer Peptic ulcer disease (acute gastritis) Endoscopy (macroscopic diagnosis) Histology of gastric biopsy for definitive diagnosis Eliminate pain (acute gastritis) Prevent progression to peptic ulcer disease or gastric cancer Re-establish normal histology Acute Gastritis: Antacids - Magnesium trisilicate 1 - 2 tablets or suspension 10 mL orally three times daily or as required Or: H receptor antagonist - Ranitidine 150 mg orally once daily as required Or: Proton Pump Inhibitors - Omeprazole 20 mg orally once daily as required TypeAgastritis: Endoscopic surveillance every 2 - 3 years for early detection of cancer Type B gastritis: Eradication of H. using triple therapy with - Clarithromycin 500 mg orally twice daily for 7 days Plus: - Amoxicillin 1g orally every 12 hours for 7 days Plus: GASTRITIS Introduction Clinical features Complications Differential diagnosis Investigations Treatment objectives Drug treatment pylori . pylori 2 Clinical features Complications Differential diagnoses Investigations Treatment objectives Drug treatment Supportive measures Notable adverse drug reactions Prevention Watery diarrhoea of varying volumes, sometimes with vomiting: this is the commonest presentation, and suggests pathology in the small intestine. Bloody mucoid stools: suggests disease in the colon Fever, abdominal pain and dehydration Fast and small volume pulse with low blood pressure: indicates significant fluid loss Hypovolaemic shock with multiple organ failure Septicaemia Intestinal perforation Gastro-intestinal bleeding Paralytic ileus Non-infectious diarrhoea e.g. drug-induced Gut allergy (e.g. gluten) Psychogenic stress Metabolic and endocrine causes (e.g. thyrotoxicosis, uraemia, diabetes mellitus) Stool examination including microscopy, culture and sensitivity Full Blood Count Urea, Electrolytes and Creatinine Serology (e.g. Widal test) Achieve adequate hydration Eliminate infectious agent (where possible) Treat complications Rehydrate with: Oral Rehydration Therapy - ORT (low osmolarity) for mild to moderate dehydration - 500 mLorally over 2 - 3 hours, 3 - 4 times daily Intravenous sodium chloride 0.9% - 1 litre 2 - 6 hourly for moderate-to- severe dehydration - Alternate with Darrow's solution depending on serum potassium Children: Use of zinc supplementation - 20 mg per day for 10 - 14 days - Under 6 months old: 10 mg per day Specific anti-infective agents for infectious diarrhoeas e.g. metronidazole for amoebiasis, giardiasis Monitor fluid intake/output Heart failure: from overhydration Initial increase in diarrhoea with ORT: this is self limiting Hyperkalaemia: from excessive use of potassium- containing fluids Provide access to safe drinking water Sanitary disposal of human waste - Omeprazole 20 mg orally for 7 days Or: - Metronidazole 400 mg orally every 8 hours for 7 days Plus: - Amoxicillin 500 mg orally every 8 hours for 7 days Plus: - Omeprazole 20 mg orally for 7 days Avoid risk factors (NSAIDs, alcohol, etc) Aparasitic infection caused by Worldwide in distribution but more common in developing countries. Spread by the faeco-oral route. Invasion of the upper small intestine by the parasite evokes inflammation, leading to progressive villous atrophy. Acute disease: watery diarrhoea with abdominal bloating Chronic disease: diarrhoea, steatorrhoea and weight loss from malabsorption syndrome- with lactose intolerance, xylose malabsorption and vitamin B deficiency Diseases related to Vitamin B deficiency Other causes of upper gastrointestinal malabsorption such as coeliac disease and tropical sprue Full blood count Stool microscopy and faecal fat assessment Jejunal biopsy Rehydrate adequately Eradicate parasite Replace malabsorbed (deficient) nutrients Metronidazole 2 g orally daily for 3 days or 400 mg 8 hourly for 5 days 1 - 3 years 500 mg orally daily; 3 - 7 years 600 - 800 mg daily; 7-10 years 1 g daily for 3 days Tinidazole 40 mg/kg orally as a single dose; repeat after 1 week 50 to 75 mg/kg as a single dose; repeat after 1 week Vitamin B supplementation Avoidance of milk every 12 hours every 12 hours Prevention GIARDIASIS Introduction Pathogenesis Clinical features Complications Differential diagnoses Investigations Treatment objectives Drug treatment Supportive Giardia lamblia. Adult: Child: Adult: Child: 12 12 12 3 4 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Notable adverse drug reactions Prevention HAEMORRHOIDS Introduction Clinical features Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment Drug treatment Surgery Caution PANCREATITIS Introduction Metallic taste and vomiting from metronidazole Good sanitary habits Uncontaminated water and food supplies Enlarged or varicose veins of the tissues at the anus or rectal outlet. Engorgement of the vascular complex or thrombus often leads to the symptoms of disease. The pathophysiologic mechanisms are complex and vary with the subject. May be external or internal. Internal haemorrhoids: typically painless but present with bright red rectal bleeding May become thrombosed and protrude into the anal canal External haemorrhoids when thrombosed cause acute perineal pain with or without necrosis and bleeding Fibrosed external haemorrhoids present as anal tags Colorectal cancer Adenomatous polyps Inflammatory bowel disease Bleeding, necrosis, perineal sepsis, mucus discharge Anoscopy Full blood count including blood film Relieve pain Prevent complications Increase fibre in foods Increase fluid intake Avoid foods that cause constipation Stool softeners Regular exercise Suppositories/ointments of preparations containing hydrocortisone acetate with or without lidocaine hydrochloride plus astringent(s) Elastic band ligation Sclerosis, photocoagulaton, cryosurgery, excisional haemorrhoidectomy Each drug treatment course should not exceed 7 days A state of inflammation of the pancreas, which can be Nasogastric tube suctioning Decrease pancreatic inflammation Prevent, identify and treat complications Caution Prevention PEPTIC ULCER DISEASE Introduction Aetiology/Predisposing factors Clinical features Complications Investigations Differential diagnoses Treatment objectives Avoid narcotic analgesics which may cause spasm of the sphincter of Oddi and worsen pancreatitis Control alcohol ingestion Caused by peptic ulceration that involves the stomach, duodenum and lower oesophagus. An increasingly common problem in developing countries. Most ulcers are duodenal . gut infection Use of NSAIDs Smoking Recurrent epigastric pain - Often radiating to the back - Worse at night - Improved by antacids - May be made worse by some food types (generally better with bland diet) Upper gastrointestinal bleeding Perforation Penetration Gastric outlet obstruction Gastric cancer Full Blood Count Liver Function Tests Urea, Electrolytes and Creatinine Occult blood test Stool microscopy Endoscopy Double contrast barium meal Direct/indirect detection of (by CLO test or by CO breath test) Gastritis Duodenitis Non-Ulcer Dyspepsia Gastro-duodenal malignancy Oesophagitis Gall bladder diseases Relieve pain Promote healing of ulcers Eradicate Prevent/reduce recurrence H pylori H. pylori H. pylori 2 acute or chronic. Varied, but most important are: Gallstones Alcohol ingestion Abdominal trauma Infections Idiopathic in as many as 20-30% cases Occurrence is worldwide, but commoner in areas of the world where gallstones and alcohol ingestion are common. Autolysis of pancreatic tissue by pancreatic enzymes as a result of “secretory block” in the pancreatic bed (often caused by stones) . Acute pancreatitis: Epigastric pain: may radiate to the back in over 50% of cases Nausea, vomiting, abdominal distension Severe abdominal tenderness with features of hypovolaemia in severe cases Peptic ulcer disease Cholecystitis Serum amylase: raised in 80% of acute cases Serum lipase: if raised is more specific than serum amylase Alanine aminotransferase: a rise above 3-fold suggests pancreatitis of gallstone origin CT scan Abdominal ultrasound: least useful in acute pancreatitis Hypovolaemic shock Acute renal and respiratory failure Phlegmos Gastrointestinal bleeding Electrolyte imbalance (hypo & hypercalcaemia) Pancreatic pseudocysts Relieve pain Prevent complications Renal failure: haemodialysis Respiratory failure: mechanical ventilation Gallstones: Endoscopic Retrograde Cholangio Pancreatography (ERCP) with sphincterotomy Pancreatic pseudocyst: surgery Analgesics Treat specific complications Aetiology Pathophysiology Clinical features Differential diagnoses Investigations Complications Treatment objectives Non-drug treatment Drug treatment Supportive measures Bed rest Monitor vital signs; fluid intaket/output Drug treatment Adjunct therapy Supportive therapy Notable adverse drug reactions Treatment of complications UPPER GASTROINTESTINALBLEEDING Introduction Symptomatic treatment with antacids may be used prior to confirming the diagnosis of peptic ulcer disease Triple therapy with: - Metronidazole 400 mg orally every 8 hours for 7 days Plus: - Amoxicillin 500 mg orally for 7 days Plus: - Omeprazole 20 mg orally every 12 hours for 7 days Or: - Clarithromycin 500 mg orally for 7 days Plus: - Amoxicillin 1g orally for 7 days Plus: - Omeprazole 20 mg orally for 7 days Magnesium trisilicate suspension 15 mL orally three times daily as required Regular meals Avoidance of provocative factors (NSAIDs, alcohol, spicy foods etc.) Gastric irritation, diarrhoea from triple therapy Diarrhoea/constipation from adjunct therapy Intravenous omeprazole 20 mg 12 hourly for 5 days then standard triple therapy Interventional endoscopic treatment Blood transfusion Surgery Surgery Rest the gut Surgery Bleeding from the lower oesophagus, stomach or duodenum up to the level of ligament of Treitz. Occurs worldwide and is responsible for significant mortality and morbidity. Major causes include bleeding from: - Peptic ulcer disease - Oesophageal and gastric varices - Mallory-Weiss tear - NSAID-related mucosal bleeding - Neoplasia Bleeding is either from rupture of engorged varices or from disruption of the oesophageal or gastro-duodenal H. pylori eradication Mild upper gastrointestinal bleeding Severe upper gastrointestinal bleeding Perforation Gastric outlet obstruction every 8 hours every 12 hours every 12 hours every 12 hours 5 6 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY mucosa with ulceration or erosion into an underlying vessel. Depends on whether the bleeding is acute or chronic, mild or severe Various presentations - Haemetemesis - Melaena - Haematochezia - Hypovolaemia - Iron deficiency anaemia (with its associated symptoms) Black stools from ingestion of iron tablets Haematemesis/melaena from previously swallowed blood (from the upper respiratory tract and oral cavity) Hypovalaemic shock Congestive heart failure from chronic severe anaemia Upper gastrointestinal endoscopy: picks up lesions in 90% of cases Upper gastrointestinal barium radiography: 80% detection rate Selective mesenteric arteriography Radio isotope scanning Stool- occult blood test Full Blood Count Restore and maintain haemodynamic status Control bleeding Prevent recurrence of bleeding Carefully monitor vital signs (pulse, blood pressure, respiration and temperature) as frequently as necessitated by the patient's condition Insert a nasogastric tube to aspirate gastric contents and/or to introduce agents to constrict the blood vessels Blood transfusion: whole blood (acute bleeding) or packed cells (chronic) bleeding. Up to 5 - 6 pints of blood may be needed in severe cases - Plasma expanders in the absence of blood Continuous Central Venous Pressure (CVP) monitoring Proton Pump Inhibitors - Omeprazole 20 mg orally once daily for 4 weeks Or: - Omeprazole 40 mg by slow intravenous injection over 5 minutes once daily until patient can take orally Anti therapy set above. Endoscopic treatment for actively bleeding ulcer or visible non-bleeding vessel - Injection therapy with 98% alcohol (total volume less than 1mL) Or: Clinical features Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment Drug treatment Bleeding peptic ulcers/erosions Helicobacter pylori Aetiology Important risk factors Clinical features Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment Drug treatment Varies, depending on geographical region: Viruses, alcohol and drugs are the commonest aetiologic agents Family history Alcohol ingestion Previous blood transfusion Contamination of food and water by sewage Drug ingestion Sexual contact Acute hepatitis: Mild-to-moderate jaundice Vague upper quadrant discomfort With or without mild fever There may be enlargement of the liver below the costal margin with varying consistency (depending on the stage of the liver disease) Chronic hepatitis: Re-occurence of jaundice may suggest a chronic illness Liver abscess Metabolic liver disease/disorder Fulminant hepatic failure Bleeding tendencies Liver Function Tests Serologic markers of HepatitisA, B, C, D and E Abdominal ultrasonography Provide supportive measures Prevent progression to chronic phase High carbohydrate and low protein diet Discontinuation of hepatotoxic medication Bed rest Self-limiting disease. No specific drug treatment Acute: Self-limiting to fulminant Treatment is supportive Chronic: Interferon alfa -2b: 10 million units subcutaneously three times weekly for 4 months Lamivudine: 100 mg orally daily for 1year Liver transplant Chronic Hepatitis C: Interferon alfa -2b - 3 million units subcutaneously 3 times weekly for 4 months Ribavirin - 400 mg orally twice daily for adults with body weight HepatitisA Hepatitis B - Injection therapy with epinephrine (1:10,000) up to 1mL Or: - Thermal coagulation with heat probe Or: - Laser therapy Intravenous vasopressin 20 units over 20 minutes bolus then infusion of 0.1 - 0.5 units/min Plus: Intravenous nitroglycerin 40 microgram/min (titrated upward to maintain systolic blood pressure above 90 mmHg) Endoscopic treatment Injection sclerotherapy: equal volume mixture of 3% sodium tetradecyl sulfate, 98% ethanol, sodium chloride 0.9% injection (2-5 mL/site; maximum 50 mL) Variceal band ligation Radiologic therapy Venous embolization Transjugular Intrahepatic Portosystemic Shunt (TIPS) Oesophageal transection and devascularization Liver transplant Surgical repair or resection as appropriate Monitor vital signs and urine output to detect early features of hypovolaemic shock Look out for features of hepatic encephalopathy Vasopressin can cause abdominal cramps. It lowers blood pressure drastically and could worsen ischaemic heart disease Avoid NSAIDs. Treat infection blockers (propranolol 40 mg orally 12 hourly and titrate up to 160 mg depending on the heart rate) Maintenance sclerotherapy Inflammation of the liver that can be caused by infective agents, drugs and other toxins The most predominant and important presentation of liver disease worldwide The suffixes acute, chronic, viral, autoimmune, alcoholic etc. define the agents causing hepatic injury or their duration as the case may be Bleeding varices Peptic ulcers/erosions/tumours Peptic ulcers/erosions related upper gastrointestinal bleeding Oesophageal varices Supportive Notable adverse drug reactions Prevention HEPATITIS Introduction H. pylori β HEPATICAND BILIARYDISORDERS less than 65 kg; 400 mg in the morning and 600 mg in the evening for adults weighing 65-85 kg; 600 mg twice daily for adults weighing over 85 kg Interferon alfa -2b: 3 million units subcutaneously 3 times weekly for 4 months Plus: Lamivudine: 100 mg orally once daily for 4 months Largely supportive Interferon alpha 2b and Ribavirin haematopoietic toxicity Flu-like illness Leucopenia Psychiatric-like symptoms Development of early resistance if therepy exceeds 1 year Prevention of faecal contamination of food and water Screen blood and blood products for hepatotrophic viruses Immunization against hepatitisA, B Reduction of drug misuse/abuse Pre-exposure prophylaxis (as for NPI/EPI) Post-exposure prophylaxis Astate of disturbed central nervous system function as a result of hepatic insufficiency Characterized by changes in personality, cognition, motor function, level of consciousness One-year survival rate is 40% Nitrogenous substances, particularly ammonia, reach the brain via portosystemic shunts leading to alteration of neurotransmission Reduced blood supply to the liver Infection of the liver Bleeding into the gut Electrolyte imbalance (hypokalaemia and hypomagnesaemia) Poor bowel evacuation Cognitive abnormalities: may be mild and recognizable only with psychometric testing but may be severe with frank confusion, altered level of consciousness and coma Hyper-reflexia Fetor hepaticus Insomnia Flapping tremor (asterixis) Intracranial lesions (haemorrhage, tumour, abscess etc.) Hepatitis D Hepatitis E Notable adverse drug reactions Prevention HEPATIC ENCEPHALOPATHY Introduction Predisposing factors Clinical features Differential diagnoses 7 8 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY CNS infections (encephalitis, meningitis) Other metabolic encephalopathies (uraemia, hyper/hypoglycaemia etc.) Hypertensive encephalopathy Alcohol intoxication Drug toxicity e.g. sedatives, heavy metals As appropriate to identify possible precipitating factors Full Blood Count Urea, Electrolytes and Creatinine, Blood sugar Microscopy and culture of the stool and blood Reverse neuropsychiatric symptoms Minimize nitrogenous substances Treat precipitating factors Lactulose syrup (10 g/15 mL) - Initially 30 - 45 mL orally three times daily titrated to either the resolution of symptoms or production of three soft stools per day Or: - As rectal retention enema 300 mL in 1 litre water retained for 1 hour; duration usually for days or weeks Or: Metronidazole 800 mg orally 12 hourly Treat underlying cause(s) e.g hypokalaemia, anaemia, infection Lactulose: excess gas, diarrheoa Metronidazole: peripheral neuropathy, dysgeusia Avoid precipitating factors Acommon clinical state of varying aetiologies Classified as haemolytic, hepatic or obstructive Clinical jaundice occurs when the level of serum bilirubin exceeds 2.5 mg/dL The bilirubin may be conjugated, unconjugated or mixed Diseases of the liver and the biliary tract Conditions that cause excessive red cells haemolysis: infections, haemoglobinopathies Discolouration of the sclerae and other mucus membranes With or without pruritus (especially with cholestatic jaundice) Investigations Treatment objectives Drug treatment Notable adverse drug reactions Prevention JAUNDICE Introduction Important causes Clinical features Supportive measures High carbohydrate, low protein diet Adequate hydration Rectal wash out Hepato-renal syndrome LFTs PT, PTTK, Serum albumin Liver biopsy Ultrasound examination of the liver Screening for aetiologic factors in chronic liver disease e.g. viral markers for hepatotrophic viruses (e.g. Hepatitis B & C) Prevent further liver damage Prevent deterioration of liver function Symptomatic relief from anaemia, fatigue and oedema Encourage high fibre and low salt diet Enhance opening of bowel Correction of anaemia Reduce oedema and ascites Spironolactone tablets 25 - 100 mg orally 12 hourly Furosemide 20 - 80 mg orally 12 hourly Salt-poor albumin for intractable ascites Propranolol 40 - 80 mg orally daily Liver transplant Lactulose 30 mLorally twice daily - Doses to be titrated upward until at least 3 bowel motions daily are achieved Saline rectal enema Immunization against hepatitis B, C Abstinence from alcohol Adequate nutrition is the intake and utilization of energy-giving and body building foods and nutrients, to maintain well-being, and productivity. “Malnutrition” includes generalized malnutrition that manifests as stunting, underweight, wasting (kwashiorkor and marasmus), obesity as well as deficiencies of micronutrients. Kwashiorkor is protein-energy malnutrition . Marasmus is malnutrition resulting from inadequate calorie intake. Obesity is a commonly nutritional disorder (results from excessive intake of calories). Investigations Treatment objectives Non-drug treatment Drug treatment Prevention KWASHIOKORAND MARASMUS Introduction Epidemiology Ascites and pedal oedema Prevention of variceal bleeding Replacement of damaged liver Prevention of encephalopathy NUTRITIONALDISORDERS Associated features of the underlying disease LFTs: determine levels and nature of bilirubin, liver enzymes (AST,ALT,Alkaline phosphotase) Abdominal ultrasound scan: look out for canalicular dilatations, biliary stones Treat underlying cause Prevent complications Specific treatment depends on the identified cause Colestyramine - 3 - 6 g orally 6 hourly in severe obstructive jaundice Phenobarbital in neonatal jaundice - 5 - 8 mg/kg orally daily Colestyramine: diarrhoea Phenobarbital may cause dose-dependent respiratory depression ERCPsphincterotomy with stone removal Stent insertion Pancreatic head/duodenal head realignment An advanced stage of chronic liver disease associated with permanent distortion of the liver architecture and replacement of some destroyed hepatocytes with fibrous tissue Accompanied by some loss of liver function leading to certain recognized symptoms and signs Similar to some causes of acute liver diseases No known aetiology in up to 30% of cases Varies with the extent of liver damage: Fatigue Ascites Pedal oedema Haematemesis Liver may be shrunken or enlarged below the costal margin; it is typically firm Granulomatous lesion of the liver Primary or secondary neoplasms of the liver Intractable oedema Upper gastrointestinal tract bleeding Coagulopathy Hepatic encephalopathy Investigations Treatment objectives Drug treatment Notable adverse drug reactions Surgical treatment LIVER CIRRHOSIS Introduction Aetiology Clinical features Differential diagnoses Complications Obstructive jaundice Supportive measures Reassurance and monitoring Phototherapy in neonatal jaundice High percentages in under-developed countries, especially sub-SaharanAfrica Kwashiorkor: Growth retardation Muscle wasting Anaemia Apathy Moon face Lack-luster skin Easily plucked hair Pedal oedema Hypo-pigmented skin patches Exfoliation, Diarrhoea Marasmus: Thin; protruding bones Hungry-looking 'old-looking face’ Whimpering cry Full Blood Count, ESR Stool microscopy Urinalysis Serum proteins Chest radiograph Mantoux test Nutritional counselling Adequate nutrient intake: may require assistance and special preparations e.g. nasogastric feeding, etc. Periodic growth monitoring May be indicated where there are specific infections/infestations Deficiencies of minerals (iron, iodine, zinc, calcium, phosphorus, magnesium, copper, potassium, sodium, chloride, fluoride etc); folic acid and vitamins Inadequate dietary intake Increased requirements Increased loss (e.g. worm infestation) Global; high percentages in under-developed countries, especially sub-SaharanAfrica Iron: anaemia Iodine: goitre Zinc, copper: manifestations of enzyme and insulin deficiencies Calcium: rickets, osteomalacia Phosphorus and fluoride: teeth and bone abnormalities Clinical features Investigations Non-drug treatment Drug treatment MICRONUTRIENT DEFICIENCIES Definition Aetiology Epidemiology Clinical features 9 10 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Vitamins: - A: keratomalacia, corneal xerosis, night blindness - B (thiamine): beri-beri - B (riboflavin): scrotal and vulval dermatoses, angular stomatitis, scars, magenta tongue, cheilosis - B (niacin): scarlet and dry tongue, pellagra - Ascorbic acid: scurvy, petechiae and musculo-skeletal haemorrhages - D: rickets, epiphyseal enlargement, muscle wasting, bossing of skull bone, 'thoracic rosary', persistently open anterior fontanelle, genu valgum or varum Blood, urine and stool tests Other investigations as appropriate Correct nutrient deficiencies Ensure adequate intake Prevent complications Administration of specific nutrients (as concentrates in foods) Food supplementation Treat underlying diseases Nutritional counselling Optimal breastfeeding and appropriate weaning practices Adequate intake of locally available, nutritious foods Personal/food/water hygiene Prophylactic therapies for malaria Amajor component of the metabolic syndrome. Being overweight or obese significantly increases the risk of morbidity and mortality from Type 2 diabetes and its co-morbidities. Successful weight reduction has a positive impact on morbidity and mortality outcomes. Constititional obesity is a result largely of diet and lifestyle. Body mass index (BMI): calculation for overall obesity Waist circumference: determination of central fat distribution BMI is calculated as follows BMI = weight in kg divided by height in m , expressed as kg/m Underweight: <18.5 kg/ m Normal weight: 18.5 - 24.9 kg/ m Overweight: 25 - 29.9 kg/ m Obesity (Class1): 30 - 34.9kg/m Obesity (Class 2): 35 - 39.9 kg/m Extreme obesity (Class 3): > 40 kg/m BMI represents overall adiposity 1 2 6 Investigations Treatment objectives Treatment Prevention OBESITY Introduction Measurements for evaluation Classification of BMI 2 2 2 2 2 2 2 2 Prevent complications Assess dietary intake, level of physical activity, BMI (total body fat) and waist circumference (abdominal fat) on presentation and at regular monitoring Assess efficacy of weight loss measures Integrate weight control measures into the overall management of diabetes mellitus and co-morbidities if - BMI is >25 - Waist circumference is more than 102 cm and 88 cm in men and women respectively Educate patients and other family members Set realistic goals Use a multi-disciplinary approach to weight control Dietary changes and increased level of physical activity are the most economical means to loose weight Maintain records of goals, instructions and weight progress charts Surgical intervention may be required in extreme cases Management The pattern of distribution of fat in the body (whether mostly peripheral or central) is assessed by the use of the waist/hip ratio (WHR) Waist/Hip ratio=Waist circumference (in cm) divided by Hip circumference (in cm) Waist circumference: measure midway between the lower rib margin and the iliac crests Hip circumference: the largest circumference of the hip Waist circumference better depicts central or upper body obesity than waist/hip ratio - Upper limits: 102 cm and 88 cm in men and women respectively Non-specific - Always bear in mind the possibility of an underlying cause: although these may not be common, specific therapy may be available - Clinical presentation may therefore require specific investigations to exclude conditions such as Hypothyroidism Hypercortisolism Male hypogonadism Insulinoma CNS disease that affects hypothalamic function Cardiovascular: Coronary disease Stroke Congestive heart failure Pulmonary: Obstructive sleep apnoea 'Obesity hypoventilation syndrome' Endocrine: Insulin resistance and type 2 diabetes mellitus Hepatobiliary: Gall stones Reproductive: Male hypogonadism Menstrual abnormalities Infertility Cancers: In males, higher mortality from cancer of the colon, rectum and prostate In females, higher mortality from cancer of the gall bladder, bile ducts, breasts, endometrium, cervix and ovaries Bone, joint and cutaneous disease: Osteoarthritis Gout Acanthosis nigricans Increased risk of fungal and yeast infections Venous stasis To educate patient and care givers Achieve an ideal body weight Investigations Complications Treatment objectives CHAPTER 2: BLOOD AND BLOOD-FORMING ORGANS ANAEMIAS Introduction Morphological classification Classification based on aetiology and pathogenesis Anaemia is a reduction in the haemoglobin concentration in the peripheral blood below the normal range expected for the age and sex of an individual The determination of haemoglobin concentration should always take the state of hydration and altitude of residence of the individual into consideration It can be classified on the basis of red cell morphology and aetiology/pathogenesis Macrocytic Megaloblastic - Folic acid deficiency - Vitamin B deficiency - Inherited disorders of DNAsynthesis Non-megaloblastic - Accelerated erythropoiesis - Increased membrane surface area - Obscure Hypochromic-microcytic Iron deficiency Disorders of globin synthesis Other disorders of iron metabolism Normochromic-normocytic Recent blood loss Haemolytic anaemias Hypoplastic bone marrow Infiltrated bone marrow Endocrine abnormality Chronic disorders - Renal disease - Liver disease Blood Loss: Acute Chronic (leads to iron deficiency) Increased red cell destruction (haemolytic anaemias): Corpuscular defects (intracorpuscular or intrinsic abnormality) Disorders of the membrane e.g elliptocytosis, spherocytosis Disorders of metabolism e.g Glucose-6-Phosphate Dehydrogenase deficiency Haemoglobinopathy e.g sickle cell disease Paroxysmal Nocturnal Haemoglobinuria Abnormal haemolytic mechanisms (extra-corpuscular or intrinsic abnormality): Autoimmune Rhesus-incompatibility, mismatched transfusion Hypersplenism Infections e.g malaria, Drugs and toxins 12 Clostridium welchii 11 12 Chapter 1: Alimentary Tract Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Others e.g. burns Decreased red cell production: Nutritional (due to deficiencies of substances essential for erythropoiesis) - Iron - Folate - Vitamin B - Various deficiencies e.g. protein, ascorbic acid Bone marrow stem cell failure: Primary (idiopathic): - Aplastic anaemia - Pure red cell aplasia Secondary: - Drugs (phenylbutazone, cytotoxic agents, etc) - Chemicals - Irradiation Anaemias associated with systemic disorders: Infection Liver disease Renal disease Connective tissue disease Cancer (including leukaemia) Marrow infiltration Thyroid or pituitary disease Depend on the degree of anaemia, severity of the causative disorder and age of the patient The clinical effects of anaemia are due to anaemia itself and the disorder(s) causing it Common: Tiredness Lassitude Weakness Dyspnoea on exertion Palpitations Pallor Less common: Angina of effort Faintness Giddiness Headache Ringing in the ears High output state Congestive cardiac failure Cardiac failure Respiratory failure Cardiac failure Death Haematologic: Haematocrit; haemoglobin concentration Red cell indices Reticulocyte count Total leukocyte and differential counts 12 Clinical features Differential diagnoses Complications Investigations Not necessary unless there is intolerance to oral iron Indications for parenteral iron: Anaemia diagnosed in late pregnancy Correction of anaemia just before an operative procedure Haemorrhage expected to continue unabated Iron preparations: Iron dextran given as “total dose" infusion Dose in mL (of 50 mg/mL preparations) = [Patient's wt. in kg X (14 Hb in g/dL)] 10 Oral iron preparations: Nausea, epigastric pain, diarrhoea, constipation, skin eruptions Reduce dosage and frequency of administration to reduce these effects Parenteral iron: Local reactions: phlebitis and lymphadenopathy Systemic reactions: may be early or late- headache, fever, vomiting; general aches and pains, backache, chest pain, dyspnoea, syncope; death from anaphylaxis A test dose should be administered: 25 mg intramuscularly or by intravenous infusion over 5 to 10 minutes Total-dose infusion should be avoided in patients with history of allergy Response to therapy is satisfactory if administered dose is limited to the minimal daily requirement Treatment with vitamin B (cobalamin) to replace body stores - Six-1000 micrograms intramuscular injections of hydroxocobalamin given at 3 - 7 day intervals Maintenance therapy: patients will need to take vitamin B for life - 1000 micrograms hydroxocobalamin intramuscularly once every 3 months Toxic reactions are very rare and are usually not due to cobalamin itself Pharmacologic doses of folic acid produce haematological response in vitamin B -deficient patients but worsen the neurological complications Large doses of vitamin B also give haematological response in folate-deficient patients Balanced diet Prompt treatment of all illnesses Blood transfusion is the administration of blood for therapy. It is potentially hazardous: blood should be given only if the dangers of not transfusing outweigh those of ÷ Notable adverse drug reactions, caution Notable adverse drug reactions, caution Prevention BLOOD TRANSFUSION Introduction Megaloblastic anaemia 12 12 12 12 Platelet count Erythrocyte sedimentation rate Blood film examination for morphology of cells Thick and thin films for malaria parasites Urine analysis: Colour, pH, clarity, specific gravity Microscopic examination of fresh urine specimen Protein Glucose Occult blood Stool: Colour, consistency Examination for ova and parasites Occult blood Plasma: Blood Urea Nitrogen (BUN) Total protein and albumin Bilirubin Creatinine (if BUN is abnormal) Others: Coombs test for the presence of antibodies to red cells Ham's test (acidified serum test) Bone marrow aspiration and trephine biopsy Haemoglobin electrophoresis Sickling test (metabisulphite and solubility) Family studies Restore haemoglobin concentration to normal levels Prevent/treat complications Bed rest in severe cases: initially necessary, especially when cardiovascular symptoms are prominent Treat cardiac failure by standard measures Balanced diet with adequate protein and vitamins Correct dietary deficiencies (e.g. iron, folic acid) Blood transfusion: a very important measure in the treatment of anaemia, but should not be used as a substitute for investigation, or specific treatment of the cause Arrest blood loss Treat any underlying systemic disorder Remove any toxic chemical agent or drug Correct anatomical gastro-intestinal abnormalities Haematinics e.g. iron, vitamin B folic acid The specific haematinic indicated should be given alone Response to adequate treatment is important in confirming diagnosis Oral iron therapy: - Ferrous sulfate 200 mg (containing 65 mg of iron) 1 tablet 2-3 times daily Treat for 3- 6 months to correct deficits in haemoglobin and in stores Parenteral therapy: Treatment objectives Supportive measures Drug treatment 12, Iron deficiency transfusion. Indication(s) must be clearly established. Transfusion of whole blood or red cell concentrates is important in the treatment of acute blood loss and of anaemia. Red cells can be stored at 4ºC for 5 weeks in media that are specially designed to maintain the physical and biochemical integrity of the erythrocytes and which maintain their viability after transfusion. Citrate Phosphate Dextrose with Adenine (CPDA) is commonly used for collections of whole blood. The use of whole blood as a therapeutic agent has been almost completely replaced by the use of blood fractions. Autologous blood transfusion: Transfusion of the patient's own blood to him /her - Safest blood for patients The three main types are: - Pre- deposit autologous transfusion - Immediate pre-operative phlebotomy with haemodilution - Intra-operative blood salvage Exchange transfusion: To remove deleterious material from the blood, for example, in severe jaundice resulting from haemolytic disease of the newborn Alternatives to red cell transfusion: Perfluorochemicals such as Fluosol-DA Polymerised haemoglobin solutions with good intravascular recovery Symptomatic anaemias: - Recurrent haemorrhage - Haemolysis - Bone stem cell failure - Pure red cell aplasia - Severe anaemia of chronic disorders - Haematological malignancies (e.g. leukaemia, lymphoma) - Chemotherapy complicated by anaemia In neonates: - Severe acute haemorrhage - Haemolytic disease of the new born - Septicaemia - Prematurity Bleeding disorders: - Congenital e.g. haemophilia - Acquired e.g. disseminated intravascular coagulopathy Prevention or treatment of shock: - Clinical situations in which there is need to restore and/or maintain circulatory volume e.g. trauma, haemorrhage To maintain the circulation (as in extracorporeal or cardiac by-pass shunts) Types of blood transfusion Indications for blood transfusion Whole blood preparations 13 14 Chapter 2: Blood and Blood-Forming Organs Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Should be limited to correction or prevention of hypovolaemia in patients with severe acute blood loss Justified by the recognition that there is a relatively rapid loss of platelets, leucocytes and some coagulation factors with liquid storage. There is also progressive increase in the levels of undesirable products such as potassium, ammonia, and hydrogen ions Four types are in common use: - Packed red blood cells - Washed red blood cells - Leucocyte-reduced red blood cells - Frozen red blood cells Obtained from liquid-stored blood by saline washing using a continuous-flow cell separator or from frozen erythrocytes extensively washed to remove the cytoprotective agents Best prepared by passing whole blood or packed cells through specifically designed filters. Three main reasons for the use of leucocyte-reduced red blood cells: - To prevent non-haemolytic febrile reactions to white cell and platelet antibodies in recipients exposed to previous transfusions or pregnancies - To prevent sensitization of patients with aplastic anaemia who may be candidates for bone marrow transplantation - To minimize risk of transmission of viruses such as HIV or cytomegalovirus Informed consent should be obtained from patients except in life-threatening emergencies The risks and benefits of the proposed transfusion therapy should be discussed with the patient and documented in the patient's medical records There may be no time available to type, select and cross-match compatible blood Arare occurrence, except for - Trauma - Unexpected intra-operative haemorrhage - Massive gastro-intestinal bleeding - Ruptured aneurysm Uncross-matched or partially cross-matched blood is administered; routine cross-match should be carried out retrospectively to identify any incompatibility Immunological: Sensitization to red cell antigens Haemolytic transfusion reactions - Immediate - Delayed Reactions due to white cell and platelet antibodies Fresh Blood Erythrocyte preparations Washed red blood cells Leucocyte-reduced red blood cells Blood for emergencies Transfusion therapy Complications of blood transfusion Prompt treatment of illnesses that could be complictated by anaemia Regular medical check-ups A heterogeneous group of diseases characterized by infiltration of the blood, bone marrow and other tissues by neoplastic cells of the haematopoietic system Two main types - Myeloid leukaemia - Lymphoid leukaemia Each is further divided into acute and chronic Acute leukaemias are defined pathologically as blast cell leukaemias or malignancies of immature haematopoietic cells. The bone marrow shows > 30% blast cells Two main groups of acute leukaemias - Acute myeloid leukaemia (AML) - Acute lymphoblastic leukaemia (ALL) Childhood leukaemias: patients aged <15 years Adult leukaemias: patients aged >15 years Leukaemias in adults aged > 60 years: an important group because - Their responses to current treatment protocols both for ALLandAMLare inferior - These patients are not usually considered for more radical treatment approaches such as autologous or allogeneic bone marrow transplantation 80% of adult cases:AML More common in industrialized than rural areas Environmental agents implicated in the induction of certain types of leukaemia: Ionising radiation: X-rays and other ionizing rays Chemical carcinogens - Benzene and other petroleum derivatives - Alkylating agents Host susceptibility e.g. genetic disorders: Bloom's syndrome Fanconi's anaemia (AML) Ataxia telangiectasia (ALL) Down's syndrome Blast transformation in pre-existing myeloproliferative disoders: Aplastic anaemia (ALL) Oncogenic viruses: HTLV-1 (Human T-cell Lymphotropic virus 1): implicated in adult T cell leukaemia /lymphoma HAEMOSTASIS AND BLEEDING DISORDERS - refer for specialist care LEUKAEMIAS Introduction Epidemiology/predisposing conditions Acute lymphoblastic leukaemia (ALL) and Acute myeloid leukaemia (AML) - Febrile transfusion reactions - Post-transfusion purpura Reactions due to white cell and plasma protein antibodies - Urticaria - Anaphylaxis Non-immunological: Transmission of disease Reactions due to bacteria and bacterial pyrogens Circulatory overload Thrombophlebitis Air embolism Transfusion haemosiderosis Complications of massive transfusion A minimum of three major procedures must be carried out: - Determine the recipient'sABO and Rhesus groups - Select compatible donor blood - Cross-match donor cells against recipient's serum Donor blood should be screened for infective agents: HIV, hepatitis B, and C viruses Haemoglobin concentration Haematocrit Red cell indices: MCH, MCV, MCHC Total leucocyte and differential counts Reticulocyte count Erythrocyte sedimentation rate Platelet count To raise haemoglobin concentration and other blood parameters to normal levels To prevent blood transfusion complications Transfusion of red blood cells, platelet concentrates or platelet rich plasma as required Provision of fresh frozen plasma or other blood products as necessary Furosemide 40 mg on administration of one unit of blood In the event of transfusion reactions, stop the transfusion immediately and administer the following: Promethazine 25 mg intramuscularly or intravenously Epinephrine 0.5 mL of 1:1000 solutions to be administered subcutaneously Hydrocortisone sodium succinate 100 mg injection Appropriate nutrition Adequate hydration Furosemide: dehydration and hypersensitivity Promethazine: drowsiness, hypersensitivity Avoid/prevent accidents Tests of Compatibility Other investigations Treatment objectives Non-drug treatment Drug treatment Supportive measures Notable adverse drug reactions, caution Prevention Clinical features Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment General symptoms of anaemia Bleeding Infections Anorexia Weight loss Lymphadenopathy (not common in AML except in the monocytic variant) Skin : Macules, papules, vesicles Pyoderma gangrenosum Neutrophilic dermatitis Leukaemic cutis Granulocytic sarcoma Septicaemia Miliary tuberculosis Malignant histiocytosis Worsening ill- health Full blood count with ESR, reticulocyte count Coomb's test Bone marrow examination Biochemical tests: serum electrolytes, urea, creatinine, uric acid Liver function tests Prothrombin time, partial thromboplasnime Human LeucocyteAntigen typing HIV I and II Cytochemical tests - Peroxidase - Sudan Black B - Non-specific esterase reaction e.g. alpha napthyl acetate esterase Bone marrow cultures Cytogenetic studies Electron microscopy Cell markers e.g. using a panel of antibodies combined with flow cytometric analysis or the alkaline phosphase- antialkaline phosphate (APAAP) technique to classify the blast cells into lymphoid or myeloid lineages Abdominal ultrasound/CT scans Immunological classification Terminal deoxynucleotidyl transferase demonstration in nuclei of B and T lymphocytes Induce remission to achieve complete remission Maintain disease-free state Appropriate nutrition Adequate hydration (at least 3 litres/24 hours) Erythrocyte transfusion as required Platelet concentrate transfusion as required Maintain electrolyte balance 15 16 Chapter 2: Blood and Blood-Forming Organs Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Drug treatment Acute lymphoblastic leukaemia Acute myeloblastic leukaemia Allopurinol 300 mg daily orally Daunorubicin 30 mg/m intravenously on days 8, 15, 22 and 29 Vincristine 1.4 mg/m to a maximum of 2 mg intravenously on days 8, 15, 22 and 29 Prednisolone 60 mg orally once daily from day 1 - 28 L-asparaginase 1000 IU/m intravenously on days 12, 15, 18, 21, 24, 27, 30 and 33 Or: Cyclophosphamide 650 mg/m intravenously on days 1 and 8; 14 and 22 Vincristine 1.4 mg/m intravenously to a maximum of 2 mg: days 1 and 8; 14 and 22 Cytosine Arabinoside 50 mg/ m subcutaneously 12 hourly for 12 days or bolus intravenous injection 100 mg/m daily for 7 days Prednisolone 40 mg/m oral for 14 days - Drugs are given every 28 days for 3 courses Nervous system prophylaxis - Methotrexate 12.5 mg/m intrathecally twice weekly to a maximum of 15 mg i.e. 5 doses over 3 weeks. To be given on day 29 COAP regime to be given once provided WBC count is = 1x10 /Land platelet count is = 100 x10 /L 6-Mercaptopurine 75 mg/m orally daily Methotrexate 20 mg/m orally weekly - For 3 years if remission is maintained, otherwise re- assessment To be given every 3 months with - Vincristine 1.4 mg/ m to a maximum of 2 mg weekly on days1 and 8 Either TAD or COAPas shown below: Cytarabine 100 mg/m (continuous infusion) on days 1 and 2, and 100 mg/m by intravenous infusion over 30 minutes on days 3 - 8 Thioguanine 100 mg/m orally on days 3 - 9 Daunorubicin 60 mg/m by intravenous infusion over one hour on days 3 - 5 Or: Cyclophosphamide 650 mg/m intravenously on days 1 and 8 Vincristine 1.4 mg/m intravenously to a maximum of 2 mg on days 1 and 8 Cytarabine 50 mg/m subcutaneously DVPRegime COAPRegime Consolidation Maintenance Pulse therapy (Intensification) TAD COAP 2 2 2 2 2 2 2 2 2 9 9 2 2 2 2 2 2 2 2 2 2 every 12 hours every 12 hours every 12 hours Clinical features Complications Investigations Treatment objectives Non-drug treatment Drug treatment Notable adverse drug reactions caution Asymptomatic Abdominal swelling/pain Lethargy Shortness of breath on exertion Weight loss Unexplained haemorrhage at various sites e.g. gums, intestinal/urinary tracts Increased sweating Visual disturbances Gout Priapism Splenomegaly Anaemia Haemorrhage Fever Lymphadenopathy (rare in chronic phase) Blastic transformation Death As above for acute leukaemia plus: Determination of Philadelphia chromosome Lactic dehydrogenase Serum calcium Induce remission to achieve complete remission Maintain disease-free state Achieve absence of Philadelphia chromosome Appropriate nutrition Adequate hydration Electrolyte balance Hydroxycarbamide (hydroxyurea) 20-30 mg/kg orally daily or 80 mg/kg every third day Not recommended Interferon alpha 9 million units subcutaneously or intravenously thrice weekly for 6 - 12 months Or: Imatinib mesylate - 400 mg orally daily , The above drugs (except the steroids) all cause profound myelosuppression Profound nausea, vomiting, diarrhoea and abdominal discomfort Secondary malignancies Steroids: Cushing's syndrome, hypertension, diabetes mellitus, immunosuppression, infections Vincristine: neurotoxicity Cylophosphamide: alopecia, haemorrhagic cystitis Daunorubicin: myelosuppression, alopecia, Adult: Child: Adult: - To be used strictly under specialist supervision for 7 days Prednisolone 40 mg/ m orally for 14 days - Nervous system prophylaxis is not required - Assess for remission after 3 courses COAPevery 6 weeks for 2 years Intrathecal treatment as forALL if there is CNS disease of the monocytic type Also Chronic Myelogenous Leukaemia; Chronic Granulocytic Leukaemia (CGL) A clonal disease that results from acquired genetic change in a pluri-potential haematopoietic stem cell Altered stem cell proliferation generates a population of differented cells, and a greatly expanded total myeloid mass Majority of patients have relatively homogenous disease characterized by: - Splenomegaly - Leucocytosis - Presence of Philadelphia (Ph) chromosone in all leukaemia cells Minority of patients have less typical disease (atypical CML) - These variants lack Ph chromosome. Examples: - Chronic myelomonocytic leukaemia - Chronic neutrophilic leukeamia - Juvenile chronic myeloid leukaemia Rare below the age of 20 years but occurs in all age groups Increased risk of developing CMLwith exposure to high doses of irradiation Abiphasic or triphasic disease, usually diagnosed in the initial “chronic”or stable phase Untreated patient: - <12% blast cells in blood or marrow Treated patient: - Normal or near-normal blood count without immature granulocytes in peripheral blood Rising leucocyte count despite treatment Rapid leucocyte doubling time Immature granulocytes in blood Blast cells >5% but <30% in marrow Anaemia (Hb <10 g/dL) not attributable to treatment Thrombocytosis (>1000 x 10 /L) Acquisition of specific new cytogenetic abnormalities Increasing marrow fibrosis More than 30% blasts Or: Blasts plus promyelocytes in blood or bone marrow 2 9 Maintenance Distinguishing features between phases of CGL Chronic phase Accelerated phase Blastic transformation Chronic Myeloid Leukaemia (CML) Classification Epidemiology, aetiology and natural history cardiotoxicity All are contraindicated in patients with history of hypersensitivity reactions to the respective medicines Avoid exposure to ionizing radiation Early detection and treatment Neoplastic proliferations of mature lymphocytes The diseases involve the blood bone marrow and other tissues Characterized by accumulation of small mature-looking CD5+ B lymphocytes in the blood, marrow and lymphoid tissues B-cell disorders are more common B-cell CLLis more common in males than females - Accounts for 60% of cases - Rarely diagnosed below the age of 40 years Asymptomatic (30% of cases) Symptoms of anaemia Lymph node enlargement (painless) Rare: pyrexia, sweating or weight loss Severe chest infection/pneumonia Splenomegaly (50% of cases) Hepatomegaly (not frequent) Low grade non-Hodgkin's lymphomas with frequent blood and bone marrow involvement (leukaemia / lymphoma syndromes) Tuberculosis Viral infections Toxoplasmosis Richter transformation Progression of disease Cell morphology: Size Nuclear: cytoplasmic (N:C) ratio Regularity or irregularity of the nuclear outline Characteristics of the cytoplasm (presence and length or absence of azurophil granules) Degree of nuclear chromatin condensation and its pattern Prominence, frequency and localization of the nucleolus As for anaemia and other leukaemias Induce remission to achieve complete remission Maintain disease-free state Appropriate nutrition Adequate hydration Maintenance of electrolyte balance Bone marrow transplant Prevention Clinical features Differential diagnoses Complications Investigations Investigations Treatment objectives Non-drug treatment Chronic Lymphocytic Leukaemia 17 18 Chapter 2: Blood and Blood-Forming Organs Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Red cell and platelet concentrate transfusion as required Allopurinol 100 mg orally every 8 hours Chlorambucil 5 mg/m orally on days 1 to 3 Prednisolone 75 mg orally on day 1; 50 mg orally on day 2 and 25 mg orally on day 3 - Repeat every 2 weeks Or: Fludarabine 25 - 30 mg intravenously over 30 minutes on days 1-5 - Repeat every 4 weeks Or: Combination chemotherapy - Cyclophosphamide 400 mg/m - Vincristine 1.4 mg/m - Prednisolone 100 mg orally days 1 - 5 Repeat every 3 weeks Or: Fludarabine 30 mg/m intravenously over 30 minutes on days 1 - 3 Cyclophosphamide 250 - 300 mg/m intravenously over 30 minutes on days 1 - 3 - Repeat every 4 weeks Appropriate nutrition Adequate hydration Same as for other leukaemias Avoid chemicals on body (e.g benzene) Avoid ionizing radiation (X rays) Early detection and treatment Solid neoplasms that originate in lymph nodes or other lymphatic tissues of the body Aheterogeneous group of disorders - Can arise at virtually any site - More often occurs in regions with large concentrations of lymphoid tissues, e.g. lymph nodes, tonsils, spleen and bone marrow Two main groups: - Hodgkin's disease - Non-Hodgkin's lymphomas Hodgkin's disease is characterized by Reed- Sternberg cells (large binucleate cells with vesicular nuclei and prominent eosinophilic nucleoli) - Reed-Sternberg cells are occasionally found in other clinical conditions e.g. hyperplastic or inflammatory lesions of lymph nodes Non-Hodgkin's lymphomas: a heterogeneous collection of lymphoproliferative malignancies Drug treatment Supportive measures Notable adverse drug reactions, caution Prevention LYMPHOMAS Introduction Chronic Lymphocytic Leukaemia 2 2 2 2 2 Vincristine 1.4 mg/m (maximum 2 mg) on days 1 and 8 Prednisolone 100 mg orally on days 1 - 8 Mechlorethamine 6 mg/m intravenously on days 1 and 8 Vincristine 1.4 mg/m (maximum 2 mg) intravenously on days 1 and 8 Procarbazine 100 mg/m orally on days 1 4 Prednisolone 40 mg orally on days 1 - 14 Chlorambucil 6 mg/m orally on days 1 and 14 Vinblastine 6 mg/m (maximum 10 mg) intravenously on days 1 and 18 Procarbazine 100 mg/m orally on days 1 and 14 Prednisolone 40 mg orally on days 1 - 14 Appropriate nutrition Adequate hydration All the drugs are contraindicated in patients with hypersensitivity reactions to the respective medicines Profound nausea, vomiting, diarrhoea and abdominal discomfort Secondary malignancies Myelosuppression (except the steroids) Steroids (prednisolone) may cause Cushing's syndrome, hypertension, diabetes mellitus, suppression of immunity, infections Vincristine: neurotoxic Cyclophosphamide: alopecia and haemorrhagic cystitis Doxorubicin: cardiotoxic Avoid unnecessary exposure to irradiation and chemicals Agroup of conditions with pathological processes resulting from the presence of Haemoglobin S Usually inherited from the parents who have themselves inherited haemoglobin S The principal genotypes include: - Homozygous sickle cell disease (SS) - Sickle cell-haemoglobin C disease (SC) - Sickle cell-ß thalassaemia (Sß thal) Sickle cell-ß+ thalassaemia Type I (Sß+thal.Type I) Sickle cell-ß+thalassaemia. Type II. (Sß+thal. Type II) 2 2 2 2 2 2 2 Hodgkin's lymphoma MOPP ChIVPP Supportive measures Notable adverse drug reactions, caution Prevention SICKLE CELLDISEASE Introduction - Vary widely according to histological subtype, stage and bulk of disease Full Blood Count (i.e. haemoglobin, haematocrit, leucocyte and differential counts; red cell indices, reticulocyte count) Erythrocyte sedimentation rate Coombs test Bone marrow aspiration and needle biopsy Serum Urea, Electrolytes Serum Uric acid Liver Function Tests: transaminases-ALT, AST, ALP; bilirubin; serum proteins HIV screening Immunoglobulins Chest X- ray Examination of post-nasal space Serum copper level Neutrophil alkaline phosphatase Tomograms of lung or mediastinum Skeletal X-ray Abdominal ultrasound scans Intravenous pyelography CT scans of chest and abdomen Supplementary node biopsy Induce remission Restore patient to disease-free state Maintain state of well being Appropriate nutrition Adequate hydration Red cell and platelet concentrate transfusions as required Malaria prophylaxis: proguanil 200 mg orally daily Antibiotics as indicated Allopurinol 300 mg orally daily (when uric acid is high) (3 weekly): Cyclophosphamide 750 mg/m intravenously on day 1 Doxorubicin 50 mg/m intravenously on day 1 Vincristine 1.4 mg/m (maximum of 2 mg) intravenously on day 1 Prednisolone 100 mg orally on days 1 - 5 (4 weekly): Cyclophosphamide 750 mg/m intravenously on days 1 and 8 Doxorubicin 25 mg/m intravenously on days 1 and 8 Investigations Treatment objectives Non-drug treatment Drug treatment Mandatory Optional Non-Hodgkin's lymphomas CHOP CHOP 2 2 2 2 2 Sickle cell-ß+thalassaemia. Type III. (Sß+thal. Type III) Inheritance of one normal gene controlling formation of ß Haemoglobin (HbA), and a sickle gene (HbS) Total haemoglobin Ais more than haemoglobin S Normal haemoglobin F Inheritance of two abnormal allelemorphic genes controlling formation of ß chains of haemoglobin, at least one of which is the sickle gene Polymerization of the sickle haemoglobin may lead to vaso-occlusion Red cells have reduced deformability and easily adhere to vascular endothelium, increasing the potential for decreased blood flow and vascular obstruction. Abnormalities in coagulation, leucocytes, vascular endothelium, and damage to the membranes of red cells contribute to sickling Haemolytic anaemia and vasculopathy are the result of the various pathophysiologic processes Organ damage is on-going and is often silent until far advanced The course of the disease is punctuated by episodes of pain Vary widely from one patient to another: Persistent anaemia/pallor Growth retardation (variable) Jaundice (variable) Bone pains (recurrent) Prominent facial bones due to increased bone marrow activity Leaner body build and less weight (on average) Some fingers are shortened as a result of infarction (destruction due to blockage of blood supply) Hand-foot syndrome (painful and swollen hands and feet) in childhood Life span on average shorter than normal Sexual development is delayed in both sexes: menarche occurs at a mean age of 15.5 years (range 12 - 20 years) compared to non-sicklers (mean 13.2years) Impotence can occur from prolonged priapism High foetal loss in pregnancy Patient has acute symptoms/signs attributable directly to sickle cell disease Two main types: Pain (vaso-occlusive) crisis Anaemia crisis Sickle cell trait Sickle cell disease Sickle cell crises Vaso-occlusive crises Pathophysiology Clinical features 19 20 Chapter 2: Blood and Blood-Forming Organs Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY Painful Painless Tender, swollen bones Acute hepatopathy Acute chest syndrome Priapism Haematuria Cerebrovascular disease (accident) - in descending order of prevalence - Thrombotic stroke - Seizures - Haemorrhage Retinopathy (commonest in SC patients) Acute splenic (or hepatic) sequestration Hyper-haemolytic (e.g. precipitated by malaria) Megaloblastic (folic acid deficiency) Hypoplastic (due to infection or renal failure) Aplastic (e.g. due to epidemic parvo virus B19) Connective tissue disorders e.g. rheumatoid arthritis Liver disease Other causes of failure to thrive Kidneys: - Hyposthenuria (reduced ability to concentrate urine/conserve body fluids) - Haematuria - Albuminuria - Reduced kidney function Leg ulcers: - Occur around ankles - Heal slowly and tend to recur Bones and Joints - Osteomyelitis - Avascular necrosis These may cause: - Hip pain - Limping gait - Kyphoscoliosis when necrosis affects spinal vertebral bones Infections: - Salmonella osteomyelitis - Pneumococcal pneumonia - Pneumoccoccal meningitis (rare in adolescents and adults) - Tonsillitis and pharyngitis Brain and nerves: - Strokes, seizures (not common in adults) - Meningitis (not common in adults) - Cerebral haemorrhage - Mental neuropathy (rare) Cardiovascular/respiratory: - Heart failure Anaemic crises Differential diagnoses Complications - Emotional stress Blood transfusion (especially red cell transfusion) Anti-pneumococcal vaccine (when patient is well with no complaints): Proguanil 200 mg orally daily under 1 year 25 mg daily; 1 - 4 years 50 mg; 5 - 8 years 100 mg; 9 - 14 years 150 mg orally daily Plus: Folic acid 5 mg orally daily Paracetamol 1 g, every 4 - 6 hours to a maximum of 4 g daily 1 - 5 years 120 - 250 mg; 6 -12 years 250 - 500 mg; 12 -18 years 500 mg every 4 - 6 hours (maximum 4 doses in 24 hours) Or: Aspirin (acetylsalicylic acid) 600 mg orally every 8 hours daily - Not recommended for children under 16 years Or: Ibuprofen 200 mg every 8 hours daily (or other non-steroidal anti-inflammatory drugs) - Not recommended for children under 16 years Parenteral therapy: Diclofenac sodium 75 mg or 100 mg intramuscularly (as necessary) - Not recommended for children Oral therapy: Paracetamol 1 - 5 years 20 mg/kg every 6 hours (maximum 90 mg/kg daily in divided doses) for 48 hours or longer if necessary and if adverse effects are ruled out Then: 15 mg/kg every 6 hours (maintenance) 6 - 12 years: 20 mg/kg (maximum 1 g) 6 hourly (maximum 90 mg/kg daily in divided doses, not to exceed 4 g for 48 hours or longer if necessary and if adverse effects are ruled out Then: 15 mg/kg every 6 hours (maximum 4 g daily) 12 - 18 years: 500 mg - 1g every 4 - 6 hour (maximum 4 doses in 24 hours) Diclofenac potassium 50 mg every 12 hours daily Or: Diclofenac sodium 100 mg once daily Or: Morphine 15 mg every 8 - 12 hours daily Adjunct treatment Drug treatment Steady state Pain crises Adult: Child: Adult: Child: Adult: Child: s Mild pain Moderate-to-severe painful crises - Pulmonary hypertension - Acute chest syndrome Full Blood Count (haemoglobin, haematocrit, total leucocyte count and differential counts, platelet counts) Erythrocyte sedimentation rate Red cell indices (MCH, MCHC, MCV) Reticulocyte count Sickling tests: solubility test; metabisulphite test Haemoglobin electrophoresis - Using cellulose acetate paper at pH 8.4 (alkaline) or citrate agar gel at pH 5.6 (acidic) Serum Electrolytes, Urea and Creatinine Liver function tests (transaminases, bilirubin, serum albumin, alkaline phosphatase and prothrombin time) Urinalysis; microscopy, culture and sensitivity: Sputum - Acid Fast Bacilli - Microscopy, culture and sensitivity Stool: - Ova and parasites - Occult blood Ultra sound scan: - Abdominal ultrasound scan - Transcranial Doppler ultrasonography Chest radiograph Maintain (or restore) a steady state of health Prevent and treat complications Provide accurate diagnosis, relevant health education and genetic counselling to patients, relatives and heterozygotes Improve quality of life Provide a positive self-image in affected persons Counselling and health education Encouraging membership of support groups Providing infection prophylaxis (antimalarial; anti- pneumococcal, hepatitis B virus vaccines) Providing folate supplementation Avoiding pain-inducing conditions Providing prompt treatment of symptoms Advising on contraception Supervising pregnancy/Labour Providing regular health checks Limiting family size Balanced diet Adequate fluid intake (at least 3 litres/24 hours) Avoidance of pain-inducing conditions - Strenous physical exertion or stress - Dehydration - Sudden exposure to extremes of temperature - Infections e.g. malaria Investigations Treatment objectives Treatment strategies Non-drug treatment Antimalarials Artemisinin-based combination therapy (see section on malaria) Counselling and health education Membership of support group Regular health checks Paracetamol should be used with caution in patients with hepatic impairment Opioid analgesics cause varying degrees of respiratory depression and hypotension - They should be avoided when intracranial pressure is suspected to be raised Advice on the risks involved in marriages between carriers, and between sicklers Anti-pneumococcal vaccine Supportive measures Notable adverse drug reactions, caution and contraindications Prevention 21 22 Chapter 2: Blood and Blood-Forming Organs Standard Treatment Guidelines for Nigeria 2008 UNIV ERSIT Y O F IB ADAN L IB RARY CHAPTER 3: CARDIOVASCULAR SYSTEM ANGINAPECTORIS Introduction Clinical features Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment A symptom complex characterised by chest pain or discomfort caused by transient myocardial ischaemia usually due to coronary heart disease Less common in this environment though current studies show increasing prevalence In 90% (or more) of cases there is a hereditary factor Major risk factors: Hypertension Diabetes mellitus Hypercholesterolemia Smoking Obesity Male sex Age Stable angina (chest discomfort on exertion and relieved by rest) Unstable angina (discomfort on exertion and at rest) Myocardial infarction (chest pain or discomfort that lasts more than 30 minutes; may be associated with symptoms of cardiac failure, shock, arrhythmias) Myalgia Pericarditis Aortic dissection Pleurisy Cardiac failure Myocardial infarction Arrhythmias Sudden death Full Blood Count and differentials Urea, Electrolytes and Creatinine Fasting blood glucose Urinalysis; urine microscopy Electrocardiograph: resting, treadmill exercise Echocardiography (resting/exercise) Radio nuclide studies Cardiac enzymes (CK-MB) Coronary angiography Relieve discomfort Improve quality of life Prevent complications Relieve the obstruction Address the risk factors present Dietary manipulation (low salt, low cholesterol diet) Exercise Stop smoking Reduce alcohol consumption Differential diagnoses Complications Investigations Treatment objectives Non-drug treatment Drug treatment Supportive measures Notable adverse drug reactions Prevention CONGENITALHEART DISEASE Introduction Clinical features Sinus arrhythmias Anxiety Cardiac failure Stroke Peripheral embolic phenomena Sudden death Electrocardiograph (resting, 24 hour Holter, 1 month Holter monitoring) Urea, Electrolytes and Creatinine Echocardiography Electrophysiology Abolish the arrhythmias Treat complications Prevent further arrhythmias Pacemaker insertion Ablation (electrophysiology) Cardioversion: acute arrhythmias Depends on the type of arrythmia Ref