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Approach to a Case of Congenital Heart Disease

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Approach to a Case of Congenital Heart Disease BAI JERBAI WADIA HOSPITAL FOR CHILDREN PEDIATRIC CLINICS FOR POST GRADUATES PREFACE This book is a compilation of the discussions carried out at the course for post-graduates on ” Clinical Practical Pediatrics” at the Bai Jerbai Wadia Hospital for Children, Mumbai. It has been prepared by the teaching faculty of the course and will be a ready-reckoner for the exam-going participants. This manual covers the most commonly asked cases in Pediatric Practical examinations in our country and we hope that it will help the students in their practical examinations. An appropriately taken history, properly elicited clinical signs, logical diagnosis with the differential diagnosis and sound management principles definitely give the examiner the feeling that the candidate is fit to be a consultant of tomorrow. Wishing you all the very best for your forthcoming examinations. Dr.N.C.Joshi Dr.S.S.Prabhu Program Directors. FOREWARD I am very happy to say that the hospital has taken an initiative to organize this CME for the postgraduate students. The hospital is completing 75 years of its existence and has 2 done marvelous work in providing excellent sevices to the children belonging to the poor society of Mumbai and the country. The hospital gets cases referred from all over the country and I am proud to say that the referrals has stood the confidence imposed on the hospital and its faculty. We do get even the rarest of the rare cases which get diagnosed and treated. I am sure all of you will be immensely benefited by this programme. Wish you all the best in your examination and career. Brig (Retd.) . Dr. K.B.N.S.Dod Chief Senior Executive Wadia Group of Hospitals TIPS FOR CANDIDATES Ten Commandments 1. Dress appropriately as a future consultant. Always wear a neatly washed and ironed apron. Sit straight and mind your body language, especially your speech and hand movements i.e. practice speaking before appearing for exams. 2. Always be able to summarize, encapsulate the essence and emphasize the major issues without losing too much detail. Hence practice case presentations. Mental rehearsal of the case helps in fluent presentation and makes you appear confident. 3. Wish the examiner/s when you enter and thank them when you leave. 3 4. Present the case boldly, confidently and clearly with an attitude of a future consultant and not a resident (poor speech affects your viva performance). 5. Maintain eye contact with the examiner/s when answering questions 6. When asked to demonstrate clinical signs, give a brief description of what you want to show the examiners. Always be brief and factual and avoid jargon, slang, abbreviations and meaning-less expressions. 7. Never antagonize or argue with the examiners .You will always lose. Remember that the examiner is the judge, jury and the final word. 8. Be clear in what you want to tell. Avoid statement like slightly pale, not looking good, maybe edematous….. 9. Always have your own equipment set which includes- pens, paper, growth charts, stethoscope, fundoscope, otoscope, measuring tape, cotton, knee hammer, tuning fork, pins, torch with batteries, Colorful toys, disposable spatulas, hand held eye charts (if available). 10. Always appear for the examination with a positive attitude. It helps. 4 List of contributors Anaita Hegde. Archana.Limaye Ira Shah. K.N. Shah. Kumud.P.Mehta. Meena. P.Desai. M. P. Colaco. N.C. Joshi. Parmananad.A. Priti Mehta. Rajesh Joshi. Ruchira Pahare. Shakuntala Prabhu. Shilpa Kamat. Sudha Rao. Sumitra Venkatesh. Uma.S.Ali. Vrajesh Udani. 5 INDEX 1. Tuberculous Meningitis 6 2. Cerebral Palsy 9 3. Acute infantile hemiplegia 12 4. Ataxia 17 5. Duchenne’s muscular dystrophy 20 6. Floppy Infant 22 7. Chorea 25 8. Hydrocephalus 28 9. Meningomyelocoele 31 10. Paraplegia 34 11. Guillian Barre Syndrome 39 12. Neuroregression 42 13. Congenital Heart Diseases 48 14. Rheumatic Heart Disease 56 15. PEM 65 16. Rickets 70 17. Short stature 73 18. Ambigious Genetalia 78 19. Bronchiectasis 81 20. Hepatosplenomegaly with anaemia 84 21. Hepatosplenomegaly with jaundice with PHT 89 22. Cholestatic syndrome of infancy 91 23. Rheumatoid Arthritis 94 24. Nephrotic Syndrome 97 25. Newborn 99 6 TUBERCULOUS MENINGITIS Name Age Sex Address Consanguinity Handedness Chief Complains Fever - mild, moderate, low grade with evening rise. Convulsions :- focal / generalised seizures (usually not along with onset of fever ,late in the course of fever) Altered sensorium :- onset - sudden / insiduous. Lethargy Vomiting Focal neurological deficit - Hemiplegia / monoplegia / cranial neuropathies. Origin/Duration/Progress Chief complains in details. Narrative history :- H/o. Abnormality of higher functions - Lethargy , altered sensorium Convulsions Cranial nerve palsies - deviation of angle of mouth, drooling of saliva, squinting, diplopia. Focal neurological deficits ( hemiplegia /monoplegia). Abnormal / involuntary movements tremors / chorea / hemiballismus H/s/o increased intracranial pressure i.e. vomiting / headache / blurring of vision. H/s/o meningeal inflammation i.e.neck pain, photophobia, restriction of neck movement. H/o bowel, bladder complaints . History for etiology :- H/o. head injury ( may precipitate TBM) H/o. otorrhoea - (pyogenic meningitis ) H/o. any treatment taken outside in f/o intramuscular / intravenous injections (Partially treated pyogenic meningitis) H/o. vaccines / drugs / sera ( Acute disseminated encephalomyelitis) H/o. rash, fever, altered sensorium, convulsions ( Viral encephalitis) H/o. fever with rash (measles) H/o. whooping cough. H/o. contact with tuberculosis. H/o. diarrhoea, fever, chronic cough (HIV) H/o. immunosuppressive drug intake. Immunisation history – BCG , Measles. History for complications :- H/o bed sores, contractures, skin changes, bladder, bowel complications. (constipation/ urinary infection ) 7 H/o. seizures. H/o. decorticate / decerebrate posturing. Drug history, procedure history. H/o. any surgery, VP shunt / reservoir Family history - of koch’s Nutritional history - malnutrition may precipitate Tuberculous meningitis. Birth History :- Developmental history. Socio economic history - Overcrowding , sanitation. Examination :- General examination :- 1] Decubitus 2] Vitals - Temperature ,Pulse , Respiration , Blood pressure. 3] Anthropometry with interpretation. 4] Pallor, cyanosis, clubbing, icterus, lymphadenopathy, edema feet, 5] Stigmata of tubercolosis – Phlycten ,Scrofuloderma ,Sinuses, erythema nodosum 6] Anterior fontanelle 7] Size & heaviness of head 8] crack pot sign 9] BCG scar - present / absent. 10] Neurocutaneous markers 11] Dysmorphic features 12] Presence or absence of IV line, Ryles tube 13] Skull, spine, scars 14] Skin - bedsores 15] Contractures 16] Signs of malnutrition & vitamin deficiency 17] Presence / absence & patency of VP shunt CNS :- Higher functions - state of conciousness Gag reflex Eye movements Pupillary reflexes Corneal / conjunctival reflexes Motor system examination Sensory system Cerebellar signs Meningeal signs Hydrocephalus : Heavy head, crackpot or sutural seperation - Signs of increased intracranial pressure. Involuntary movements Fundus - papilloedema / choroid tubercules / optic atrophy. 8 Diagnosis :- ---years old M/F child with chronic meningoencephalitis with / without hemi / monoparesis with / without cranial nerve palsy with / without involuntary movement with / without signs of increased intracranial pressure. Probable etiology being TBM. Investigations :- Specific for diagnosis of tuberculous meningitis 1] CSF examination (after fundus examination} CSF for PCR , Tubercular antigen & ADA levels , Tubercular stearic acid and Bromide partition test. 2] Neuroimaging - CT scan with contrast (in c/o increased intracranial pressure CT should be done prior to lumbar puncture & LP should be guarded /LP under cover of mannitol). 3] MT 4] X ray chest 5] Gastric lavage for Acid fast bacillus. 6] CBC - with lymphocytosis & ESR 7] HIV 8] Liver function tests ( prior to treatment & for treatment monitoring} 9] Renal function test 10] Eletrolyte - baseline as well as monitoring to rule out SIADH Commonly asked questions : 1] Discussion of differential diagnosis 2] Stages of coma 3] Stages of TBM & prognosis in each stage. 4] Signs of meningeal irritation. 5] Signs of increased intracranial pressure 6] Types of herniation 7] Management of TBM - supportive + definitive 8] Types of shunt & complications of shunt 9] Complication of TBM 10] Pathology in TBM & lesion localization 11] CT correlates in TBM 12] Precipitating factors in TBM 13] Poor prognostic factors in TBM 14] Role of steroids 15] Newer modalities of diagnosis of TBM 9 CEREBRAL PALSY Name Age Sex Handedness Consanguinity Chief complaints: - delayed milestones, - convulsions. O.D.P. - Convulsions - Generalised tonic clonic / myoclonic / focal Infantile spasms. - Detailed birth history. Antenatal period - maternal drugs, Xrays, illnesses-like rash , PIH ,DM , fall - Milestone History Gross motor , fine adaptive ,social , language (with rough DQ to each category). - Hand preference. - Scissoring (difficulty in putting diaper). - Floppiness of body . - Power in limbs. - Impairment of vision, hearing. - Squinting, CN palsies. - Swallowing difficulties. - Involuntary movement - Dystonia, tremors, chorea, dyskinesia. - limb dyskinesia , oromotor dyskinesia , jark in the box tongue. - Mannerisms, stereotypies. - Bladder, bowel involvement. For etiology :- Birth details :Antenatal Infections, twins , trauma,drugs Neonatal sepsis, kernicterus Meconium, asphyxia, hypoglycemia.,NICU
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