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BMS Study Smart Pediatric Review

BMS Study Smart Pediatric Review. Dr. Jinan Darwish MBBCh , LRCPSI , BAO ( NUI) CABP 18 th May, 2013. Cleft lip& palate ( OSCE).

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BMS Study Smart Pediatric Review

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  1. BMS Study Smart Pediatric Review Dr. Jinan Darwish MBBCh , LRCPSI, BAO ( NUI) CABP 18th May, 2013

  2. Cleft lip& palate ( OSCE) • Represents failure of the palatal shelves to approximate or fuse. • incidence of cleft palate is ≈1/2,500 births • Possible causes include maternal drug exposure, a syndrome-malformation complex, or genetic factors (autosomal dominant) • The timing of surgical correction should be individualized. Usually closure of the palate is usually done before 1 yr of age • Recurrent otitis media and hearing loss, malposition of the teeth, speech defects,velopharyngeal incompetence.

  3. Anemia ( MCQs/ OSCE) • A- Aplastic Hb , Plt, neutrophils Normal MCV Low/absent retics • B-Hemolytic HbMCV (retics) LDH Unconjugated bilirubin haptoglobin C-Iron deficiency Hbferriten Iron TIBC HCT MCV

  4. Cushing’s syndrome: Common iatrogenic problem in paeds ex. Steroids in Asthma

  5. congenital hypothyroidism:( OSCE)

  6. Congenital Hypothyroidism • Investigations: Universal neonatal screening Blood Wrist & hand xray Radio-active technetium scan Echocardiogram • Management: • Thyroxine Replacement • Regular Follow up • Complications: Excess thyroxine –advanced bone age Severe mental neurological delay

  7. Cystic Fibrosis (MCQs) Autosomal recessive inheritence Defective CFTR Neonates 15% meconium ileus presentation Sweat test ( gold standard) Complications: Resp (corpulmonale, pneumthorax) GI (Cirrhosis, portal HTN, DIOS) Endocrine ( DM, infertility 99% men & subfertility in females) Psychological/Behavioural

  8. Cystic Fibrosis sweat chloride Chloride concentration > 60mEq/L CF( gene CFTR mutation F508

  9. Developmental Dysplasia of the hip (MCQs) • Associations: FHx ( first degree relative , 20%); first born ( smaller uterus), oligohydramnios, breech dlivery, LSCS • Examination : • OrtolAni: Abduct&Anterior ; Relocate a dislocated hip • Barlow : Adduct & posterior; Dislocates unstable hip.

  10. Developmental Dysplasia of the hip • Diagnosis: USS hip: High false + rate in infants <6 weeks Xray hip: >4 months ( 2 views: adduction & abduction) Management: Supportive: Pavlikharness abduction splints until 5-6 months.USS monitoring Surgical correction : Late diagnosis ( > 6 months)

  11. Diabetes mellitus 1 ( MCQs) • RBS > 11.0 mmol/L • Fasting > 7.0 mmol/L • HbA1c >7.5% • Urinalysis: Ketonuria, glycosuria ( Stable product of non-enzymatic irreversible glycosylation of β-chain of HB by plasma glucose) • DKA

  12. Down Sydrome Trisomy 21 (OSCE)

  13. Febrile Seizures • Rule of 6 : peak 18 months • Usually arise from infection/inflammation outside the CNS in an otherwise neurologically normal child. • Seizures arising from fever due to meningitis/encephalitis are not included in the definition. • Simple: Isolated,brief,generalizedclonic/tonic seizure. • Complex : > 15 mins, focal features,repeat seizure within same illness or incomplete recovery from seizure < 1 hour. • Febrile status epilepticus: > 30mins ( up to 5% present s status epilepticus) • Differential Diagnosis: CNS infection, epilepsy ( 1-2% will develop it)

  14. G6PD deficiency ( MCQ)

  15. Fluids ( MCQ)

  16. Pearl of wisdom

  17. Henoch-Schönleinpurpura (MCQ/OSCE)

  18. Hydrocele (OSCE) • -Small hydroceles in infancy are benign and spontaneously resolve by 9-12 months of age. -Large hydroceles rarely resolve and may cause vascular compromise and testicular atrophy; these should be resected. • -A communicating hydrocele (which changes in size) indicates a completely patent processusvaginalis and has the potential for hernia formation. This variety should also be repaired

  19. Hydrocephalus ( OSCE) • Obstructive • Communicating • CSF production • Mx: Shunt with a one-way valve from from ventricle to peritoneum or right atrium. • Complications: Shunt: obstruction,infection(staph.epidermidis, over drainage subdural haemorrhage. Long-term : Global developmental delay, impaired memory & vision, precocious puberty.

  20. Hypospadias ( OSCE) • With / without chordee • Most are distal • Usually isolated • If assoc with undesc. testis: ambiguous genitalia workup. • Surgical correction between 6-12 months

  21. Infantile Glaucoma (OSCE) • Triad: tearing, Photophobia, blepharospasm • Signs: Large corneas, injection, edema. • Primary: a)Congenital b)Syndromes: Sturge Weber, NF-1,Marfan, Stickler, HCR, OCR(Lowe) C)Ocular abnormalities: • Secondary: Infection(TORCH)/cataracts surgery/trauma/tumor • Treatment primarily surgical

  22. Inflammatory Bowel Disease (MCQ)

  23. Intussusception (MCQ) • - The most common cause of intestinal obstruction between 3 mo and 6 yr of age. • -80% of the cases occur before 24 mo • - The male: female ratio is 4:1. • - if left untreated, most will lead to intestinal infarction, perforation, peritonitis, and death. • - Air, hydrostatic (saline), and, less often, water-soluble contrast enemas have replaced barium examinations • - Air reduction is associated with fewer complications and lower radiation exposure than traditional contrast hydrostatic techniques.

  24. Kawasaki Disease (MCQ/ OSCE) • KAWASAKI DISEASE DIAGNOSTIC CRITERIAKawasaki is also called Muco Cutaneous Lymph Node Syndrome or MCLNSDiagnostic criteria are Fever for > 5 days • + at least 4 of the following 5 criteriaMCLNSM = Mucous membrane changes: injected pharynx, strwberry tongue, injected dry cracked lips • C = Conjunctivitis: non purulent and bilateral (D/D w scarlet fever) • L = Limb changes: edema, erythema and periungueal desquamation • N = Nodes enlargement in the Neck: usulally > 1.5 cm in diameter • S = Scarlattiniform rash 

  25. Labial adhesions (MCQ/ OSCE) • -Common disorder in prepubertal females. Peak incidence around the age of 13-23 months • -The disorder is usually asymptomatic • - May be associated with postvoid dribbling, also called vaginal voiding and occasionally UTIs • -Treatment of labial adhesions is typically conservative. If left untreated, labial adhesions usually spontaneously resolve (80% within 1 year) • - If treatment is necessary based on symptoms or parental request, estrogen cream is indicated twice daily for 2-4 weeks .Once the labia separate, apply emollient 3-5 times a day for several months to allow complete healing and prevention of recurrence.

  26. Measles (MCQ/ OSCE) • Measles: complications "MEASLES COMP" (complications): MyocarditisEncephalitisAppendicitisSubacutesclerosingpanencephalitisLaryngitisEarly deathSh!ts (diarrhoea)Corneal ulcerOtis mediaMesenteric lymphadenitisPneumonia and related (bronchiolitis-bronchitis-croup) 7 Cs of Measles? • Cough • Coryza • Conjunctivitis • Concurrent fever and rash • Coplik (koplik spot • Cephalocaudal spreading rash • Cervical lymphadenopathy

  27. Measles • Measles is caused by the paramyxovirus, and is highly contagious.  • The incubation period is on average 10 days (7-18 days), and patients are usually considered infectious for several days before and after the onset of the rash when the viral load is highest. • There is a prodromal illness which consists of the 3 C's (cough, coryza and conjunctivitis). • Koplikspots (seen in the buccal mucosa) are pathognomonic for measles and are usually seen several days after onset of the ilness. Described as being like "grains of sand" • Rash starts 3-4 days after onset of symptoms. Starts behind the ears and forehead, spreading over the whole body from head to foot. The rash is dark red macules and papules which become confluent and blotchy. • Rash usually lasts for 5-10 days and classically desquamates as it resolves. • Patients should be isolated for at least 5 days after appearance of rash. Any contacts if not immune should be vaccinated within 72 hours. • Anti-measles IgM is generally detectable 3 days after onset of rash. • Anti-measles IgG has a delayed onset before becoming positive - usually peaks at 14 days after onset of rash. • Other diagnostic tests include NPA for measles IF and first pass urine for measles PCR.

  28. Meningitis (OSCE)

  29. Neonatal Jaundice (MCQ)

  30. Pneumonia (OSCE/ MCQ)

  31. Pneumothorax (OSCE) • What is this five week old baby’s chest xray showing? • Answer: spontaneous right-sided tension pneumothorax • Name objects labeled by arrows? • Answer: Blue- ECG lead • Red – Endotracheal tube (ETT) • Green- Nasogastric tube (NGT) • How would you treat? • Answer: Insert right intercostral drain (ICD)

  32. Pyloric Stenosis (MCQ) • Multifactorial inheritence • 30% first-born males • M:F 4:1 • 7% FHx (parental) • 12% Associated Nephrotic Syndrome • Progressive non-bilous vomiting within 30 mins of feed; may become projectile. • Hypochloraemic Hypokalemic Alkalosis • USS abdomen: Pyloric muscle diameter > 3-4 mm & pyloric channel > 18mm in length. • Rx: Ramstedtpyloromyotomy

  33. Rickets ( MCQ/ OSCE)

  34. Retinoblastoma (OSCE) • Leukocoria • Absent red reflex • Should not be missed

  35. Scabies (OSCE) • Sarcoptesscabei • Mechanism of spread: prolonged direct human contact( >20mins)- holding hands/playing contact games.Fomite (towels,underclothing,toilet seats) • Rx: Permethrin 5% dermal cream applied to all areas below the neck overnight. • Malathion 0.5% (Allergic to Permethrin) • Mittens < 2 years prevents excoriation & secondary infection.

  36. SCDmode of inheritence; premarital & new born screening, how to read HPLC

  37. TalipesEquinovarus (Club foot) • Features: CAVE (cavus, adductus, varus, equinus). 1) Plantar flexion of foot at ankle 2) Inversion of heel 3) Medial Deviation of forefoot. • Categories: 1) Idiopathic 2) Neurogenic 3) Syndromic(Larsen sn, Arthrog) • Always check for assoc. anomalies, specially spine. • Early treatment(first day): Casting , rapid correction • Late treatment: Surgical

  38. Tetralogy of Fallot (MCQ) • Most common cyanotic disease. • components PROVe Pulmonary HypertensionRight Ventricular HypertrophyOverriding AortaVSD

  39. Thalassemia( OSCE)

  40. Transposition of Great Arteries • Most common cyanotic disease in first 24 hours

  41. Turner Syndrome (MCQ/ OSCE) • Turner Syndrome Patients look like CLOWNS (only to remember its features):C - Cardiac anomalies (most common - coarctation of aorta)L - Lymphoedema, low thyroidO - Ovaries under developed (streak ovaries), primary AmenorrheaW - Webbed neckN - Nipples widely placedS - Short stature, Sensoneural hearing loss, Short 4th metacarpal

  42. Undescended Testis (MCQ/ OSCE) • 4.5% of boys have undesc. Testis at birth • 0.8% by age 6 months • Will not descend after 4 months of age • Histopathologic changes demonstratable by 6-12 months of age. • Surgical correction at 6months of age(no later than 9-15months)- success rate of 98% • LHRH, HCG: Not effective

  43. Varicella ( chicken pox) ( MCQ/ OSCE)

  44. MCQs

  45. 1. A patient with acute asthma is most likely to have decreased • Forced expiratory volume in 1 second • Residual volume • Functional residual capacity • Total lung capacity • Tidal volume

  46. 2. A 14-month old infant has had a clear nasal discharge and a mild cough for about two weeks. Despite therapy with Amoxicillin, his condition has not improved. The cough has worsened and the infant has been vomiting at each feeding. The leukocyte count is 20,000/mm3 with 80% lymphocytes, 15% neutrophils and 5% band forms. X-ray study of the chest shows bilateral perihilar infiltrates with shaggy heart border. The most likely diagnosis is • Acute bronchial asthma • Acute lympocytic leukemia with pulmonary infiltration • Influenza virus infection • Pertusis • Hemophilusinfluenzae pneumonia

  47. 3.Regarding steroid inhaler therapy for a 4-year old child with asthma, you should tell the mother • It is most effective with acute exacerbation • It should be administered every two hours when the child is having an acute episode of asthma • The primary value is in the prevention of episodes of asthma • It will not be of value in the management of the child’s exercise-induced episodes of asthma • The most common side effect is the development of oral candidiasis.

  48. 4. A 2-1/2 year old child has had cough for two weeks after having been hospitalized one month ago for pneumonia in the right lower lobe. X-ray study of the chest reveals persistence of pneumonia in the right lower lobe. Of the following , the most likely diagnosis is • Staphylococcal pneumonia • Foreign body aspiration • Cystic Fibrosis • Pneumonia due to Mycoplasma pneumoniae • Pneumonia due to respiratory synctial virus

  49. 5. School phobias (school refusals) in grade-school children most commonly result from • Fear of a strict teacher • Concern about failing school • Fear of separation from parent • Desire to avoid unfriendly classmates • Inability to do school work

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