17.6.2013(pediatrics)40 Flashcards
Epilepsy in autism
1/5 to 1/3
Mental retardation in children with autism
75%
Onset of autism
Before age of 3 years
Specific marker for autism
Two or more trophoblastic inclusions
3 essential criteria for diagnosis of autism
Impaired social interaction
Impaired communication
Restricted, repetitive range of interests,behaviours,activities
Seizure disorder with loss of language skills
Landu kleffner syndrome
Age of onset of landu kleffner syndrome
5 1/2 years
Landu kleffner syndrome is common in
Boys
DD for landu kleffner syndrome
Autism
DOC for landu kleffner syndrome
Sodium valproate
CT and MRI in landu kleffner syndrome
Normal
Rx of Lennox gestaut syndrome
Ketogenic diet
Rolandic epilepsy
Benign childhood partial epilepsy with centrotemporal spikes
Age of onset of rolandic epilepsy
9-10yrs
Presentation of rolandic epilepsy
Partial seizures
Motor and sensory symptoms are often confined to face
Prognosis of rolandic epilepsy
Excellent
Spontaneous remission after 14-16yrs
Rx of rolandic epilepsy
Carbamazepine for 2 years
Rasmussen encephalitis
Subacute inflammatory encephalitis
Rasmussen encephalitis presents as
Epilepsia partialis continua
Cause of Rasmussen encephalitis
Autoantibodies that stimulate glutamate receptors
Prognosis of Rasmussen encephalitis
Poor
Progressive Myoclonic epilepsy
Lafora disease
Mental deterioration is characteristic feature of
Lafora disease
Presentation of Lafora disease
Initially GTCS,later Myoclonic jerks
Lafora bodies are seen in
Eccrine sweat gland duct cells
Gene involved in Lafora disease
6p
Codes for tyrosine phosphatase
Cause of renal artery stenosis in young
India(Takayasu)
West(Fibromuscular dysplasia)
Bleeding from umbilical stump suggests
Factor XIII deficiency
Test to detect factor XIII deficiency
Clot stability in 5M urea
Why Von willi brand disease pts don’t present in neonatal period
Plasma Von willi brand levels are high(maternal)
Presentation of factor XIII deficiency
Bleeding
Delayed wound healing
Infertility in males
Abortion in females
Physiological jaundice,duration
Phase 1 5 days(term infants) 12mg/dl 7 days(preterm infants) 15mg/dl Phase 2 Values decline to 2mg/dl which lasts for 2 weeks
Breast feeding jaundice,onset
1 week
Cause of breast feeding jaundice
Reduced feed
Dehydration
Unconjugated hyperbilirubinemia
Breast milk jaundice,onset
4-7 days
May continue till 3rd month
Cause of breast milk jaundice
Inhibitors of conjugation present in breast milk
Beta pregananediol
Free fatty acids
Primary prophylaxis should be given within
9 days
Drugs not used for primary prophylaxis
Tetracyclines
Sulpha drugs
Secondary prophylaxis of rheumatic fever
Prevention of colonisation of URI in pts who already had a previous attack of RF or established RHD
Drugs used for primary prophylaxis of RF
Benzathine pencillin 1.2M units i.m single dose Penicillin V for 10 days Erythromycin Cephalexin Cefadroxil Clindamycin Azithromycin Erythromycin Clarithromycin
Secondary prophylaxis duration
21 years of age or 5 years after the last attack of RF whichever is later
If heart disease is present life long prophylaxis or atleast till 40 yrs of age
Secondary prophylaxis,drugs
Benzathine pencillin 1.2MU every 3-4 weeks
Pencillin V 500mg bd
Secondary prophylaxis in penicillin allergic pt
1.SULPHADIAZINE
Penicillin and sulfonamide allergy
Macrolides
Type of rickets not associated with myopathy
X linked hypophosphatemic rickets
Diff btw hypophosphatemic and calcium deficient rickets
No tetany No dental enamel hypoplasia No profound myopathy No rachitic rosary and Harrison sulcus In hypophosphatemic rickets