4th Pediatric Infectious Diseases Conference
 
 
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Specialist Answers
Question
Dear sir a young couple closely related to each other has a big problem. They have 6 children 3 alive & 3 died due to unknown cause. Those children who died usually had problem at the age 4-6month. They suffered from a chest infection which is resistant to treatment. Those who died were male & female both. The 1st died at 4m, 2nd died at 6m, 3rd died 2year after a protracted course of illness started as LRT infection & lymphadenopathy . The age of the alive children are 8years (female) , 3years(female), 18months(male).The 1st & 3rd are totally normal.The 2nd child ie 3year(female) run the same illness LRT infection , lymphadenopathy, FTT progress to chronic lung disease + clubbing of finger+ chronic exuding ,fascial widespreading skin infection . Those who died & this patient. are fully vaccinated ,while those survived are not vaccinated . On examination chronically ill child was under weight dyspenic with + clubbing of fingers Vital sign are normal apart from tachypenia. Generalized lymphadenopathy, Resp. system: bilateral rhonchi & crepition Cvs. System: normal Soft abdomen, mild hepatosplenomegaly Investigation: CBC was normal GOT+GPT normal. Blood urea & creatinin was normal Immunoglobulin assay showed normal ESR 50. Sweat test was normal Toxoplasmosis titer: -ve Blood , urine, stool culture are –ve Tt reaction –ve ACE normal Sputum for Tb bacilli –ve Gastric aspirate for Tb bacilli –ve Bone scan was normal. Abdominal sonar was normal Cxr & CAT scan chest infection & hilar lymphadenopathy +lung fibrosis. Lymph node & lung biopsy :Follcular hyperplastic lymphadenitis with evidence of progressive transformation of germinal centers & occasional mini clusters of epitheloid & giant cells without true granuloma formation or caseation necrosis. This is most consistent with: a. progressive transformation of germinal centers. b. Toxoplasmosis & other specific(none-Tb) infection. The skin lesion as seen by an expert dermatologist to be mostly as Tb of skin. What is your opinion about this case? Waiting your advice.
Answer
What is serum IgE? Also is there persistent neutrophilia. One should rule out Hyper IgE and Chronic granulomatous disease. A serum IgE level, NBT test and peripheral smear for neutrophil granules is advisable.
 
 
 
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