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Sundaram B.,Kanchi Kamakoti CHILDS Trust Hospital | Sundaram B.,CHILDS Trust Medical Research Foundation | Amperayani S.,Kanchi Kamakoti CHILDS Trust Hospital | Amperayani S.,CHILDS Trust Medical Research Foundation | And 4 more authors.
Indian Journal of Pediatrics | Year: 2014

Failure of response to therapy in childhood tuberculosis may be due to resistance, paradoxical response or immunodeficiency. Interferon-γ (IFNγ) plays a major role during host defense against Mycobacterium tuberculosis (Mtb). An 8-y-old boy presented with fever, hepatosplenomegaly and parotid abscess. He had been earlier treated for BCG adenitis in infancy and at 5 y for TB osteomyelitis of iliac bone and recovered. Investigations confirmed disseminated Mycobacterium Avium Intracellulare infection. He was investigated for immunodeficiency because of recurrent mycobacterial disease and a partial defect of γ-interferon receptor was identified. He required a 2 y course of therapy with a 7 drug regimen and recovered. The authors report this case because of its rarity and to highlight the need to consider γ-interferon receptor defect in the presence of recurrent tuberculosis (TB) and also review the options for therapy. © Dr. K C Chaudhuri Foundation 2013.

Ramachandran P.,Institute of Liver Disease and Transplantation | Ramachandran P.,Childs Trust Medical Research Foundation | Safwan M.,Institute of Liver Disease and Transplantation | Safwan M.,Childs Trust Medical Research Foundation | And 4 more authors.
Indian Pediatrics | Year: 2015

Need and purpose of review: Biliary atresia is a progressive obstructive cholangiopathy and is fatal if left untreated within 2 years of life. Delay in referral is because of difficulties in differentiating it from physiologic jaundice and identifying an abnormal stool color. This paper presents an overview on the diagnosis and discusses the current strategies in the management of this disease in developing countries. Methods: Articles were retrieved from the PubMed database using the terms ‘biliary atresia’, ‘Kasai portoenterostomy’ and ‘pediatric liver transplantation’. Contents of the article are also based on personal experience of the authors. Conclusion: A national screening program using stool color cards as part of standard care in the neonatal period will greatly improve early detection of biliary atresia. Outcomes will improve if it is diagnosed at the earliest after birth, the child is referred to an experienced pediatric hepatobiliary unit for evaluation, and undergoes an early Kasai procedure. If an early Kasai portoenterostomy is performed, nearly half of all children survive into adolescence, and about one-third are likely to have a long-term, symptom-free life with normal liver biochemistry. Sequential treatment combining Kasai as first line and liver transplantation as second line results in 90% survival for children with biliary atresia. © 2015, Indian Academy of Pediatrics.

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