Conjugated Hyperbilirubinemia in a Neonate with Meconium lleus and Congenital Hypothyroidism

Mekonyum ileusu kistik fibrozisin eşsiz belirtisidir. Mekonyum ileus ile çeşitli birliktelikler, özellikle kistik fibrozis rapor edilmiştir; ancak hastamız kistik fibrozis ile beraber rapor edilen ve konjenital hipotiroidism ile konjuge hiperbiiirubinemiyi bulunduran ilk hastadır. Bu iki durumun mekanizmaları için detaylı açıklamalar bulunmaktadır; ancak literatür araştırması sonrasında muhtemel ortak mekanizma için bir açıklama bulunamamıştır. Bu durumun sadece birliktelik olduğu düşünülmüştür. Konjenital hipotiroidizm kistik fibrozis olan ve kojuge hiperbilirubinemi olan hastalarda göz ardı edilmemeli ve araştırılmalıdır.

Anahtar Kelimeler:

Mekonyum ileus, konjuge hiperbilirubinemi, çocuk, kistik fibrosis.

Mekonyum ileus ve Konjerıital Hipotroidizm Olan Bir Yenidoğanda Konjuge Hiperbilirubirıemi

Meconium ileus is a unique manifestation of cystic fibrosis. Different anomalies associated with meconium ileus, conse-quently in cystic fibrosis, were reported but our case was the first reported with congenital hypothyroidism demonstrating signi-ficant conjugated hyperbilirubinemia. There are detailed explanations about the mechanisms of these two entities but we found no possible common pathvvay for these diseases in the evaluation of the literatüre. We suggest that this unique formation may only be coexistence. Congenital hypothyroidism may be evaluated for the patients with cystic fibnosis when conjugated hyperbilirubinemia is detected.

Keywords:

Meconium ileus, conjugated hyperbilirubinemia, children, cystic fibrosis.,

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Chaudry G, Navarro OM, Levine DS, Oudjhane K. Abdominal manifestations o f cystic fibrosis in children. Pediatr Radiol 2006;36:233-40.
Li Z, Lai HCJ, Kosorok MR, et. al. Longitudinal Pulmonary
Status o f Cystic Fibrosis Children With Meconium lleus. Pediatric Pulmonology 2004; 38:277-84.
Munck A, Gerardin M, Alberti C, et al. Clinical outcome of cystic fibrosis presenting with or without meconium ileus: A matched cohort study. Journal of Pediatric Surgery 2006; 41:1556-60.
Escobar MA, Grosfeld JL, Burdick JJ, et al. Surgical consid
erations in cystic fibrosis: A 32-year evaluation of outcomes. Surgery 2005;138:560-72.
Olivieri A, Medda E, De Angeiis S, et al. The Study Group
for Congenital Hypothyroidism. High risk o f congenital hypothyroidism in multiple pregnancies J Clin Endocrin Metab. First published ahead o f print May 8, 2007.
Olıvıerı A, Stazı MA, Mastroıacovo P, Fazım C, Medda E,
Spagnolo A, De Angelıs S, Grandolfo ME, Taruscıo D, Cordeddu V, Sorcını M and The Study Group For Congenital Hypothyroidism. Poputation-Based Study on the Freçuency ofAdditional Congenital Malformations in Infants with Congenital Hypothyroidism: Data from the Italian Registry for Congenital Hypothyroidism (1991-1998). The Journal o f Clinical Endocrinology & Metabolism 2002;87: 557-62.
Beardsall K, Ogilvy-Stuart AG. Congenital hypothyroidism. Current Paediatrics 2004;14:422-9.
Kenna JG, Neuberger J, Mieli-Vergani G, Mowat AP, VVİlliams R. Halothane hepatitis in children. Brıtısh Medıcal Journal Volüme 1987;294:1209-11.
Gupta R, Nagdeve NG, Sarin YK. Neonatal Surgical Jaundice Revisited. Indian J Pediatr 2005;72:415-23.
Harris KB, Pass KA. Increase in congenital hypothyroidism in New York State and in the United States. Molecular Genetics and Metabolism 2007;91:268-77.
Kopp P. Perspective: Genetic Defects in the Etiology of Congenital Hypothyroidism. Endocrinology, June 2002;143: 2019-24.