Abstract
Yenidoğan kolestaz nedenleri geniş bir hastalık spektrumu- nu içine alır. Bu spektrum içinde yer alan hastalıklar eks- trahepatik safra yolları tıkanıklığı, infeksiyonlar, metabolik hastalıklar ve panhipopituitarizm gibi hastalıklardır. Sekonder adrenal yetmezliğe bağlı yenidoğan kolestaz vakası çok az sayıda bildirilmiştir. Burada 29. gestasyon haftasında doğan ve “respiratuar distress” sendromu nedeniyle izlemi sırasında direkt hiperbiluribinemisi orta- ya çıkan bir vaka sunulmuştur. Vaka neonatal kolestaz ayırıcı tanısına giren hastalıklar yönünden araştırıldı ve sekonder adrenal yetmezlik tanısı konuldu. Hastanın direkt hiperbilirubinemisi fizyolojik dozda 15 mg/m2/gün hidro- kortizon tedavisi ile iki hafta içerisinde tamamen düzeldi. Sekonder adrenal yetmezlik, neonatal kolestaz nedenleri arasında ender görüldüğünden ayırıcı tanıda akılda tutul- ması gereği vurgulandı
Keywords
References
- Sheehan AG, Martin SR, Stephure D, Scott RB. Neonatal cholestasis, hypoglycemia, and congenital hypopituitarism. J Pediatr Gastroenterol Nutr 1992; 14:426-30.
- hypoglycemia: Manifestations of congenital anterior hypopi- tuitarism. J Clin Endocrinol Metab 1996; 81:2786-9.
- and hypogonadism in the newborn infant. J Pediatr 1956; 48:782-92.
- Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr 2000; 89:951-4.
- Gönç EN, Kandemir N, Andıran N, Özön A, Yordam N. Cholestatic hepatitis as a result of severe cortisol deficiency in early infancy: report of two cases and review of literature. Turk J Pediatr 2006; 48:376-9.
- resolves persistent neonatal jaundice in multiple hormone deficiencies. Helv Paediatr Acta 1988; 43:219-23.
- Neustein HB. Neonatal cholestasis and hypopituitarism. Arch Dis Child 1984; 59:787-9.
Abstract
Neonatal cholestasis is a disease secondary to a large spectrum of diseases, which includes extrahepatic biliary atresia or obstruction, infectious diseases, inborn errors of metabolism and panhypopituitarism. There are few reports on neonatal cholestasis associated with secondary adrenal failure. We reported here a case of neonatal cholestasis. The baby was born at 26-weeks of gestational age and followed up because of respiratory distress syndrome and neonatal cholestasis. The investigations revealed secon- dary adrenal failure as the etiology of neonatal cholestasis. Cholestasis resolved after hydrocortisone replacement at physiological doses 15 mg/m2/day though two weeks. Secondary adrenal failure, is extremely rare, should be considered in the differential diagnosis of neonatal choles- tasis
References
- Sheehan AG, Martin SR, Stephure D, Scott RB. Neonatal cholestasis, hypoglycemia, and congenital hypopituitarism. J Pediatr Gastroenterol Nutr 1992; 14:426-30.
- hypoglycemia: Manifestations of congenital anterior hypopi- tuitarism. J Clin Endocrinol Metab 1996; 81:2786-9.
- and hypogonadism in the newborn infant. J Pediatr 1956; 48:782-92.
- Kelly DA. Investigation and outcome of neonatal hepatitis in infants with hypopituitarism. Acta Paediatr 2000; 89:951-4.
- Gönç EN, Kandemir N, Andıran N, Özön A, Yordam N. Cholestatic hepatitis as a result of severe cortisol deficiency in early infancy: report of two cases and review of literature. Turk J Pediatr 2006; 48:376-9.
- resolves persistent neonatal jaundice in multiple hormone deficiencies. Helv Paediatr Acta 1988; 43:219-23.
- Neustein HB. Neonatal cholestasis and hypopituitarism. Arch Dis Child 1984; 59:787-9.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Research Articles |
| Authors | |
| Publication Date | July 1, 2008 |
| Published in Issue | Year 2008 Volume: 8 Issue: 3 |