선천성 부신피질 과형성증(Congenital Adrenal Hyperplasia)에 동반된 신생아 담즙정체증 1례
A Case of Neonatal Cholestasis Associated with Congenital Adrenal Hyperplasia
1
조회
0
다운로드
1
인용
0
참고문헌
키워드
초록
저자들은 색소침착과 기력저하, 담즙정체증을 보인 2개월 여아에서 호르몬 검사와 복부 MRI 검사로 선천성 부신피질 과형성증으로 진단하고 히드로코르티손과 광물부신겉질호르몬을 투여하여 담즙정체증과 색소 침착의 완전한 회복을 경험하였기에 문헌 고찰과 함께 보고하는 바이다.
Abstract
It has been suggested that cortisol deficiency may be responsible for the cholestasis. Although congenital hypopituitarism was reported as a possible cause of neonatal cholestasis, congenital adrenal hyperplasia with neonatal cholestasis is a very rare report in the literature. We experienced a case presenting with neonatal cholestasis associated with congenital adrenal hyperplasia. This case provides a clinical evidence supporting the notion that cortisol has an important physiological role in bile acid synthesis and transport
참고문헌 (11)
- Herman SP, "Liver dysfunction and histologic abnormalities in neonatal hypopituitarism", 87, pp.892~5 (1975)
- Sheehan AG, "Neonatal cholestasis,hypoglycemia,and congenital hypopituitarism", 14, pp.426~30 (1992)
- Kaufman FR, "Neonatal cholestasis and hypopituitarism", 59, pp.787~9 (1984)
- Drop SL, "Hyperbilirubinemia and idiopathic hypopituitarism in the newborn period", 68, pp.277~80 (1979)
- Leblanc A, "Neonatal cholestasis and hypoglycemia:possible role of cortisol deficiency", 99, pp.577~80 (1981)
- 양혜란, "선천성 뇌하수체 기능저하증과 동반된 신생아 담즙정체 1례", Pediatric Gastroenterology, Hepatology & Nutrition, 5(2), pp.199~205 (2002), 대한소아소화기영양학회
- Labrune P, "Bilirubin uridine diphosphate glucuronosyltransferase hepatic activity in jaundice associated with congenital hypothyroidsm", 14, pp.79~82 (1992)
- Berberoglu M, "Isolated deficiency of glucocorticoids presenting with cholestasis", 40, pp.378~80 (1998)
- Olsson RG, "Liver involvement in Addison's disease", 85, pp.435~8 (1990)
- Bauman JW, "The effect of adrenalectomy and hypophysectomy on bile flow in the rats", 52, pp.404~ (1966)
- Macarol V, "Hydrocortisone cholestasis in the dog", 49, pp.1714~23 (1970)