Isolated deficiency of glucocorticoids presenting with cholestasis

Pediatrics International

Volumn 40, Issue 4, 1998, Pages 378-380

  Berberoǧlu, Merih ^a   Yiǧit, Sevket ^a   Öcal, Gönül ^a   Kansu, Aydan ^a   Tarcan, Aylin ^a   Girgin, Nurten ^a   Suskan, Emine ^a  

^a ANKARA UNIVERSITY   (Turkey)

Author keywords

Cholestasis; Congenital unresponsiveness to ACTH; Isolated deficiency of glucocorticoids

Indexed keywords

ALANINE AMINOTRANSFERASE; ALDOSTERONE; ASPARTATE AMINOTRANSFERASE; BILIRUBIN; CORTICOTROPIN; GAMMA GLUTAMYLTRANSFERASE; GLUCOCORTICOID; HYDROCORTISONE; HYDROXYPROGESTERONE; LACTATE DEHYDROGENASE; PRASTERONE SULFATE;

ARTICLE; CASE REPORT; CHOLESTASIS; CONVULSION; HEPATOMEGALY; HORMONE DEFICIENCY; HUMAN; HYPERPIGMENTATION; INFANT; JAUNDICE; LIVER BIOPSY; MALE; PRIORITY JOURNAL; SPLENOMEGALY;

ADRENOCORTICOTROPIC HORMONE; ALDOSTERONE; CHOLESTASIS; GLUCOCORTICOIDS; HUMANS; HYDROCORTISONE; INFANT; MALE;

EID: 0031817418     PISSN: 13288067     EISSN: 1442200X     Source Type: Journal    
DOI: 10.1111/j.1442-200X.1998.tb01953.x     Document Type: Article

References (12)

1. Di George Am. Disorders of adrenal glands. In: Behrman Re. Kliegman Rm. Nelso We. Vaughan Vc (eds). Textbook of Pediatrics. 14th edn. Philadelphia. WB Saunders. 1992: 1 p. 1440.
2. Werbel Ss, Ober Kp. Acute adrenal insufficiency. Endocrinol. Metub. Clin. North Am. 1993; 22: 303–8. 1.
3. Tobin Mv, Aldridge Sa. Morris Al. Belchetz Pe, Gilmore It. Gastrointestinal manifestations of Addison’s disease. Am. J. Gastroenterol. 1989:84: 1302–5.
4. Olsson Rg, Lindgren A., Zettergren L. Liver involvement in Addison’s disease. Am. J. Gastroenterol. 1990; 85: 435–8.
5. Shepard Th, Landing Bh, Mason Dg. Familial Addison’s disease. Case reports of two sisters with corticoid deficiency unassociated with hypoaldosteronism. Am. J. Dis. Child 1959; 97. 154–62.
6. Migeon Cj, Kenny Fm, Kowarski A. et al. The syndrome of congenital adrenocortical unresponsiveness to ACTH. Report of six cases. Pediatr. Res. 1968; 2: 501–13.
7. Grant Db, Barnes Nd, Dumic B. et al. Neurological and adrenal dysfunction in the adrenal insufficiency, alacrima, achalasia (3A) syndrome. Arch. Dis. Child 1993; 68: 779–82.
8. Geffner Me, Lippe Bm, Kaplan Sa. Selective ACTH insen-sitivity, achalasia and alacrima: A multisystem disorder presenting in childhood. Pediatr. Res. 1983; 17: 532–6.
9. Allgrove J., Clayden Gs, Grant Db, Macaulay Jc. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet 1978; 1: 1284–6.
10. Grant Db, Dunger Db, Smith I., Hyland K. Familial glucocorticoid deficiency with achalasia of the cardia associated with mixed neuropathy, long tract degeneration and mild dementia. Eur. J. Pediatr. 1992; 151: 85–9.
11. Leblanc A., Odievre M., Hadchouel M., Gendrel D., Chaussain Jl, Rappaport R. Neonatal cholestasis and hypoglycemia: Possible role of Cortisol deficiency. J. Pediatr. 1981; 99: 577–80.
12. Boulton R., Hamilton M., Dhillon Ap, Kinloch D., Burroughs Ak. Subclinical Addison’s disease: A cause of persistent abnormalities in transaminase values. Gastroenterology 1995; 109: 1324–7.