Isolated deficiency of glucocorticoids presenting with cholestasis

BERBEROĞLU M., Yigit S., Ocal G., KANSU TANCA A., Tarcan A., Girgin N., ...More

ACTA PAEDIATRICA JAPONICA, vol.40, no.4, pp.378-380, 1998 (SCI-Expanded) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 40 Issue: 4
  • Publication Date: 1998
  • Doi Number: 10.1111/j.1442-200x.1998.tb01953.x
  • Journal Name: ACTA PAEDIATRICA JAPONICA
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus
  • Page Numbers: pp.378-380
  • Keywords: cholestasis, congenital unresponsiveness to ACTH, isolated deficiency of glucocorticoids, ADDISONS-DISEASE, ADRENAL INSUFFICIENCY, ACHALASIA, ALACRIMA, CARDIA
  • Ankara University Affiliated: Yes

Abstract

Background: Isolated deficiency of glucocorticoids is characterized by elevated levels of adrenocorticotropin (ACTH) and normal aldosterone production. It is rare for isolated deficiency of glucocorticoids to be associated with liver involvement. A case of an infant with isolated deficiency of glucocorticoids presenting with cholestasis is presented in this article. A male infant on his 39th postnatal day was referred to our hospital for evaluation of prolonged jaundice and convulsion. He had two episodes of hypoglycemic convulsion on postnatal 8th and 39th day, after which he was admitted to our hospital.