Neonatal cholestasis in congenital pituitary hormone deficiency and isolated hypocortisolism: characterization of liver dysfunction and follow-up.

Abstract

INTRODUCTION:

Neonatal cholestasis due to endocrine diseases is infrequent and poorly recognized. Referral to the pediatric endocrinologist is delayed.

OBJECTIVE:

We characterized cholestasis in infants with congenital pituitary hormone deficiencies (CPHD), and its resolution after hormone replacement therapy (HRT).

SUBJECTS AND METHODS:

Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism.

RESULTS:

Onset of cholestasis occurred at a median age of 18 days of life (range 2-120). Ten and nine patients had elevated transaminases and γGT, respectively. Referral to the endocrinologist occurred at 32 days (range 1 - 72). Remission of cholestasis occurred at a median age of 65 days, whereas liver enzymes occurred at 90 days. In our cohort isolated, hypocortisolism was a transient disorder.

CONCLUSION:

Cholestasis due to hormonal deficiencies completely resolved upon introduction of HRT. Isolated hypocortisolism may be a transient cause of cholestasis that needs to be re-evaluated after remission of cholestasis.

• Authors: Débora Braslavsky, Ana Keselman, Marcela Galoppo, Carol Lezama, Ana Chiesa, Cristina Galoppo, Ignacio Bergadá
• Journal: Arquivos brasileiros de endocrinologia e metabologia (Arq Bras Endocrinol Metabol) Vol. 55 Issue 8 Pg. 622-7 (Nov 2011) ISSN: 1677-9487 [Electronic] Brazil
• PMID: 22218445 (Publication Type: Journal Article)

Chemical References

• Pituitary Hormones, Anterior
• Thyroxine
• Hydrocortisone

Topics

• Adrenal Insufficiency (etiology, physiopathology)
• Age of Onset
• Cholestasis (etiology, physiopathology)
• Female
• Follow-Up Studies
• Hormone Replacement Therapy (methods)
• Humans
• Hydrocortisone (deficiency, therapeutic use)
• Hypopituitarism (congenital, drug therapy)
• Infant
• Liver Diseases (etiology, physiopathology)
• Male
• Pituitary Hormones, Anterior (deficiency)
• Remission Induction
• Retrospective Studies
• Thyroxine (therapeutic use)
• Treatment Outcome