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

BRASLAVSKY D; KESELMAN A; GALOPPO M; LEZAMA C; CHIESA A, ; GALOPPO C; BERGADÁ I

ARQUIVOS BRASILEIROS DE ENDOCRINOLOGIA E METABOLOGIA

SBEM-SOC BRASIL ENDOCRINOLOGIA & METABOLOGIA

Año: 2011 vol. 55 p. 622 - 627

0004-2730

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: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: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:Subjects and methods: Sixteen patients (12 males) were included; eleven with CPHD, and five with isolated central hypocortisolism. Results:Results: Onset of cholestasis occurred at a median age of 18 days of life (range 2120). Ten and nine patients had elevated transaminases and gGT, 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 reevaluated after remission of cholestasis.gGT, 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 reevaluated after remission of cholestasis.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 reevaluated after remission of cholestasis.