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


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.


CONCLUSIONS
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.
División de Endocrinología, Centro de Investigaciones Endocrinológicas, Buenos Aires, Argentina. dbraslavsky@cedie.org.ar
Débora
D
Braslavsky
Ana
A
Keselman
Marcela
M
Galoppo
Carol
C
Lezama
Ana
A
Chiesa
Cristina
C
Galoppo
Ignacio
I
Bergadá
D000309:Adrenal Insufficiency / Q000209:etiology* / Q000503:physiopathology; D017668:Age of Onset; D002779:Cholestasis / Q000209:etiology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D020249:Hormone Replacement Therapy / Q000379:methods; D006801:Humans; D006854:Hydrocortisone / Q000172:deficiency / Q000627:therapeutic use*; D007018:Hypopituitarism / Q000151:congenital* / Q000188:drug therapy; D007223:Infant; D008107:Liver Diseases / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D010908:Pituitary Hormones, Anterior / Q000172:deficiency; D012074:Remission Induction; D012189:Retrospective Studies; D013974:Thyroxine / Q000627:therapeutic use*; D016896:Treatment Outcome
D010908:Pituitary Hormones, Anterior; D013974:Thyroxine; D006854:Hydrocortisone