Document Detail

The course of neonatal cholestasis in congenital combined pituitary hormone deficiency.
MedLine Citation:
PMID:  17663294     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Neonatal cholestatic hepatitis is frequently associated with congenital combined pituitary hormone deficiency (CCPHD). Data on the course of this hepatopathy are scarce. AIM: We retrospectively analyzed the data of all CCPHD infants with cholestasis who presented at the University Children's Hospital, Tuebingen. RESULTS: All infants (n = 9; 2 females) presented with early and prolonged jaundice, failure to thrive and recurrent hypoglycemia. All males had micropenis and 3/7 cryptorchidism. Median age at diagnosis was 1.4 months. Cholestasis began at a median age of 13 days (range 5-31) and resolved at 88 days (54-174). Maximum direct bilirubin level was 6.9 mg/dl (2.4-11.6). Peaks of ALP (median 721 U/l), ALT (148 U/l) and AST (195 U/l) occurred 2-4 weeks later, while GGT levels were elevated in only two infants (167 U/l). Functional liver parameters were always normal. Liver biopsies (n = 4) showed canalicular cholestasis and mild portal eosinophilic infiltration. TEBIDA radioisotope excretion into the intestinal tract was blocked. Substitution with Lthyroxine, hydrocortisone and growth hormone seemed to accelerate the cure from cholestasis. Liver function at follow-up (median 4 yr) stayed normal. CONCLUSION: Cholestasis in CCPHD follows the course described here, frequently with normal GGT levels.
G Binder; D D Martin; I Kanther; C P Schwarze; M B Ranke
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric endocrinology & metabolism : JPEM     Volume:  20     ISSN:  0334-018X     ISO Abbreviation:  J. Pediatr. Endocrinol. Metab.     Publication Date:  2007 Jun 
Date Detail:
Created Date:  2007-07-31     Completed Date:  2007-08-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9508900     Medline TA:  J Pediatr Endocrinol Metab     Country:  England    
Other Details:
Languages:  eng     Pagination:  695-702     Citation Subset:  IM    
University Children's Hospital, Tuebingen, Germany.
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MeSH Terms
Child, Preschool
Cholestasis / congenital,  diagnosis,  etiology*,  physiopathology*
Cryptorchidism / complications,  physiopathology
Failure to Thrive / complications,  drug therapy,  physiopathology
Hormone Replacement Therapy
Human Growth Hormone / therapeutic use
Hydrocortisone / therapeutic use
Hypoglycemia / congenital,  etiology,  physiopathology
Hypopituitarism / complications*,  congenital,  drug therapy,  physiopathology*
Infant, Newborn
Infant, Newborn, Diseases / drug therapy,  physiopathology*
Optic Nerve / abnormalities
Penis / abnormalities
Retrospective Studies
Thyroxine / therapeutic use
Reg. No./Substance:
12629-01-5/Human Growth Hormone; 50-23-7/Hydrocortisone; 7488-70-2/Thyroxine

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