| The course of neonatal cholestasis in congenital combined pituitary hormone deficiency. | |
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MedLine Citation:
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PMID: 17663294 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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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. |
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Authors:
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G Binder; D D Martin; I Kanther; C P Schwarze; M B Ranke |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of pediatric endocrinology & metabolism : JPEM Volume: 20 ISSN: 0334-018X ISO Abbreviation: J. Pediatr. Endocrinol. Metab. Publication Date: 2007 Jun |
Date Detail:
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Created Date: 2007-07-31 Completed Date: 2007-08-27 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9508900 Medline TA: J Pediatr Endocrinol Metab Country: England |
Other Details:
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Languages: eng Pagination: 695-702 Citation Subset: IM |
Affiliation:
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University Children's Hospital, Tuebingen, Germany. gerhard.binder@med.uni-tuebingen.de |
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| MeSH Terms | |
Descriptor/Qualifier:
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Child Child, Preschool Cholestasis / congenital, diagnosis, etiology*, physiopathology* Cryptorchidism / complications, physiopathology Failure to Thrive / complications, drug therapy, physiopathology Female Hormone Replacement Therapy Human Growth Hormone / therapeutic use Humans Hydrocortisone / therapeutic use Hypoglycemia / congenital, etiology, physiopathology Hypopituitarism / complications*, congenital, drug therapy, physiopathology* Infant Infant, Newborn Infant, Newborn, Diseases / drug therapy, physiopathology* Male Optic Nerve / abnormalities Penis / abnormalities Retrospective Studies Thyroxine / therapeutic use |
| Chemical | |
Reg. No./Substance:
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12629-01-5/Human Growth Hormone; 50-23-7/Hydrocortisone; 7488-70-2/Thyroxine |
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