2004;25;388 Pediatrics in Review Frederick J. Suchy Neonatal Cholestasis http://pedsinreview.aappublications.org/content/25/11/388 located on the World Wide Web at: The online version of this article, along with updated information and services, is http://pedsinreview.aappublications.org/content/suppl/2005/01/26/25.11.388.DC1.html Data Supplement (unedited) at: Pediatrics. All rights reserved. ...

during a period of three years from 1996 to 1998, 124 infants (64 male and 60 female) with an age range of 1-6 months (mean age 1.5 months) with cholestasis were studied. idiopathic neonatal hepatitis was the most common cause of cholestasis, accounting for 48 cases with a rate of 3'8.70% in a total of 124 patients, followed by galactosemia in 29 patients (23.38%) and extrahepatic biliary ...

Cholestasis results from impairment in the excretion of bile, which may be due to mechanical obstruction of bile flow or impairment of excretion of bile components into the bile canaliculus. When present, cholestasis warrants prompt diagnosis and treatment. The differential diagnosis of cholestasis beyond the neonatal period is broad and includes congenital and acquired etiologies. It is impera...

Optic nerve hypoplasia with hypopituitarism and intact septum pellucidum is a variant of septo-optic dysplasia or deMorsier's syndrome.' Although neonatal jaundice has been seen with this syndrome, the association with severe prolonged cholestatic jaundice has not been emphasised. We describe three patients who presented with cholestatic jaundice and were found to have optic nerve hypoplasia an...

Congenital hypopituitarism is a rare condition associated with possible serious complications and long-term neurological sequelae, if not promptly recognized and treated.1 Neonates with congenital hypopituitarism may present with or without associated developmental defects, such as ocular, midline, and genital abnormalities. They may also present with nonspecific symptoms, including hypoglycemi...

Neonatal cholestasis is a potentially life-threatening condition requiring prompt diagnosis. Mutations in several different genes can cause progressive familial intrahepatic cholestasis, but known genes cannot account for all familial cases. Here we report four individuals from two unrelated families with neonatal cholestasis and mutations in NR1H4, which encodes the farnesoid X receptor (FXR),...

Background and objectivesLiver damage in cholestasis is multifactorial, yet bile acid-mediated hepatotoxicity pivotal. Honey consumption has many physiological effects; it influences detoxification process (phase I, II III), antioxidant, anti-inflammatory, immune-stimulant, anti-ulcer, wound/burn healing effects others. The acid ursodeoxycholic (UDCA) currently used off-label to treat neonatal ...

BACKGROUND AND OBJECTIVE Rapidly establishing the cause of neonatal cholestasis is an urgent matter. The aim of this study was to report on the prevalence and mortality of the diverse disorders causing neonatal cholestasis in an academic center in Germany. METHODS Clinical chemistry and cause of disease were retrospectively analyzed in 82 infants (male n = 42, 51%) that had presented with neo...

OBJECTIVE Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of...