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...

Background: Jaundice is frequently associated with extrahepatic systemic infections. The important causes include increased bilirubin levels due to hemolysis, hepatic dysfunction, reduced excretion and cholestasis. Sepsis induced cholestasis has a unique pathophysiologic basis. Although common in gram negative sepsis among neonates, the clinical features of sepsis induced cholestasis are not wi...

BACKGROUND Parenteral nutrition-associated liver disease can be a progressive and fatal entity in children with short-bowel syndrome. Soybean-fat emulsions provided as part of standard parenteral nutrition may contribute to its pathophysiology. METHODS We compared safety and efficacy outcomes of a fish-oil-based fat emulsion in 18 infants with short-bowel syndrome who developed cholestasis (s...

decreased cardiac responsiveness to adrenergic stimulation has been observed in cholestatic liver disease, but the cause remains unclear. previous reports have suggested that nitric oxide overproduction might have a role in cholestasis-induced bradycardia via inhibition of l-type calcium channels. in the present study, the digoxin has been used to increase cardiac ca2+ transient in male sprague...

Prolonged neonatal jaundice is defined as a jaundice lasting more than 14 days of life in the full-term infants [1,2]. Etiologically it is helpful to distinguish jaundice related to unconjugated (indirect) or conjugated (direct) hyperbilirubinemia. A prolonged unconjugated hyperbilirubinemia may be related to breastfeeding or to some pathological conditions as hemolytic diseases (due to Rh or A...

Hereditary or familial hiperbilirubinemia comprises a group of syndromes (Dubin-Johnson’s, Rotor’s, hepatic storage disease) in which hyperbilirubinemia, predominantly unconjugated or conjugated, occurs as an isolated biochemical abnormality without evidence of either hepatocellular necrosis or cholestasis. We present a patient with Dubin-Johnson syndrome, one of the familial disorders associat...

The mechanism of cholestasis (decreased bile flow) induced by taurolithocholate in the isolated perfused hamster liver was investigated. Taurocholate was infused to maintain bile acid output, and sulfobromophthalein (BSP) was administered to establish a BSP transport maximum in bile. The effects of taurolithocholate on bile flow and on the biliary secretion of BSP and bile acid anions were dete...

Mitochondrial impairment is hypothesized to contribute to the pathogenesis of chronic cholestatic liver diseases. Mitofusin 2 (Mfn2) regulates mitochondrial morphology and signaling and is involved in the development of numerous mitochondrial-related diseases; however, a functional role for Mfn2 in chronic liver cholestasis which is characterized by increased levels of toxic bile acids remain u...

Intrahepatic cholestasis represents a heterogeneous group of disorders that begin during childhood, most commonly manifesting as neonatal cholestasis, and lead to ongoing liver dysfunction in children and adults. For children, inherited pathogenic factors of cholestasis have gained increasing attention owing to the rapid development of molecular biology technology. However, these methods have t...

We report a case of Dubin-Johnson syndrome presenting with neonatal cholestasis. Liver histology was studied during the neonatal period and at 6 years of age. Distinct brownish pigment granules in hepatocytes were noted. This case confirms that Dubin-Johnson syndrome is a cause of neonatal cholestasis.