Biomaterial Database

Liver disease in association with neonatal lupus erythematosus. 1993

N Evans, and K Gaskin

Department of Perinatal Medicine, King George V Hospital, Camperdown, New South Wales, Australia.

This report describes a patient with neonatal lupus erythematosus (NLE) in whom there was multisystem involvement including neonatal hepatitis. The hepatitis, defined pathologically as a giant cell hepatitis, presented with severe cholestasis, was unrelated to other known causes of neonatal hepatitis and resolved spontaneously by 6 months of age. Both mother and infant were positive for Sjögren syndrome A+B antibodies (SS-A[Ro] and SS-B[La]) as well as having high titres of antinuclear antibody (ANA). Three of the four cases described in the one previous report of this association were also ANA positive. This antibody may be a marker for the development of the hepatitis. Maternal and infant ANA status should be determined in cases labelled as idiopathic neonatal hepatitis to exclude undiagnosed maternal SLE as a cause of neonatal cholestasis.

UI	MeSH Term	Description	Entries
D007231	Infant, Newborn	An infant during the first 28 days after birth.	Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D007567	Jaundice, Neonatal	Yellow discoloration of the SKIN; MUCOUS MEMBRANE; and SCLERA in the NEWBORN. It is a sign of NEONATAL HYPERBILIRUBINEMIA. Most cases are transient self-limiting (PHYSIOLOGICAL NEONATAL JAUNDICE) occurring in the first week of life, but some can be a sign of pathological disorders, particularly LIVER DISEASES.	Icterus Gravis Neonatorum,Neonatal Jaundice,Physiological Neonatal Jaundice,Severe Jaundice in Neonate,Severe Jaundice in Newborn,Jaundice, Physiological Neonatal,Neonatal Jaundice, Physiological
D008180	Lupus Erythematosus, Systemic	A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow.	Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D008297	Male		Males
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002779	Cholestasis	Impairment of bile flow due to obstruction in small bile ducts (INTRAHEPATIC CHOLESTASIS) or obstruction in large bile ducts (EXTRAHEPATIC CHOLESTASIS).	Bile Duct Obstruction,Biliary Stasis,Bile Duct Obstructions,Biliary Stases,Cholestases,Duct Obstruction, Bile,Duct Obstructions, Bile,Obstruction, Bile Duct,Obstructions, Bile Duct,Stases, Biliary,Stasis, Biliary
D005260	Female		Females
D006327	Heart Block	Impaired conduction of cardiac impulse that can occur anywhere along the conduction pathway, such as between the SINOATRIAL NODE and the right atrium (SA block) or between atria and ventricles (AV block). Heart blocks can be classified by the duration, frequency, or completeness of conduction block. Reversibility depends on the degree of structural or functional defects.	Auriculo-Ventricular Dissociation,A-V Dissociation,Atrioventricular Dissociation,A V Dissociation,A-V Dissociations,Atrioventricular Dissociations,Auriculo Ventricular Dissociation,Auriculo-Ventricular Dissociations,Block, Heart,Blocks, Heart,Dissociation, A-V,Dissociation, Atrioventricular,Dissociation, Auriculo-Ventricular,Dissociations, A-V,Dissociations, Atrioventricular,Dissociations, Auriculo-Ventricular,Heart Blocks
D006505	Hepatitis	INFLAMMATION of the LIVER.	Hepatitides
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man