PMID: 8583141Nov 1, 1995Paper

Copper-associated liver disease in childhood

Journal of Hepatology
A BakerA P Mowat

Abstract

Indian childhood cirrhosis is associated with high liver copper concentrations and progressive liver disease with a high mortality. Early treatment with penicillamine was found to reduce mortality and reverse liver damage. We aimed to define the clinical features of copper-associated liver disease outwith the Indian subcontinent and encourage the earlier consideration of the syndrome in cryptogenic liver disease. Three European children presented between 10 and 29 months of age with abdominal distension, pyrexia and hepatosplenomegaly. Over 1-5 weeks their condition deteriorated rapidly due to liver failure. Two died within 2 months of onset and one received a successful liver transplant. In two cases consideration of the diagnosis occurred only on examination of the liver after orthotopic liver transplant or death. Light microscopy was used, with haematoxylin and eosin, reticulin and orcein stains. Tissue, plasma and water copper levels were measured by flame atomic absorption spectrometry. All had micronodular cirrhosis and severe hepatocellular necrosis with Mallory bodies and copious-orcein positive material. Liver copper concentrations ranged from 1100-1310 micrograms/g dry weight. For two patients domestic water with high...Continue Reading

References

Jan 1, 1979·Human Heredity·S S AgarwalP C Bajpai
Jan 1, 1987·Virchows Archiv. A, Pathological Anatomy and Histopathology·J Müller-HöckerG Hübner
Nov 1, 1987·Archives of Disease in Childhood·M S TannerA N Pandit
Jul 1, 1987·Journal of Pediatric Gastroenterology and Nutrition·S A BhaveM S Tanner
Nov 1, 1987·Journal of Pediatric Gastroenterology and Nutrition·G MaggioreG R Burgio
Jun 1, 1973·Archives of Disease in Childhood·J Walker-Smith, J Blomfield
Nov 1, 1983·Indian Journal of Pediatrics·B S TomarC Verma
Aug 29, 1980·Biochemical and Biophysical Research Communications·G SimoninA Paraf
Jul 29, 1982·The New England Journal of Medicine·J H LefkowitchJ W Hagstrom
Apr 1, 1980·Journal of Comparative Pathology·S Haywood
Aug 1, 1994·Seminars in Liver Disease·G Mieli-Vergani, D Vergani
Oct 1, 1994·Gut·S P HorslenM F Lowry

❮ Previous
Next ❯

Citations

Jan 28, 2003·Journal of Hepatology·Thomas MüllerCisca Wijmenga
Jun 25, 2003·Toxicology·Lisa M Gaetke, Ching Kuang Chow
Jun 6, 1998·European Journal of Clinical Investigation·G S Fell
Dec 1, 1996·European Journal of Pediatrics·K E Mühlendahl
Apr 22, 2014·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Anna HordyjewskaJoanna Kocot
May 12, 2006·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Kishor B RajaSaman Warnakulasuriya
Feb 26, 2013·The British Journal of Oral & Maxillofacial Surgery·Gururaj ArakeriPeter A Brennan
Aug 5, 1998·Pathology, Research and Practice·J Müller-HöckerB Rodeck
Dec 27, 2005·Clinics in Liver Disease·Nanda KerkarFrederick J Suchy
Oct 1, 2004·The Journal of Biological Chemistry·Marina ZeleninaAnita Aperia
Dec 16, 1998·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·R Jaffe
Jul 30, 1999·Journal of Oral Pathology & Medicine : Official Publication of the International Association of Oral Pathologists and the American Academy of Oral Pathology·C TrivedyN W Johnson

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.