Biomaterial Database

Hepatic pathology in porphyria cutanea tarda. 1983

J H Lefkowitch, and M E Grossman

We examined clinical and laboratory data and the liver pathology of 48 patients in whom porphyria cutanea tarda was related to alcohol ingestion, estrogen use and pregnancy, or was idiopathic. Biochemical test results, when abnormal, tended to be mild in most cases, with less than two-fold elevations of serum aminotransferases and alkaline phosphatase and mild hyperbilirubinemia. Fatty change, liver cell and Kupffer cell hemosiderosis and glycogenation of hepatocyte nuclei were frequent histologic findings in the 58 liver specimens studied. Alcoholic hepatitis, chronic hepatitis and cirrhosis were uncommon. Granuloma-like lobular aggregates consisting of iron- and ceroid-laden Kupffer cells, chronic inflammatory cells and fat droplets ("lobular lesions of porphyria cutanea tarda") were found in nearly two-thirds of specimens and appeared to be the most characteristic form of parenchymal damage in this form of porphyria. These lesions may be associated with pericentral fibrosis in alcoholic as well as estrogen-treated patients and may remit following therapeutic phlebotomy.

UI	MeSH Term	Description	Entries
D007728	Kupffer Cells	Specialized phagocytic cells of the MONONUCLEAR PHAGOCYTE SYSTEM found on the luminal surface of the hepatic sinusoids. They filter bacteria and small foreign proteins out of the blood and dispose of worn out red blood cells.	Kupffer Cell,Cell, Kupffer,Cells, Kupffer
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008103	Liver Cirrhosis	Liver disease in which the normal microcirculation, the gross vascular anatomy, and the hepatic architecture have been variably destroyed and altered with fibrous septa surrounding regenerated or regenerating parenchymal nodules.	Cirrhosis, Liver,Fibrosis, Liver,Hepatic Cirrhosis,Liver Fibrosis,Cirrhosis, Hepatic
D008107	Liver Diseases	Pathological processes of the LIVER.	Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008111	Liver Function Tests	Blood tests that are used to evaluate how well a patient's liver is working and also to help diagnose liver conditions.	Function Test, Liver,Function Tests, Liver,Liver Function Test,Test, Liver Function,Tests, Liver Function
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D011164	Porphyrias	A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.	Porphyria,Porphyrin Disorder,Disorder, Porphyrin,Disorders, Porphyrin,Porphyrin Disorders
D011247	Pregnancy	The status during which female mammals carry their developing young (EMBRYOS or FETUSES) in utero before birth, beginning from FERTILIZATION to BIRTH.	Gestation,Pregnancies
D011248	Pregnancy Complications	Conditions or pathological processes associated with pregnancy. They can occur during or after pregnancy, and range from minor discomforts to serious diseases that require medical interventions. They include diseases in pregnant females, and pregnancies in females with diseases.	Adverse Birth Outcomes,Complications, Pregnancy,Adverse Birth Outcome,Birth Outcome, Adverse,Complication, Pregnancy,Outcome, Adverse Birth,Pregnancy Complication