Systemic mastocytosis with portal hypertension. Autopsy findings and ultrastructural study of the liver. 1985

L Ghandur-Mnaymneh, and E Gould

Systemic mastocytosis is a rare cause of portal hypertension with only two previously reported cases to our knowledge. A 51-year-old man who was seen with intractable diarrhea and abdominal distention was found to have this combination at autopsy. At autopsy the liver showed bridging portal fibrosis. Subendothelial collagen deposition was demonstrated ultrastructurally. We present the possible mechanisms of portal hypertension in systemic mastocytosis.

UI MeSH Term Description Entries
D006975 Hypertension, Portal Abnormal increase of resistance to blood flow within the hepatic PORTAL SYSTEM, frequently seen in LIVER CIRRHOSIS and conditions with obstruction of the PORTAL VEIN. Cruveilhier-Baumgarten Disease,Cruveilhier-Baumgarten Syndrome,Cruveilhier Baumgarten Disease,Cruveilhier Baumgarten Syndrome,Disease, Cruveilhier-Baumgarten,Portal Hypertension,Portal Hypertensions,Syndrome, Cruveilhier-Baumgarten
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
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D014582 Urticaria Pigmentosa The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules. Maculopapular Cutaneous Mastocytosis,Cutaneous Mastocytoses, Maculopapular,Cutaneous Mastocytosis, Maculopapular,Maculopapular Cutaneous Mastocytoses

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