Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0030305 (pancreatitis)
 16,014 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients with profound decrease of alpha1-antitrypsin (PiZZ) presented with severe pannicultis (Weber-Christian disease); one had systemic panniculitis including pancreatitis. Another possible case is quoted from the literature. Although milder forms of panniculitis can have normal Pi phenotypes and alpha1-antitrypsin levels, the marked reduction of antiproteolytic activity found in PiZZ homozygotes may predispose to or aggravate the lesions of Weber-Christian disease.
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PMID:Alpha1-antitrypsin deficiency with severe panniculitis. Report of two cases. 30 70

The authors report 5 cases of subcutaneous cytosteatonecrosis (Weber-Christian syndrome of pancreatic origin) and review 68 cases in the world literature. The skin, bone joint and general manifestations may appear without any clinical or radiological sign of pancreatitis. The rise in blood and urinary amylase and lipase, the skin lesions, the joint pleural and peritoneal effusions, orient the diagnosis towards the pancreas and suggest a full radiological arteriographic and echotomographic investigation. The pancreatic disease was pancreatitis in 50 cases, cancer in 18 cases. Too long a delay between the initial signs and the diagnosis may lead to early operation even in the absence of pancreatic signs. The cancer may still be limited and removable. The pancreatitis was in 18 cases a false cyst of the head in 2/3rds, whether obvious clinically or not. Operation led to its discovery usually but may miss the lesion which is then only discovered on autopsy. Removal or early by pass operations transform the prognosis which is otherwise fatal. Analysis of these cases illustrates well the problems of indication operative management and efficacy of surgery in a disease still relatively unfamiliar both to physicians and surgeons.
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PMID:[Subacute cytosteatonecrosis of pancreatic origin. Surgical problem in 5 cases (author's transl)]. 60 49

We report a 4-year-old child with Weber-Christian panniculitis who subsequently developed histologically proven chronic active hepatitis, pancreatitis and extensive lipoatrophy. An "LKM" variant autoantibody was detected in the serum and a favourable response has been seen with immunosuppressive therapy. These findings lend support to the concept of Weber-Christian being an "auto-immune" disease.
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PMID:Weber-Christian panniculitis and chronic active hepatitis. 376 82

A case of acute Weber-Christian disease is reported, in which pancreatitis was accompanied by evidence of dissemination of pancreatic enzymes causing necrosis of fat and vessels. There is clinical and experimental evidence in the literature to suggest that widespread vascular dissemination of lipase occurs in cases of pancreatitis or pancreatic carcinoma. Review of the autopsy literature of cases of Weber-Christian disease shows that a majority had pancreatitis and systemic involvement of fat. A minority showed lesions confined to the panniculus, which tended to ulcerate; these lesions were in other ways not typical of Weber-Christian disease. In this group none had autopsy evidence of pancreatitis.The opinion is expressed that Weber-Christian disease results from disruption of pancreatic tissue and subsequent vascular dissemination of pancreatic enzymes.
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PMID:The Relation of Pancreatic Disease to Weber-Christian Disease. 2032 82