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)

Fibrocalculous pancreatic diabetes (FCPD) is a form of diabetes secondary to tropical calcific pancreatitis (TCP). The usual age of onset of FCPD is between 15 and 50 years. This paper reports on two unusual cases of FCPD with age of onset below 5 years. This is the first report of FCPD in infancy and raises intriguing questions about the rapidity with which calcification occurs in this entity.
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PMID:Fibrocalculous pancreatic diabetes in infancy--two case reports. 782 Nov 93

Fibrocalculous pancreatic diabetes (FCPD) is a form of diabetes associated with tropical chronic calcific pancreatitis, seen mostly in developing countries. FCPD is likely to be a multifactorial disease with both environmental and genetic components. The reg 1A gene encodes a protein associated with regeneration of pancreatic islets and has a sequence identical to that of pancreatic stone protein. Since FCPD is associated with both diabetes and pancreatitis, we tested the hypothesis that FCPD may be the result of mutations in the coding regions of the reg 1A gene. Restriction length polymorphisms (RFLPs) and possible sequence variants of the reg 1A gene were studied by RFLP analysis, looking for single-stranded conformational polymorphisms (SSCPs) and direct nucleotide sequencing. In 20 patients with FCPD and 20 control subjects, no RFLPs were detected using 10 restriction enzymes. In 50 patients with FCPD and 50 control subjects, no SSCP variants were detected. Finally, direct nucleotide sequencing of the reg 1A gene from 30 patients with FCPD did not show any differences from the published human reg 1A gene sequence. In conclusion, it seems unlikely that mutations in the coding region of the reg 1A gene are a common cause of FCPD.
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PMID:Analysis of islet regenerating (reg) gene polymorphisms in fibrocalculous pancreatic diabetes. 905 83

Fibrocalculous pancreatopathy is a form of diabetes, associated with tropical chronic calcific pancreatitis, in which islet beta-cell loss and pancreatic stone formation are found. It is likely to be a multifactorial disease with both genetic and environmental components. Regenerating (reg) gene encodes protein that has been involved in pancreatic lithogenesis and the regeneration of islet cells and therefore the abnormality of reg genes could be associated with fibrocalculous pancreatopathy. In this study, regla and reg1beta mRNAs were isolated from peripheral blood lymphocytes obtained from 16 patients with fibrocalculous pancreatopathy, 42 patients with type 1 diabetes, 37 patients with type 2 diabetes, and 22 normal controls. mRNAs were amplified by reverse-transcription polymerase chain reaction (RT-PCR) and analysed by a single strand conformation polymorphism (SSCP) technique. The reg1alpha and reg1beta mRNAs were isolated, indicating the ectopic expression of these genes in peripheral blood lymphocytes; however, variation among mobility patterns was not observed in the SSCP analysis of the RT-PCR products. The results indicated that there was no abnormality of the regla and reg1beta mRNAs obtained from the study groups.
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PMID:Analysis of the reg1alpha and reg1beta gene transcripts in patients with fibrocalculous pancreatopathy. 1223 39

Fibrocalculous pancreatic diabetes (FCPD) is an uncommon form of diabetes that occurs as a result of chronic calcific pancreatitis, in the absence of alcohol abuse. The disease is restricted to tropical regions of the world, and southern India has the highest known prevalence of FCPD. The typical patient with FCPD is a lean adolescent or young adult of either sex, presenting with history of recurrent bouts of abdominal pain and steatorrhea. Demonstration of large, discrete pancreatic calculi by plain radiographs or ultrasonography of the abdomen is diagnostic. While the exact etiology of FCPD is unknown, genetic, nutritional and inflammatory factors have been hypothesized to play a role. Diabetes in FCPD is often brittle and difficult to control; most patients require multiple doses of insulin for control of glycemia. However, in spite of high blood glucose levels, patients rarely develop ketosis. Malabsorption responds to pancreatic enzyme supplementation. Surgical removal of stones is indicated for symptomatic relief of intractable pain. While patients with FCPD develop microvascular complications as frequently as those with type 2 diabetes, macrovascular disease is uncommon. Development of pancreatic malignancy is the most dreaded complication and should be suspected in any patient who complains of weight loss, back pain or jaundice.
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PMID:Fibrocalculous pancreatic diabetes (FCPD). 2539 47