Genetic mechanisms underlying the pathogenesis of tropical calcific pancreatitis

Mahurkar, Swapna; Reddy, D. Nageshwar; Rao, G. V.; Chandak, Giriraj Ratan

doi:10.3748/wjg.15.264

Cited by 35 publications

(8 citation statements)

References 54 publications

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“…Tropical calcific pancreatitis (TCP) is an idiopathic, juvenile, nonalcoholic form of chronic pancreatitis with a unique geographical distribution, while fibro-calculous pancreatic diabetes (FCPD) is a condition, characterized by the development of diabetes secondary to TCP. A genetic etiology for TCP and FCPD has been suggested from its familial aggregation and geographical distribution (94)(95)(96). However, comprehensive screening for Reg IA gene rules out association with TCP and FCPD (97,98).…”

Section: Islet Regeneration and Diabetesmentioning

confidence: 99%

Pancreatic Stone Protein/Regenerating Protein Family in Pancreatic and Gastrointestinal Diseases

Jin

Hayakawa

et al. 2011

Intern. Med.

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Pancreatic stone protein (PSP; reported in 1979), pancreatitis-associated protein (PAP; 1984) and regenerating protein (Reg I;1988) were discovered independently in the fields of the exocrine (pancreatitis) and endocrine (diabetes) pancreas. Subsequent analysis revealed that PSP and Reg I are identical and PAP belongs to the same protein family. PSP/Reg I and PAP share a selective and specific trypsin cleavage site and result in insoluble fibrils (PTP, PATP). Search for a functional role of PSP had led to the idea that it might serve as an inhibitor in pancreatic stone formation and PSP was renamed lithostathine. Inhibitory effects of lithostathine in stone formation have been questioned. Evidence so far obtained can support a lithogenic role rather than a lithostatic role of PSP. PAP and its isoforms have been investigated mainly regarding responses to inflammation and stress. Reg I and its isoforms have been examined on regeneration, growth and mitogenesis in gastrointestinal neoplastic diseases as well as diabetes. Evidence obtained can be applied in the prediction of prognosis and therapy for inflammatory and neoplastic diseases.

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Section: Islet Regeneration and Diabetesmentioning

confidence: 99%

Pancreatic Stone Protein/Regenerating Protein Family in Pancreatic and Gastrointestinal Diseases

Jin

Hayakawa

et al. 2011

Intern. Med.

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“…Recently, Mahurkar et al [12] have proposed a two hit model for the pathogenesis of diabetes in TCP. The first hit includes the mutation of one or more genes, resulting in the formation of supertrypsin in the acinar cell of the pancreas.…”

Section: Changing Disease Spectrummentioning

confidence: 99%

Fibrocalculous pancreatic diabetes—current scenario in developing countries

Praveen

Mohan

2018

Int J Diabetes Dev Ctries

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“…Studies from various parts of the Indian subcontinent have shown mutations in this gene to be associated with not only FCPD, but also other pancreatic diseases as well [26][27][28][29]. The presence of a mutation is in itself not sufficient to cause disease, requiring a ''second hit'' in the form of an environmental insult to set the pathophysiological mechanism in motion [30].…”

Section: The Cassava Hypothesismentioning

confidence: 99%

Fibrocalculous pancreatic diabetes (FCPD)

Unnikrishnan

Mohan

2014

Acta Diabetol

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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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Genetic mechanisms underlying the pathogenesis of tropical calcific pancreatitis

Cited by 35 publications

References 54 publications

Pancreatic Stone Protein/Regenerating Protein Family in Pancreatic and Gastrointestinal Diseases

Pancreatic Stone Protein/Regenerating Protein Family in Pancreatic and Gastrointestinal Diseases

Fibrocalculous pancreatic diabetes—current scenario in developing countries

Fibrocalculous pancreatic diabetes (FCPD)

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