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• W2100738603 abstract "We read with interest the article of Haldorsen et al. on the lack of the pancreatic body and tail in human HNF1B mutation carriers [1]. Absence of the pancreatic body and tail, namely agenesis of the dorsal pancreas, is a rare congenital pancreatic malformation. During the last 100 years in the medical literature we found 53 patients with clinical descriptions of agenesis of the dorsal pancreas and we have recently summarized systematically all reports of agenesis of the dorsal pancreas, including associated medical problems. Renal duplication, left kidney absent, kidney stones and retro-aortic left renal vein were described, but none of the patients had renal insufficiency [2]. In 1911 the first description of agenesis of the dorsal pancreas was published as an autopsy finding. New imaging technologies have been developed and improved, and the number of patients reported to show agenesis of the dorsal pancreas has increased rapidly over recent years. So far, the findings in autopsy and radiological descriptions of the anatomical pancreatic structures are highly variable, and in most cases a diagnosis is made without description of the pancreatic duct system [2]. We support the valuable clinical descriptions of Haldorsen et al. [1] and confirm that the diagnosis of agenesis of the dorsal pancreas is inconclusive without demonstration of the absence of the dorsal pancreatic duct, either with endoscopic retrograde or magnetic resonance pancreatography. Diabetes mellitus comprises a group of metabolic diseases characterized by hyperglycaemia resulting from defects in insulin secretion and/or insulin action. We found that > 50% of reported patients with this agenesis of the dorsal pancreas were hyperglycaemic. Thorough assessment of hyperglycaemia and diabetes mellitus in all patients with agenesis of the dorsal pancreas, including description of fasting blood glucose, oral glucose tolerance test, glycated haemoglobin and medical treatment, would be helpful. In one family with agenesis of the dorsal pancreas, a marked defect in hepatic glycogen metabolism was demonstrated, even in non-diabetic offspring. An impaired index of first-phase insulin secretion in the diabetic and in both non-diabetic family members was described. Mutations of HNF1B [2] and nephropathy were not found in this family [3] and the search for genes responsible is still ongoing. It is known that the majority of islets are located in the pancreatic tail, and β-cells of the dorsal pancreas respond better to glucose stimulation [4,5], and so it is said that agenesis of the dorsal pancreas may cause diabetes mellitus. The root cause of the most common forms, diabetes mellitus, Type 1 and Type 2 diabetes, is a decrease in β-cell mass. It seems that decreased β-cell mass and limited in vivo replication capacity of β-cells after surgical resection [6], as in agenesis of the dorsal pancreas, lead to diabetes mellitus in a high number of affected patients [2]. We suggest that more family studies, including imaging techniques to demonstrate the pancreatic duct system, are needed. Full investigation of hyperglycaemia in patients with agenesis of the dorsal pancreas, exploration of all possible accompanying medical problems and genetic testing for known gene mutations would be a future goal." @default.
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• W2100738603 date "2009-01-01" @default.
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• W2100738603 title "Agenesis of the dorsal pancreas" @default.
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• W2100738603 doi "https://doi.org/10.1111/j.1464-5491.2008.02615.x" @default.
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