Agenesis of dorsal pancreas in a patient with weight loss and diabetes mellitus

Klein, W; Dabezies, Marta A.; Friedman, Arnold C.; Caroline, Dina F.; Boden, Guenther; Cohen, Sidney

doi:10.1007/bf02087781

Cited by 16 publications

(22 citation statements)

References 25 publications

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“…In a complete agenesis, the minor papilla, accessory pancreatic duct, the body and tail of pancreas are absent. However in partial agenesis, the minor papilla with a remnant of the accessory pancreatic duct and a variable amount of pancreatic body are present (7). The diagnosis can be made by ultrasound, CT scan, magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP).…”

Section: Discussionmentioning

confidence: 99%

Periampullary carcinoma in a patient with agenesis of dorsal pancreas

Kapoor

Singh

2011

Journal of Surgical Case Reports

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Section: Discussionmentioning

confidence: 99%

Periampullary carcinoma in a patient with agenesis of dorsal pancreas

Kapoor

Singh

2011

Journal of Surgical Case Reports

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“…The tetraspanin Tm4sf3 is localized to the ventral pancreas and regulates fusion of the dorsal and ventral pancreatic buds in life, such as diabetes mellitus or pancreatitis (Gilinsky et al, 1985;Guntz et al, 1976;Gurson et al, 1970;Klein et al, 1994;Lechner and Read, 1966;Shah et al, 1987;Wang et al, 1990;Wildling et al, 1993). On the other hand, there are no reports of ventral pancreas agenesis.…”

Section: Introductionmentioning

confidence: 99%

The tetraspanin Tm4sf3 is localized to the ventral pancreas and regulates fusion of the dorsal and ventral pancreatic buds

et al. 2009

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During embryogenesis, the pancreas develops from separate dorsal and ventral buds, which fuse to form the mature pancreas. Little is known about the functional differences between these two buds or the relative contribution of cells derived from each region to the pancreas after fusion. To follow the fate of dorsal or ventral bud derived cells in the pancreas after fusion, we produced chimeric Elas-GFP transgenic/wild-type embryos in which either dorsal or ventral pancreatic bud cells expressed GFP. We found that ventral pancreatic cells migrate extensively into the dorsal pancreas after fusion, whereas the converse does not occur. Moreover, we found that annular pancreatic tissue is composed exclusively of ventral pancreas-derived cells. To identify ventral pancreas-specific genes that may play a role in pancreatic bud fusion, we isolated individual dorsal and ventral pancreatic buds, prior to fusion, from NF38/39 Xenopus laevis tadpoles and compared their gene expression profiles (NF refers to the specific stage of Xenopus development). As a result of this screen, we have identified several new ventral pancreas-specific genes, all of which are expressed in the same location within the ventral pancreas at the junction where the two ventral pancreatic buds fuse. Morpholinomediated knockdown of one of these ventral-specific genes, transmembrane 4 superfamily member 3 (tm4sf3), inhibited dorsalventral pancreatic bud fusion, as well as acinar cell differentiation. Conversely, overexpression of tm4sf3 promoted development of annular pancreas. Our results are the first to define molecular and behavioral differences between the dorsal and ventral pancreas, and suggest an unexpected role for the ventral pancreas in pancreatic bud fusion.

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“…Most reports describe single, but not familiar, patients who were diagnosed because of jaundice (36), pancreatitis (11), weight loss, and diabetes mellitus (10). Partial agenesis of the dorsal pancreas like that in the older son has been reported (37), but was not familiar.…”

Section: Discussionmentioning

confidence: 96%

“…Metabolic parameters derived from the FSIGT and AUC for C-peptide and insulin after a mixed meal in patients with agenesis of the dorsal pancreas (DPA) and healthy control (CON) subjects K G [10 -20] K G [20 -40] S See text for parameter meaning. K G , Glucose disappearance (%⅐min Ϫ1 ); S I , insulin sensitivity index (10 Ϫ4 ⅐min Ϫ1 ⅐U Ϫ1 ⅐ml); S G , glucose effectiveness (min Ϫ1 ); AUC CP [0 -10] , first-phase C-peptide secretion (ng⅐ml Ϫ1 ⅐min Ϫ1 ); AUC CP [0 -180] , total C-peptide secretion (ng⅐ml Ϫ1 in 180 min); AUC Ins [0 -10] , first-phase insulin secretion (mU⅐ml Ϫ1 ⅐min Ϫ1 ); ⌬AIR G [3][4][5][6][7][8][9][10] , insulin secretion index (U/ml); Clear ins, insulin clearance (ml⅐min Ϫ1 ⅐kg Ϫ1 ); Disp index, disposition index (S I ϫ ⌬AIR G [3][4][5][6][7][8][9][10] ) (10 Ϫ4 ⅐min Ϫ1 ); Adapt index, adaptation index (S I ϫ AUC CP [0 -10] ) (10 Ϫ4 ⅐ng⅐U Ϫ1 ); AUC CP [dinner], total C-peptide secretion after dinner (ng⅐ml Ϫ1 ⅐in 13.75 h); AUC Ins [dinner], total insulin secretion after dinner (mU⅐ml Ϫ1 ⅐in 13.75 h). a P ϭ 0.05 DPA vs. CON.…”

Section: Discussionmentioning

confidence: 99%

Reduction of Hepatic Glycogen Synthesis and Breakdown in Patients with Agenesis of the Dorsal Pancreas

Stingl

Schnedl

Krššák

et al. 2002

The Journal of Clinical Endocrinology &Amp; Metabolism

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In a family with agenesis of the dorsal pancreas only the mother presents with insulin-dependent diabetes mellitus, whereas her sons are glucose tolerant. We examined whether metabolic defects can be detected early in this disease. Plasma glucose profiles were obtained from patients with dorsal pancreas agenesis and from matched healthy subjects. Hepatic glycogen synthesis and breakdown were determined from the time course of glycogen concentrations using noninvasive (13)C nuclear magnetic resonance spectroscopy. Gluconeogenesis was calculated from the difference between glucose production (measured with D-[6,6-(2)H(2)]glucose) and glycogen breakdown. Frequently sampled iv glucose tolerance tests were performed to assess insulin secretion and sensitivity. The mean plasma glucose level was higher (12.9 +/- 0.4 vs. 5.9 +/- 0.1 mmol/liter), whereas the peak plasma insulin level was lower (236 vs. 397 +/- 23 pmol/liter) in the diabetic mother than in her nondiabetic sons and healthy subjects. In all patients, however, glycogen synthesis and breakdown were reduced by approximately 55% (P < 0.05) and 40% (P < 0.02), respectively. Gluconeogenesis (6.8 +/- 0.8 vs. 4.2 +/- 0.3 micro mol/kg.min; P < 0.05) and hepatic insulin clearance (6.8 +/- 1.3 vs. 2.8 +/- 1.0 ml/kg.min) were increased in all patients. In conclusion, patients with complete agenesis of the dorsal pancreas exhibit marked defects in hepatic glycogen metabolism, which are present even in the nondiabetic offspring.

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Agenesis of dorsal pancreas in a patient with weight loss and diabetes mellitus

Cited by 16 publications

References 25 publications

Periampullary carcinoma in a patient with agenesis of dorsal pancreas

Periampullary carcinoma in a patient with agenesis of dorsal pancreas

The tetraspanin Tm4sf3 is localized to the ventral pancreas and regulates fusion of the dorsal and ventral pancreatic buds

Reduction of Hepatic Glycogen Synthesis and Breakdown in Patients with Agenesis of the Dorsal Pancreas

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