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Pancreatic developmental defect evaluated by celiac artery angiography in a patient with MODY5.

ABSTRACT: The hepatocyte nuclear factor 1? gene (HNF1B) is responsible for maturity-onset diabetes of the young type 5 (MODY5), which is characterized by early-onset diabetes mellitus and urogenital malformations. HNF1B is expressed during visceral endoderm formation. We identified a disruption of the great pancreatic artery in a patient with MODY5 with no pancreatic body or tail. Our finding supports the significance of HNF1B in the development of the pancreas.

SUBMITTER: Iwasaki N

PROVIDER: S-EPMC4944010 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature

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Pancreatic developmental defect evaluated by celiac artery angiography in a patient with MODY5.

Iwasaki Naoko N Tsurumi Masashi M Asai Kuniya K Shimuzu Wataru W Watanabe Atsushi A Ogata Makiko M Takizawa Miho M Ide Risa R Yamamoto Toshiyuki T Saito Kayoko K

Human genome variation 20160714

The hepatocyte nuclear factor 1β gene (HNF1B) is responsible for maturity-onset diabetes of the young type 5 (MODY5), which is characterized by early-onset diabetes mellitus and urogenital malformations. HNF1B is expressed during visceral endoderm formation. We identified a disruption of the great pancreatic artery in a patient with MODY5 with no pancreatic body or tail. Our finding supports the significance of HNF1B in the development of the pancreas. ...[more]

PMID: 27468355

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Project description:Abstract Celiac artery (CA) occlusion or stenosis is identified in up to almost half of all patients undergoing abdominal angiography, and the resulting increased collateral blood flow from the superior mesenteric artery to the pancreaticoduodenal artery (PDA) may cause PDA aneurysms (PDAAs). PDAAs are rare but could be fatal if they rupture. However, treatment of the PDAA could block this important collateral blood flow pathway, leading to ischemic organ damage. Treatment of such aneurysms is therefore difficult, especially in patients with multiple PDAAs. Successful treatment of PDAAs requires establishing blood flow in the CA region and selecting which aneurysm(s) to treat. We present four patients who underwent surgery for unruptured PDAAs caused by CA obstruction. Blood flow in the CA region was established by bypassing the splenic artery and by anastomosing it either directly to the left renal artery (n = 1) or to the abdominal aorta using a graft (saphenous vein: n = 1; artificial vessel: n = 2). Three patients had multiple PDAAs: all PDAAs were treated in one patient with PDAAs of similar size and shape, but only the largest PDAA with the highest risk of rupture was treated in the other two patients to simplify the procedure. The median observation period was 19.5 months (range: 11‐28 months), and all patients were alive without recurrence at the time of writing. Surgical treatment including splenic artery bypass may thus be a viable option for treating patients with unruptured PDAAs. Since celiac artery occlusion represents a common attributable aetiology for these aneurysms, restoration of blood flow to the regions supplied by the celiac artery should also be taken into account when designing a treatment plan. Here, we present 4 cases of PDA aneurysms coexisting with coeliac artery occlusion, all of which were managed with bypass surgery using the splenic artery. Furthermore, in this study, we showed that preserving either arcade of the PDA without treating a small aneurysm may provide a secondary pathway and avoid complications in case of bypass problems.

Dataset Information

Pancreatic developmental defect evaluated by celiac artery angiography in a patient with MODY5.

Publications

Pancreatic developmental defect evaluated by celiac artery angiography in a patient with MODY5.

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