Agenesis of the Dorsal Pancreas and Associated Diseases

Schnedl, Wolfgang J.; Piswanger-Soelkner, Claudia; Wallner, Silvia; Reittner, Pia; Krause, Robert; Lipp, Rainer W.; Hohmeier, Hans E.

doi:10.1007/s10620-008-0370-3

Cited by 75 publications

(92 citation statements)

References 54 publications

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“…Moreover, the association of congenital heart defects such as septal defects, tetralogy of Fallot or pulmonary artery stenosis is common [4]. Although few cases of agenesis of the dorsal pancreas describe features similar to our patient, the individual symptoms may vary from case to case [1]. Most cases describe single, but not familiar, patients.…”

Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

“…Several mechanisms such as sphincter of Oddi dysfunction and dyskinesia accompanied by compensatory hypertrophy and hypersecretion of the remaining ventral pancreas with raised intraductal pressure have been proposed to explain the pancreatitis [2]. In addition to abdominal pain, it is reported that approximately 50% of patients with agenesis of the dorsal pancreas are hyperglycaemic [1] because the majority of islets are located in the pancreatic tail, and b-cells of the dorsal pancreas respond positively to glucose stimulation. In addition to a decrease in b-cell mass, insulin resistance is also the root cause in the most common forms of diabetes.…”

Section: Discussionmentioning

confidence: 99%

“…Pancreas divisum is the most common anomaly. However, agenesis of the dorsal pancreas, with the absence of the pancreatic body and tail is very rare; only 53 cases have been described in medical literature in the last 100 years [1].The embryology of the pancreas is complex. During gastrulation, the epiblast comprised multipotential cells that differentiate into three embryonic layers: ectoderm, endoderm and mesoderm.…”

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Agenesis of the dorsal pancreas: a rare cause of diabetes

et al. 2011

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A 19-year-old Japanese man visited our department presenting with glycosuria that had been detected during a routine school urinalysis. The patient had undergone cardiac surgery at the age of 4 years for atrial and ventricular septal defects, and pulmonary artery stenosis. He denied any history of abdominal trauma, drug or alcohol intake, or gastrointestinal problems. His maternal grandmother had mild diabetes that began in middle age; whereas, his parents had no significant medical history including diabetes.The physical examination revealed an emaciated young man with a body mass index of 19.3, but otherwise a normal examination. Laboratory studies were: fasting hyperglycaemia of 19.7 mmol/l (354 mg/dl) without ketosis. The HbA1c was 12.1%, urinary excretion of C-peptide 43.9 lg/day (normal range 50-100 lg/day). The anti-islet cell and anti-glutamic acid decarboxylase antibodies were negative. The serum total amylase level was normal, 49 U/l (normal range 40-180 U/l); whereas, the levels of serum pancreatic enzymes p-type amylase, lipase, trypsin and phospholipase A 2 were decreased to 8 U/l (normal range 17-50 U/l), 10 U/l (13-49 U/l), 50 U/l (100-550 U/l) and 100 U/l (130-400 U/l), respectively. Ultrasonography (US) of the abdomen could not identify the body of the pancreas, although the head and uncus were clearly visible. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed that most of the body and the tail of the pancreas were absent, and that the pancreatic head was slightly enlarged (Fig. 1). Magnetic resonance cholangiopancreatography (MRCP) revealed that the dorsal pancreatic duct was absent. Based on these findings, a diagnosis of agenesis of the dorsal pancreas was made. Insulin replacement therapy was initiated, and currently, the diabetes is well controlled with 4 U of insulin per day. There are no signs of any diabetes-related renal, retinal or neurological changes. His blood glucose levels have improved after beginning insulin therapy, and he is being followed up as a hospital outpatient. DiscussionDevelopmental anomalies of the pancreas are rarely seen. Pancreas divisum is the most common anomaly. However, agenesis of the dorsal pancreas, with the absence of the pancreatic body and tail is very rare; only 53 cases have been described in medical literature in the last 100 years [1].The embryology of the pancreas is complex. During gastrulation, the epiblast comprised multipotential cells that differentiate into three embryonic layers: ectoderm, endoderm and mesoderm. The gastrointestinal organs, including the pancreas, are derived from the endoderm. The pancreas develops embryologically from a dorsal and a ventral bud on opposite sides of the foregut. Morphogenetically, the ventral pancreatic bud goes on to form the posterior part of the head, and the dorsal pancreatic bud contributes mainly to the formation of the tail and body. During the growth and the rotation of the gut tube in the seventh week of gestation, the two buds fuse. As these two buds grow, each bud develops a ...

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Section: Discussionmentioning

confidence: 65%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

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Agenesis of the dorsal pancreas: a rare cause of diabetes

et al. 2011

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“…This anomaly was first described in 1911, and 53 cases were reported up to 2008 (3,4). Several authors have reported an association between DPA and acinar-ductal metaplasia (ADM), and neoplasia of the remnant pancreas (ventral pancreas) and diabetes (5)(6)(7)(8)(9).…”

Section: Introductionmentioning

confidence: 99%

Agenesis of the dorsal pancreas: systematic review of a clinical challenge

Cienfuegos¹,

Rotellar²,

Salguero³

et al. 2016

Rev Esp Enferm Dig

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Background: Agenesis of the dorsal pancreas is a rare malformation. Since 1911 and until 2008, 53 cases have been reported. Several authors have recently described the association of this anomaly with neoplasia of the ventral pancreas, thus we performed a systematic review of the literature from 2008 to 2015.Methods: A systematic review of the Medline and ISI Web of Science Databases from 2008 until 2015 was carried out, and 30 articles which met the inclusion criteria were identified that included a total of 53 patients: 7 children and 46 adults.Conclusions: Although dorsal pancreatic agenesis is a rare malformation, given its association with non-alcoholic pancreatitis and neoplasia of the residual pancreas, physicians should maintain an expectant attitude.

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Pancreas

Watanabe¹

2016

Bergman's Comprehensive Encyclopedia of Human Anatomic Variation

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Agenesis of the Dorsal Pancreas and Associated Diseases

Cited by 75 publications

References 54 publications

Agenesis of the dorsal pancreas: a rare cause of diabetes

Agenesis of the dorsal pancreas: a rare cause of diabetes

Agenesis of the dorsal pancreas: systematic review of a clinical challenge

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