Agenesis of dorsal pancreas in a young adult: a rare cause of diabetes mellitus

Sonkar, Satyendra Kumar; Kumar, Satish; Singh, Neeraj

doi:10.1136/bcr-2017-223301

Cited by 6 publications

(5 citation statements)

References 5 publications

(6 reference statements)

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“…However, these patients may also have a single-lobed spleen or even a normal spleen. Affected patients have a low prevalence of congenital heart diseases and less severe defects than patients with HS and asplenia, but when cardiac lesions are associated with this syndrome, the 1-year mortality rate can rise up to 50% (15)(16)(17).…”

Section: Discussionmentioning

confidence: 99%

Heterotaxy syndrome with agenesis of dorsal pancreas and diabetes mellitus: case report and review of the literature

Riguetto

Pelichek

Moura

2019

Archives of Endocrinology and Metabolism

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Heterotaxy syndrome (HS) is a rare congenital condition with multifactorial heritance, characterized by an abnormal arrangement of thoraco-abdominal organs and vessels. Patients present with multiple cardiac, gastrointestinal, hepatosplenic, pancreatic, renal, neurological and skeletal disorders without any pathognomonic alteration. Despite the described increased risk of diabetes mellitus (DM) in patients with altered pancreatic anatomy, just one case was reported in Korea regarding the association of HS and DM in a 13-year-old girl. Our report refers to a 40-year-old female Brazilian patient with a history of DM and HS with polysplenia and agenesis of dorsal pancreas without cardiac abnormalities. She presented a worsening glycemic control associated with weight gain and signs of insulin resistance. After a proper clinical management of insulin and oral medications, our patient developed an improvement in glycemic control. Although it is a rare disease, HS with polysplenia and pancreatic disorders can be associated with an increased risk of DM. This case highlights the importance of investigating DM in patients with HS, especially those with pancreatic anatomical disorders, for proper clinical management of this rare condition.

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Section: Discussionmentioning

confidence: 99%

Heterotaxy syndrome with agenesis of dorsal pancreas and diabetes mellitus: case report and review of the literature

Riguetto

Pelichek

Moura

2019

Archives of Endocrinology and Metabolism

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“…Associations between embryogenesis of the pancreas and genes such as HNF1B [3] and GATA6 [4], and signalling pathways such as retinaldehyde dehydrogenase 2 (Raldh2) [5] have been reported in the literature. Yet this condition remains rare with less than one hundred and twenty cases reported in the literature [6][7][8][9].…”

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

“…Approximately half of patients with ADP will develop diabetes mellitus, and up to half of them will require insulin therapy [7][8][9]. The age of onset of diabetes mellitus associated with ADP ranges between 28 to 39 years [7][8][9]. Although beta cell dysfunction and insulin deficiency have been implicated in the pathophysiology of diabetes mellitus among patients with ADP, there have only been three studies that reported a correlation between this congenital malformation and diabetic ketoacidosis [7,9].…”

Section: Discussionmentioning

confidence: 99%

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Agenesis of the Dorsal Pancreas

Manoharan¹,

Mv²,

E³

2021

JCR

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Background: Agenesis of the dorsal pancreas (ADP) is a rare entity, which is often diagnosed incidentally on abdominal imaging. It can present with abdominal pain or with symptoms of exocrine and endocrine deficiency. Approximately half of patients with ADP will have a diagnosis of diabetes mellitus, and up to half of them will require insulin therapy. Case Report: Abdominal CT in a 39-year-old man with antibody negative type 1 diabetes and chronic abdominal pain revealed dorsal pancreatic agenesis without any focal pancreatic lesions or evidence of pancreatitis. An MRCP showed normal hepatobiliary anatomy. He was replete for fat soluble vitamins. Investigations at the time of diabetes diagnosis demonstrated relative insulin deficiency. Conclusion: The case highlights that ADP is a rare cause of abdominal pain, which is often diagnosed incidentally on abdominal imaging.

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“…Dorsal pancreatic agenesis is a rare congenital disorder characterized by abnormal embryogenesis and is compatible with life whereas complete agenesis of the pancreas or ventral agenesis is incompatible with life [ 1 ]. Dorsal pancreatic agenesis can be asymptomatic or cause epigastric discomfort, hyperglycemia, and acute or chronic pancreatitis [ 2 ]. The first case of dorsal pancreatic agenesis was noticed during an autopsy in 1911 [ 3 ].…”

Section: Introductionmentioning

confidence: 99%

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

Jha¹,

Prasad²,

Kumar³

et al. 2023

Cureus

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Background Agenesis of the dorsal pancreas is a rare entity and is caused due to the defective embryological development of the pancreas. The clinical manifestations may range from diabetes mellitus, pancreatitis, and abdominal pain to no symptoms at all. We here present a case series of 10 cases with complete or partial agenesis of the dorsal pancreas. Objectives To correlate the clinical symptoms in the patients with the dorsal agenesis of the pancreas, to study any biochemical abnormality present with the dorsal agenesis of the pancreas, and to look for other coexistent finding in the patients. Results We observed that out of 10 patients, six were males and four were females. Four had symptoms related to the pancreas and six were discovered incidentally. Diabetes mellitus was present in five patients, seven patients had pain in the abdomen, and jaundice was seen in three patients. Out of 10 patients, four had complete agenesis and six had partial agenesis of the dorsal pancreas. Conclusions We conclude that the diagnosis of this rare entity and establishing its association with clinical conditions like diabetes mellitus and non-specific abdomen pain with the aid of cross-sectional imaging helps in the better evaluation and management of the patients.

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Agenesis of dorsal pancreas in a young adult: a rare cause of diabetes mellitus

Cited by 6 publications

References 5 publications

Heterotaxy syndrome with agenesis of dorsal pancreas and diabetes mellitus: case report and review of the literature

Heterotaxy syndrome with agenesis of dorsal pancreas and diabetes mellitus: case report and review of the literature

Agenesis of the Dorsal Pancreas

The Role of Cross-Sectional Imaging in the Diagnosis of Agenesis of the Dorsal Pancreas: A Case Series

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