Coincidence of Polysplenia, Kartagener Syndrome, Dorsal Pancreas Agenesis, and Polycystic Kidney Disease in an Adult

Demir, Mustafa Kemal; Furuncuoglu, Yavuz

doi:10.5152/eurasianjmed.2017.17067

Cited by 8 publications

(6 citation statements)

References 14 publications

(14 reference statements)

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“…The association between tumorigenesis and ADP is unclear, but chronic pancreatitis caused by the latter is indeed one of the risk factors for cancer. In addition, ADP associated with other organ malformations has also been reported, including polycystic kidney disease, Kartagener syndrome, multiple splenic deformities, congenital choledochal cysts and biliary atresia [19,20].…”

Section: Discussionmentioning

confidence: 99%

Two cases of agenesis of the dorsal pancreas and a review of the literature

Mei

Cao

2020

BMC Gastroenterol

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Background Agenesis of the dorsal pancreas (ADP) is a very rare disease with no specific symptoms, and the pathogenesis is not clear. Some patients will be accompanied by other diseases, such as pancreatic tumor or pancreatitis. But most cases are very atypical and difficult to distinguish. Some syndromes of pancreatic exocrine insufficiency are common in patients with ADP. Here, we report two cases of ADP and summarize the clinical features, diagnosis, and treatment of ADP. Case presentation Case A is a 65-year-old Chinese woman who presented with abdominal pain accompanied by nausea, bloating and acid reflux. The enhanced abdominal CT scan found nothing meaningful except the absence of the body and tail of the pancreas. The diagnosis was considered as gastrointestinal dysfunction cause by exocrine pancreatic insufficiency and recovered after symptomatic treatment. Case B is a 61-year-old Chinese woman who presented with abdominal pain accompanied by fever, vomiting and bloating. The abdominal CT showed multiple stones in the gallbladder, and the body and tail of the patient’s pancreas were absent. She was diagnosed with cholelithiasis and recovered after laparoscopic cholecystectomy. Conclusion Agenesis of the dorsal pancreas (ADP) is a rare congenital disease with an unclear pathogenesis that presents multiple symptoms. It should be considered when the patients have non-specific, persistent and unexplained symptoms such as bloating or uncontrolled blood sugar. Imaging examination is helpful for diagnosis. And it does not require surgical intervention unless it accompanies other diseases, EPI need to be considered when the non-specific gastrointestinal symptoms appear.

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Section: Discussionmentioning

confidence: 99%

Two cases of agenesis of the dorsal pancreas and a review of the literature

Mei

Cao

2020

BMC Gastroenterol

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“…According to a literature review by Krott et al, that is a very rare anomaly and described with various terms, such as duplex pancreas, pancreas bifidum, bifid pancreatic duct, and double pancreatic ducts. [1][2][3][4][5] In addition, although different concepts, BMPD is easily confused with bifid pancreas, as the latter refers to a separated pancreatic parenchyma of the tail. Because of poor recognition, detailed knowledge, such as frequency, is not clear.…”

Section: Discussionmentioning

confidence: 99%

“…Other clinical presentations are pancreatitis, pseudocysts, neoplasms, or a combination of these 2 . It may rarely be associated with heterotaxy syndrome, Kartagener syndrome, polycystic kidney disease, congenital choledochal cysts, and biliary atresia 4,5 …”

Section: Discussionmentioning

confidence: 99%

Agenesis of Dorsal Pancreas With Chronic Calcific Pancreatitis

Mahey

Chawla

2022

Pancreas

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“…Adults with ADPKD have been shown to have a higher prevalence of bronchiectasis, a pathognomonic finding in PCD . Furthermore, case reports describing patients presenting with both PCD and PKD have been published . Two explanatory hypotheses have been proposed.…”

Section: Overlapping Motor and Sensory Cilia Disordersmentioning

confidence: 99%

“…20,21,63 Furthermore, case reports describing patients presenting with both PCD and PKD have been published. 90,91 Two explanatory hypotheses have been proposed.…”

Section: Organ Laterality Defectsmentioning

confidence: 99%

To beat, or not to beat, that is question! The spectrum of ciliopathies

Kempeneers

Chilvers

2018

Pediatric Pulmonology

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Cilia are widely distributed throughout the human body, and have numerous roles in physiology, development, and disease. Ciliary ultrastructure is complex, consisting of nine parallel microtubules doublets, with or without motor dynein arms and a central pair of microtubules. Classification of cilia has evolved over time, and currently, four main classes are described: motile and non-motile cilia with a "9 + 2" structure, and motile and non-motile cilia with a "9 + 0" structure, which depend on the presence or absence of dynein arms and a central pair. Ciliopathies are inherited multisystem disorders of cilia, and may present with a varied spectrum of genotypes and phenotypes. Motor and sensory ciliopathies were historically considered as distinct dysfunctions of motile and non-motile cilia, but recent data indicate that the classical features of motor and sensory cilia may overlap.

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Coincidence of Polysplenia, Kartagener Syndrome, Dorsal Pancreas Agenesis, and Polycystic Kidney Disease in an Adult

Cited by 8 publications

References 14 publications

Two cases of agenesis of the dorsal pancreas and a review of the literature

Two cases of agenesis of the dorsal pancreas and a review of the literature

Agenesis of Dorsal Pancreas With Chronic Calcific Pancreatitis

To beat, or not to beat, that is question! The spectrum of ciliopathies

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