Global InformationDorsal pancreatic agenesis information
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| Embryonic development of pancreas. Showing the dorsal rotation and fusion of ventral bud with dorsal bud during the sixth and seventh week of gestation. |
Dorsal pancreatic agenesis is a congenital anomaly characterised by the absence of the duct of Santorini, tail and body of the pancreas.[1] It is regarded as asymptomatic and the most common clinical manifestation is non-specific abdominal pain.[2] While the cause is unclear, its mechanism is thought to be impaired dorsal buds or mutation of genes that regulate organogenesis during embryogenesis.[1]
Early diagnostic methods are laparotomy and autopsy.[1] Endoscopic retrograde cholangiopancreatography (ERCP) and computed tomography scan (CT scan) are combined for diagnosis of this agenesis in recent years.[1][3] No specific medications are needed for relieving symptoms, but pancreatic enzymes capsule and insulin are prescribed.[4]
Scholars have studied several transcription factors and proteins that can manipulate pancreatic growth and related to dorsal pancreatic agenesis.[5][6] This malformation is associated with other pancreatic disorders including pancreatitis and pancreatic tumors.[2][7] Also, patients with this dysgenesis are usually accompanied with pancreatic exocrine dysfunction such as diabetes mellitus.
The prevalence and geographical pattern are unknown.[2] First reported in 1911 by Heiberg in an autopsy.[2]
- ^ a b c d Sakpal, Sujit Vijay; Sexcius, Lucretia; Babel, Nitin; Chamberlain, Ronald Scott (May 2009). "Agenesis of the Dorsal Pancreas and Its Association With Pancreatic Tumors". Pancreas. 38 (4): 367–373. doi:10.1097/MPA.0b013e318196c401. ISSN 0885-3177. PMID 19390403.
- ^ a b c d A.-Cienfuegos, Javier; Rotellar, Fernando; Salguero, Joseba; Benito, Alberto; Solórzano, José Luis; Sangro, Bruno (2016). "Agenesis of the dorsal pancreas: systematic review of a clinical challenge". Revista Española de Enfermedades Digestivas. 108 (8): 479–484. doi:10.17235/reed.2016.4474/2016. ISSN 1130-0108. PMID 27468966.
- ^ Zhong, Jin Wei; Yu, Ling Min; Ying, Qing Ji; Peng, Wen Wen; Xu, Chang Zhao; Cai, Zhen Zhai; Xue, Zhan Xiong; Zhou, Yu Hui (May 2020). "Performance of endoscopic ultrasound for diagnosis of agenesis of the dorsal pancreas: a case report". Journal of International Medical Research. 48 (5): 030006051988414. doi:10.1177/0300060519884148. ISSN 0300-0605. PMC 7265568. PMID 31680602.
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:8was invoked but never defined (see the help page). - ^ Schnedl, Wolfgang J.; Piswanger-Soelkner, Claudia; Wallner, Sandra J.; Reittner, Pia; Krause, Robert; Lipp, Rainer W.; Hohmeier, Hans E. (March 2009). "Agenesis of the Dorsal Pancreas and Associated Diseases". Digestive Diseases and Sciences. 54 (3): 481–487. doi:10.1007/s10620-008-0370-3. ISSN 0163-2116. PMID 18618254.