Congenital pancreas malformations: a clinical case report

Bento, Ana; Baptista, Hamilton; Oliveira, Filipa

doi:10.1016/s2255-4823(13)70427-2

Cited by 6 publications

(2 citation statements)

References 22 publications

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“…A low-fat diet and diabetes control are recommended for the patients of ADP with DM. [ 36 ] If the ADP patients have pancreatitis, pancreatic enzymes could be administrated to reduce the pancreatic secretion and promote pain relief. [ 37 ] When pancreatic tumors or other malignant medical problems are suspected in ADP patients, surgical therapy like pancreaticoduodenectomy, even total pancreatectomy should be taken into consideration.…”

Section: Discussionmentioning

confidence: 99%

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Agenesis of the dorsal pancreas with chronic suppurative pancreatitis

Xia

Jiang

et al. 2021

Medicine

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Rationale: Agenesis of the dorsal pancreas (ADP) is a rare congenital anomaly of the pancreas. ADP is associated with some other medical problems such as diabetes mellitus, abdominal pain/bloating, pancreatitis, pancreatic neuroendocrine tumor and so on. In this study, we present a case of ADP with chronic suppurative pancreatitis, summarize the clinical characteristics of the reported cases in China and review the correlative literature. Patient concerns: A 51-year-old Chinese man, with a history of impaired fasting glucose, presented with jaundice, pruritus and dark urine. Laboratory analysis showed abnormal liver function and elevated carbohydrate antigen 19-9. Diagnoses: Contrast-enhanced computed tomography demonstrated a mass located at the head of pancreas and complete absence of the body and tail of pancreas. Endoscopic retrograde cholangiopancreatography demonstrated an eccentric malignant stricture about 1.6cm of distal common bile duct. Interventions: The patient underwent pancreaticoduodenectomy because of the suspicion of pancreatic tumor. The postoperative pathological result was chronic suppurative pancreatitis, with moderate hyperplasia in focal ductal epithelium. Outcomes: A long-term follow-up shows that the patient is asymptomatic with well-controlled diabetes mellitus and pancreatic exocrine insufficiency. Lessons: ADP is a quite rare congenital malformation of the pancreas with poorly-understood pathogenesis. The diagnosis of ADP depends on the imaging examination. The therapeutic strategy varies from person to person due to the different accompanying conditions.

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Section: Discussionmentioning

confidence: 99%

“…The main goal of the therapy for ADP is to relieve the associated symptoms. A low-fat diet and diabetes control are recommended for the patients of ADP with DM [36] . If the ADP patients have pancreatitis, pancreatic enzymes could be administrated to reduce the pancreatic secretion and promote pain relief [37] .…”

Section: Discussionmentioning

confidence: 99%

Agenesis of the dorsal pancreas with chronic suppurative pancreatitis

Xia

Jiang

et al. 2021

Medicine

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Embryonic exposure to Mono(2-ethylhexyl) phthalate (MEHP) disrupts pancreatic organogenesis in zebrafish (Danio rerio)

et al. 2018

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Mono(2-ethylhexyl) phthalate (MEHP) is the bioactive metabolite of di(2-ethylhexyl) phthalate, a plasticizing agent and persistent environmental contaminant associated with obesity, developmental abnormalities, and oxidative stress. Nrf2 (Nfe2l2) is a transcription factor that regulates cytoprotective genes as part of the adaptive antioxidant response. We previously identified the pancreas as a sensitive target of oxidative stress during embryonic development. The goals of this study were to 1) characterize the effects of MEHP exposure on pancreatic development, and 2) determine whether oxidative stress contributes to MEHP embryotoxicity. Zebrafish (Danio rerio) embryos from AB wildtype and Tg(ins:GFP;nrf2a) were exposed to 0 or 200 μg/L MEHP at 3 h post fertilization (hpf) through 168 hpf to assess pancreatic organogenesis. MEHP exposure significantly decreased β-cell area at all timepoints (48, 72, 96, 168 hpf), but Nrf2a did not significantly protect against islet hypomorphism. Tg(gcga:GFP) embryos exposed to MEHP showed a decrease in α-cell area in the islet across the same timepoints. Tg(ptf1a:GFP) embryos were assessed at 80 and 168 hpf for exocrine pancreas length. MEHP exposure decreased growth of the exocrine pancreas. Expression of pancreas genes insa, sst2 and ptf1a was significantly reduced by MEHP exposure compared to controls. Glutathione (GSH) concentrations and redox potentials were quantified at 72 hpf by HPLC, but no significant changes were observed. However, expression of the GSH-related genes gstp1 and gsr were significantly altered by MEHP exposure. These data indicate that the developing pancreas is a sensitive target tissue of embryonic exposure to MEHP.

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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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Congenital pancreas malformations: a clinical case report

Cited by 6 publications

References 22 publications

Agenesis of the dorsal pancreas with chronic suppurative pancreatitis

Agenesis of the dorsal pancreas with chronic suppurative pancreatitis

Embryonic exposure to Mono(2-ethylhexyl) phthalate (MEHP) disrupts pancreatic organogenesis in zebrafish (Danio rerio)

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

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