Project description:Pancreatic divisum (PD) is caused by the lack of fusion of the pancreatic duct during the embryonic period. Considering the incidence rate of PD, clinicians lack an understanding of the disease, which is usually asymptomatic. Some patients with PD may experience recurrent pancreatitis and progress to chronic pancreatitis. Recently, a 13-year-old boy presented with pancreatic pseudocyst, recurrent pancreatitis, and incomplete PD, and we report this patient's clinical data regarding the diagnosis, medical imagining, and treatment. The patient had a history of recurrent pancreatitis and abdominal pain. Magnetic resonance cholangiopancreatography was chosen for diagnosis of PD, pancreatitis, and pancreatic pseudocyst, followed by endoscopic retrograde cholangiopancreatography, minor papillotomy, pancreatic pseudocyst drainage, and stent implantation. In the follow-up, the pseudocyst lesions were completely resolved, and no recurrent pancreatitis has been observed.
Project description:Pancreas divisum (PD) is the most common developmental anatomic variant of pancreatic duct. Endoscopic ultrasound (EUS) is often performed to evaluate idiopathic pancreatitis and has been shown to have high accuracy in diagnosis of PD. The different techniques to identify PD by linear EUS have been described differently by different authors. If EUS is done with a proper technique it can be a valuable tool in the diagnosis of PD. The anatomical and technical background of different signs has not been described so far. This article summarizes the different techniques of imaging of pancreatic duct in a suspected case of PD and gives a technical explanation of various signs. The common signs seen during evaluation of pancreatic duct in PD are stack sign of linear EUS, crossed duct sign on linear EUS, the dominant duct and ventral dorsal duct (VD) transition. Few other signs are described which include duct above duct, short ventral duct /absent ventral duct, separate opening of ducts with no communication, separate opening of ducts with filamentous communication, stacking of duct of Santorini and indirect signs like santorinecele. The principles of the sign have been explained on an anatomical basis and the techniques and the principles described in the review will be helpful in technical evaluation of PD during EUS.
Project description:BACKGROUND: We performed duct-to-mucosa pancreaticojejunostomy with resection of jejunal serosa in 55 patients, and here compare the clinical results between duct-to-mucosa pancreaticojejunostomies with a non-dilated pancreatic duct and those with a dilated duct. PATIENTS AND METHODS: In the period 1999 to 2005, 55 patients (27 F, 28 M; mean age 63.4 years) underwent duct-to-mucosa pancreaticojejunostomy with resection of jejunal serosa. A non-dilated pancreatic duct was observe in 29 patients in group A and a dilated pancreatic duct in 26 patients in group B. Clinical characteristics (age, gender, benign or malignant condition, presence of diabetes mellitus, anastomotic time) were analyzed in both groups and postoperative complications were compared between groups. RESULTS: In a comparison of clinical characteristics, all factors were similar between groups. In group A, the postoperative complication occurred in 4 (wound infection in 2, pulmonary embolism in 1, gastric ulcer in 1) of 29 patients (13.8%), and in group B in 1 (pneumothorax) of 26 patients (3.8%). No pancreatic leakage was observed in either group. The difference between group A and group B in the rate of postoperative complication was not statistically significant. CONCLUSIONS: There was no statistical difference in the rate of postoperative complications, including pancreatic leakage, between duct-to-mucosa pancreaticojejunostomies with a dilated pancreatic duct and those with a non-dilated duct. We consider that the diameter of the pancreatic duct is irrelevant to results of duct-to-mucosa pancreaticojejunostomy.
Project description:Development of systems that reconstitute hallmark features of human pancreatic intraepithelial neoplasia (PanINs), the precursor to pancreatic ductal adenocarcinoma, could generate new strategies for early diagnosis and intervention. However, human cell-based PanIN models with defined mutations are unavailable. Here, we report that genetic modification of primary human pancreatic cells leads to development of lesions resembling native human PanINs. Primary human pancreas duct cells harbouring oncogenic KRAS and induced mutations in CDKN2A, SMAD4 and TP53 expand in vitro as epithelial spheres. After pancreatic transplantation, mutant clones form lesions histologically similar to native PanINs, including prominent stromal responses. Gene expression profiling reveals molecular similarities of mutant clones with native PanINs, and identifies potential PanIN biomarker candidates including Neuromedin U, a circulating peptide hormone. Prospective reconstitution of human PanIN development from primary cells provides experimental opportunities to investigate pancreas cancer development, progression and early-stage detection.
Project description:Introduction:In this study, we describe for the first time a Neurofibromatosis type 1 patient with pancreas divisum, multiple periampullary tumors and germline pathogenic variants in NF1 and CFTR genes. Case report:A 62-year-old female NF1 patient presented with weakness, choluria, nausea, and diffuse abdominal pain to an emergency room service. Magnetic resonance imaging revealed an abdominal mass involving the periampullary region and pancreas divisum. After surgical resection, three synchronous neoplasms were detected including two ampullary tumors (adenocarcinoma of the major ampulla and a neuroendocrine tumor of the minor ampulla) and a gastrointestinal stromal tumor (GIST). Germline multigene panel testing (MGPT) identified two pathogenic heterozygous germline variants: NF1 c.838del and CFTR c.1210-34TG[12]T[5]. Conclusion:This is the first report of a Neurofibromatosis type 1 patient with pancreas divisum and multiple periampullary tumors harboring pathogenic germline variants in NF1 and CFTR genes. The identification of two germline variants and a developmental anomaly in this patient may explain the unusual and more severe findings and underscores the importance of comprehensive molecular analyses in patients with complex phenotypes.
Project description:BackgroundThe reported success rates for treatments of kidney stones with either extracorporeal shock wave lithotripsy (ESWL) or flexible ureterorenoscopy (URS) are conflicting. We aimed to compare the efficacy and safety of ESWL and URS for previously untreated renal calculi.MethodsAll patients treated with ESWL or URS at our tertiary care centre between 2003 and 2015 were retrospectively identified. Patients with previously untreated kidney stones and a stone diameter of 5-20?mm were included. Stone-free, freedom from reintervention and complication rates were recorded. Independent predictors of stone-free and freedom from reintervention rates were identified by multivariable logistic regression and a propensity score-matched analysis was performed.ResultsA total of 1282 patients met the inclusion criteria, of whom 999 (78%) underwent ESWL and 283 (22%) had URS. During post-operative follow-up, only treatment modality and stone size could independently predict stone-free and freedom from reintervention rates. After propensity score matching, ESWL showed significantly lower stone-free rates [ESWL (71%) versus URS (84%)] and fewer patients with freedom from reintervention [ESWL (55%) versus URS (79%)] than URS. Complications were scarce for both treatments and included Clavien Grade 3a in 0.8% versus 0% and Grade 3b in 0.5% versus 0.4% of ESWL and URS treated patients, respectively.ConclusionsTreatment success was mainly dependent on stone size and treatment modality. URS might be the better treatment option for previously untreated kidney stones 5-20?mm, with similar morbidity but higher stone-free rates and fewer reinterventions than ESWL.