• Asian Pacific Journal of Cancer Prevention
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• v.16 no.1
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• pp.373-376
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• 2015

Background: EVA1A (eva-1 homolog A) is a novel gene that regulates programmed cell death through autophagy and apoptosis. Our objective was to investigate the expression profiles and potential role of EVA1A in normal and neoplastic human pancreatic tissues. Materials and Methods: The expression pattern of EVA1A in normal pancreatic tissue was examined by indirect immunofluorescence and confocal microscopy. Protein levels in paraffin-embedded specimens from normal and diseased pancreatic and matched non-tumor tissues were evaluated by immunohistochemistry. Results: EVA1A colocalized with glucagon but not with insulin, demonstrating production in islet alpha cells. Itwas strongly expressed in chronic pancreatitis, moderately or weakly expressed in the plasma membrane and cytoplasm in pancreatic acinar cell carcinoma, and absent in normal pancreatic acinar cells. Although the tissue architecture was deformed, EVA1A was absent in the alpha cells of pancreatic ductal adenocarcinomas, intraductal papillary mucinous neoplasms, mucinous cystadenomas, solid papillary tumors and pancreatic neuroendocrine tumors. Conclusions: EVA1A protein is specifically expressed in islet alpha cells, suggesting it may play an important role in regulating alpha-cell function. The ectopic expression of EVA1A in pancreatic neoplasms may contribute to their pathogenesis and warrants further investigation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.13-17
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• 2018

Methylmalonic acidemia is an autosomal recessive disorder caused by complete (mut0) or partial (mut-) deficiency of methylmalonyl-CoA mutase (MUT) or by defects in the synthesis of adenosylcobalamin (cblA, cblB, cblD variant 2). Long term complications of methylmalonic acidemia include tubulointerstitial nephritis with progressive renal failure, intellectual impairment, pancreatitis, and growth failure. We report a case of methylmalonic acidemia in a girl who diagnosed at 6 days after birth. She has developed recurrent metabolic crises with hyperammonemia and metabolic acidosis. In addition, she suffered from the chronic complications including tubulointerstitial nephritis, electrolyte imbalance associated with renal dysfunction, growth failure and fracture of femur shaft. At the age of 10 years, hypercalcemia and severe osteoporosis were noted, and pamidronate therapy was given for two years, which relieved hypercalcemia and osteoporosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.4
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• pp.332-339
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• 2022

Purpose: Although endoscopic retrograde cholangiopancreatography (ERCP) has been used for more than five decades, its applicability in Bangladeshi children has recently become more common. Therefore, this manuscript aims to describe our experience in performing ERCPs in Bangladeshi children with hepatopancreaticobiliary diseases, focusing on presenting diseases, as well as the diagnostic and therapeutic efficacy. Methods: Between 2018 and 2021, 20 children underwent 30 ERCP procedures at the Bangladesh Specialized Hospital, Dhaka. A single trained adult gastroenterologist performed all procedures using a therapeutic video duodenoscope. The indications for ERCP, diagnostic findings, therapeutic procedures, and complications were documented. Results: The median age of the study patients was 10 years (range, 1.7-15 years). Successful cannulation of the papilla was achieved in 28 procedures and failed in 2 cases. Repeated ERCP was required in seven patients. Nine patients had biliary indications and 11 had pancreatic indications. Choledocholithiasis was the most common indication for ERCP in patients with biliary disease, while chronic pancreatitis was common among patients with pancreatic indications. Pancreatic divisum was observed in only one patient. Pancreatic and biliary sphincterotomy was performed in 14 and 9 cases, respectively. A single pigtail or straight therapeutic stent was inserted in seven cases and removed in five cases. Stone extraction was performed in six procedures, and balloon dilatation was performed in five procedures. The post-procedural period for these patients was uneventful. Conclusion: We found that ERCP is a practical and successful therapeutic intervention for treating hepatopancreaticobiliary disorders in children when performed by experienced endoscopists.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.28 no.1
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• pp.53-58
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• 2024

Backgrounds/Aims: Double duct sign (DDS) (dilated common bile and pancreatic duct) is synonymous with pancreatic head/peri-ampullary tumor (PHPAT). There is limited evidence on whether incidental DDS (I-DDS) is associated with an increased risk of malignancy. This study aimed to evaluate 5-year outcomes of I-DDS. Methods: Patients were categorized according to their risk of malignancy. 'Low-risk' patients, including those with I-DDS between 2010 and 2015, were analyzed in this study. The primary outcome was incidence of PHPAT within five years of identification of DDS. Histology results from endoscopic ultrasound-guided biopsy were considered diagnostic. Secondary outcomes were incidence of benign causes, extent of follow-up investigations, and clinical indicators of malignancy in patients with DDS. Results: Among 103 patients with DDS, 20 had I-DDS. Subsequent follow-up of these 20 patients found no patient with PHPAT, two (10%) patients with chronic pancreatitis, and 18 (90%) patients with no cause found. The median follow-up duration for 'low-risk' patients was 7.3 years (range, 6-11 years). The mean number of follow-up investigations per patient was two (range, 0-9). Investigations included computed tomography (n = 27), magnetic resonance cholangiopancreatography (n = 23), endoscopy (n = 16), and ultrasound (n = 14). Patients with jaundice were more likely to have malignancy (p < 0.01). Those with abdominal pain were more likely to have a benign cause (p < 0.01). Hyperbilirubinemia and/or deranged liver enzymes and raised CA19-9 were more likely to be associated with PHPAT (p < 0.01). Conclusions: Patients with I-DDS have a low risk of developing PHPAT within five years.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.2
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• pp.141-147
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• 2011

Purpose: Ultrasonography (US) is widely used as a screening test in patients with abdominal pain (AP). We investigated the usefulness of US by a pediatrician in children with AP. Methods: We retrospectively analysed the medical records of children with AP who undertook US from December, 2008 to July, 2010. Results: A total of 628 patients (325 male, 303 female) were enrolled in this study. The mean age of patients was $8.08{\pm}4.61$ years. Duration of AP was acute in 427 and chronic in 201 patients. Localization of AP was diffuse (36.9%), periumbilical (24.4%), epigastric (21.0%), and right lower quadrant (8.1%). On the examination, there were no abnormal findings in 327 patients (52.1%). Abnormal ultrasonographic findings were mesenteric lymphadenitis (27.1%), intestinal mural thickening (10.0%), intussusception (3.0%), appendicitis (2.6%), choledochal cyst (1.6%), and pancreatitis (0.3%). We performed additional imaging studies such as computed tomography (CT) or magnetic resonance imaging (MRI) in 39 patients who showed obscure findings on the US. In 33 patients (84.6%), the same results were obtained from CT or MRI. Two cases of appendicitis, one case of pancreatitis and one case of Henoch-Sh$\ddot{o}$nlein purpura were diagnosed by the CT examination. However, there were two cases of appendicitis diagnosed by US thathad no evidence of appendicitis on the CT. Diagnostic accuracy of initial US in children with abdominal pain was 99.4%. Conclusion: US by a pediatrician as a screening test in children with AP provides a rapid and accurate diagnostic indication and has non-invasive and radiation-free advantages.

• The Korean Journal of Pharmacology
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• v.14 no.1_2
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• pp.47-54
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• 1978

Recently, a major resection of the pancreas has been carried out not only to treat carcinoma of pancreas but also chronic pancreatitis. But limited and often contradictory reports have been made on the exocrine effects after partial surgical pancreatectomy in mammals. It was suggested that the growth of the residual tissue in pancreatectomized rat is very active, because pancreas has the great power of regeneration after partial pancreatectomy, while others observed that rat pancreas after partial surgical resection revealed a perplexing mixture of atrophy and regeneration of acinar tissue. On the other hand, another results showed that the amount of insulin required to control diabetes after partial resection of pancreas is much greater than that needed after total pancreatectomy. Because the anti-insulin system, such as glucagon secretion and hypophyseoadrenal function, is probably depressed after total pancreatectomy. Furthermore, minimal resection line which will not influence the normal function of pancreas is not agreeable, such 75%, 80% or 95% resection of the total pancreas in rat. So far, studies on the exocrine function other than endocrine function after partial pancreatectomy have been limited. Therefore, the main purpose of this study is to examine the changes of exocrine as well as endocrine function of pancreas at the different time interval after 60% or 80% pancreatectomy in rats. The results summerized as follow: 1) In both 60% and 80% resected groups, a slight decrease of the total body weight was observed at a day after partial pancreatectomy in rats, but the body weight was continued to increase for following 100 days. 2) The weight of residual pancreas was continuously increased during experiment in both 60% and 80% resected groups. But the content of tissue protein in residual pancreas was significantly decreased comparing with those of resected pancreas. 3) The flow rate of pancreatico-biliary juice was significantly decreased immediately after pancreatectomy in both resected groups. But it was recovered to control level after a day in 60% resected group, after 30 days in 80% resected group. 4) The output of amylase and lipase in resected groups were significantly decreased right after pancreatectomy comparing with control group. In the 60% resected group, the output of amylase was recovered during the following 100 days after pancreatectomy, while lipase output in 3 days. However, in the 80% resected group, the output of amylase and lipase were not recovered during 100 days after pancreatectomy. 5) In order to examine the endocrine function, blood sugar level were examined at all experimental periods after partial pancreatectomy. There was no difference between control and 60% resected group in the sugar level. But in the 80% resected group the level was significantly incresed immediately after pancreatectomy, and reached the highest level at 3 days. Then it was decreased to control level during the next 10 days after pancreatectomy. The above results showed that in 60% resected group little changes were observed on pancreatic function, but severe functional impairments were observed in 80% resected group. This results suggested that the endocrine function was recovered within a short period, although the exocrine function was not recovered for a long time after 80% pancreatectomy in rats.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.174-180
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• 2002

Purpose: Ursodeoxycholic acid (UDCA) is known to decrease hepatic injury by promoting the biliary secretion of retained toxic endogenous bile acids in hepatobiliary diseases complicated by total parenteral nutrition (TPN). However, most studies have focused on treatment for complications after TPN. We investigated the preventive role of early administration of UDCA in TPN-induced hepatobiliary complications by a randomized, double-blind, placebo-controlled trial. Methods: Between May 2000 and May 2002, thirteen patients, who were given TPN more than 10 days in the hospital, were assigned randomly to two groups. One was the case group (7 patients) who were given UDCA simultaneously with TPN regimen, and the other, the control group (6 patients) who were given placebo. Their age ranged from 1 day to 13 years. They were affected with diseases impossible for enteral nutrition, such as prematurity, cerebral palsy, chronic diarrhea, anorexia nervosa, pancreatitis, and cyclic vomiting. The duration of TPN ranged from 10 to 70 days. Hematologic parameters including liver function test were measured at regular intervals, and the duration, composition, administration rate, total calorie of TPN were recorded. The serum levels of total bilirubin, aspartate aminotransferase (AST), alanine aminotransferase (ALT), and alkaline phosphatase were compared between groups after cessation of the study. Results: The autoregressive coefficient of the control group was 0.4419 (p=0.0651) in bilirubin, -0.0431 (p=0.7923) in AST, 0.2398 (p=0.2416) in ALT, and 0.2459 (p=0.1922) in alkaline phosphatase by mixed procedure model when the parameters were referred to the case group. Conclusion: The serum level of total bilirubin did not increase in comparison with that of the control group, but statistically insignificant, when both TPN and UDCA were administered simultaneously from the beginning.