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exocrine pancreatic insufficiency

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https://www.readbyqxmd.com/read/29317564/pdx1-gene-mutation-with-permanent-neonatal-diabetes-mellitus-with-annular-pancreas-duodenal-atresia-hypoplastic-gall-bladder-and-exocrine-pancreatic-insufficiency
#1
Abhishek Kulkarni, Varun K Sharma, Fazal Nabi
BACKGROUND: Neonatal diabetes mellitus is a rare condition. CASE CHARACTERISTICS: A small for gestational age male, presented with neonatal onset diabetes mellitus, duodenal atresia, annular pancreas and gall bladder hypoplasia. OBSERVATION: Observation: A novel homozygous mutation p.K163R (c.488A>G) in the PDX1 gene was found. Parents were heterozygous for the same. MESSAGE: This case highlights the importance of establishing the genetic diagnosis in all cases of neonatal diabetes mellitus...
December 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29313579/pancreatic-exocrine-insufficiency-in-type-1-and-2-diabetes-therapeutic-implications
#2
Rupjyoti Talukdar, D Nageshwar Reddy
The objective of the present review is to focus on pancreatic exocrine insufficiency that is associated with Type 1 and 2 diabetes, its clinical and therapeutic implications, including the utility and efficacy of pancreatin supplementation. A literature search was conducted on Pubmed / Medline to identify relevant articles using terms pancreatic exocrine insufficiency in diabetes mellitus patients, pathophysiology, prevalence, treatment and management published between 2006-2016 in English language. Meta-analysis has revealed the prevalence of PEI in patients with type-1 and type-2 diabetes mellitus to be 37...
September 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/29303286/-cystic-fibrosis-in-adults
#3
Libor Fila
Cystic fibrosis (CF) is an inherited disease caused by mutations in the transmembrane conductance regulator (CFTR) gene. The disease leads to dysfunction of the exocrine glands with high concentration of chloride in the sweat and formation of abnormally viscous mucus in the respiratory, digestive and reproductive tract. Chronic sinopulmonary disease, exocrine pancreatic insufficiency, liver disease, intestinal obstruction, impaired nutritional status, salt loss syndrome and male infertility dominates in the clinical presentation...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29299757/benign-tumors-of-the-pancreas-radical-surgery-versus-parenchyma-sparing-local-resection-the-challenge-facing-surgeons
#4
EDITORIAL
Hans G Beger
Pancreaticoduodenectomy and left-sided pancreatectomy are the surgical treatment standards for tumors of the pancreas. Surgeons, who are requested to treat patients with benign tumors, using standard oncological resections, face the challenge of sacrificing pancreatic and extra-pancreatic tissue. Tumor enucleation, pancreatic middle segment resection and local, duodenum-preserving pancreatic head resections are surgical procedures increasingly used as alternative treatment modalities compared to classical pancreatic resections...
January 3, 2018: Journal of Gastrointestinal Surgery: Official Journal of the Society for Surgery of the Alimentary Tract
https://www.readbyqxmd.com/read/29285795/ataluren-driven-restoration-of-shwachman-bodian-diamond-syndrome-protein-function-in-shwachman-diamond-syndrome-bone-marrow-cells
#5
Valentino Bezzerri, Donatella Bardelli, Jacopo Morini, Antonio Vella, Simone Cesaro, Claudio Sorio, Andrea Biondi, Cesare Danesino, Piero Farruggia, Baroukh Maurice Assael, Giovanna D'Amico, Marco Cipolli
Shwachman-Diamond syndrome (SDS) is a rare inherited recessive disease mainly caused by mutations in the Shwachman-Bodian-Diamond syndrome (SBDS) gene, which encodes for the homonymous protein SBDS, whose function still remains to be fully established. SDS affects several organs causing bone marrow failure, exocrine pancreatic insufficiency, skeletal malformations, and cognitive disorders. About 15% of SDS patients develop myelodysplastic syndromes (MDS) and are at higher risk of developing acute myeloid leukemia (AML)...
December 29, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29277262/pancreatectomies-for-pancreatic-neoplasms-in-pediatric-and-adolescent-age-a-single-institution-experience
#6
Chiara Scandavini, Roberto Valente, Elena Rangelova, Ralf Segersvärd, Urban Arnelo, Johan Permert, Pär-Johan Svensson, Jakob Stenman, Marco Del Chiaro
BACKGROUND: There are very few data in the current literature regarding the short- and long-term outcome of surgery for pediatric pancreatic tumors (PPT). No data are available on the impact of pancreatic surgery on the children's growth. METHODS: This is a retrospective cohort study on a consecutive series of pediatric/adolescent patients who underwent pediatric surgery at Karolinska University Hospital from January 2005 to July 2017. RESULTS: Overall 14 pancreatic operations were performed in 13 patients...
December 19, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/29275422/is-central-pancreatectomy-truly-recommendable-a-9-year-single-center-experience
#7
Ang Lv, Hong-Gang Qian, Hui Qiu, Jian-Hui Wu, Chun-Yi Hao
AIMS: To compare the short- and long-term outcomes in patients with pancreatic benign or borderline neoplasm who underwent central pancreatectomy (CP) and distal pancreatectomy (DP). METHODS: The inclusion criteria were as follows: (1) single benign or low-grade malignant tumor; (2) tumor confined to the pancreatic neck or proximal body; and (3) tumor amenable to either CP or DP. Short and long-term outcomes, including complications, pancreatic exocrine and endocrine function, and quality of life (QoL) were analyzed retrospectively...
December 22, 2017: Digestive Surgery
https://www.readbyqxmd.com/read/29248457/malnutrition-in-pancreatic-ductal-adenocarcinoma-pda-dietary-pancreatic-enzymes-improve-short-term-health-but-stimulate-tumor-growth
#8
Yalda Zolghadri, Shreoshi Pal Choudhuri, Ozhan Ocal, Somayeh Layeghi- Ghalehsoukhteh, Feaven Berhe, Michael A Hale, Thomas M Wilkie
Pancreatic ductal adenocarcinoma (PDA) is a deadly cancer that resists efforts to identify better chemotherapeutics. PDA is associated with chronic pancreatitis and acinar cell dedifferentiation. This reduces enzyme production by the exocrine pancreas, resulting in digestive insufficiencies. Malabsorption of partially digested food can cause bloating, overfilled intestines, abdominal pain, excessive feces, steatorrhea, and malnutrition. These maladies affect quality of life and restrict treatment options for pancreatitis and PDA...
December 14, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/29230485/-importance-of-functional-diagnostics-in-gastroenterology
#9
REVIEW
M Hollenbach, A Hoffmeister, J Rosendahl, J Mössner
In this review article important and frequently used investigation methods for gastrointestinal functional diagnostics are presented. Some other rarely used special investigations are also explained. The hydrogen breath test is simple to carry out, ubiquitously available and enables the detection of lactose, fructose and sorbitol malabsorption. Furthermore, by the application of glucose, the test can be carried out when there is a suspicion of abnormal intestinal bacterial colonization and using lactulose for measuring small intestinal transit time...
December 11, 2017: Der Internist
https://www.readbyqxmd.com/read/29221631/a-population-based-evaluation-of-the-natural-history-of-chronic-pancreatitis
#10
Jorge D Machicado, Suresh T Chari, Lawrence Timmons, Gong Tang, Dhiraj Yadav
BACKGROUND: Based on reports from tertiary care centers, chronic pancreatitis (CP) is considered to be a painful and debilitating disease frequently requiring invasive interventions. Our primary aim was to assess the natural course of CP in a population-based cohort using endoscopic and surgical interventions as surrogates for disease aggressiveness. METHODS: We identified all patients (n = 89, alcoholic [ACP = 46], non-alcoholic [NACP] = 43) with newly diagnosed definite CP from Olmsted County, Minnesota between 1977 and 2006...
November 24, 2017: Pancreatology: Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]
https://www.readbyqxmd.com/read/29217490/evaluation-of-pancreatic-exocrine-insufficiency-by-cine-dynamic-mrcp-using-spatially-selective-inversion-recovery-ir-pulse-correlation-with-severity-of-chronic-pancreatitis-based-on-morphological-changes-of-pancreatic-duct
#11
Kazuya Yasokawa, Katsuyoshi Ito, Akihiko Kanki, Akira Yamamoto, Teruyuki Torigoe, Tomohiro Sato, Tsutomu Tamada
PURPOSE: To evaluate the correlation between the pancreatic exocrine insufficiency estimated by cine-dynamic MRCP using spatially selective IR pulse and the severity stages (modified Cambridge classification) based on morphological changes of the pancreatic duct in patients with suspected chronic pancreatitis. MATERIALS AND METHODS: Thirty-nine patients with suspected chronic pancreatitis underwent cine-dynamic MRCP with a spatially selective IR pulse. The secretion grading score (5-point scale) based on the moving distance of pancreatic juice inflow on cine-dynamic MRCP was assessed, and compared with the stage of the severity of chronic pancreatitis based on morphological changes of pancreatic duct...
December 4, 2017: Magnetic Resonance Imaging
https://www.readbyqxmd.com/read/29212278/efficacy-and-safety-of-pancreatic-enzyme-replacement-therapy-on-exocrine-pancreatic-insufficiency-a-meta-analysis
#12
Can Gan, Yan-Hua Chen, Ling Liu, Jin-Hang Gao, Huan Tong, Cheng-Wei Tang, Rui Liu
Background: Pancreatic enzyme replacement therapy (PERT) is widely applied to patients with exocrine pancreatic insufficiency (EPI), but its effect and safety has not been quantified. Therefore we performed a meta-analysis to determine the efficacy and tolerance of PERT on patients with EPI. Materials and Methods: PubMed, Medline, Cochrane library database, Evidence-based medicine/clinical trials published before December 2016 were searched by two independent reviewers to identify prospective randomized controlled trials (RCTs)...
November 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/29178640/expanding-the-mutational-spectrum-in-johanson-blizzard-syndrome-identification-of-whole-exon-deletions-and-duplications-in-the-ubr1-gene-by-multiplex-ligation-dependent-probe-amplification-analysis
#13
Maja Sukalo, Eva Schäflein, Ina Schanze, David B Everman, Nima Rezaei, Jesús Argente, Isabel Lorda-Sanchez, Charu Deshpande, Tsutomu Takahashi, Alexander Kleger, Martin Zenker
BACKGROUND: Johanson-Blizzard syndrome (JBS, MIM #243800) is a very rare autosomal recessive disorder characterized by exocrine pancreatic insufficiency, nasal wing hypoplasia, hypodontia, and other abnormalities. JBS is caused by mutations of the UBR1 gene (MIM *605981), encoding a ubiquitin ligase of the N-end rule pathway. METHODS: Molecular findings in a total of 65 unrelated patients with a clinical diagnosis of JBS who were previously screened for UBR1 mutations by Sanger sequencing were reviewed and cases lacking a disease-causing UBR1 mutation on either one or both alleles were included in this study...
November 2017: Molecular Genetics & Genomic Medicine
https://www.readbyqxmd.com/read/29170072/pancreatic-enzyme-replacement-therapy-after-gastric-resection-an-update
#14
REVIEW
Filippo Antonini, Stefano Crippa, Massimo Falconi, Giampiero Macarri, Raffaele Pezzilli
Exocrine pancreatic insufficiency (EPI) is one of the possible mechanisms of fat maldigestion following gastric surgery, together with reduced food intake, loss of gastric reservoir, small bowel bacterial overgrowth and rapid small bowel transit. Oral pancreatic enzyme replacement therapy (PERT) is the mainstay of treatment for EPI. The efficacy and safety of pancreatic enzyme substitution in patients following gastric resection remains unclear. This review article summarizes relevant studies addressing PERT after gastric resection...
November 10, 2017: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29155861/pancreatic-exocrine-insufficiency-in-patients-with-chronic-heart-failure-and-its-possible-association-with-appetite-loss
#15
Tingting Xia, Xichen Chai, Jiaqing Shen
BACKGROUND: Appetite loss is one complication of chronic heart failure (CHF), and its association with pancreatic exocrine insufficiency (PEI) is not well investigated in CHF. AIM: We attempted to detect the association between PEI and CHF-induced appetite. METHODS: Patients with CHF were enrolled, and body mass index (BMI), left ventricular ejection fraction (LVEF), New York Heart Association (NYHA) cardiac function grading, B-type natriuretic peptide (BNP), serum albumin, pro-albumin and hemoglobin were evaluated...
2017: PloS One
https://www.readbyqxmd.com/read/29138633/influence-of-spk-with-enteric-drainage-on-the-pancreatic-exocrine-function-in-diabetic-patients-with-uremia
#16
Guanghui Pei, Wu Lv, Xiaohang Li, Guoqing Zhang, Jialin Zhang
Objective: This study aimed to determine the use of fecal elastase in evaluating the effect of simultaneous pancreas-kidney transplantation with enteric drainage on the pancreatic exocrine function of diabetic patients with uremia. Methods: A total of 19 patients with simultaneous pancreas-kidney transplantation (SPK) with enteric drainage, 31 diabetic patients with uremia (chronic renal failure (CRF)), 22 diabetic patients with uremia who underwent renal transplantation (RT), and 20 normal individuals (CON) were included in the study...
2017: International Journal of Endocrinology
https://www.readbyqxmd.com/read/29137169/pancreatic-exocrine-insufficiency-after-bariatric-surgery
#17
REVIEW
Miroslav Vujasinovic, Roberto Valente, Anders Thorell, Wiktor Rutkowski, Stephan L Haas, Urban Arnelo, Lena Martin, J-Matthias Löhr
Morbid obesity is a lifelong disease, and all patients require complementary follow-up including nutritional surveillance by a multidisciplinary team after bariatric procedures. Pancreatic exocrine insufficiency (PEI) refers to an insufficient secretion of pancreatic enzymes and/or sodium bicarbonate. PEI is a known multifactorial complication after upper gastrointestinal surgery, and might constitute an important clinical problem due to the large number of bariatric surgical procedures in the world. Symptoms of PEI often overlap with sequelae of gastric bypass, making the diagnosis difficult...
November 13, 2017: Nutrients
https://www.readbyqxmd.com/read/29137016/binding-pancreaticogastrostomy-anastomosis-in-central-pancreatectomy-a-single-center-experience
#18
Pengcheng Kang, Zhidong Wang, Kaiming Leng, Xiangyu Zhong, Hao Wang, Ming Wan, Sheng Tai, Yunfu Cui
A growing number of central pancreatectomies are performed. However, reconstruction of pancreaticoenteral digestive continuity after central pancreatectomy remains debated. This study evaluates the short-term outcomes of binding pancreaticogastrostomy anastomosis in central pancreatectomy.We have reviewed our experience with 52 patients who underwent binding pancreaticogastrostomy following central pancreatectomy from February 2009 to March 2015. Indication includes 6 noninvasive intraductal papillary mucinous neoplasms, 11 neuroendocrine tumors, 12 solid pseudopapillary tumor, 9 serous cystadenoma, 6 mucinous cystadenoma, and 8 focal pancreatic traumas...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29134356/secretin-stimulated-ultrasound-estimation-of-pancreatic-secretion-in-cystic-fibrosis-validated-by-magnetic-resonance-imaging
#19
Trond Engjom, Erling Tjora, Gaute Wathle, Friedemann Erchinger, Birger N Lærum, Odd H Gilja, Ingfrid Salvesen Haldorsen, Georg Dimcevski
OBJECTIVES: Secretin-stimulated magnetic resonance imaging (s-MRI) is the best validated radiological modality assessing pancreatic secretion. The purpose of this study was to compare volume output measures from secretin-stimulated transabdominal ultrasonography (s-US) to s-MRI for the diagnosis of exocrine pancreatic failure in cystic fibrosis (CF). METHODS: We performed transabdominal ultrasonography and MRI before and at timed intervals during 15 minutes after secretin stimulation in 21 CF patients and 13 healthy controls...
November 13, 2017: European Radiology
https://www.readbyqxmd.com/read/29106956/supplementation-of-pancreatic-digestive-enzymes-alters-the-composition-of-intestinal-microbiota-in-mice
#20
Hiroki Nishiyama, Tomoyuki Nagai, Masatoshi Kudo, Yoshihisa Okazaki, Yoshinao Azuma, Tomohiro Watanabe, Susumu Goto, Hiroyuki Ogata, Toshiharu Sakurai
Although pancreatic enzyme replacement therapy (PERT) is effective in the alleviation of pancreatic exocrine insufficiency (PEI)-related symptoms in patients with chronic pancreatitis, its mechanism of action is poorly understood. Recent studies suggest that the intestinal microbiota is associated with the pathogenesis of chronic pancreatitis. Therefore, we hypothesized that PERT exerts its effect by modifying the intestinal microbiota in addition to its presumed role in promoting fat and protein absorption...
October 26, 2017: Biochemical and Biophysical Research Communications
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