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Pediatric Gastroenterology, Hepatology & Nutrition

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https://www.readbyqxmd.com/read/28401059/bannayan-riley-ruvalcaba-syndrome-in-a-patient-with-a-pten-mutation-identified-by-chromosomal-microarray-analysis-a-case-report
#1
Sun Hwa Lee, Eell Ryoo, Hann Tchah
Bannayan-Riley-Ruvalcaba syndrome (BRRS) is one of the phosphatase and tensin homolog hamartoma tumor syndrome with a PTEN gene mutation. It is a rare dominant autosomal disorder characterized by cutaneous lipomas, macrocephaly, intestinal polyps, and developmental delay. Diagnosing this syndrome is important, because it may represent the pediatric phenotype of Cowden syndrome, in which there is an increased risk for malignant tumors in children. Until now, the prevalence of BRRS is unknown. Several dozen cases have been reported in the medical literature, but no case has been reported in Korea...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401058/a-rare-cause-of-recurrent-acute-pancreatitis-in-a-child-isovaleric-acidemia-with-novel-mutation
#2
Elif Sag, Alper Han Cebi, Gulay Kaya, Gulay Karaguzel, Murat Cakir
Recurrent acute pancreatic attacks is a rare clinical condition (2-5% of all acute pancreatis) in children and is mainly idiopathic in most cases. Sometimes it may be associated with congenital anomalies, metabolic diseases or hereditary conditions. Isovaleric acidemia (IVA) is a rare autosomal recessive amino acid metabolism disorder associated with isovaleryl coenzyme A dehydrogenase deficiency presenting the clinical findings such metabolic acidosis with increased anion gap, hyperammonemia, ketonemia, hypoglycemia, "the odor of sweaty feet," abdominal pain, vomiting, feeding intolerance, shock and coma...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401057/intestinal-hypoganglionosis-leading-to-intestinal-failure-and-the-compassionate-use-of-omegaven%C3%A2
#3
Racha Khalaf, Sara Karjoo, Paul Danielson, Michael Wilsey, Fauzia Shakeel
Intestinal hypoganglionosis is a rare innervation disorder that provides numerous nutritional, medical and surgical challenges. In this case report, we present a case of a newborn with intestinal hypoganglionosis leading to intestinal failure and intestinal failure-associated liver disease who responded to Omegaven™, a fat emulsion comprised of omega-3 fatty acids. Omegaven™ has been shown to be beneficial in the management of cholestatic liver injury. Clinical success with Omegaven™ was seen in this patient with a clear decrease in aspartate aminotransferase, alanine aminotransferase, alkaline phosphatase and complete resolution of cholestasis with a direct bilirubin of zero within two weeks of initiation of Omegaven™...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401056/effect-of-metronidazole-in-infants-with-bowel-habit-change-irrelative-to-the-clostridium-difficile-colonization
#4
Eun Jin Kim, Sung Hyun Lee, Hann Tchah, Eell Ryoo
PURPOSE: Clinical symptoms associated with Clostridium difficile infection (CDI) can vary widely. Carrier state without apparent symptoms is relatively common during infancy. The objective of this study was to determine the association of C. difficile colonization with bowel habit change and the effect of C. difficile colonization treatment on restoration of normal bowel habit. METHODS: Between 2006 and 2014, infants at 1 to 12 months of age with diarrhea for more than 2 weeks who did not improve with conservative care were recruited from Gachon University Gil Medical Center...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401055/effect-of-the-baseline-vitamin-d-level-on-growth-outcome-in-pediatric-crohn-disease
#5
Eun Joo Lee, Jin Soo Moon, Jae Sung Ko, Hye Ran Yang, Ju Young Jang, Ju Whi Kim, Kyung Jae Lee
PURPOSE: Vitamin D deficiency is common in Crohn disease (CD). The aim of the study was to examine the prevalence of vitamin D deficiency and evaluate the association between vitamin D status and growth outcome in Korean pediatric CD patients. METHODS: In this retrospective study, 17 children younger than 18 years old diagnosed with CD were enrolled and their serum 25-hydroxy vitamin D (25[OH]D) was checked between 2011 and 2015. We categorized the patients into two groups, Group 1 and Group 2...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401054/single-center-experience-with-gastrostomy-insertion-in-pediatric-patients-a-10-year-review
#6
Jiyoung Kim, Hong Koh, Eun Young Chang, Sun Yeong Park, Seung Kim
PURPOSE: This study was performed to review the outcomes of gastrostomy insertion in children at our institute during 10 years. METHODS: A retrospective chart review was performed on 236 patients who underwent gastrostomy insertion from October 2005 to March 2015. We used our algorithm to select the least invasive method for gastrostomy insertion for each patient. Long-term follow-up was performed to analyze complications related to the method of gastrostomy insertion...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401053/comparison-of-percutaneous-endoscopic-gastrostomy-and-surgical-gastrostomy-in-severely-handicapped-children
#7
June Kim, Min Lee, Soon Chul Kim, Chan Uhng Joo, Sun Jun Kim
PURPOSE: Gastrostomy is commonly used procedures to provide enteral nutrition support for severely handicapped patients. This study aimed to identify and compare outcomes and complications associated with percutaneous endoscopic gastrostomy (PEG) and surgical gastrostomy (SG). METHODS: A retrospective chart review of 51 patients who received gastrostomy in a single tertiary hospital from January 2000 to May 2016 was performed. We analyzed the patients and the complications caused by the procedures...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401052/pooled-analysis-of-the-cow-s-milk-related-symptom-score-comiss%C3%A2-as-a-predictor-for-cow-s-milk-related-symptoms
#8
Yvan Vandenplas, Philippe Steenhout, Anette Järvi, Anne-Sophie Garreau, Rajat Mukherjee
PURPOSE: The diagnosis of cow's milk (CM) allergy is a challenge. The Cow's Milk-related-Symptom-Score (CoMiSS™) was developed to offer primary health care providers a reliable diagnostic tool for CM related symptoms. The predictive prospective value of the CoMiSS™ was evaluated in three clinical trials. METHODS: Pooled analyses of the three studies were conducted based on regressing the results of the month-1 challenge test on the month-1 CoMiSS™, adjusting for baseline CoMiSS™ using a logistic regression model...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401051/surgery-in-pediatric-crohn-s-disease-indications-timing-and-post-operative-management
#9
REVIEW
Seung Kim
Pediatric onset Crohn's disease (CD) tends to have complicated behavior (stricture or penetration) than elderly onset CD at diagnosis. Considering the longer duration of the disease in pediatric patients, the accumulative chance of surgical treatment is higher than in adult onset CD patients. Possible operative indications include perianal CD, intestinal stricture or obstruction, abdominal abscess or fistula, intestinal hemorrhage, neoplastic changes and medically untreatable inflammation. Growth retardation is an operative indication only for pediatric patients...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28401050/the-new-rome-iv-criteria-for-functional-gastrointestinal-disorders-in-infants-and-toddlers
#10
REVIEW
Judith Zeevenhooven, Ilan J N Koppen, Marc A Benninga
Functional gastrointestinal disorders (FGIDs) are common worldwide and cover a wide range of disorders attributable to the gastrointestinal tract that cannot be explained by structural or biochemical abnormalities. The diagnosis of these disorders relies on the symptom-based Rome criteria. In 2016 the Rome criteria were revised for infants/toddlers and for children and adolescents. In this review, we discuss the novel Rome IV criteria for infants and toddlers. The criteria for infant colic were drastically changed, whereas only minor changes were made for regurgitation, cyclic vomiting syndrome, functional diarrhea, infant dyschezia and functional constipation...
March 2017: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090476/correction-middle-east-consensus-statement-on-the-diagnosis-and-management-of-functional-gastrointestinal-disorders-in-12-months-old-infants
#11
Yvan Vandenplas, Muath Abdurrahman Alturaiki, Wafaa Al-Qabandi, Fawaz AlRefaee, Ziad Bassil, Bassam Eid, Ahmed El Beleidy, Ali Ibrahim Almehaidib, Pierre Mouawad, Maroun Sokhn
[This corrects the article on p. 153 in vol. 19, PMID: 27738596.].
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090475/use-of-omega-3-polyunsaturated-fatty-acids-to-treat-inspissated-bile-syndrome-a-case-report
#12
Woo Young Jun, Min Jeng Cho, Hye Seung Han, Sun Hwan Bae
Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090474/post-laparoscopic-cholecystectomy-biloma-in-a-child-managed-by-endoscopic-retrograde-cholangio-pancreatography-and-stenting-a-case-report
#13
Charu Tiwari, Om Prakash Makhija, Deepa Makhija, Shalika Jayaswal, Hemanshi Shah
Laparoscopic cholecystectomy, though an uncommon surgical procedure in paediatric age group is still associated with a higher risk of post-operative bile duct injuries when compared with the open procedure. Small leaks from extra hepatic biliary apparatus usually lead to the formation of a localized sub-hepatic bile collection, also known as biloma. Such leaks are rare complication after laparoscopic cholecystectomy, especially in paediatric age group. Minor bile leaks can usually be managed non-surgically by percutaneous drainage combined with endoscopic retrograde cholangio-pancreatography (ERCP)...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090473/three-year-old-male-with-multiple-dieulafoy-lesions-treated-with-epinephrine-injections-via-therapeutic-endoscopy
#14
Christina L Baldwin, Michael Wilsey
Dieulafoy lesions, vascular anomalies typically found along the gastrointestinal tract, have been viewed as rare and obscure causes of sudden intestinal bleeding, especially in pediatric patients. Since their discovery in the late 19th century, the reported incidence has increased. This is due to an increased awareness of, and knowledge about, their presentation and to advanced endoscopic diagnosis and therapy. Our patient was a three-year-old male, without a complex medical history. He presented to the emergency department with acute hematemesis with blood clots and acute anemia requiring blood transfusion...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090472/prevalence-and-risk-factors-for-the-weight-loss-during-hospitalization-in-children-a-single-korean-children-s-hospital-experience
#15
Eun Ha Hwang, Jae Hong Park, Peter Chun, Yeoun Joo Lee
PURPOSE: Undernutrition during hospitalization increases the risk of nosocomial infection and lengthens the disease courses. The aim of this study was to evaluate the risk factors of weight loss during hospitalization in children. METHODS: All the patients who were admitted in general wards between April and May 2014 were enrolled. Patients aged >18 years and discharged within 2 days were excluded. Weight loss during hospitalization was defined as a decrease in body weight of >2% in 8 hospital days or on the day of discharge...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090471/hair-zinc-level-analysis-and-correlative-micronutrients-in-children-presenting-with-malnutrition-and-poor-growth
#16
Tae Hwan Han, Jin Lee, Yong Joo Kim
PURPOSE: Zinc deficiency can induce serious clinical problems in the gastrointestinal (GI) system and immune system and can affect growth and development. It is more severe in younger patients. Chronic zinc deficiency is reflected more precisely in hair than in serum. We studied hair zinc levels and other hair and serum micronutrients in chronic malnourished children to identify which micronutrients are affected or correlated with the other ones. METHODS: Hair mineral analyses were performed in 56 children (age, 1-15 years) presenting with malnutrition, poor growth, poor appetite, anorexia, with/without other GI symptoms (diarrhea, abdominal pain, constipation) from August 2012 to March 2015...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090470/acanthosis-nigricans-as-a-clinical-predictor-of-insulin-resistance-in-obese-children
#17
Young Kwon Koh, Jae Hee Lee, Eun Young Kim, Kyung Rye Moon
PURPOSE: This study aimed to evaluate the utility of acanthosis nigricans (AN) severity as an index for predicting insulin resistance in obese children. METHODS: The subjects comprised 74 obese pediatric patients who attended the Department of Pediatrics at Chosun University Hospital between January 2013 and March 2016. Waist circumference; body mass index; blood pressure; fasting glucose and fasting insulin levels; lipid profile; aspartate transaminase, alanine transaminase, glycated hemoglobin, C-peptide, and uric acid levels; and homeostatic model assessment insulin resistance (HOMA-IR) and quantitative insulin check sensitivity index (QUICKI) scores were compared between subjects with AN and those without AN...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090469/the-relationships-between-respiratory-virus-infection-and-aminotransferase-in-children
#18
Jun Suk Oh, Jun Sik Choi, Young Hyuk Lee, Kyung Og Ko, Jae Woo Lim, Eun Jung Cheon, Gyung Min Lee, Jung Min Yoon
PURPOSE: We sought to examine the relationship between the clinical manifestations of nonspecific reactive hepatitis and respiratory virus infection in pediatric patients. METHODS: Patients admitted to the pediatric unit of Konyang University Hospital for lower respiratory tract disease between January 1, 2014 and December 31, 2014 and who underwent reverse transcriptase polymerase chain reaction tests were examined. The patients were divided into those with increased levels of alanine aminotransferase (ALT) or aspartate aminotransferase (AST) and those with normal ALT or AST levels...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090468/clinical-features-and-extraintestinal-manifestations-of-crohn-disease-in-children
#19
Young Ah Lee, Peter Chun, Eun Ha Hwang, Sang Wook Mun, Yeoun Joo Lee, Jae Hong Park
PURPOSE: The aim of this study was to investigate the clinical features and extraintestinal manifestations (EIMs) of Crohn disease (CD) in Korean pediatric patients. METHODS: The medical records of 73 children diagnosed with CD were retrospectively reviewed. Data regarding baseline demographic and clinical characteristics, including CD phenotype at diagnosis based on the Montreal classification, and clinical features and course of EIMs were investigated. RESULTS: Fifty-two (71...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
https://www.readbyqxmd.com/read/28090467/early-infant-feeding-practices-may-influence-the-onset-of-symptomatic-celiac-disease
#20
Shailja Vajpayee, Shiv Dayal Sharma, Rajkumar Gupta, Alok Goyal, Aakash Sharma
PURPOSE: To study whether breastfeeding and breastfeeding status during gluten introduction influences the age at diagnosis of celiac disease (CD). In addition to study, whether the timing of gluten introduction influences the age at diagnosis of CD. METHODS: It was a hospital based observational study. Total 198 patients diagnosed with CD as per modified European Society of Pediatric Gastroenterology, Hepatology and Nutrition (2012) criteria, aged between 6 months to 6 years were included...
December 2016: Pediatric Gastroenterology, Hepatology & Nutrition
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