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Meconium ileus

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https://www.readbyqxmd.com/read/29692998/early-diagnosis-from-newborn-screening-maximises-survival-in-severe-cystic-fibrosis
#1
Gloria Tridello, Carlo Castellani, Ilaria Meneghelli, Anna Tamanini, Baroukh M Assael
Newborn screening (NBS) for cystic fibrosis (CF) has been gradually established in several countries, but scant data are available on its long-term effects on survival. Our objective was to evaluate the long-term effects of CF NBS on survival. 586 patients, diagnosed and followed between 1971 and 2014 at the Verona CF Centre were analysed. Eligibility was confirmed in 342 cases diagnosed by NBS, 101 with meconium ileus and 143 through symptoms (44 out of 143 were NBS false negatives). The primary end-point was the 30-year overall survival in patients diagnosed by NBS...
April 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29562160/meconium-ileus-in-a-neonate-with-cystic-fibrosis
#2
Kelly E Wood
No abstract text is available yet for this article.
March 22, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29487495/meconium-ileus
#3
REVIEW
John H T Waldhausen, Morgan Richards
Cystic fibrosis is one of the most common inheritable traits in Caucasians. Meconium ileus and its potential complications are the most likely reasons that these patients will need surgical care. Surgical intervention is usually needed in the neonatal period but may also be required later in life. This article discusses the various ways cystic fibrosis can affect the gastrointestinal tract. Both the operative and nonoperative management of complicated and uncomplicated meconium ileus are discussed in the neonatal period as well as long-term issues, such as distal intestinal obstructive syndrome, fibrosing colonopathy, and rectal prolapse, all of which may be seen in older children and adults...
March 2018: Clinics in Colon and Rectal Surgery
https://www.readbyqxmd.com/read/29396025/early-life-growth-patterns-persist-for-12-years-and-impact-pulmonary-outcomes-in-cystic-fibrosis
#4
Don B Sanders, Zhumin Zhang, Philip M Farrell, HuiChuan J Lai
BACKGROUND: In children with cystic fibrosis (CF), recovery from growth faltering within 2 years of diagnosis (Responders) is associated with better growth and less lung disease at age 6 years. This study examined whether these benefits are sustained through 12 years of age. METHODS: Longitudinal growth from 76 children with CF enrolled in the Wisconsin CF Neonatal Screening Project was examined and categorized into 5 groups: R12 , R6 , and R2 , representing Responders who maintained growth improvement to age 12, 6, and 2 years, respectively, and I6 and N6, representing Non-responders whose growth did and did not improve during ages 2-6 years, respectively...
January 30, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29224788/long-term-intestinal-obstruction-sequelae-and-growth-in-children-with-cystic-fibrosis-operated-for-meconium-ileus-expectancies-and-surprises
#5
Anastasia Mentessidou, Ioanna Loukou, Georgios Kampouroglou, Anastasia Livani, Ioannis Georgopoulos, Petros Mirilas
BACKGROUND/PURPOSE: In the few studies on intestinal complications and growth of cystic fibrosis (CF) patients with a history of meconium ileus (MI), operated MI has not been investigated separately. We aimed to investigate the incidence of long-term intestinal obstruction sequelae [constipation, distal intestinal obstruction syndrome (DIOS)] and growth in CF patients operated for MI. METHODS: Retrospective study (1989-2016) including operative diagnoses and procedures, constipation and DIOS events, yearly Body Mass Index (BMI) measurements...
November 15, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28986020/meconium-ileus-in-cystic-fibrosis
#6
Meghana Sathe, Roderick Houwen
Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology of MI and its clinical presentation. It focuses on the medical and surgical management emphasizing the importance of nutrition and a multidisciplinary approach to improve both short-term and long-term outcomes for CF patients with MI.
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28978796/guanylate-cyclase-2c-agonism-corrects-cftr-mutants
#7
Kavisha Arora, Yunjie Huang, Kyushik Mun, Sunitha Yarlagadda, Nambirajan Sundaram, Marco M Kessler, Gerhard Hannig, Caroline B Kurtz, Inmaculada Silos-Santiago, Michael Helmrath, Joseph J Palermo, John P Clancy, Kris A Steinbrecher, Anjaparavanda P Naren
Cystic fibrosis (CF) is a genetic disorder in which epithelium-generated fluid flow from the lung, intestine, and pancreas is impaired due to mutations disrupting CF transmembrane conductance regulator (CFTR) channel function. CF manifestations of the pancreas and lung are present in the vast majority of CF patients, and 15% of CF infants are born with obstructed gut or meconium ileus. However, constipation is a significantly underreported outcome of CF disease, affecting 47% of the CF patients, and management becomes critical in the wake of increasing life span of CF patients...
October 5, 2017: JCI Insight
https://www.readbyqxmd.com/read/28932729/a-novel-and-simple-method-using-a-transanal-intestinal-long-tube-for-protecting-intestinal-anastomosis-and-decompressing-the-small-bowel
#8
So Hyun Nam
PURPOSE: I introduce the use of transanal intestinal long tube (TILT) using nasogastric tube. TILT passes from anus to the anastomosis, helping to decompress a dilated bowel loop. METHODS: TILT procedure was limited to those patients predicting a severe luminal size discrepancy after intestinal anastomosis, and who had postoperative prolonged ileus. We retrospectively reviewed the medical records of 10 infants (7 male an 3 female patients) who were treated using the TILT procedure between 2012 and 2016...
September 2017: Annals of Surgical Treatment and Research
https://www.readbyqxmd.com/read/28920016/comparison-of-t-tube-ileostomy-and-bishop-koop-ileostomy-for-the-management-of-uncomplicated-meconium-ileus
#9
Md Samiul Hasan, Ashrarur Rahman Mitul, Sabbir Karim, Kazi Md Noor-Ul Ferdous, M Kabirul Islam
BACKGROUND: Meconium ileus is a common cause of neonatal intestinal obstruction. Various surgical procedures are in practice for uncomplicated meconium ileus. Bishop Koop ileostomy allows distal passage of gut content and uses the distal absorptive area. T tube ileostomy avoids the need for gut resection and formal closure of stoma. The aim of this prospective interventional study was to compare the outcome of T-tube ileostomy and Bishop Koop ileostomy for the treatment of uncomplicated meconium ileus...
July 2017: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/28472055/abdominal-symptoms-in-cystic-fibrosis-and-their-relation-to-genotype-history-clinical-and-laboratory-findings
#10
Harold Tabori, Christin Arnold, Anke Jaudszus, Hans-Joachim Mentzel, Diane M Renz, Steffen Reinsch, Michael Lorenz, Ruth Michl, Andrea Gerber, Thomas Lehmann, Jochen G Mainz
BACKGROUND & AIMS: Abdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. METHODS: A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1...
2017: PloS One
https://www.readbyqxmd.com/read/28437538/effects-of-diagnosis-by-newborn-screening-for-cystic-fibrosis-on-weight-and-length-in-the-first-year-of-life
#11
MULTICENTER STUDY
Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey
Importance: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. Design, Setting, and Participants: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015...
June 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28420732/modulation-of-tmem16a-channel-activity-by-the-von-willebrand-factor-type-a-vwa-domain-of-the-calcium-activated-chloride-channel-regulator-1-clca1
#12
Monica Sala-Rabanal, Zeynep Yurtsever, Kayla N Berry, Colin G Nichols, Tom J Brett
Calcium-activated chloride channels (CaCCs) are key players in transepithelial ion transport and fluid secretion, smooth muscle constriction, neuronal excitability, and cell proliferation. The CaCC regulator 1 (CLCA1) modulates the activity of the CaCC TMEM16A/Anoctamin 1 (ANO1) by directly engaging the channel at the cell surface, but the exact mechanism is unknown. Here we demonstrate that the von Willebrand factor type A (VWA) domain within the cleaved CLCA1 N-terminal fragment is necessary and sufficient for this interaction...
June 2, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28409283/body-composition-and-lung-function-in-children-with-cystic-fibrosis-and-meconium-ileus
#13
Artemis Doulgeraki, Argyri Petrocheilou, Glykeria Petrocheilou, George Chrousos, Stavros-Eleftherios Doudounakis, Athanasios G Kaditis
The aim of this study was to explore whether history of meconium ileus (MI) at birth in children and adolescents with cystic fibrosis (CF) adversely affects body composition and lung function in later life. Data of children and adolescents with CF who underwent spirometry and DXA as part of their routine care were analyzed. Associations between MI (explanatory variable) and areal bone mineral density (total body less head-TBLH aBMD), lean tissue mass (LTM), and fat mass (FM) (outcomes) were assessed using general linear models...
June 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28247106/timing-and-outcome-of-stoma-closure-in-very-low-birth-weight-infants-with-surgical-intestinal-disorders
#14
Kyoko Mochizuki, Masahiro Hayakawa, Naoto Urushihara, Hiromu Miyake, Akiko Yokoi, Jun Shiraishi, Hideshi Fujinaga, Kensuke Ohashi, Genshiro Esumi, Satoko Ohfuji, Shintaro Amae, Toshihiro Yanai, Taizo Furukawa, Yuko Tazuke, Kyoko Minagawa, Hiroomi Okuyama
PURPOSE: Very low birth weight infants (VLBWIs) are at risk of surgical intestinal disorders including necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI). We conducted this study to verify whether the timing of stoma closure and that of enteral nutrition establishment after stoma closure in VLBWIs differ among the most common disorders. METHODS: A retrospective multicenter study was conducted at 11 institutes. We reviewed the timing of stoma closure and enteral nutrition establishment in VLBWIs who underwent stoma creation for intestinal disorders...
August 2017: Surgery Today
https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#15
S V Minaev, N I Bykov, A V Isaeva, A V Kachanov, E A Tovkan, N V Filip'yeva, I N Gerasimenko
AIM: To investigate the complications of intestinal stoma in children and to develop measures for decrease of their incidence. MATERIAL AND METHODS: The study included 152 children with congenital and acquired gastrointestinal pathology requiring surgical treatment with the imposition of intestinal stoma. Atresia of intestinal tube was observed in 28 (18.4%) children, meconium ileus - in 10 (6.6%) cases, Hirschsprung's disease - in 11 (7.2%)cases, anorectal malformations - in 39 (25...
2017: Khirurgiia
https://www.readbyqxmd.com/read/28003230/prenatal-intestinal-volvulus-look-for-cystic-fibrosis
#16
Taieb Chouikh, Nicolas Mottet, Christelle Cabrol, Yann Chaussy
Intestinal volvulus is a life-threatening emergency requiring prompt surgical management. Prenatal intestinal volvulus is rare, and most are secondary to intestinal atresia, mesenteric defect or without any underlying cause. Cystic fibrosis (CF) is known to cause digestive tract disorders. After birth, 10-15% of newborns with CF may develop intestinal obstruction within a few days of birth because of meconial ileus.1 This obstruction is a result of dehydrated thickened meconium obstructing the intestinal lumen...
December 21, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27896154/use-of-t-tube-enterostomy-in-neonatal-gastro-intestinal-surgery
#17
Maher Al-Zaiem, Abdulhai F Al-Garni, Abdulrahman Al-Maghrebi, Asim A Asghar
Aim: To evaluate the results of the use of the T-tube ileostomy in neonatal intestinal surgery cases. Materials and Methods: A retrospective review of sixty two neonates underwent intestinal obstruction surgery by using T-tube ileostomy was conducted between January 1990 and January 2013.The pathologies of the intestinal obstruction were; thirty four of jejunoileal atresia cases, thirteen case meconium ileus, eight cases perforated necrotizing enterocolitis (NEC), three cases meconium peritonitis, three cases with bowel resection due to intestinal volvulus, and one case of gastroschisis...
October 2016: Journal of Neonatal Surgery
https://www.readbyqxmd.com/read/27806795/-infantile-cholestasis-caused-by-cftr-mutation-case-report-and-literature-review
#18
REVIEW
L Li, N L Wang, J Y Gong, J S Wang
Objective: To study the clinical presentation, biochemical features and genetic analysis of an infant with cholestasis related to the CFTR mutations. Method: The clinical presentation, laboratory investigations and management of a case with infantile cholestasis caused by CFTR mutations were summarized and the relevant literature was reviewed. Result: (1) The patient was a 5 months old boy with cholestasis which developed in neonatal period with delayed meconium exclusion.The laparoscopic exploration was performed to exclude biliary atresia because of acholic stool when he was two months old...
November 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/27793338/cystic-fibrosis-is-associated-with-adverse-neonatal-outcomes-in-washington-state-1996-2013
#19
Kathleen J Ramos, Coralynn S Sack, Kristina H Mitchell, Christopher H Goss, Jacqueline R Starr
OBJECTIVE: To determine whether cystic fibrosis (CF) is associated with adverse neonatal outcomes in a recent birth cohort in the US. STUDY DESIGN: A retrospective matched cohort study of infants born in Washington State from 1996 to 2013 was identified through birth certificate data and linked to statewide hospital discharge data. Infants with CF were identified by hospitalization (through age 5 years) in which a CF-specific International Classification of Diseases, Ninth Revision code was recorded...
January 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/27781254/-application-of-bishop-koop-stoma-in-refractory-congenital-intestinal-atresia
#20
Hong Zhang, Wei Zhong, Jing Sun, Qiuming He, Yong Wang, Le Li, Jiakang Yu, Zhe Wang, Zhihua Ye, Kailin Tang, Huimin Xia
OBJECTIVE: To explore the feasibility and safety of Bishop-Koop stoma procedure in the treatment of neonates with refractory congenital intestinal atresia. METHODS: Clinical and follow-up data of 25 neonates with refractory congenital intestinal atresia undergoing Bishop-Koop stoma procedure in our center from January 2011 to December 2014 were retrospectively analyzed. Of 25 neonates, 13 (52%) were male, 12(48%) were female, the birth weight was 1600-3800 g (mean 2920 g), the age of admission was 10 hours to 20 days, and the age of operation was 1-58 d (mean 7 d)...
October 25, 2016: Zhonghua Wei Chang Wai Ke za Zhi, Chinese Journal of Gastrointestinal Surgery
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