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Meconium ileus

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https://www.readbyqxmd.com/read/28472055/abdominal-symptoms-in-cystic-fibrosis-and-their-relation-to-genotype-history-clinical-and-laboratory-findings
#1
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
#2
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
#3
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
#4
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
#5
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...
February 28, 2017: Surgery Today
https://www.readbyqxmd.com/read/28209955/-the-complications-of-intestinal-stoma-in-children
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/27740724/innovative-assessment-of-inpatient-and-pulmonary-drug-costs-for-children-with-cystic-fibrosis
#12
Joseph F Levy, Marjorie A Rosenberg, Philip M Farrell
BACKGROUND: Previous estimates of the cost of care for pediatric Cystic fibrosis (CF) showed wide variation, without specific summary of pulmonary drug costs. METHODS: Enrolled CF children from the Wisconsin newborn screening trial were evaluated quarterly per protocol. Assessments systematically included all treatments, hospitalizations, and nutritional and pulmonary outcomes. Direct medical costs from hospital billing and medical records from 1989 to 2010 were used to describe costs by age-ranges and subgroups throughout follow-up...
December 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27696212/neonatal-gastrointestinal-perforation-in-japan-a-nationwide-survey
#13
MULTICENTER STUDY
Masahito Sato, Yoshinori Hamada, Miyuki Kohno, Kazuya Ise, Keiichi Uchida, Hiromi Ogata, Hiroaki Masuyama, Yoshiki Morotomi, Masao Yasufuku, Motoshi Wada
PURPOSE: The purpose of this study is to identify the current clinical features of neonatal gastrointestinal perforation in Japan. METHODS: A questionnaire about cases of neonatal gastrointestinal perforation treated in recent 5 years was sent to participating institutions of the Japanese Society of Pediatric Surgeons (JSPS). RESULTS: Five hundred and thirty-six neonates with gastrointestinal perforation were treated. They consisted of 42 patients with gastric rupture/perforation (GR), 33 patients with intestinal atresia/stenosis (IA), 3 patients with malrotation (ML), 118 patients with necrotizing enterocolitis (NEC), 160 patients with focal intestinal perforation (FIP), 46 patients with meconium-related ileus (MRI), 77 patients with meconium peritonitis (MP), and 57 patients with other conditions...
January 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/27639560/onset-of-persistent-pseudomonas-aeruginosa-infection-in-children-with-cystic-fibrosis-with-interval-censored-data
#14
Wenjie Wang, Ming-Hui Chen, Sy Han Chiou, Hui-Chuan Lai, Xiaojing Wang, Jun Yan, Zhumin Zhang
BACKGROUND: Persistent Pseudomonas aeruginosa (PPA) infection promotes lung function deterioration in children with cystic fibrosis (CF). Although early CF diagnosis through newborn screening (NBS) has been shown to provide nutritional/growth benefit, it is unclear whether NBS lowers the risk of PPA infection and how the effect of NBS vary with age. Modeling the onset age of PPA infection is challenging because 1) the onset age of PPA infection is interval censored in patient registry data; and 2) some risk factors such as NBS may have time-varying effects...
September 17, 2016: BMC Medical Research Methodology
https://www.readbyqxmd.com/read/27555301/prevalence-of-elevated-liver-enzymes-in-children-with-cystic-fibrosis-diagnosed-by-newborn-screen
#15
Samantha A Woodruff, Marci K Sontag, Frank J Accurso, Ronald J Sokol, Michael R Narkewicz
BACKGROUND: Prevalence and risks for elevated liver enzymes have not been studied systematically in children with CF identified by newborn screen. METHODS: 298 CF children identified by newborn screen since 1982. AST, ALT and GGT tested at annual visits. Percent of children with 1 or ≥2 values of elevated AST, ALT and GGT determined. Relationship of liver enzymes to clinical factors or subsequent liver disease was analyzed RESULTS: At least one abnormal value for AST (63%), ALT (93%) and ALT ≥1...
January 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27510896/transient-spontaneous-remission-in-congenital-mll-af10-rearranged-acute-myeloid-leukemia-presenting-with-cardiorespiratory-failure-and-meconium-ileus
#16
Tobias Gyárfás, Juergen Wintgens, Wolfgang Biskup, Ilske Oschlies, Wolfram Klapper, Reiner Siebert, Susanne Bens, Claudia Haferlach, Roland Meisel, Michaela Kuhlen, Arndt Borkhardt
BACKGROUND: Neonatal leukemia is a rare disease with an estimated prevalence of about one to five in a million neonates. The majority being acute myeloid leukemia (AML), neonatal leukemia can present with a variety of symptoms including hyperleucocytosis, cytopenia, hepatosplenomegaly, and skin infiltrates. Chromosomal rearrangements including mixed lineage leukemia (MLL) translocations are common in neonatal AML. CASE PRESENTATION: A female neonate born at 34 weeks gestation presented with cardiorespiratory failure, hepatosplenomegaly, pancytopenia, and coagulopathy...
December 2016: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/27502879/clinical-spectrum-of-neonates-presenting-with-pneumoperitoneum-a-retrospective-study
#17
Ahmed Hosni Morsi, Hossam Rashad Omar, Ahmed Osama, Ahmed Refaat Khodary
BACKGROUND: Neonatal pneumoperitoneum is attributed, in most of the reported cases, to necrotising enterocolitis (NEC). There are also other causes leading to free intraperitoneal air. The aim of this study is to describe the clinical spectrum, causes, management and outcome of neonates admitted with pneumoperitoneum in the paediatric surgery unit of a university hospital. SUBJECTS AND METHODS: This retrospective study included neonates having radiographic evidences of pneumoperitoneum from 2012 to the end of 2014...
July 2016: African Journal of Paediatric Surgery: AJPS
https://www.readbyqxmd.com/read/27461434/risk-factors-and-prevention-for-surgical-intestinal-disorders-in-extremely-low-birth-weight-infants
#18
Masaya Yamoto, Yusuke Nakazawa, Koji Fukumoto, Hiromu Miyake, Hideaki Nakajima, Akinori Sekioka, Akiyoshi Nomura, Kei Ooyama, Yutaka Yamada, Katsushi Nogami, Yuko Van, Chisako Furuta, Reiji Nakano, Yasuhiko Tanaka, Naoto Urushihara
PURPOSE: Surgical intestinal disorders (SID), such as necrotizing enterocolitis (NEC), focal intestinal perforation (FIP), and meconium-related ileus (MRI), are serious morbidities in extremely low birth weight (ELBW, birth weight <1000 g) infants. From 2010, we performed enteral antifungal prophylaxis (EAP) in ELBWI to prevent for SID. The aim of this study was to identify disease-specific risk factors and to evaluate the efficacy of prevention for SID in ELBW infants. METHODS: A retrospective chart review of all consecutive patients between January 2006 and March 2015, which included 323 ELBW infants who were admitted to Shizuoka Children's Hospital, was conducted...
September 2016: Pediatric Surgery International
https://www.readbyqxmd.com/read/27374658/cystic-fibrosis-a-report-of-33-pediatric-tunisian-cases
#19
Sonia Halioui-Louhaichi, Amel Ben Chehida, Rim Hassouna, Tayeb Massaoud, Marie Françoise Ben Dridi, Sihem Barsaoui, Azza Gharbi-Sammoud, Neji Tebib, Ahmed Maherzi
BACKGROUND: The frequency of cystic fibrosis is unknown in Tunisia, regarding the limited number of reported surveys and patients. AIM: to determine the clinical characteristics, outcome and genetic data of cystic fibrosis in Tunisian pediatric patients. METHODS: Cases of cystic fibrosis managed at pediatric departments of Tunis, during 15 years (1997-2012), were reviewed. RESULTS: 33 children (23 males and 10 females) were enrolled...
August 2015: La Tunisie Médicale
https://www.readbyqxmd.com/read/27244789/pubertal-height-growth-and-adult-height-in-cystic-fibrosis-after-newborn-screening
#20
RANDOMIZED CONTROLLED TRIAL
Zhumin Zhang, Mary J Lindstrom, Philip M Farrell, HuiChuan J Lai
BACKGROUND: To examine long-term growth benefit of newborn screening (NBS), adolescent peak height velocity (PHV), and adult height were compared between the screened (diagnosed early via NBS) and the control (identified generally by symptoms) in the Wisconsin Randomized Clinical Trial. METHODS: Data from 107 children born in 1985-1994 and followed through 2012 were analyzed. PHV was estimated by a semiparametric growth curve model and compared with Tanner reference...
May 2016: Pediatrics
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