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Meconium ileus

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...
October 14, 2016: Pediatric Pulmonology
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...
September 30, 2016: Pediatric Surgery International
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...
2016: BMC Medical Research Methodology
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...
August 20, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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
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
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
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
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
Małgorzata Olszowiec-Chlebna, Agnieszka Koniarek-Maniecka, Włodzimierz Stelmach, Katarzyna Smejda, Joanna Jerzyńska, Paweł Majak, Monika Białas, Iwona Stelmach
INTRODUCTION: Severity of lung disease varies in patients with the same CFTR genotype. It suggests that other factors affect the severity of cystic fibrosis (CF). The aim of the study was to identify risk factors that determine lung function decline in Polish cystic fibrosis children. MATERIAL AND METHODS: The follow-up time was no less than 5 years of respiratory status observation based on the forced expiratory volume in 1 s value (FEV1). The socio-economic data, perinatal interview, presence of meconium ileus (MI), time of CF diagnosis, initiation of tobramycin inhalation solution (TIS), pancreatic function, sensitization to Aspergillus fumigatus, presence of impaired glucose tolerance (IGT) or diabetes mellitus, chronic bacterial colonization and number of exacerbations and hospitalizations were assessed...
April 1, 2016: Archives of Medical Science: AMS
Chee Y Ooi, Christina Jeyaruban, Jasmine Lau, Tamarah Katz, Angela Matson, Scott C Bell, Susan E Adams, Usha Krishnan
AIMS: Distal intestinal obstruction syndrome (DIOS) and constipation in cystic fibrosis (CF) are conditions associated with impaction and/or obstruction by abnormally viscid mucofaecal material within the intestinal lumen. Dehydration has been proposed as a risk factor for DIOS and constipation in CF. The study primarily aimed to determine whether warmer ambient temperature and lower rainfall are risk factors for DIOS and constipation in CF. METHODS: Hospitalisations for DIOS (incomplete or complete) and/or constipation were retrospectively identified (2000-2012)...
April 2016: Journal of Paediatrics and Child Health
Stephanie Demeyer, Kris De Boeck, Peter Witters, Katrien Cosaert
UNLABELLED: Cystic fibrosis is a life shortening hereditary disease, primarily leading to progressive pulmonary infection and exocrine pancreatic dysfunction. Several gastrointestinal complications other than malabsorption can arise during the disease course and with the progressively increasing life span of patients with CF; new and more rare complications are being recognized. We review the literature on gastrointestinal manifestations in CF, excluding the liver and pancreas. CONCLUSION: We describe the clinical presentation and treatment of more common conditions like gastroesophageal reflux, small intestinal bacterial overgrowth, intussusception, meconium ileus, distal intestinal obstruction syndrome, and constipation, and we also discuss what is known on celiac disease, appendicitis, fibrosing colonopathy, inflammation and inflammatory bowel disease and gastrointestinal cancer...
July 2016: European Journal of Pediatrics
Anjali Verma, Kamal Nain Rattan, Ravi Yadav
INTRODUCTION: Neonatal intestinal obstruction is the most common surgical emergency in a newborn requiring prompt intervention. There are only very few studies done in world regarding pattern of neonatal intestinal obstruction. AIM: This study was conducted to see the aetiological spectrum of neonatal intestinal obstruction and to find out the problems and outcome of surgical management. MATERIALS AND METHODS: This study was done retrospectively to include all operated cases of neonatal intestinal obstruction from June 2000 to May 2015 and various factors affecting outcome were analysed...
February 2016: Journal of Clinical and Diagnostic Research: JCDR
Gordana Vilotijević-Dautović, Vesna Stojanović
INTRODUCTION: Pseudo-Bartter syndrome (PBS) is characterized by hyponatremic, hypochloremic metabolic alkalosis that mimics Bartter syndrome but with no pathology in the renal tubules. We present five patients with cystic fibrosis (CF) and PBS. CASES OUTLINE: Four children aged between three and five-and-one-half months with previously diagnosed CF and one aged 17 months with previously undiagnosed disease, were hospitalized during the summer season, with severe dehydration, oliguria, apathy and adynamia...
November 2015: Srpski Arhiv za Celokupno Lekarstvo
Anne Munck, Corinne Alberti, Carla Colombo, Nataliya Kashirskaya, Helmut Ellemunter, Maria Fotoulaki, Roderick Houwen, Eddy Robberecht, Priscilla Boizeau, Michael Wilschanski
BACKGROUND: Distal intestinal obstruction syndrome (DIOS) is a specific complication of cystic fibrosis. METHODS: A study was performed in 10 countries to prospectively evaluate the incidence, associated factors, and treatment modalities in children and adults. RESULTS: 102 patients presented 112 episodes. The incidence of DIOS was similar in children and adults. Medical treatment failed only in cases of complete DIOS (11%). Children with meconium ileus had a higher rate of surgery for DIOS (15% vs...
July 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
A G Carroll, R G Kavanagh, C Ni Leidhin, N M Cullinan, L P Lavelle, D E Malone
RATIONALE AND OBJECTIVES: The purpose of this study was to critically appraise and compare the diagnostic performance of imaging modalities that are used for the diagnosis of upper and lower gastrointestinal (GI) tract obstruction in neonates and infants. METHODS: A focused clinical question was constructed and the literature was searched using the patient, intervention, comparison, outcome method comparing radiography, upper GI contrast study, and ultrasound in the detection of upper GI tract obstruction such as duodenal atresia and stenosis, jejunal and ileal atresia, and malrotation and volvulus...
May 2016: Academic Radiology
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
Mercy Nkuba Nassali, Tadele Melese Benti, Moreri Bandani-Ntsabele, Elly Musinguzi
BACKGROUND: Despite advances in diagnostic imaging and focused antenatal care, cases of undiagnosed abdominal pregnancies at term are still reported in obstetric practice. It is atypical and very rare for a patient to be asymptomatic late in pregnancy and for the pregnancy to result in a live birth with no evidence of intrauterine growth restriction despite the unfavourable implantation site. This late term asymptomatic presentation despite routine antenatal care demonstrates a diagnostic challenge...
2016: BMC Research Notes
Antoine Guillon, Claire Chevaleyre, Celine Barc, Mustapha Berri, Hans Adriaensen, François Lecompte, Thierry Villemagne, Jérémy Pezant, Rémi Delaunay, Joseph Moënne-Loccoz, Patricia Berthon, Andrea Bähr, Eckhard Wolf, Nikolai Klymiuk, Sylvie Attucci, Reuben Ramphal, Pierre Sarradin, Dominique Buzoni-Gatel, Mustapha Si-Tahar, Ignacio Caballero
BACKGROUND: Cystic Fibrosis (CF) is the most prevalent autosomal recessive disease in the Caucasian population. A cystic fibrosis transmembrane conductance regulator knockout (CFTR-/-) pig that displays most of the features of the human CF disease has been recently developed. However, CFTR-/- pigs presents a 100% prevalence of meconium ileus that leads to death in the first hours after birth, requiring a rapid diagnosis and surgical intervention to relieve intestinal obstruction. Identification of CFTR-/- piglets is usually performed by PCR genotyping, a procedure that lasts between 4 to 6 h...
2015: PloS One
Hae-Young Kim, Soo-Hong Kim, Yong-Hoon Cho, Shin-Yun Byun, Young-Mi Han, Ah-Young Kim
PURPOSE: One of the major causes of bowel obstruction in extremely premature infants is a meconium obstruction. However, there are many challenges not only in the recognition and diagnosis, but also in the management of meconium obstruction. This study aimed to find perioperative clinical features and determine the postoperative course of meconium-related ileus in very low birth weight (VLBW) and extremely low birth weight (ELBW) infants. METHODS: We retrospectively reviewed the clinical data of premature infants (n = 11, VLBW infnats; n = 16, ELBW infants) with a meconium-related ileus who underwent operation for intractable ileus between January 2009 and May 2013...
September 2015: Annals of Surgical Treatment and Research
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