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Meconium Ileus (meconium + ileus)
Selected AbstractsA NOVEL WAY TO DIAGNOSE CYSTIC FIBROSIS IN THE NEONATE WITH A BOWEL OBSTRUCTION AND POSSIBLE MECONIUM ILEUSJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 9 2003V Sung No abstract is available for this article. [source] Meconium ileus,it is time to act now!PEDIATRIC PULMONOLOGY, Issue 10 2010Matthias Kappler MD Meconium ileus is a life-threatening presentation of neonates with cystic fibrosis (CF). Notwithstanding, today the long-term prognosis of such patients is comparable to that of CF patients not diagnosed in screening programs and not suffering from this insult,1,3 as confirmed by the article of Johnson et al. in this issue of Pediatric Pulmonology. Good news then for CF patients and CF caretakers, working with the modern interdisciplinary setting of neonatal intensive care, including radiology, anesthesiology, and pediatric surgery experts. State of the art management of life-threatening neonatal ileus during this fragile early phase of life obviously balances all disadvantages associated with neonatal ileus and provides an outcome not different from that in CF patients without meconium ileus, but diagnosed later during early childhood. Pediatr Pulmonol. 2010; 45:949,950. © 2010 Wiley-Liss, Inc. [source] Abdominal manifestations of cystic fibrosis in adults: A reviewJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 4 2004S Constantine Summary Gastrointestinal manifestations of disease are present in most adults with cystic fibrosis. Radiologists are familiar with the classical imaging characteristics of end-stage pulmonary disease and the radiological findings of meconium ileus in neonates. As most patients now live into adulthood, recognition of the imaging appearances of abdominal disease is important to enable prompt diagnosis and treatment. Accordingly, this article presents typical imaging appearances of the adult gastrointestinal manifestations of cystic fibrosis. [source] Bilious vomiting in the newborn: 6 years data from a Level III CentreJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 5 2010Atul Malhotra Background: Bilious vomiting in the newborn is an urgent condition that frequently requires neonatal and paediatric surgical involvement. Investigations involve abdominal X-ray and contrast imaging in most cases. We aimed to describe the prevalence of surgical intervention in this cohort and assess the reliability of contrast imaging in accurate prediction of underlying condition. Methods: A retrospective audit of data from December 2001 to October 2007 was undertaken. Data on newborns admitted to a level III unit with bilious vomiting was extracted. Infants with bilious aspirates but no vomiting were excluded. Results: Sixty-one infants were admitted to the unit during the period with bilious vomiting. Most of them were out born (83.6%). Mean (and standard deviation) gestation was 38.3 weeks (±3.2); weight was 3173.5 grams (±717.6); day of admission was 3.68 days (1,28); and length of stay in the unit was 9.96 days (1,48). There were 52 (85.2%) abnormal X-rays and 21 (34.4%) abnormal contrast studies. Sixteen (26.6%) babies had laparotomies of which 6 were malrotations with volvulus, 2 small bowel obstructions, 2 meconium ileus, 2 Hirschsprung's disease, 2 other findings, while 2 were normal. Positive predictive value (number of accurate predictions of surgical findings) for barium contrast studies was 85.7% in this series. Conclusion: Bile stained vomiting is a surgical emergency and prompt investigation is the key in the management. Contrast studies still form the backbone of such investigations. [source] Meconium ileus,it is time to act now!PEDIATRIC PULMONOLOGY, Issue 10 2010Matthias Kappler MD Meconium ileus is a life-threatening presentation of neonates with cystic fibrosis (CF). Notwithstanding, today the long-term prognosis of such patients is comparable to that of CF patients not diagnosed in screening programs and not suffering from this insult,1,3 as confirmed by the article of Johnson et al. in this issue of Pediatric Pulmonology. Good news then for CF patients and CF caretakers, working with the modern interdisciplinary setting of neonatal intensive care, including radiology, anesthesiology, and pediatric surgery experts. State of the art management of life-threatening neonatal ileus during this fragile early phase of life obviously balances all disadvantages associated with neonatal ileus and provides an outcome not different from that in CF patients without meconium ileus, but diagnosed later during early childhood. Pediatr Pulmonol. 2010; 45:949,950. © 2010 Wiley-Liss, Inc. [source] Does presenting with meconium ileus affect the prognosis of children with cystic fibrosis?PEDIATRIC PULMONOLOGY, Issue 10 2010Jo-Anne Johnson MBChB Abstract It is a matter of debate as to what extent the long-term outcome of cystic fibrosis (CF) is affected by presenting with meconium ileus (MI). We compared long-term clinical outcomes of CF children who presented with MI, to those presenting with other symptoms (non-MI) in an era of non new-born-screening (NBS). We collected annual lung function data between the ages of 8,15 years in terms of percent predicted first second forced expired volume (FEV1%pr), percent predicted forced vital capacity (FVC%pr), and between the ages of 2,15 years annual height and weight Z-scores (HtZ and WtZ respectively) for children attending the Royal Brompton Hospital CF clinic. To be included in the study, subjects had to have at least five pulmonary function tests and five anthropometric measurements recorded over this period. Thirty-eight MI and 76 non-MI subjects were compared. There were no significant differences in genotype, sex, chronic Pseudomonas infection, or pancreatic enzyme use between the two groups. The median age of diagnosis was 1 day (MI) versus 7 months (non-MI). There was a decline in spirometry and anthropometric variables over the study period for both MI and non-MI groups apart from WtZ score in the non-MI group. Mixed model analysis adjusting for potential confounders including genotype, pancreatic status, sex, chronic Pseudomonas aeruginosa lung infection, and age of diagnosis revealed no difference between the two groups in terms of lung function and growth during the time period of the study, however there was a non-significant trend for subjects presenting with MI to do better in all four parameters. We conclude that babies presenting with MI have no worse long-term outcome than those presenting symptomatically later in infancy, despite having undergone invasive procedures in the newborn period. This underscores the importance of early diagnosis and treatment in CF. Pediatr Pulmonol. 2010; 45:951,958. © 2010 Wiley-Liss, Inc. [source] Longitudinal pulmonary status of cystic fibrosis children with meconium ileusPEDIATRIC PULMONOLOGY, Issue 4 2004Zhanhai Li PhD Abstract Although meconium ileus (MI) is the earliest manifestation of cystic fibrosis (CF), and is associated with poorer growth, the longitudinal pulmonary progression of CF children with MI is not clear. To test the hypothesis that MI is associated with worse pulmonary outcomes, we prospectively compared from diagnosis to 12 years of age 32 CF children with MI to 50 CF children without MI who were diagnosed during early infancy through neonatal screening. Pulmonary outcome measures included respiratory symptoms, respiratory infections, pathogens, antibiotic usage, hospitalizations, quantitative chest radiology, spirometry, and lung volume determinations. Obstructive lung disease was defined as percent predicted spirometry values below the lower limits of normal. Longitudinal analyses revealed no significant differences in cough, wheezing, respiratory infections, prevalence of and median times to acquisition of Pseudomonas aeruginosa or Staphylococcus aureus, antibiotic usage, and chest radiograph scores between the two groups. However, MI children showed significantly worse forced expiratory volume in 1 sec (FEV1), forced vital capacity (FVC), forced expiratory flow between 25,75% of FVC (FEF25,75), % predicted FEV1, % predicted FEF25,75, and total lung capacity (TLC). These differences were particularly apparent beginning at age 8,10 years. MI children also had higher rates of and shorter median times to obstructive lung disease. Subgroup analyses showed MI children treated surgically and those treated medically had similar pulmonary outcomes. In conclusion, MI children have worse lung function and more obstructive lung disease than those without MI. Such abnormalities are accompanied by reduced lung volume. MI is a distinct CF phenotype with more severe pulmonary dysfunction. Pediatr Pulmonol. 2004; 38:277,284. © 2004 Wiley-Liss, Inc. [source] |