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Korean Journal of Radiology Jan 2022Gastrointestinal (GI) emergencies in neonates and infants encompass from the beginning to the end of the GI tract. Both congenital and acquired conditions can cause... (Review)
Review
Gastrointestinal (GI) emergencies in neonates and infants encompass from the beginning to the end of the GI tract. Both congenital and acquired conditions can cause various GI emergencies in neonates and infants. Given the overlapping or nonspecific clinical findings of many different neonatal and infantile GI emergencies and the unique characteristics of this age group, appropriate imaging is key to accurate and timely diagnosis while avoiding unnecessary radiation hazard and medical costs. In this paper, we discuss the radiological findings of essential neonatal and infantile GI emergencies, including esophageal atresia and tracheoesophageal fistula, hypertrophic pyloric stenosis, duodenal atresia, malrotation, midgut volvulus for upper GI emergencies, and jejunoileal atresia, meconium ileus, meconium plug syndrome, meconium peritonitis, Hirschsprung disease, anorectal malformation, necrotizing enterocolitis, and intussusception for lower GI emergencies.
Topics: Diagnostic Imaging; Duodenal Obstruction; Humans; Infant; Infant, Newborn; Intestinal Atresia; Intussusception
PubMed: 34983099
DOI: 10.3348/kjr.2021.0111 -
Channels (Austin, Tex.) Dec 2023SLC26A9 is one out of 11 proteins that belong to the SLC26A family of anion transporters. Apart from expression in the gastrointestinal tract, SLC26A9 is also found in... (Review)
Review
SLC26A9 is one out of 11 proteins that belong to the SLC26A family of anion transporters. Apart from expression in the gastrointestinal tract, SLC26A9 is also found in the respiratory system, in male tissues and in the skin. SLC26A9 has gained attention because of its modifier role in the gastrointestinal manifestation of cystic fibrosis (CF). SLC26A9 appears to have an impact on the extent of intestinal obstruction caused by meconium ileus. SLC26A9 supports duodenal bicarbonate secretion, but was assumed to provide a basal Cl- secretory pathway in airways. However, recent results show that basal airway Cl- secretion is due to cystic fibrosis conductance regulator (CFTR), while SLC26A9 may rather secrete HCO-, thereby maintaining proper airway surface liquid (ASL) pH. Moreover, SLC26A9 does not secrete but probably supports reabsorption of fluid particularly in the alveolar space, which explains early death by neonatal distress in Slc26a9-knockout animals. While the novel SLC26A9 inhibitor S9-A13 helped to unmask the role of SLC26A9 in the airways, it also provided evidence for an additional role in acid secretion by gastric parietal cells. Here we discuss recent data on the function of SLC26A9 in airways and gut, and how S9-A13 may be useful in unraveling the physiological role of SLC26A9.
Topics: Animals; Biological Transport; Cystic Fibrosis; Intestines; Respiratory System; Sulfate Transporters; Antiporters
PubMed: 36866602
DOI: 10.1080/19336950.2023.2186434 -
World Journal of Gastroenterology Sep 2023Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious... (Review)
Review
Delayed passage of meconium or constipation during the perinatal period is traditionally regarded as a signal to initiate further work up to evaluate for serious diagnoses such as Hirschsprung's disease (HD), meconium ileus due to Cystic Fibrosis, The diagnosis of HD particularly warrants invasive testing to confirm the diagnosis, such as anorectal manometry or rectal suction biopsy. What if there was another etiology of perinatal constipation, that is far lesser known? Cow's milk protein allergy (CMPA) is often diagnosed in infants within the first few weeks of life, however, there are studies that show that the CMPA allergen can be passed from mother to an infant in-utero, therefore allowing symptoms to show as early as day one of life. The presentation is more atypical, with perinatal constipation rather than with bloody stools, diarrhea, and vomiting. The diagnosis and management would be avoidance of cow's milk protein within the diet, with results and symptom improvement in patients immediately. Therefore, we discuss whether an alternative pathway to address perinatal constipation should be further discussed and implemented to potentially avoid invasive techniques in patients. This entails first ruling out CMPA with safe, noninvasive techniques with diet modification, and if unsuccessful, then moving forward with further diagnostic modalities.
Topics: Animals; Cattle; Female; Infant; Pregnancy; Humans; Milk Hypersensitivity; Constipation; Biopsy; Diarrhea; Hirschsprung Disease
PubMed: 37731998
DOI: 10.3748/wjg.v29.i33.4920 -
The Journal of Pediatric Pharmacology... 2019With the improving life expectancy of cystic fibrosis patients, new manifestations of the disease are emerging. Distal intestinal obstruction syndrome is one of the... (Review)
Review
With the improving life expectancy of cystic fibrosis patients, new manifestations of the disease are emerging. Distal intestinal obstruction syndrome is one of the increasingly noted complications. Traditionally this syndrome was treated surgically. -acetylcysteine is sometimes used as a non-surgical treatment option despite lack of definitive evidence for its efficacy and safety and not being mentioned in current treatment guidelines. The existing case reports suggest that -acetylcysteine may have a place in therapy for older patients with incomplete distal intestinal obstruction syndrome to relieve the initial obstruction or following disimpaction to ensure clearance of remaining ileus and to prevent obstruction recurrence. In younger patients (e.g., <3 years of age), efficacy of -acetylcysteine has been controversial and its use has been associated with drug-induced liver injury and hypernatremia. In the cases included in this review, 4% -acetylcysteine was the formulation most commonly used. Since higher concentrations have been associated with increased adverse effects and mucosal injury, lower concentrations and dosages should be used when using -acetylcysteine until further evidence becomes available. Proper administration technique and monitoring parameters are not well defined in current literature. Prospective, well-designed clinical trials are lacking and would be helpful to better define the role of -acetylcysteine in distal intestinal obstruction syndrome.
PubMed: 31598102
DOI: 10.5863/1551-6776-24.5.390 -
Journal of Cystic Fibrosis : Official... Nov 2019
Topics: Cohort Studies; Correlation of Data; Cystic Fibrosis; Delayed Diagnosis; Humans; Infant, Newborn; Meconium Ileus; Mortality; Patient Care Management; Prognosis; Severity of Illness Index
PubMed: 31668933
DOI: 10.1016/j.jcf.2019.10.003 -
PloS One 2022Meconium-related ileus in very low birth weight infants can lead to increased morbidity or mortality and prolonged hospitalization without prompt diagnosis and...
BACKGROUND
Meconium-related ileus in very low birth weight infants can lead to increased morbidity or mortality and prolonged hospitalization without prompt diagnosis and treatment. This study primarily aimed to identify the incidence of and factors associated with meconium-related ileus and secondarily sought to investigate clinical and growth outcomes after water-soluble contrast media (Gastrografin) enema.
METHODS
We retrospectively reviewed medical records of very low birth weight infants born between February 2009 and March 2019 in the neonatal intensive care unit of a single medical center. Perinatal factors, clinical outcomes, and growth outcomes were compared between the group with meconium-related ileus that received Gastrografin enema and the control group.
RESULTS
Twenty-four (6.9%) patients were diagnosed with meconium-related ileus among 347 very low birth weight infants. All achieved successful evacuation of meconium with an average of 2.8 (range: 1-8) Gastrografin enema attempts without procedure-related complications. Initiation of Gastrografin enema was performed at mean 7.0 days (range: 2-16) after birth. Incidences of moderate to severe bronchopulmonary dysplasia were higher and the duration of mechanical ventilation and need for oxygen were longer in the meconium-related ileus group (P = 0.039, 0.046, 0.048, respectively). Meconium-related ileus infants took more time to start enteral feeding and the nothing per oral time was longer (P = 0.001 and 0.018, respectively). However, time to achieve full enteral feeding and Z-scores for weight and height at 37 weeks and at 6 months corrected age did not differ between the two groups.
CONCLUSIONS
Gastrografin enema in very low birth weight infants with meconium-related ileus was an effective and safe medical management. Following Gastrografin enema, very low birth weight infants with meconium-related ileus achieved similar subsequent feeding progress and similar growth levels as the control groups without meconium-related ileus.
Topics: Diatrizoate Meglumine; Enema; Humans; Ileus; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Retrospective Studies
PubMed: 35951504
DOI: 10.1371/journal.pone.0272915 -
Cureus Mar 2022Background Anatomical abnormalities leading to bowel movement failure are the major cause of intestinal obstruction. This study was done to assess the diagnostic...
Background Anatomical abnormalities leading to bowel movement failure are the major cause of intestinal obstruction. This study was done to assess the diagnostic efficacy of contrast enema in neonates with lower intestinal obstruction. Methodology This prospective study was conducted in The Children's Hospital and University of Child Health Sciences, Lahore from February 2021 to July 2021. Patients presenting with constipation, abdominal distension, vomiting, and unable to pass meconium, evaluated clinically and by X-rays, were included in the study. Patients with lower intestinal obstruction (i.e., meconium ileus, Hirschsprung's disease, ileal atresia, meconium plug syndrome, and small left colon) were given contrast enema (gastrografin) after hydration. The radiologist was blinded by the clinical diagnosis or reference standard diagnosis, which was labeled prior to image interpretation. Sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated for major contrast enema diagnosis. Results During this period, a total of 34 patients were included in the study. The mean age of presentation was 72.0 ± 24 hours. There were 21 (61.8%) boys and 13 (38.2%) girls, representing a male to female ratio of 1.7:1. In four cases, our diagnosis on the basis of contrast enema was proven wrong. Sensitivity, specificity, PPV, and NPV of Hirschsprung's disease were found to be 93.3%, 50%, 87.5%, and 66.7%, while these were 84.6%, 66.7%, 91.7%, and 50.0%, respectively, for meconium ileus. Conclusion The diagnostic efficacy of contrast enema was found to be good in neonates with lower intestinal obstruction. The spectrum of intestinal obstruction among neonates shows diversity while contrast enema seems to play a major role in the identification and remodeling of the diagnostic plan in a major proportion of cases.
PubMed: 35495017
DOI: 10.7759/cureus.23458 -
International Journal of Neonatal... Jun 2020Screening metrics are essential to both quality assessment and improvement, but are highly dependent on the way positive tests and cases are counted. In cystic fibrosis... (Review)
Review
Screening metrics are essential to both quality assessment and improvement, but are highly dependent on the way positive tests and cases are counted. In cystic fibrosis (CF) screening, key factors include how mild cases of late-presenting CF and CF screen positive, inconclusive diagnosis (CFSPID) are counted, whether those at prior increased risk of CF are excluded from the screened population, and which aspects of the screening pathway are considered. This paper draws on the New Zealand experience of almost forty years of newborn screening for CF. We demonstrate how different definitions impact the calculation of screening sensitivity. We suggest that, to enable meaningful comparison, CF screening reports should clarify what steps in the screening pathway are included in the assessment, as well as the algorithm used and screening target.
PubMed: 33073037
DOI: 10.3390/ijns6020047