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Indian Journal of Pediatrics Sep 2019The bowel is a challenging abdominal organ to image. The main reason is the variable location, convoluted morphology and motility. A variety of bowel disorders such as... (Review)
Review
The bowel is a challenging abdominal organ to image. The main reason is the variable location, convoluted morphology and motility. A variety of bowel disorders such as congenital, developmental, inflammatory, infectious and neoplastic lesions can affect children and most of them are either unique to this age group or have a distinct clinico-radiological appearance compared to adults. Imaging plays a very important role in characterizing these lesions and further guiding the management. This is the first part of the series on imaging of bowel disorders in children. This article will cover the imaging modalities used for the evaluation of bowel and the imaging features of congenital /developmental disorders.
Topics: Abdominal Cavity; Anorectal Malformations; Child; Diagnostic Imaging; Duodenal Diseases; Duodenal Obstruction; Esophageal Atresia; Gastrointestinal Diseases; Humans; Intestinal Atresia; Magnetic Resonance Imaging; Meconium Ileus; Pediatrics; Pyloric Stenosis, Hypertrophic; Tomography, X-Ray Computed; Tracheoesophageal Fistula; Ultrasonography
PubMed: 30767163
DOI: 10.1007/s12098-019-02877-9 -
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 -
Clinical Radiology Mar 2021Term neonatal bowel obstruction is common, and absence of treatment is potentially catastrophic. There is a relatively narrow differential diagnosis, with causes... (Review)
Review
Term neonatal bowel obstruction is common, and absence of treatment is potentially catastrophic. There is a relatively narrow differential diagnosis, with causes categorised as either low or high bowel obstruction. The commonest causes of low bowel obstruction include anorectal malformations (ARM), Hirschsprung's disease, ileal atresia, meconium ileus, meconium plug, and colonic atresia. The commonest causes of high bowel obstruction include duodenal atresia, duodenal stenosis/web, jejunal atresia, and malrotation with volvulus (and hypertrophic pyloric stenosis usually presenting in slightly older infants). Diagnosis can be decided using a step-wise binary decision tool that includes the appropriate imaging steps and evaluation of bowel calibre. This paper presents the decision-making tool from the presenting features, through plain radiographic findings and, where necessary, the additional radiological investigations to assist the general radiologist, novice paediatric radiologist and paediatric surgeon. The tool is pictorial, with the radiological findings accompanied by eight schematics, serving as a simplified visual aid for memorizing the imaging patterns of the differential diagnosis. The imaging and decision-making steps allow for a rapid, simplified diagnosis that can benefit patients by recommending when to perform surgery, when to perform further imaging, and when imaging can act in a therapeutic manner.
Topics: Clinical Decision-Making; Diagnostic Imaging; Humans; Infant, Newborn; Intestinal Obstruction; Intestines
PubMed: 33097229
DOI: 10.1016/j.crad.2020.09.016 -
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 -
Expert Review of Respiratory Medicine 2023Gastrointestinal (GI)-related symptoms, complications, and comorbidities in cystic fibrosis (CF) are common and research to reduce their burden is a priority for the CF... (Review)
Review
INTRODUCTION
Gastrointestinal (GI)-related symptoms, complications, and comorbidities in cystic fibrosis (CF) are common and research to reduce their burden is a priority for the CF community. To enable future research, this review aimed to summarize the range of GI symptoms, complications and comorbidities seen in CF, the underlying pathophysiology, and treatments.
AREAS COVERED
This was a rapid systematic review undertaken using the recommendations from the Cochrane Rapid Reviews Methods Group. We searched databases including PubMed, Embase, Medline and the Cochrane database and identified those studies reporting GI-related symptoms, complications, or comorbidities in CF or their treatment. Our searches identified 2,930 studies and a total 119 studies met our inclusion criteria. Where a prevalence could be determined, GI symptoms were reported in 33.7% of study participants. The range of symptoms reported was broad and the highest median prevalence included flatulence (43.5%), bloating and abdominal distension (36%), and fatty stool (36%). Meconium ileus was reported in 12% and distal intestinal obstruction syndrome in 8.5.
EXPERT OPINION
GI-related symptoms, complications, and comorbidities in CF are common. More consistent characterization and recording of these symptoms in clinical studies may help achieve the priority of reducing the burden of GI disease in CF.
Topics: Humans; Cystic Fibrosis; Intestinal Obstruction; Comorbidity; Prevalence
PubMed: 37345513
DOI: 10.1080/17476348.2023.2228194 -
Journal of Pediatric Surgery Jul 2021therapeutic strategy for meconium-related ileus (MRI) in very-low-birth-weight infants (VLBWs) has not been established. This study aims to clarify the optimum...
BACKGROUND/PURPOSE
therapeutic strategy for meconium-related ileus (MRI) in very-low-birth-weight infants (VLBWs) has not been established. This study aims to clarify the optimum therapeutic strategy for MRI in VLBWs.
METHODS
MRI was defined as delayed meconium excretion and microcolon on contrast enema with Gastrografin (diatrizoate acid). Forty-two infants with MRI were treated at our institution between 2009 and 2019, and are reviewed here. They were classified into two groups: in group A (n=21), Gastrografin regurgitated into the dilated intestine during the first or second round of Gastrografin enema (GaE), while in group B (N = 21), Gastrografin did not regurgitate. Laparotomy was indicated if the intestine was perforated, or if abdominal distention was not relieved by two rounds of GaE.
RESULTS
in group A, meconium was excreted in all cases within 24 h after GaE, and no cases required laparotomy. In group B, twelve cases (57%) underwent laparotomy (P < 0.01), six cases in this group (29%), showed free air on X-ray images (P < 0.01). The median hospital stay in groups A and B were 89.0 and 136.5 days, respectively (P < 0.05). Overall mortality was 2.4%.
CONCLUSIONS
early therapeutic diagnosis by GaE followed by early surgery is suggested as the optimum strategy for MRI in VLBWs.
Topics: Diatrizoate Meglumine; Enema; Humans; Ileus; Infant, Newborn; Infant, Very Low Birth Weight; Intestinal Obstruction; Meconium; Meconium Ileus
PubMed: 33896618
DOI: 10.1016/j.jpedsurg.2021.03.029 -
Pediatric Pulmonology May 2022Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established...
BACKGROUND
Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease.
METHODS
Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data.
RESULTS
There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively).
CONCLUSIONS
The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.
Topics: Bartter Syndrome; Cystic Fibrosis; Cystic Fibrosis Transmembrane Conductance Regulator; Female; Humans; Infant, Newborn; Male; Patient Care; Registries; Turkey
PubMed: 35102722
DOI: 10.1002/ppul.25852