Meconium Ileus: A Ten-Year Review of Thirty-Six Patients

Murshed, R.; Spitz, L; Kiely, Edward M.; Drake, D G

doi:10.1055/s-2008-1071170

Cited by 28 publications

(12 citation statements)

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“…Treatments for the management of meconium ileus in cystic fibrosis were introduced to treat MO of prematurity [14] , and this approach has been reported to be successful [3,7] . In our unit, medical management was successful in 77% of patients described here.…”

Section: Discussionmentioning

confidence: 99%

Recognition, Diagnosis and Treatment of Meconium Obstruction in Extremely Low Birth Weight Infants

Kim

et al. 2011

Neonatology

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Background: Meconium obstruction (MO) of prematurity can result in increased morbidity or mortality and prolonged hospitalization if not diagnosed and treated appropriately. Objectives: The aims of our study were to identify the incidence and risk factors associated with MO and to review the treatment outcomes. Methods: A retrospective analysis was undertaken of 101 extremely low birth weight infants (ELBWIs) who were born between January 1, 2007, and April 1, 2009, at Seoul National University Hospital. Prenatal and neonatal factors were compared between the MO and control groups. The treatment outcomes were also reviewed. Results: Twenty-two (22%) patients were diagnosed as having MO. Eighteen of these patients (82%) had prenatal risk factors for MO. Respiratory distress syndrome was more prevalent in the MO group than in the control group (p = 0.001). Overall, 17 of the 22 patients (77%) were relieved with medical treatment and the rest underwent ileostomy. The times to full enteral feeding did not differ between the medically treated group and the control group. However, the surgically treated group required more time to achieve full enteral feeding, and some patients had persistent gastrointestinal problems. Conclusion: MO is not a rare condition in ELBWIs, and the majority of ELBWIs have prenatal risk factors. Medical management was effective, and medically manageable cases had good prognoses for subsequent feeding, whereas some surgically managed cases had persistent gastrointestinal problems.

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Section: Discussionmentioning

confidence: 99%

Recognition, Diagnosis and Treatment of Meconium Obstruction in Extremely Low Birth Weight Infants

Kim

et al. 2011

Neonatology

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“…It has been estimated to occur with an incidence of 6.2 episodes per 1000 patient-years and is usually treated conservatively. In CF pigs, intestinal disease resembles meconium ileus in humans with development in utero, occasional intestinal perforation prior to birth, and atretic bowel distal to the obstruction (7,(14)(15)(16). In addition, after the meconium ileus has been surgically bypassed, CF pigs may also develop a DIOS-like syndrome (14).…”

Section: Cftr Expression In the Intestine Is Sufficient To Rescue Mecmentioning

confidence: 99%

“…In addition, up to 20% of babies with CF are born with meconium ileus (4,5). Intestinal obstruction most commonly occurs in the distal ileum to proximal colon and often requires surgical intervention to relieve the obstruction (6,7). The pathogenesis of meconium ileus remains uncertain, but may result from loss of CFTR-mediated Cl − and/or HCO 3 − transport by the intestinal epithelium and/or from pancreatic dysfunction (8)(9)(10)(11)(12).…”

Section: Introductionmentioning

confidence: 99%

Intestinal CFTR expression alleviates meconium ileus in cystic fibrosis pigs

Stoltz¹,

Rokhlina²,

Ernst³

et al. 2013

J. Clin. Invest.

115

127

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Cystic fibrosis (CF) pigs develop disease with features remarkably similar to those in people with CF, including exocrine pancreatic destruction, focal biliary cirrhosis, micro-gallbladder, vas deferens loss, airway disease, and meconium ileus. Whereas meconium ileus occurs in 15% of babies with CF, the penetrance is 100% in newborn CF pigs. We hypothesized that transgenic expression of porcine CF transmembrane conductance regulator (pCFTR) cDNA under control of the intestinal fatty acid-binding protein (iFABP) promoter would alleviate the meconium ileus. We produced 5 CFTR -/-;TgFABP>pCFTR lines. In 3 lines, intestinal expression of CFTR at least partially restored CFTR-mediated anion transport and improved the intestinal phenotype. In contrast, these pigs still had pancreatic destruction, liver disease, and reduced weight gain, and within weeks of birth, they developed sinus and lung disease, the severity of which varied over time. These data indicate that expressing CFTR in intestine without pancreatic or hepatic correction is sufficient to rescue meconium ileus. Comparing CFTR expression in different lines revealed that approximately 20% of wild-type CFTR mRNA largely prevented meconium ileus. This model may be of value for understanding CF pathophysiology and testing new preventions and therapies.

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“…Volvulus is not uncommon in CF with complicated meconium ileus, presenting alone or in association with intestinal atresia in 17 to 35% of all cases with meconium ileus. 10,11 It has been suggested that volvulus in CF results from hyperperistaltic twisting of the proximal bowel in an attempt to overcome distal obstruction caused by inspissated meconium. 12 Case reports suggest that midgut volvuli presenting late in pregnancy bear a significant risk of intrauterine mortality.…”

Section: Discussionmentioning

confidence: 99%