Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises

Mentessidou, Anastasia; Loukou, Ioanna; Καμπούρογλου, Γεώργιος; Livani, Anastasia; Georgopoulos, Ioannis; Mirilas, Petros

doi:10.1016/j.jpedsurg.2017.11.040

Cited by 15 publications

(11 citation statements)

References 19 publications

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“…According to the literature, anastomotic leakage following BK formation occurs in up to 5% of patients. [17,20,21] In contrast, prior studies demonstrate a variable rate of anastomotic complications following PA if created without protective ostomy ranging from 15% to 30% for MI, [1,2,24,25] from 6% to 10% for CIA, [26–28] and from 5% to 12% for NEC. [5,7,10,29] We hypothesize that the lower rate of anastomotic complications in BK compared to PA is due to presence of a diverting ostomy, which serves as a vent and thereby protects the anastomosis.…”

Section: Discussionmentioning

confidence: 99%

The outcome of Bishop-Koop procedure compared to divided stoma in neonates with meconium ileus, congenital intestinal atresia and necrotizing enterocolitis

et al. 2019

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To determine the potential value and suitability of Bishop-Koop procedure (BK) compared to divided stoma (DS) in neonates with meconium ileus (MI), congenital intestinal atresia (CIA), and necrotizing enterocolitis (NEC). A retrospective data collection from 2000 to 2019 on neonates undergoing BK and DS formation and closure for MI, CIA, and NEC was conducted. Ostomy related complications following both procedures were analyzed. One hundred two consecutive patients managed with a BK (n = 57, 55.8%) and DS (n = 45, 44.2%) for MI (n = 38, 37.2%), CIA (n = 31, 30.5%), and NEC (n = 33, 32.3%) were analyzed. Mean operating time for ostomy creation did not differ significantly between BK and DS groups (156 ± 54 vs 135 ± 66.8 min, P = .08). The prevalence of stoma-related complications following BK and DS formation was 8.7% and 31.1%, respectively ( P = .005). The complication rate after BK and DS closure was 3.5% and 6.7%, respectively ( P = .65). The operating time for ostomy reversal and length of hospital stay after stoma closure were significantly shorter in BK group (82.2 ± 51.4 vs 183 ± 84.5 min and 5.5 ± 2.7 vs 11.3 ± 3.9 days, P < .001). BK procedure is safe, reliable, and suitable technique in neonatal surgery with low complications rate following ostomy creation as well as shorter operating time and length of hospital stay after ostomy closure compared to DS ostomies. Surgeons should keep this technique as an alternative approach in their repertoire.

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Section: Discussionmentioning

confidence: 99%

The outcome of Bishop-Koop procedure compared to divided stoma in neonates with meconium ileus, congenital intestinal atresia and necrotizing enterocolitis

et al. 2019

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“…The addition of PEG as a dietary supplement improved life expectancy to 70% (P = 0.1573 Figure 1F). Together, CFTR-G542X knockin rats exhibited developmental abnormalities, retarded growth, and intestinal obstruction as observed in established CFTR knockout rats (Tuggle et al, 2014) and human subjects (Ferrazzano et al, 2012;Mentessidou et al, 2018).…”

Section: Cftr G542x Knockin Rats Exhibit Developmental Defectsmentioning

confidence: 90%

A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay

et al. 2020

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Nonsense mutations that lead to the insertion of a premature termination codon (PTC) in the cystic fibrosis transmembrane conductance regulator (CFTR) transcript affect 11% of patients with cystic fibrosis (CF) worldwide and are associated with severe disease phenotype. While CF rat models have contributed significantly to our understanding of CF disease pathogenesis, there are currently no rat models available for studying CF nonsense mutations. Here we created and characterized the first homozygous CF rat model that bears the CFTR G542X nonsense mutation in the endogenous locus using CRISPR/Cas9 gene editing. In addition to displaying severe CF manifestations and developmental defects such as reduced growth, abnormal tooth enamel, and intestinal obstruction, CFTR G542X knockin rats demonstrated an absence of CFTR function in tracheal and intestinal sections as assessed by nasal potential difference and transepithelial short-circuit current measurements. Reduced CFTR mRNA levels in the model further suggested sensitivity to nonsense-mediated decay, a pathway elicited by the presence of PTCs that degrades the PTC-bearing transcripts and thus further diminishes the level of CFTR protein. Although functional restoration of CFTR was observed in G542X rat tracheal epithelial cells in response to single readthrough agent therapy, therapeutic efficacy was not observed in G542X knockin rats in vivo. The G542X rat model provides an invaluable tool for the identification and in vivo validation of potential therapies for CFTR nonsense mutations.

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“…The most commonly prescribed is Polyethylene Glycol (PEG) administered at a dose of 20-40 ml/Kg/H, up to a maximum of 1 L/h for a total of 8 hours, achieving fecal effluent consisting of clear fluid along with resolution of abdominal pain and constipation [ 1 , 6 ]. If the diagnosis remains unclear and thus requires surgical intervention, ileocecal valve resection should be considered to prevent the recurrence of intestinal obstruction sequalae and growth, especially in adolescents [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Acute Appendicitis Masquerading Distal Intestinal Obstruction Syndrome in Adult Cystic Fibrosis

Nanavati

Patel

Melki

et al. 2018

Case Reports in Gastrointestinal Medicine

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Overshadowed by Sino-pulmonary infections, Cystic Fibrosis (CF) commonly affects gastrointestinal organs because of secretory and motility dysfunction. Infrequently, these changes result in Distal Intestinal Obstruction Syndrome (DIOS), an increasingly diagnosed gastrointestinal entity in adult Cystic Fibrosis patients. We present a case 22-year-old male who presented to our hospital with right lower quadrant abdominal pain with suspicion of acute appendicitis and was subsequently diagnosed as DIOS. Our case highlights the importance of DIOS as one of the differential diagnosis of right lower quadrant abdominal pain in a patient with a CF, especially for physicians working at community hospitals which may not have a Cystic Fibrosis care program available.

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Long-term intestinal obstruction sequelae and growth in children with cystic fibrosis operated for meconium ileus: expectancies and surprises

Cited by 15 publications

References 19 publications

The outcome of Bishop-Koop procedure compared to divided stoma in neonates with meconium ileus, congenital intestinal atresia and necrotizing enterocolitis

The outcome of Bishop-Koop procedure compared to divided stoma in neonates with meconium ileus, congenital intestinal atresia and necrotizing enterocolitis

A Novel G542X CFTR Rat Model of Cystic Fibrosis Is Sensitive to Nonsense Mediated Decay

Acute Appendicitis Masquerading Distal Intestinal Obstruction Syndrome in Adult Cystic Fibrosis

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