Congenital intestinal atresias with multiple episodes of sepsis: A case report and review of literature.

INTRODUCTION: Hereditary multiple intestinal atresia associated with severe combined immunodeficiency (MIA-SCID) is a very rare disease caused by deleterious mutations in the tetratricopeptide repeat domain-containing protein 7A gene TTC7A. It is characterized by intestinal obstruction, sepsis, and a poor prognosis. Insights into phenotype-genotype correlations could help to guide genetic counseling and increase our knowledge of the natural history of this disease. CASE PRESENTATION: We report the case of a newborn in which his fetal magnetic resonance imaging showed jejunal atresia and microcolon and an abdominal x-ray at birth confirmed intestinal obstruction. The clinical course was complicated by multiple episodes of sepsis, and laboratory investigations showed SCID. The genetic analysis identified a homozygous c.53344_53347 mutation in the TTC7A gene compatible with MIA-SCID syndrome. The patient required 3 operations because of new intestinal atresias in the first months of life. She underwent bone marrow transplantation at 8 months of age but died of liver failure secondary to graft-versus-host disease. CONCLUSION: Immunologic assessment and genetic screening for TTC7A mutations are important in patients with MIA. Greater knowledge of the functions of the TTC7A protein will have important therapeutic implications for patients with MIA-SCID syndrome.

Impact of bacterial translocation in calves with atresia coli.

OBJECTIVE: To identify whether enteric bacteria pass into the mesenteric lymph nodes (MLNs) and peritoneal cavity in calves with atresia coli and to evaluate whether the presence of bacterial translocation (BT) has an impact on the success of surgical treatment. DESIGN: Prospective clinical study. ANIMALS: Twenty-six client-owned calves. INTERVENTIONS: During laparotomy, swab samples were collected from the peritoneal cavity and MLNs using a sterile swab stick and were submitted for microbiological analysis. MEASUREMENTS AND MAIN RESULTS: Bacterial cultures of swab samples revealed that 65% (n = 17) of the calves experienced BT. Of these, 14 calves experienced BT to the MLNs, 9 to the peritoneal cavity, and 5 to both regions. Of the bacteria isolated from the MLNs, 72% (n = 10) were Escherichia coli. Of the samples isolated from the peritoneal fluid, 33% (n = 3) contained E. coli and 33% (n = 3) contained E. coli + coagulase-negative Staphylococcus (CNS). In calves with BT that were discharged (n = 13) and without BT that were discharged (n = 7), the median survival was 30 days; these data were found to be similar in the 2 groups. CONCLUSIONS: This study revealed that BT is observed in the majority of atresia coli cases. E. coli is more common in BT, and translocation occurs primarily through the lymphatic route. These results suggest that the presence of BT is closely related to the success of the operation for correction of atresia coli.

Immunoregulation effects of different γδT cells and toll-like receptor signaling pathways in neonatal necrotizing enterocolitis.

The aim of the study was to observe cytokine and T-cell-related toll-like-receptor (TLR) changes in intestinal samples of neonatal necrotizing enterocolitis patients.Four necrotic bowels were collected from neonatal NEC patients with gestational ages of 28 to 29 weeks in our hospital, whereas 4 neonatal patients who underwent intestinal atresia surgery served as the controls. Intestinal flora was examined and IL-1, IL-2, IL-4, IL-6, IL-8, IL-10, TNF-α, IFN-γ, and IL-17 expressions in resected intestine samples, as well as in isolated gamma delta T (γδT) cells, were analyzed immunohistochemically and via quantitative RT-PCR. γδT cells were isolated from the intestinal intraepithelial lymphocytes (IELs) and their TLR4/TLR9 distribution in the intestinal tissues was determined by flow cytometry.The bacterial flora of the neonatal NEC patients' contained significantly higher amounts of Gram-negative Enterobacteriaceae, Klebsiella, and Bacteroides but anaerobic Gram-positive Bifidobacteria occurred significantly less in the NEC than the control group. IL-1, IL-2, IL-4, IL-6, IL-8, IL-10, TNF-α, IFN-γ, and IL-17 expressions in the resected intestine samples and in isolated γδT cells were enhanced in NEC samples compared to the controls. γδT cells were less prevalent in NEC-derived intestinal tissues, but their TLR4/TLR9 expressions were significantly enhanced.The changed bacterial flora in preterm neonatal NEC patients led to an obvious inflammation of the intestines, which was accompanied by reductions of γδT cell localizations to the intestine and a shift of their surface expressions to TLR4 and TLR9.

The pediatric intestinal mucosal microbiome remains altered after clinical resolution of inflammatory and ischemic disease.

BACKGROUND: The pediatric intestinal microbiome is impacted by many factors, including age, diet, antibiotics, and environment. We hypothesized that in operative patients, alterations to antibiotics and mechanoluminal stimulation would demonstrate measurable changes in the intestinal microbiome and that microbial diversity would be reduced without normal mechanoluminal stimulation and with prolonged antibiotic treatment. METHODS: Bacterial 16s rRNA was extracted from swabbed samples of 43 intestines from 29 patients, aged 5 days to 13 years old. Swabs were obtained during initial resection or later stoma closure. Samples were compared using phylogenetic diversity whole tree alpha diversity and unweighted UniFrac distance beta diversity and by comparing significantly different taxonomic groups. RESULTS: Microbial community structure varied significantly between obstructive and inflammatory diseases (P = .001), with an effect size of 0.99 (0.97, 1.00). This difference persisted even 6 weeks after return to health. Family Enterobacter and Clostridiaceae predominated in patients with necrotizing enterocolitis or focal intestinal perforation; patients with an obstructive pathology had an abundance of Bacteroides. Comparison of UniFrac distance between paired proximal and distal intestines demonstrated that paired samples were significantly closer than any other comparison. CONCLUSION: In infants, inflammatory and ischemic intestinal pathologies treated with prolonged courses of antibiotics durably alter the intestinal mucosal microbiome. Diversion of mechanoluminal stimulation, however, does not.

Risk factors for small bowel bacterial overgrowth and diagnostic yield of duodenal aspirates in children with intestinal failure: a retrospective review.

BACKGROUND: Children with intestinal failure (IF) are at risk for small bowel bacterial overgrowth (SBBO) because of anatomical and other factors. We sought to identify risk factors for SBBO confirmed by quantitative duodenal culture. METHODS: A single-center retrospective record review of children who had undergone endoscopic evaluation for SBBO (defined as bacterial growth in duodenal fluid of >10(5) colony-forming unit per mL) was performed. RESULTS: We reviewed 57 children with median (25th-75th percentile) age 5.0 (2.0-9.2) years. Diagnoses included motility disorders (28%), necrotizing enterocolitis (16%), atresias (16%), gastroschisis (14%), and Hirschsprung disease (10.5%). Forty patients (70%) had confirmed SBBO. Univariate analysis showed no significant differences between patients with and without SBBO for the following variables: age, sex, diagnosis, presence of ileocecal valve, and antacid use. Patients receiving parenteral nutrition (PN) were more likely to have SBBO (70% vs 35%, P = .02). Multiple logistic regression analysis confirmed that PN administration was independently associated with SBBO (adjusted odds ratio, 5.1; adjusted 95% confidence interval, 1.4-18.3; P = .01). SBBO was not related to subsequent risk of catheter-related bloodstream infection (CRBSI). CONCLUSION: SBBO is strongly and independently associated with PN use. Larger prospective cohorts and more systematic sampling techniques are needed to better determine the relationship between SBBO and gastrointestinal function.

Early colonization of intestinal bifidobacteria and lactobacilli in the postoperative neonates with congenital intestinal atresia.

OBJECTIVE: To observe the early colonization of bifidobacteria and lactobacilli (B&L) in the postoperative neonate patients (NPs) with congenital intestinal atresia (CIA). METHODS: A prospective study was conducted in Shanghai Children's Hospital, Shanghai, China between February 2009 to August 2010 on 18 postoperative NPs with CIA (NP group), and 20 healthy full-term neonates raised by breastfeeding (healthy group). The fecal B&L in the 2 groups of neonates were consecutively quantified by real-time fluorescence quantitative polymerase chain reaction on 6 different time points. RESULTS: The mean levels (log/g feces) of B&L in the NPs group were significantly lower than in the healthy group at the end point of study (bifidobacteria: NPs [6.3] versus healthy [9.9]; lactobacilli: NPs [6.9] versus healthy [7.6]). Significant differences between the 2 groups also existed on the colonization time (days) of the intestinal B&L (bifidobacteria: NPs [5.8] versus healthy [3.0]; lactobacilli: NPs [4.2] versus healthy [1.2]). Both groups colonized lactobacilli earlier than bifidobacteria. During the study period, levels of bifidobacteria in the NPs group were continuously decreasing compared to lactobacilli, which was opposite to the healthy group. CONCLUSION: The colonization of the intestinal B&L in the postoperative NPs with CIA was severely interfered. The proliferation of bifidobacterium was more restrained than lactobacillus.

Duodenal atresia: late follow-up.

In this study, 41 randomly chosen patients aged 15 to 35 years (mean 22 years) were carefully examined. As primary operations there were 13 membrane excisions, five duodenoduodenostomies, 22 duodenojejunostomies, and one gastrojejunostomy. Twenty-eight patients were symptom-free, ten admitted some discomfort, three had major pains, including one with a history of duodenal ulcer. Reoperation for adhesion ileus had been performed in six patients, in the early postoperative phase in one instance. At late follow-up barium meals (N = 41) showed completely normal findings in two cases only, hiatal hernia in two, gastritis in three, duodenogastric reflux in 12, slight dilation of the duodenum with good emptying and no reflux in 16, a huge duodenal sac in nine, diminished peristalsis in eight, delayed emptying in five, slight luminal narrowing in three, duodenal diverticuli in nine, bezoars in two, and a polyp in the duodenum of one patient. Ultrasound (N = 35) revealed a gallbladder septum in one patient and a dilated common bile duct in another; in one subject the gallbladder was not visualized satisfactorily. Isotope biligraphy (N = 15) showed biliary reflux to the stomach in 12 cases. Endoscopy (N = 20) findings were: esophagitis (1), hiatal hernia (2), gastric mucosa in the lower esophagus (2), biliary reflux (9), gastritis (7), gastric polyps (2), dilated duodenum of variable degree (19), diminished peristalsis (4), marked retention (2), abnormal papilla (3), diverticuli (4), and a persistent membrane (1). Histology showed superficial gastritis in three patients. E coli was cultured from the duodenal juice in five patients and Candida found in two.(ABSTRACT TRUNCATED AT 250 WORDS)

[The infectious risk in neonatal surgery : an evaluation of frequency and consequences from a study of 300 cases (author's transl)]. / Evaluation et interprétation du risque infectieux en chirurgie néo-natale. A propos de 300 observations.

This study is an evaluation of the infectious risk related to neonatal surgery in 300 patients between 1968 and 1978, and its consequences to mortality and morbidity. Bacteriological species, circumstances, chronology of infection, related to each type of surgical pathology prove the endogenous way of contamination to be usual and predominant. When intestinal obstruction occurs, the risk of hematogenous diffusion is directly dependent from local stasis and bacterial pullulation which can be evaulated with duodenal, jejunal or fecal samples. Both mechanical factors and antibiotictherapy can induce qualitative and quantitative changes in bacterial flora of the bowel, and then increase the incidence of endogenous septicemia.