Growth, Body Composition, and Micronutrient Abnormalities During and After Weaning Off Home Parenteral Nutrition.

OBJECTIVES: The aim of the study is to assess growth, body composition, and micronutrient abnormalities in children with intestinal failure (IF) over time, both during and after weaning off parenteral nutrition (PN). METHODS: Retrospective study in children on home PN between 2001 and 2015. Weight-for-age (WFA) and height-for-age (HFA) SD scores (SDS) were calculated, as well as fat mass (FM) and fat-free mass (FFM) SDS obtained by dual energy x-ray absorptiometry. The course of growth parameters and body composition was analyzed with linear-mixed models. All micronutrient measurements during the study period were obtained. RESULTS: Fifty-two patients were included with a median follow-up of 3.4 years. Seventy-one percent weaned off after a median PN duration of 0.9 years. One year after the start of PN, 28 patients were still PN-dependent with median WFA-SDS of -0.66 and median HFA-SDS of -0.96, both significantly lower than zero. Catch-up growth was achieved during PN, but HFA-SDS decreased after weaning (P = 0.0001). At a median age of 6.2 years, median %FM SDS was 0.30 and FFM SDS was -1.21, the latter significantly lower than zero. Frequent micronutrient abnormalities during PN were vitamin A (90%), zinc (87%), and iron (76%) and after weaning vitamin A (94%), E (61%), and 25-OH vitamin D (59%). CONCLUSIONS: Children with IF demonstrate abnormal growth and body composition and frequent micronutrient abnormalities. Longitudinal evaluation showed that catch-up growth occurs during PN, but height SDS decreases after weaning. This underlines the need for close monitoring, also after reaching enteral autonomy.

Chlamydia trachomatis Is Responsible for Lipid Vacuolation in the Amniotic Epithelium of Fetal Gastroschisis.

BACKGROUND: Vacuolated amniotic epithelium with lipid droplets in gastroschisis placentas is an unusual finding. Mass spectrometry of lipid droplets identified triglycerides, ester-linked to an unusual pattern of fatty acids. We hypothesize that these findings result from a Chlamydia trachomatis infection during the periconceptional period. The rising incidence of chlamydia infections has paralleled the increasing prevalence of gastroschisis among women less than 25 years of age. Histologically, young women are at greatest risk for a chlamydia infection due to their immature columnar epithelium, the preferential site for attachment of Chlamydia trachomatis infectious particle (elementary body). METHODS: Chlamydia trachomatis survive in an inclusion, relying on its host to acquire essential nutrients, amino acids, and nucleotides for survival and replication. If essential nutrients are not available, the bacteria cannot replicate and may be trafficked to the lysosome for degradation or remain quiescent, within the inclusion, subverting innate immunologic clearance. RESULTS: Chlamydiae synthesize several lipids (phosphatidylethanolamine, phosphatidylserine, and phosphoatidylglycerol); however, their lipid content reveal eukaryotic lipids (sphingomyelin, cholesterol, phosphatidylcholine, and phosphatidylinositol), evidence that chlamydiae "hijack" host lipids for expansion and replication. CONCLUSION: The abnormal amniotic epithelial findings are supported by experimental evidence of the trafficking of host lipids into the chlamydiae inclusion. If not lethal, what harm will elementary bodies inflict to the developing embryo? Do these women have a greater pro-inflammatory response to an environmental exposure, whether cigarette smoking, change in partner, or a pathogen? Testing the hypothesis that Chlamydia trachomatis is responsible for amniotic epithelium vacuoles will be a critical first step. Birth Defects Research 109:1003-1010, 2017. © 2017 Wiley Periodicals, Inc.

Maternal genitourinary infections and the risk of gastroschisis.

Genitourinary infections (GUIs) have been associated with increased risk of gastroschisis in 2 studies. Using data collected in the Slone Epidemiology Center Birth Defects Study, we examined the association between GUI and gastroschisis. From 1998 to 2010, mothers of 249 gastroschisis cases and 7,104 controls were interviewed within 6 months of delivery about pregnancy events, including vaginal infections, genital herpes, urinary tract infections (UTIs), and other sexually transmitted diseases (STDs). Women were considered exposed if they reported at least 1 instance of a GUI in the first trimester. Logistic regression models were used to calculate odds ratios and 95% confidence intervals. Women who reported having any GUI had an adjusted odds ratio of 1.8 (95% confidence interval (CI): 1.3, 2.4). The highest risk was seen among women who reported a UTI only (adjusted odds ratio = 2.3, 95% CI: 1.5, 3.5), while the odds ratio for an STD only was slightly elevated (adjusted odds ratio = 1.2, 95% CI: 1.0, 1.5). Among women under 25 years of age, the odds ratio for UTI only was 2.6 (95% CI: 1.7, 4.0), and among older women it was 1.8 (95% CI: 0.6, 5.9). When we considered the joint association of UTIs and young maternal age, a synergistic effect was observed. The results of this study add further evidence that UTIs may increase the risk of gastroschisis.

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.

Septicaemia due to enteric organisms is a later event in surgical infants requiring parenteral nutrition.

INTRODUCTION: The purpose of this study was to determine whether, in surgical infants requiring parenteral nutrition (PN), septicaemia due to enterococci or Gram-negative bacilli occurs later than septicaemia due to coagulase-negative staphylococci (CNS). PATIENTS/MATERIAL AND METHODS: We retrospectively studied 112 consecutive surgical infants (corrected gestational age up to 3 months) receiving PN for at least 5 days for congenital or acquired intestinal anomalies over a 2-year period (July 2007-June 2009). Data collected included diagnosis, duration of PN, episodes of septicaemia (defined as growth of bacteria from blood culture), and organisms cultured. We compared the time to first occurrence of septicaemia due to CNS with the times to first occurrence of septicaemia due to enterococci, Gram-negative bacilli, or other micro-organisms, using Kruskal-Wallis nonparametric ANOVA test and Dunn's multiple comparisons test. Data are given as median (range). RESULTS: 31 patients (28%) had a total of 65 episodes of septicaemia. Septicaemia due to CNS was most common, occurring in 22% of patients, after 17 days (1-239) of PN. Septicaemia due to enteric organisms was less common and occurred significantly later, at 59 (24-103) days for enterococci (p<0.01), and at 55 (30-106) days for Gram-negative bacilli (p<0.05). CONCLUSIONS: Septicaemia due to enterococci or Gram-negative bacilli occurs later in the course of PN than septicaemia due to CNS, in surgical infants. This suggests that these infants become more vulnerable to the translocation of enteric micro-organisms after a longer period of parenteral nutrition.

Case-control study of self reported genitourinary infections and risk of gastroschisis: findings from the national birth defects prevention study, 1997-2003.

OBJECTIVE: To assess the association between genitourinary infections in the month before conception to the end of the first trimesterand gastroschisis. DESIGN: Case-control study with self reported infections from a computer assisted telephone interview. SETTING: National birth defects prevention study, a multisite, population based study including 10 surveillance systems for birth defects in the United States. PARTICIPANTS: Mothers of 505 offspring with gastroschisis and 4924 healthy liveborn infants as controls. MAIN OUTCOME MEASURE: Adjusted odds ratios for gastroschisis with 95% confidence intervals. RESULTS: About 16% (n=81) of case mothers and 9% (n=425) of control mothers reported a genitourinary infection in the relevant time period; 4% (n=21) and 2% (n=98) reported a sexually transmitted infection and 13% (n=67) and 7% (n=338) reported a urinary tract infection, respectively. Case mothers aged <25 years reported higher rates of urinary tract infection alone and in combination with a sexually transmitted infection compared with control mothers. In women who reported both types of infection, there was a greater risk of gastroschisis in offspring (adjusted odds ratio 4.0, 95% confidence interval 1.4 to 11.6). CONCLUSION: There is a significant association between self reported urinary tract infection plus sexually transmitted infection just before conception and in early pregnancy and gastroschisis.