Unexpectedly Weak Anti-B in 2 Group O Pediatric Patients on Parenteral Nutrition and Disease Specific Supplemental Enteral Feeds.

Anti-A and anti-B antibodies are naturally occurring and develop from exposure to intestinal bacteria after age 4 to 6 months. In the laboratory, strong agglutination with A1 and B cells, or B cells only and A1 cells only, on reverse typing in a healthy person with immunocompetence is expected for patients with ABO types O, A, and B, respectively. However, absent or weak anti-A and anti-B antibodies can be observed in some clinical scenarios, such as patients with immunodeficiencies, newborns, elderly patients, and patients who have recently received bone marrow transplants. In this article, we report the cases of 2 pediatric patients with group O blood type who were receiving total parenteral nutrition (TPN) and disease-specific enteral feeds and who have strong anti-A and absent/weak anti-B.

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.

Heightened Immune Activation in Fetuses with Gastroschisis May Be Blocked by Targeting IL-5.

The development of the fetal immune system during pregnancy is a well-orchestrated process with important consequences for fetal and neonatal health, but prenatal factors that affect immune activation are poorly understood. We hypothesized that chronic fetal inflammation may lead to alterations in development of the fetal immune system. To test this hypothesis, we examined neonates with gastroschisis, a congenital abdominal wall defect that leads to exposure of the fetal intestines to amniotic fluid, with resultant intestinal inflammation. We determined that patients with gastroschisis show high systemic levels of inflammatory cytokines and chemokines such as eotaxin, as well as earlier activation of CD4(+) and CD8(+) effector and memory T cells in the cord blood compared with controls. Additionally, increased numbers of T cells and eosinophils infiltrate the serosa and mucosa of the inflamed intestines. Using a mouse model of gastroschisis, we observed higher numbers of eosinophils and both type 2 and type 3 innate lymphoid cells (ILC2 and ILC3), specifically in the portion of organs exposed to the amniotic fluid. Given the role of IL-5 produced by ILC2 in regulating eosinophil development and survival, we determined that maternal or fetal administration of the anti-IL-5 neutralizing Ab, or a depleting Ab against ILCs, can both effectively reduce intestinal eosinophilia. Thus, a congenital anomaly causing chronic inflammation can alter the composition of circulating and tissue-resident fetal immune cells. Given the high rate of prenatal and neonatal complications in these patients, such changes have clinical significance and might become targets for fetal therapy.

Do infants with gastroschisis may have a high incidence of non-IgE-mediated cow's milk protein allergy?

BACKGROUND/PURPOSE: To determine the frequency and characteristics of suspected cow's milk protein allergy (CMPA) in infants with gastroschisis and response to change in milk. METHODS: A retrospective cohort study of 111 consecutive infants with gastroschisis. RESULTS: 64 episodes suggesting non-IgE-mediated CMPA occurred in 50 infants (45 %) at a median age of 44 days (9-186) and during the primary admission in 38 %. At the time of the episode the infant feed was breast milk (BM, n = 24), term formula (TF, n = 20) or extensively hydrolysed formula (EHF, n = 6). The feed was changed to EHF (34), amino acid formula (AAF) (14) or BM with maternal CMP-free diet (2). Partial or complete resolution of symptoms occurred in all. There was histological evidence of an allergic reaction to CMP in all four infants in whom tissue was available. Recurrent episodes occurred in 13/50 infants (26 %), 10 of whom were receiving EHF. There were no recurrent episodes in infants being fed with AAF. CONCLUSION: Features suggesting non-IgE-mediated CMPA appear common in infants with gastroschisis.

Changes in intestinal waste products during the antenatal management of gastroschisis by serial amniotic fluid exchange and infusion.

Despite the good survival rate of fetuses with gastroschisis, the length and cost of hospitalization for surgically repaired gastroschisis are high. In gastroschisis, prolonged exposure of the intestine to amniotic fluid (AF) containing intestinal waste products results in intestinal damage, including intestinal wall thickening and fibrous peel formation. The deleterious effects of AF on gastroschisis can be prevented by lowering the concentration of intestinal waste products. We describe the treatment of a case of fetal gastroschisis by repeated AF exchange and infusion. Following repeated, successful transabdominal AF exchange and infusion, the concentrations of various intestinal waste products were decreased. AF exchange and infusion may prevent intestinal damage and improve postnatal outcome in gastroschisis by diluting the AF, probably by lowering the concentrations of intestinal waste products.

Biochemical investigations of bowel inflammation in gastroschisis.

Neonates with gastroschisis have perivisceritis resulting from contact between the bowel and amniotic fluid. Here, we characterized the mediators involved in this inflammatory process in humans and ewes, to find a reliable marker of this process. We have diagnosed 41 cases of gastroschisis since 1995. Amniotic fluid sampled for karyotyping between 15 and 32 wk of gestation was also used to assay cytokines and inflammatory proteins. The findings were compared with those in 93 age-matched controls. Amniotic fluid cells were analyzed by means of cytology. Histologic examination of the bowel was performed when neonatal appendectomy was performed. The findings were compared with those obtained in a ewe model of gastroschisis. In gastroschisis, amniotic total protein, IL-6, IL-8, and ferritin levels were significantly higher than in controls. Gastroschisis was associated with significantly higher cell counts (mainly mononuclear cells) in amniotic fluid. At birth, macrophages were abundant in the fibrous peel covering the bowel. Similar results were obtained in the ewe model. Gastroschisis may be associated with a subchronic inflammatory process of variable intensity. This inflammation is restricted to the bowel wall and involves inflammatory cells such as macrophages, which may secrete ferritin, neopterin, and calprotectin.