The value of Integrated Pulmonary Index (IPI) monitoring during endoscopies in children.

The Integrated Pulmonary Index (IPI) is an algorithm included in commercially available monitors that constitutes a representation of 4 parameters: EtCO2, RR, SpO2 and PR. The IPI index has been validated for adults and children older than 1 year of age. In this study we aimed to study the value of IPI monitoring during pediatric endoscopic procedures. Our data consisted of 124 measurements of 109 patients undergoing different procedures (upper endoscopy 84 patients, colonoscopy 6 patients, both 9 patients). The data was divided into 3 groups based on the drug type used: propofol only, 5 patients (group 1); propofol & midazolam, 89 patients (group 2); propofol, midazolam and Fentanyl, 15 patients (group 3). Patients in group 2 and 3 had significantly higher IPI levels than group 1. Significantly lower IPI values were found between ages 4-6 compared to 7-12 years old. High midazolam dose was associated with lower IPI levels during the procedure. No significant differences were found for propofol doses. Patients who had an anesthetist present had lower IPI levels during the procedure compared to those who did not. No differences were noted between the different procedures. IPI alerted all apnea episodes (58 events, IPI = 1) and hypoxia (26 events, IPI ≤ 3) episodes, whereas pulse oximetry captured only the hypoxia episodes (IPI sensitivity = 1, specificity 0.98, positive predictive value 0.95). Younger patient age, use of propofol alone, higher midazolam doses and presence of anesthetist are all associated with lower IPI levels.

Prolidase deficiency associated with systemic lupus erythematosus (SLE): single site experience and literature review.

INTRODUCTION: Prolidase deficiency (PD) is a rare autosomal recessive disorder which may have a wide spectrum of clinical features. These features include a characteristic facies, cognitive impairment, rashes or skin ulceration, splenomegaly, recurrent infections involving mainly the respiratory system, and iminodipeptiduria. The disorder is caused by a mutation in the PEPD gene. OBJECTIVE: To describe a cohort of unrelated PD patients from Northern Israel whose inborn error of metabolism was associated with systemic lupus erythematosus (SLE) and to identify in the medical literature all PD cases mimicked by and/or associated with SLE. METHODS: Three patients with PD associated with SLE were clinically, biochemically and genetically investigated. These patients were from 3 unrelated consanguineous families residing in Northern Israel. A computer-assisted (PubMed) search of the medical literature from 1975 to 2011 was performed using the following key words: Prolidase deficiency, SLE, and systemic lupus erythematosus. RESULTS: An association between PD and SLE was found in 10 PD patients. These 10 patients included three from our cohort of 23 PD patients, and seven out of just under 70 PD patients previously reported in the literature. CONCLUSION: The present findings underscore the relatively high incidence of the association between SLE and PD, suggesting that this association may not be coincidental. The phenotypic similarities between SLE and PD might suggest that the PEPD gene constitutes a modifier gene or a genetic risk factor in the causation of SLE.

Atypical Henoch-Schonlein purpura: a forerunner of familial Mediterranean fever.

Insuception is the most common cause of intestinal obstruction in early childhood. The cause of most intussusceptions is unknown but it can complicate the course of Henoch-Schonlein purpura (HSP) as a result of the vasculitic process. Familial Mediterranean fever (FMF), a common disease in Israel, is also associated with HSP. In a few patients, particularly in children, HSP has been reported to precede the diagnosis of FMF. We describe two patients with an unusual clinical course of severe abdominal pain as a result of intusucception. The correlation between intusucception, HSP and FMF are discussed.

Nutritional supplementation with polymeric diet enriched with transforming growth factor-beta 2 for children with Crohn's disease.

BACKGROUND: A polymeric diet rich in transforming growth factor-beta 2 used as a single nutrient has been shown to induce remission in 79% of children with Crohn's disease. OBJECTIVES: To summarize the experience of several pediatric gastroenterology units in Israel using a TGFbeta2-enriched polymeric diet (Modulen IBD) supplementation in children and adolescents with Crohn's disease. METHODS: In a retrospective study we reviewed the charts of 28 children with Crohn's disease (10 girls, 18 boys) who received, in addition to conventional treatment, Modulen IBD as a supplement to their regular nutrition. These children were compared with 18 children supplemented with standard polymeric formula (Ensure Plus) and 18 children without formula supplementation. We recorded clinical manifestations, growth, and the Pediatric Crohn's Disease Activity Index before and after initiation of the polymeric diet. RESULTS: The Modulen-treated children showed a significant decrease in PCDAI from 34.3 to 15.7 (P< 0.0001). A significant decrease in PCDAI was recorded also in the Ensure Plus group, from 35 to 22 (P= 0.02) but not in the non-supplemented group. Significant improvements in body mass index (P = 0.01) and erythrocyte sedimentation rate (P= 0.03) were recorded at follow-up (median 3.4 months) only in the Modulen IBD group. CONCLUSIONS: In this cohort of children with Crohn's disease, supplementation of the diet with Modulen IBD as well as supplementation with Ensure Plus was associated with a decrease in PCDAI. The children supplemented with Modulen IBD also showed improvement in BMI, suggesting an additional advantage of nutritional therapy in children with this disease.

Influence of co-administration of oral insulin and docosahexaenoic acid in mice.

Insulin and docosahexaenoic acid are both present in human milk. The aim of this study was to examine the effect of co-administration of oral insulin and DHA in mice. Immediately after weaning, Balb C mice were divided into four groups of seven mice each for a period of 4 weeks. Group 1 received a chow diet only. Group 2 received a chow diet and also was given human insulin (1 unit/mL of drinking water) without docosahexaenoic acid. Group 3 received a chow diet supplemented with docosahexaenoic acid (500 mg/kg/day in the chow) and no insulin. Group 4 received a chow diet and supplementation with both human insulin and docosahexaenoic acid. At 28 days, fasting blood levels of glucose, insulin, lipids, lipid peroxidation analysis, docosahexaenoic acid plasma levels, and docosahexaenoic acid content in red blood cells were determined. We found that glucose levels were lower in the group that was supplemented with insulin only (group 2, 61.4 mg/dL +/- 2.8,mean +/- SD) and in the group that was supplemented with DHA only (group 3, 61.1 mg/dL +/- 2.0) compared to controls (group 1, 71 mg/dL +/- 6.9, P < 0.0001). Supplementation of both insulin and docosahexaenoic acid (group 4) resulted in significantly lower glucose levels (56.4 mg/dL +/- 2.6) compared to those in groups 2 and 3 (P < 0.01). No significant differences were found in lipid profile or lipid peroxidation between the groups. We conclude that adding insulin or docosahexaenoic acid to the diet of weaned Balb C mice reduces glucose blood levels. Supplementation with both substances has a synergistic effect. The presence of insulin and docosahexaenoic acid in human milk may be the cause for reduced glucose levels in breast-fed infants, in addition to the known effects of DHA on insulin sensitivity.