The management and course of eosinophilic oesophagitis in Israeli children.

AIM: Eosinophilic oesophagitis (EoE) is a chronic inflammatory oesophageal disease, which has become more recognised in the past decade. We wanted to characterise our patients and review their course of disease and response to treatment. METHODS: We retrospectively reviewed the medical records of EoE patients from January 2010 to May 2018 in our Gastroenterology Institute. A hundred and two children were included in this study. We investigated the characteristics of patients and the response to three treatment options: proton pump inhibitors, elimination diet and topical steroids. The response to treatment was analysed according to 3 aspects: clinical, endoscopic appearance and histological features. RESULTS: Clinical improvement was noted in 55%, 75% and 87.5% on PPIs, diet and budesonide, respectively. Endoscopic improvement was noted in 38.4%, 51.4% and 65.4% on PPIs, diet and budesonide, respectively. Histological improvement was noted in 43.7%, 62.2% and 88.5% on PPIs, diet and budesonide, respectively. CONCLUSION: Our findings suggest that Israeli paediatric EoE patients have characteristics that resemble previous reports. Although there is a correlation between symptoms, endoscopic and histological appearance, we cannot rely on patients reports alone, and therefore, repeated endoscopy and biopsies are mandated. Topical steroids seem to be the most effective treatment option.

Transient Exocrine Pancreatic Insufficiency in Children: An Existing Entity?

OBJECTIVES: Pancreatic insufficiency in children is usually associated with diseases such as cystic fibrosis, Shwachman-Diamond syndrome, or chronic pancreatitis. Fecal elastase-1 is a reliable laboratory test for the diagnosis of exocrine pancreatic insufficiency (EPI). Transient pancreatic insufficiency has been rarely described and data on this entity are lacking in the medical literature. In this retrospective study we report 17 cases of transient pancreatic insufficiency presented mainly with failure to thrive and/or diarrhea. METHODS: We followed 43 children (age range 1 month-18 years) with low fecal elastase-1 in our institution between the years 2009 and 2017. We followed growth and laboratory results (particularly, complete blood count, albumin, transaminases, celiac serology, sweat test, and fat-soluble vitamins). Elastase levels <200 mg/g were considered as pancreatic insufficiency. RESULTS: Twenty-six were excluded due to missing data, a comorbidity or being syndromatic. Enrolled children (17) were all otherwise healthy.The median age at diagnosis was 3 years (range 0.2-15 years), 11 girls and 6 boys. Their main presenting symptoms were failure to thrive and/or diarrhea. Median fecal elastase-1 levels were 71 mg/g (range 18-160). Median time for normalization was 6 months (range 1-48 months). Abdominal sonography, celiac serology, and sweat test were normal for all patients. Most patients were treated with pancreatic enzymes until resolution. CONCLUSIONS: Transient EPI without clear etiology should be in the differential diagnosis of EPI after ruling out known etiologies. The resolving course pattern may be attributed to an unidentified infectious agent. Further studies to assess the etiology are mandated.

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.

Pegylated interferon alfa and ribavirin for children with chronic hepatitis C.

AIM: To study current treatment options for pediatric hepatitis C infection and their associated success rates. METHODS: We retrospectively reviewed charts of thirty children who had been treated with combination therapy of pegylated interferon alfa plus ribavirin for chronic hepatitis C infection. Patients had been treated with ribavirin (15 mg/kg per day) and either pegylated interferon alfa 2a (180 mg/m(2) once weekly) or pegylated interferon alfa 2b (1.5 mg/kg once weekly). Patients' follow-up included subjective assessment of complaints, physical examination including weight and height, as well as laboratory evaluations for viral load [before treatment, at 12 wk, and 6 mo following treatment completion, as determined by sustained viral response (SVR)], complete blood count, liver enzymes, alkaline phosphatase, bilirubin, renal function tests, and thyroid function tests. For patients not achieving a two log decrease in viral load at treatment week 12, treatment was discontinued and the patient was considered a treatment non-responder. RESULTS: Thirty children aged 3-18 years were included in the study. Twenty patients (11 males, 9 females) received pegylated interferon alfa 2b and ten patients (6 males, 4 females) received pegylated interferon alfa 2a. Twenty-three patients were infected with genotype 1, six patients were infected with genotype 3, and one patient was infected with genotype 2. Twenty patients (67%) achieved SVR. Treatment success rates were 90% with pegylated interferon alfa 2a vs 55% with pegylated interferon alfa 2b. Although a trend was noted for improved outcomes in the group receiving pegylated interferon alfa 2a, there were no statistically significant outcome differences between the two treatment groups (P = 0.1). Treatment success was 56.5% for patients infected with genotype 1 virus, compared to 100% for patients infected with other genotypes (P = 0.064). There was no difference in treatment response between males and females. A cut-off age of twelve years was used to dichotomize younger vs older participants; however, no difference in treatment response was observed between these groups. Using multivariate regression analysis, we could not determine predictors for achieving SVR from among the variables we examined (age, sex, and viral genotype). Although we noted a trend toward SVR with peginterferon alfa-2a, there was no statistical difference between the two peginterferons. A high incidence of adverse reactions to treatment was noted. Twenty-five patients (83%) suffered from at least one adverse reaction, but most experienced more than one adverse reaction. All patients except one became leukopenic (white blood cell count less than 5500 leukocytes/µL), six (20%) became anemic (hemoglobin less than 110 g/L), and one (3.3%) became thrombocytopenic (platelets less than 100 000/µL). CONCLUSION: Combination therapy to treat hepatitis C in children is as effective as in adults. There may be a benefit for treatment with pegylated interferon alfa 2a.

Salivary antioxidants and metalloproteinases in juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease in children; joint inflammation is the hallmark of the disease. Thirty-five children with JIA were studied, of whom 26 had active disease and 14 were receiving anti-TNF therapy (5 with Infliximab, 9 with Etanercept). Sixteen healthy controls also were studied. Saliva samples were obtained for analysis of anti-oxidant status, metalloproteinases (MMPs) and sialochemistry. The total antioxidant status was significantly higher in the saliva of all JIA patients, whether treated (P = 0.014) or not treated (P = 0.038) with anti-TNF agents. The increase in antioxidant status (TAS) in the saliva of the active patients was nearly two times higher than that of non-active patients (P = 0.01). MMP levels were significantly lower in JIA patients than in controls. MMP-9, MMP-3 and MMP-2 were lower in JIA patients without anti-TNF treatment by 36.7% (P = 0.01), 30.0% (P = 0.0001) and 10.7% (P = 0.0001), respectively. A greater reduction in MMP levels was observed in the group of patients treated with anti-TNF drugs: MMP-9, MMP-3 and MMP-2 were lower than in controls by 51.1% (P = 0.0001), 61.5% (P = 0.0001) and 55.4% (P = 0.0001), respectively. Children with JIA exhibited a significantly higher salivary antioxidant activity and significantly lower MMP levels. Anti-TNF treatment was associated with a further decrease in MMP levels in the saliva of JIA patients while an active state of JIA was associated with a further increase in the salivary antioxidant activity. Anti-TNF treatment may modulate the degradation process during the course of arthritis by inhibition of the activity of MMP.

Mutations in lipase H cause autosomal recessive hypotrichosis simplex with woolly hair.

BACKGROUND: Mutations in lipase H (LIPH) are a rare cause of autosomal recessive hypotrichosis (HT) simplex. OBJECTIVE: In this study, we investigated the clinical and molecular basis of HT simplex with woolly hair in 3 nonrelated families. METHODS: Three families of Jewish, Arab Muslim, and Italian origin that presented with HT with woolly hair were studied. The phenotype was confirmed by clinical, microscopic, and histologic examination. Polymorphic microsatellite genotyping and direct automated DNA sequencing of the LIPH gene were used to identify the mutations in our probands. RESULTS: All patients had woolly hair since birth. At presentation, scalp hair density was reduced or normal. Sequencing of the LIPH gene revealed two homozygous mutations: a large recurrent 90-base pair duplication mutation in exon 2 in the Jewish and Arab families, and a novel deletion/insertion mutation in exon 4 in the Italian family. LIMITATIONS: Only 3 families were studied. CONCLUSION: Mutations in LIPH result in variable degrees of HT. Woolly hair is an essential component of the clinical spectrum. A hot spot in the LIPH gene may be c.280_369dup in exon 2.

Patient approach and experience regarding complementary medicine: survey among hospitalized patients in a university hospital.

PURPOSE: To characterize the local population that turns to complementary medicine. METHODS: Participants were selected randomly: Medicine [A] (201 patients); Surgery [B] (100 patients); Control [C] (128 patients). The answers to the first two questions determined whether the questionnaire was to be completed. RESULTS: More females sought complementary medicine treatment, with no significant demographic differences among the groups. Nearly 20% of the subjects had academic education while 43% had completed high school. The results of the 16 significant questions were statistically different when comparing groups C and A (p = 0.025) and B (p = 0.011) respectively. A total of 16%, 12.4% and 9% of the subjects respectively preferred a physician MD as their complementary medicine practitioner and 49% [C], 34% [A] and 29% [B] respectively, favored teaching complementary medicine in medical and nursing schools. CONCLUSIONS: A total of 37% [C], 21% [B] and 27% [A] of the subjects experienced complementary medicine intervention at least once. The percentage of individuals holding academic degrees was higher in the group utilizing complementary medicine than those who did not. Only 21% of C and A groups and 12% of B group knew about adverse reactions to complementary therapies. More than 30% favored 'legalizing' complementary medicine.