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Background The aim of this study was to assess the pediatric population that suffered from inflammatory bowel disease (IBD) in the Czech Republic and to determine the incidence of Crohn disease (CD) in children up to 15 years age between 1990 and 2001. Methods Diagnostic criteria for CD, ulcerative colitis (UC), and indeterminate colitis (IC) were defined. Medical records provided a source of basic information about the children. A standardized protocol was filled out and sent to the coordinator of the study. All protocols were checked to see whether the data corresponded to the defined criteria and then were processed further. The study was retrospective in character for the years 1990 to 1999 and prospective for the years 2000 and 2001. Results Diagnostic criteria were met in 470 patients with IBD; 201 of them turned 18 years old during the study period. CD was diagnosed in 223 patients. The incidence of CD in children up to 15 years of age increased from 0.25/100,000 in 1990 to 1.25/100,000 in 2001. Eighty-two percent of children with CD were treated with aminosalicylates in combination with corticosteroids; 29% of patients received azathioprine. Severe growth retardation was recorded in 6.4% of adolescents with CD at the age of 18. UC was diagnosed in 202 patients. Therapy with aminosalicylates only was sufficient for control of the disease in 23% patients; 68% children were treated with corticosteroids, 15 of them (23% of the whole group) received additional azathioprine. Criteria for IC were met in 9.8% of all patients with IBD. Conclusion This study confirmed an increase in incidence of CD in children younger than 15 years in the Czech Republic.

5-aminosalicylate compounds (mesalazine, sulfasalazine) are widely used in therapy of inflammatory bowel diseases. Mesalazine-induced interstitial nephritis is a rare complication; however, the morbidity in an affected individual is high. Regular renal screening in patients treated with 5-aminosalicylate compounds is important. A 15-year-old boy with treated idiopathic proctocolitis, consequent mesalazine-induced nephritis, and a favorable response to corticotherapy is presented.

Human transferrin (Tf) shows genetic polymorphisms, which may interfere in the screening of congenital disorders of glycosylation (CDG). Isoelectric focusing followed by direct immunofixation was used for Tf analysis in controls and several groups of patients. Equivocal results in one case have been recognized as a rare Tf CD variant. A higher incidence of some genetic variants has been reported in connection with certain diseases; of the seven Tf phenotypes detected in our set of samples, an apparently higher frequency of Tf C1C2 variant found in some groups of patients was not significant.

We report on a 5.5-year-old boy with a multiple malformation syndrome and biochemical abnormalities suggesting a combined defect in the biosynthesis of Nand O-glycans. The patient was followed prenatally for intrauterine growth retardation and a finding of intestinal obstruction (double bubble sign). Born full term as the first child of healthy, non-related Czech parents without remarkable family history, his Apgar scores were 6, 6, and 7; birth weight 2,310 g; body length 45 cm; and head circumference 32.5 cm. He presented with dysmorphic features, including dolichocephaly, low hairline, high nasal bridge, thin lips, small chin, gothic palate, blepharophimosis and epicanthus, in addition to other malformations, such as arachnodactyly, unusual palmar creases, sinus sacralis, pes equinovarus, hypospadias, inguinal hernia, cryptorchidism, vesicoureteral reflux, peripheral pulmonary stenosis, and patent foramen ovale. Shortly after birth a surgical correction of intestinal malrotation and duodenal stenosis (Ladd’s syndrome) was done. Neonatal brain ultrasonography, ophthalmologic examination, a skeletal survey and an electrocardiogram were normal. Chromosome analysis was normal. Laboratory tests including serum lactate, ammonia, and immunological investigations were within normal limits. Serum biotinidase, plasma and urinary amino acids, organic acids, purines and pyrimidines, sugars, oligoand mucopolysaccharides did not reveal any pathological changes. At age 4 months, the brain ultrasonography showed hypogenesis of the posterior part of the corpus callosum (later confirmed by MRI), and abnormal EEG delta waves were noted occipitally, without epilepsy. In addition to anemia (90 g/l, normal 105–135), elevated LDH (12.7 μkat/l, normal< 4.4) and elevated transaminases (1.1–2.2 μmol/l, normal

GOALS: To determine the efficacy of triple therapy supplemented with a specially designed fermented milk product containing specific probiotic Lactobacillus casei (L. casei) DN-114 001 strain on Helicobacter pylori eradication in children. BACKGROUND: Lactobacillus species possess in vitro activity against H. pylori. There are no consistent data on the impact of eradication therapy supplemented with probiotics on H. pylori cure rates in childhood in vivo. STUDY: Multicenter, prospective, randomized, double-blind controlled study. Eighty-six symptomatic H. pylori-positive children were randomized either to receive the control treatment of omeprazole, amoxicillin, and clarithromycin (OAC) for 7 days or the test treatment of omeprazole, amoxicillin, and clarithromycin for 7 days supplemented with fermented milk (Actimel) containing L. casei DN-114 001 (OAC-LC), for 14 days. H. pylori status was assessed at 4 weeks following therapy using two noninvasive tests. RESULTS: Intention-to-treat (ITT) based eradication rates for the OAC-LC group were 84.6% (95% CI, 71.2%-95.5%), and 91.6% (95% CI, 76.9%-98.2%) by per-protocol (PP) analysis. Eradication in the OAC group was 57.5% (95% CI, 42.2%-72.3%) in the ITT set and 61.3% (95% CI, 44.4%-75.0%) in the PP group. Eradication success was higher in the OAC-LC group compared with the OAC group in both ITT (P=0.0045) and PP analysis (P=0.0019). Primary resistance for clarithromycin could be determined in 21.2%. Side effects were infrequent. Drug compliance was good throughout the study. CONCLUSION: Supplementation with fermented milk, containing live special probiotic L. casei DN-114 001, confers an enhanced therapeutic benefit on H. pylori eradication in children with gastritis on triple therapy.

This paper describes the synthesis and monolayer properties of two porous surfactants derived from the calix[5]arene framework that exist as stable cone conformers [i.e., 5,11,17,29,29-pentaformyl-91,92,99,94,95-pentakis(n-octyloxy)calix[5]arene (1a) and an N,N-dimethylthioamide derivative 2a] plus calix[4]arene and calix[6]arene analogs. The introduction of 1a, in and of itself, represents a significant advance in the calixarene area because it provides access to a wide-cavity calixarene that is amenable for further modification at its wider rim

In this paper, the authors document their discovery of extraordinary permeation properties of composite membranes derived from Langmuir-Blodgett (LB) multilayers of calix[6]arene I plus poly[1-(trimethylsilyl)-1-propyne] (PTMSP) film. Specifically, it is shown that the intrinsic permeability and selectivity of such composites toward He and N{sub 2} increase with increasing permeant pressures. In striking contrast, analogous composites prepared with a `conventional` polymeric surfactant, poly(1-octadecene-co-maleic anhydride) (II) show unexceptional behavior, i.e., permeation characteristics that are independent of pressure. The theoretical and practical implications of these findings are briefly discussed. 8 refs., 2 figs.

In this paper we provide evidence for uniqueness of calix[6]arene surfactants as building blocks for the construction of permeation-selective composites. Specifically, we show that membranes made from poly[1-(trimethylsilyl)-1-propyne] (PTMSP) plus multilayers of calix[6]arene I exhibit significant permeation selectivity toward He and N{sub 2}, but that those based on arachidic acid (AA), cadmium arachidate (AA/Cd{sup 2+}), a polyion complex of AA plus poly(ethyleneimine) (AA-PEI), and stearamide oxime (SA) show no permeation selectivity and no alteration in the barrier properties of the support. We also report results of surface analyses of PTMSP/I and PTMSP/SA by X-ray photoelectron spectroscopy (XPS) that offer insight into the origin of these striking differences. 40 refs., 2 figs., 1 tab.