[Biliary atresia - signs and symptoms, diagnosis, clinical management].

Biliary atresia is a chronic cholangiopathy leading to progressive fibrosis of both intra- and extrahepatic bile ducts. The cause of the condition is unknown. Fundamental management of biliary atresia is surgical intervention and the outcomes of the treatment depend on the child's age with best results when performed within the first 2 months of life. Thus, the main role of pediatric healthcare is an urgent differential diagnosis and prompt qualification for the surgery, optimal postoperative management and early qualification for the liver transplantation in patients with persistent cholestasis. The authors discuss the clinical presentation, diagnosis and management of biliary atresia.

Regeneration of comparative genomic hybridization oligonucleotide microarrays with dimethylurea.

Reuse of materials in DNA hybridization-based methods has been known since the advent of Southern membranes. Array-based comparative genomic hybridization is essentially Southern hybridization with multiple probes immobilized on a solid surface. We show that comparative genomic hybridization microarrays fabricated with maskless array synthesizer technology can be used up to four times with the application of 1,3-dimethylurea as an array-stripping agent. We reproducibly detected chromosomal aberrations (0.6-22.4Mb in size) in four hybridization rounds using regenerated microarray slides. We also demonstrated that regenerated arrays can detect smaller alterations (16-200kbp), such as common copy number variants, as well as complex aberration profiles in tumor DNA.

Assessment of Lactobacillus casei rhamnosus (LGG) therapy in children with biliary atresia - Randomized placebo controlled trial.

BACKGROUND: The role of microbiota in biliary atresia (BA) remains unclear. The aim of our study was to assess efficacy and safety of LGG treatment in children with BA after HPE with special focus on bacterial cholangitis (BCH) and quantitative assessment of the gut microbiota composition and metabolism. METHODS: We performed double-blind placebo controlled trial with patients randomized into treatment group who received LGG (n = 14) and placebo (n = 16). The gut microbiota and short-chain fatty acids (SCFA) were assessed at baseline and after 6 months of treatment. Clinical and laboratory parameters including episodes of bacterial cholangitis (BCH) were collected during the study period and after 2-year follow-up. Additionally, stool composition of BA patients was compared with healthy age-matched control group. RESULTS: There were lower concentration of SCFA in children with BA compared to control group and significant increase in the number of Enterococcus bacteria. After 6 months of treatment, neither laboratory parameters nor gut microbiota composition differed between LGG group and placebo. PP analysis results were similar to ITT analysis, no significant differences between study and control group. Overall, there were 11 (36%) patients who developed at least one episode of bacterial cholangitis; 3 (21%) in the LGG group compared to 8 (50%) placebo group (p = 0.14). Bacterial cultures were positive in 22% of cases and recurrence after the first episode was observed in 27% of patients. The level of total bilirubin decreased below 2 mg/dl after 6 months of the study in 6 (42.8%) patients in the LGG group and in 8 (50%) patients in the placebo group (p = 0.73). During 2-year follow-up 6 out of 14 patients (42.8%) in the LGG group and 11 out of 16 placebo patients (68.7%) underwent liver transplantation (p = 0.27). CONCLUSIONS: Patients with BA present with specific microbiota profiles and decreased SCFA what gives opportunities to implement novel therapeutic options based on modulation of  microbiota. Whether LGG is an effective therapy needs to be studied in a larger group with similar outcome parameters.

[Efficacy and safety of cyclosporine in the treatment of inflammatory bowel disease in children - a retrospective study]. / Retrospektywna ocena skutczscii bezpieczenstwa leczenia cyklosporyna dzieci z nieswoistym zapaleniem jelit.

AIM: the aim of this study was to report single centre experience with cyclosporine used in treatment of children with inflammatory bowel disease with regard to safety and efficacy. METHODS: retrospective analysis included 23 patients, 21 with ulcerative colitis and 2 with Crohn's disease, aged 2.75 to 18.5 years. They were treated with cyclosporine during the last 5 years. Before cyclosporine therapy was started they received steroids and azathioprine. Cyclosporine treatment was given in severe steroid-resistant exacerbation of the disease (n = 10) or steroid-dependence (n = 13). Cyclosporine dose was set to obtain therapeutic levels (serum concentration > 100 ng/ml and < 200 ng/ml). RESULTS: Cyclosporine treatment was continued up to 2 months in 6 cases, 2 to 6 months in 8 patients and more than 6 months in 9 patients. Complications were reported in 2 patients: hirsutism and gingival hypertrophy. Cyclosporine treatment was stopped in the second case. None of the two patients with Crohn's disease improved during the treatment. Short-term improvement was observed in 11 patients with ulcerative colitis. Long-term recovery (> 6 months) was obtained in 6 cases. In 10 patients with severe exacerbation of ulcerative colitis colectomy was performed, in 4 of them elective surgery was performed when the clinical status improved. CONCLUSION: cyclosporine appears to be a safe and relatively effective treatment of ulcerative colitis in children. Cyclosporine is less effective in maintaining remission and it did not allow to avoid colectomy in severe exacerbation. Still case controlled studies are needed to show the efficacy of this treatment.