Wirus zapalenia wątroby typu C jako stymulator autoprzeciwciał we wczesnym okresie życia dzieci zakażonych wertykalnie

Zakażenie wirusem zapalenia wątroby typu C (HCV) wykazuje związek ze stymulacją procesów autoimmunologicznych. Oceniano częstość występowania niespecyficznych narządowo autoprzeciwciał (NOSA) u 19 dzieci zakażonych wertykalnie HCV, dotychczas nieleczonych. Autoprzeciwciała wykryto u 10 spośród 19 dzieci (52,6%); przeciwciała przeciw mięśniom gładkim (SMA) — u 3; przeciwciała przeciwjądrowe (ANA) — u 3; przeciwciała przeciw mikrosomom wątroby i nerki (LKM-1) — u 4. Średnia aktywność aminotransferazy alaninowej (ALT) u pacjentów z NOSA(+) — 77,17 jm./l, u pacjentów NOSA(–) — 51,72 jm./l. Stężenie g-globulin w obu grupach było prawidłowe. Badanie histopatologiczne bioptatu wątroby wykonano u 2 dzieci z NOSA(+), u 1 z NOSA(–). Nie stwierdzono cech histologicznych procesu autoimmunizacyjnego w wątrobie. Dzieci zakażone wertykalnie HCV będą leczone interferonem α i rybawiryną. Interferon α może indukować procesy autoimmunizacyjne. Pacjenci, szczególnie z NOSA(+), powinni podczas terapii podlegać ścisłemu nadzorowi i częstym badaniom kontrolnym, które pozwolą jak najszybciej wyodrębnić pacjentów uaktywniających proces autoimmunizacyjny. Forum Medycyny Rodzinnej 2011, tom 5, nr 6, 485–49

Symptoms of ectodermal dysplasia according to Freire-Maia and Pinheiro

Ectodermal dysplasia (ED) is a congenital syndrome of developmental disorders in tissues derived from the ectoderm. The inheritance of ED may be autosomal dominant or recessive. The distinctive features of this syndrome involve the skin and appendages, dentition, eye, ear, adrenal glands, nervous system and facial part of the skull. Over 200 forms of this disease are described, about 120 include symptoms located in the oral cavity and dentition. One of the several classifications, by Freire-Maia and Pinheiro, is based on the presence of four leading clinical symptoms: hair disorders, dental anomalies, nail disorders and dyshidrosis. ED needs multidisciplinary treatment because of the multitude and variety of its symptoms

O222 Treatment interruption in children with chronic HIV-infection: the results of the paediatric European network for treatment of AIDS (PENTA) 11 trial

ss Open Acce Oral presentation O222 Treatment interruption in children with chronic HIV-infection: the results of the paediatric European network for treatment of AIDS (PENTA) 11 trial DM Gibb*1, A Compagnucci2, H Green3, M Lallemant4, Y Saidi2, N NgoGiang-Huong4, C Taylor3, L Mofenson5, F Monpoux6, MIG Tome7, M Marczynska8, D Nadal9, U Wintergerst10, S Kanjavanit11, H Lyall12, C Giaquinto13 and J Moye

Outcomes of etravirine-based antiretroviral treatment in treatment-experienced children and adolescents living with HIV in Europe and Thailand

BACKGROUND: Etravirine (ETR) is approved as a component of second or third-line antiretroviral treatment (ART) for children living with HIV. We assessed the outcomes of ETR-based ART in children in routine care in Europe and Thailand. METHODS: Data on children aged <18 years at ETR start were pooled from 17 observational cohorts. Characteristics at ETR start, immunological and virological outcomes at 12 months, discontinuations, adverse events (AEs) and serious adverse events (SAEs) were described. Follow-up was censored at ETR discontinuation, death or last visit. RESULTS: 177 children ever received ETR. At ETR start, median [IQR] age was 15 [12,16] years, CD4 count 480 [287, 713] cells/mm3, 70% had exposure to ≥3 ART classes and 20% had viral load (VL) 3. Overall, 81 (46%) discontinued ETR by last follow-up. Median time to discontinuation was 23 [8, 47] months. Common reasons for discontinuation were treatment simplification (19%), treatment failure (16%) and toxicity (12%). Eight children (5%) had AEs causally associated with ETR, all dermatological/hypersensitivity reactions. Two were SAEs, both Stevens-Johnson Syndrome in children on regimens containing ETR and darunavir and were causally related to either drugs; both resolved following ART discontinuation. CONCLUSION: Children receiving ETR were predominantly highly treatment-experienced, over two-thirds were virally suppressed at 12 months

HBV and HCV Infection in Children and Adolescents

Hepatitis B (HBV) and C (HCV) infections are the major causes of chronic liver disease and are associated with significant morbidity and mortality [...

One-Year Outcomes after Ledipasvir/Sofosbuvir Treatment of Chronic Hepatitis C in Teenagers with and without Significant Liver Fibrosis—A Case Series Report

One-year outcomes after therapy with ledipasvir/sofosbuvir (LDV/SOF) in children with chronic hepatitis C (CHC) presenting with and without significant liver fibrosis were analyzed. We included patients aged 12–17 years treated with LDV/SOF, presenting with significant fibrosis (F ≥ 2 on the METAVIR scale) in transient elastography (TE) at the baseline and we compared the outcomes with that of patients without fibrosis. Patients were followed every 4 weeks during the treatment, at the end of the therapy, at week 12 posttreatment, and one year after the end of treatment. Liver fibrosis was established using noninvasive methods: TE, aspartate transaminase-to-platelet ratio index (APRI), and Fibrosis-4 index (FIB-4). There were four patients with significant fibrosis at baseline: one with a fibrosis score of F2 on the METAVIR scale, and three with cirrhosis (F4) at baseline. One year after the end of treatment, the hepatitis C viral load was undetectable in three of them. One patient was lost to follow-up after week 4. In two out of the four patients, a significant improvement and regression of liver fibrosis was observed (from stage F4 and F2 to F0-F1 on the METAVIR scale). In one patient, the liver stiffness measurement median increased 12 weeks after the end of the treatment and then decreased, but still correlated with stage F4. An improvement in the APRI was observed in all patients. In four patients without fibrosis, the treatment was effective and no progression of fibrosis was observed. A one-year observation of teenagers with CHC and significant fibrosis treated with LDV/SOF revealed that regression of liver fibrosis is possible, but not certain. Further observations in larger groups of patients are necessary to find predictors of liver fibrosis regression

Novel serum biomarkers modified by the body mass index z-score for the detection of liver fibrosis and steatosis in children with chronic hepatitis C

Abstract Background There is a need for validation of noninvasive alternatives to liver biopsy for the evaluation of fibrosis in children with chronic hepatitis C (CHC). The aim of this study was to evaluate the diagnostic performance of serum biomarkers modified by the body mass index z-score (BMI z-score) for the detection of fibrosis and steatosis in children with CHC. Methods Thirty children aged 9.4 ± 3.7 years (14 males, 16 females) with CHC underwent liver biopsy. Fibrosis was scored using a 5-point METAVIR scale (≥2 = significant fibrosis). For all the children, the following noninvasive markers were calculated: The aspartate transaminase (AST)-to-platelets ratio index (APRI), the modified APRI (M-APRI: BMI z-score × APRI), the Fibrosis-4 index (FIB-4), the modified FIB-4 (M-FIB-4: BMI z-score × FIB-4), and a novel marker, B-AST (BMI z-score × AST). The area under the receiver operator characteristic curve (AUROC) was calculated to detect significant fibrosis and steatosis. Results In the histopathological evaluation, 22/30 (73%) patients presented with fibrosis, and 8/30 (27%) presented with steatosis. For the detection of significant fibrosis, the AUROCs for M-APRI, M-FIB-4 and B-AST were 0.842, 0.823, and 0.848, respectively. For significant steatosis, the AUROCs were more than 0.9 for all markers that included the BMI z-score. B-AST, with a cut-off of 92.8, showed 71% sensitivity and 95% specificity for detecting significant fibrosis. For predicting severe steatosis, B-AST had 100% sensitivity and 92% specificity. Negative values of all three markers that included BMI z-scores excluded all patients with both significant fibrosis and significant steatosis. Conclusions Including the BMI z-score in serum biomarker formulas enhances their diagnostic ability to detect significant fibrosis and steatosis. B-AST may thus act as an effective alternative to liver biopsy

The prevalence and predictors of pulmonary lesions in paediatric patients with coronavirus disease 2019 : a brief report

Purpose: There are currently only scarce data available describing imaging manifestations in children with COVID-19. The aim of this study was to analyse pulmonary lesions on chest radiography (CXR) in paediatric patients infected with SARS-CoV-2 and to compare the CXR results with clinical and laboratory data. Material and methods: In this prospective single-centre study we included 118 consecutive paediatric patients with COVID-19. CXR was performed in 107 patients. Clinical and laboratory evaluations were performed on the same day as CXR, immediately (0 to 2 days) after the COVID-19 diagnosis had been established. Results: Pulmonary lesions were found in 24/107 (23%) children, including 14/24 (58%) with bilateral abnormalities. Compared to patients with normal CXR, children presenting with pulmonary lesions were significantly younger (7.0 ± 4.5 vs. 9.5 ± 4.5 years, p = 0.03) and more commonly presented with an elevated D-dimer level (6/24, 25% vs. 5/81, 7%; p = 0.008). Almost half (46%) of the children with pulmonary lesions were asymptomatic, and 11/60 (18%) of all asymptomatic patients presented with abnormal CXR. Conclusions: Pulmonary lesions in the course of COVID-19 are more common in younger children and those presenting with an elevated D-dimer level. A significant proportion of asymptomatic COVID-19 patients develop CXR abnormalities