Assessment of Liver Fibrosis with the Use of Elastography in Paediatric Patients with Diagnosed Cystic Fibrosis.

Background: Complications of cystic fibrosis-associated liver disease (CFLD) are a leading nonpulmonary cause of death. Noninvasive tests enabling early detection of liver changes, especially in children are sought. The aim of the study was to assess the scale of liver fibrosis with the use of elastography in paediatric patients with diagnosed cystic fibrosis (CF) and its comparison with other tests (APRI and Fibrotest).

Assessment of Selected Parameters of Liver Fibrosis and Inflammation in Patients with Diagnosed Cystic Fibrosis.

Changes in the liver and bile ducts observed in patients diagnosed with cystic fibrosis result from inflammatory processes as well as fibrosis, remodeling, apoptosis, and cholestasis. As a consequence, portal hypertension, cirrhosis, and hepatic failure may develop. So far, the complexity of these processes has not been elucidated.

Serum Level of D-Lactate in Patients with Cystic Fibrosis: Preliminary Data.

D-Lactate is produced by the intestinal biota and later absorbed into circulation. Some patients with cystic fibrosis (CF) develop exocrine pancreatic insufficiency that may disturb the gut microbiome and enhance the production of D-lactate. However, this concept has not been studied yet.

The concentration of calprotectin in the stools of children with diagnosed cystic fibrosis.

Introduction: Calprotectin is a protein that plays a regulatory role in inflammatory reactions as an antibacterial and antiproliferative factor.

Aim: To assess the concentration of calprotectin in the stools of patients with diagnosed cystic fibrosis.

Material And Methods: Forty-one patients were included in the study, 24 boys and 17 girls, aged from 7 weeks to 18 years.

Does the mutation of the SERPINA1 gene contribute to liver damage and cholestasis in patients with diagnosed cystic fibrosis? preliminary study.

Unlabelled: Mutation of the SERPINA1 gene is present in about 2% of patients with cystic fibrosis but is more common and accounts for about 5% in patients with cystic fibrosis and co-existing liver lesions. The SERPINA1 gene is responsible for the synthesis of a serine protease inhibitor. The protein related with this gene is accumulated within the endoplasmic reticulum of hepatocytes causing their damage, inflammation and cirrhosis.

Diagnostic problems in cystic fibrosis - specific characteristics of a group of infants and young children diagnosed positive through neonatal screening, in whom cystic fibrosis had not been diagnosed.

Introduction: Neonatal cystic fibrosis screening contributes to an early diagnosis of cystic fibrosis and to implementing appropriate therapeutic management. Long-standing screening tests have made it possible to identify a group of newborns in whom the diagnosis was ambiguous and required further specialised tests.

Aim: The aim is to present cases of patients with a positive result of newborn screening for cystic fibrosis who were found to be carriers of the mutation in both alleles, however the lack of clinical symptoms and correct sweat testing values did not lead doctors to diagnosing cystic fibrosis and by the same token implementing the treatment.

Children's allergic diseases and microbial contamination of indoor air--a case report.

The assessment of exposure to bioaerosols in damp houses of two children who suffered from perennial rhinitis and asthma was performed. The paper presents an approach to the complex (i.e.

[Hereditary angioedema in a 16-year-old girl].

The angioedema may be connected with immunological or allergic reactions, rarely appears as a genetically determined hereditary disorder. The cause of hereditary genetically determined angioedema is the defect of complement due to lack or decreased activity of Cl esterase inhibitor with the low serum C4 complement. The acquired angioedema is the most frequently the effect of lymphoproliferative and autoimmunological diseases.