Flow cytometric analysis of HLA-DR antigen in conjunctival epithelial cells of patients with cystic fibrosis.

PURPOSE: Cystic fibrosis (CF) is an autosomal-recessive genetic disorder. The disease affects all secretory epithelia including the eye and belongs to the group of ocular surface epithelial diseases, termed keratoconjunctivitis sicca that develop in dry eye. In the pathogenesis of dry eye, inflammation plays a crucial role. The aim of this study was to investigate the expression of HLA-DR on conjunctival epithelial cells from patients with CF. MATERIALS AND METHODS: Twenty-five patients with CF and 25 normal subjects underwent ocular examination. Tear film break-up time (TBUT), Schirmer test, lissamine green staining, and conjunctival impression cytology were carried out. Cells were processed for flow cytometry, by using monoclonal antibodies to HLA-DR. RESULTS: The Schirmer test and TBUT scores were significantly lower in CF patients compared with controls. A significant increase of HLA-DR expression on epithelial cells was found in patients with CF compared with normal eyes. The Schirmer and TBUT test were positively correlated with HLA-DR expression for the percentage of cells. CONCLUSION: These results suggest that conjunctival epithelial cells play an important proinflammatory role in ocular changes in CF patients. Our findings confirm the presence of an inflammatory background and the immune nature of this disease. HLA-DR measurement might be a useful method for monitoring of inflammatory processes in the conjunctiva and could be helpful in the use of anti-inflammatory drugs in the treatment of ocular findings in CF patients.

Assessment of dental status and oral hygiene in the study population of cystic fibrosis patients in the Podlasie province.

PURPOSE: Cystic fibrosis (CF) is one of the most common genetic diseases worldwide. It is caused by mutations of the gene situated on the long-arm of the 7th chromosome coding Cystic Fibrosis Transmembrane Conductance Regulator (CFTCR) which is responsible for the synthesis of cAMP-dependent membrane chloride channel located on the top surface of epithelial cells of exocrine glands. Accumulation of the secretion in the outlet ducts caused by a dysfunction or lack of CFTR proteins leads to abnormal activity of exocrine glands, especially in the respiratory and alimentary tracts. Carbohydrates, the main dietary component, supply energy to the body, but at the same time are the major cariogenic agent. The aim of the current study was to assess dental caries disease and oral hygiene in CF patients in the region of Podlasie. MATERIAL AND METHODS: The study involved 23 patients with cystic fibrosis, aged 2.5-24 years, from the Podlasie Province treated in the Outpatient Cystic Fibrosis Department of the Children's University Hospital in Bialystok. Three age groups were distinguished: 1-5, 6-12, 13-24 years. The following were evaluated: caries incidence (percentage of patients with caries CI), caries intensity--based on the mean dmf/DMF score, oral hygiene--based on the dental plaque index (OHI-pl). RESULTS: The incidence rate of caries was found to be very high both in the CF population and in the control group. In children with mixed dentition it was 100%. For permanent teeth, mean DMF score was 3.55 in group II and 10.9 in group III. In CF patients, dental plaque index was the highest in group III. CONCLUSIONS: In CF patients, there is a serious risk of caries due to severe course of the disease, long-term administration of medications and high carbohydrate diet. CF patients should remain under constant dental care according to the individually designed programmes of oral health promotion and caries prophylaxis.

[Congenital megaloblastic anemia in a 22-month-old boy]. / Postac dzieciéca niedokrwistosci megaloblastycznej u 22-miesiecznego chlopca.

A 22-month old boy with the congenital form of megaloblastic anaemia is presented. The child was admitted to hospital with moderate-to-severe hematological and neurological symptoms. Very low serum vitamin B12 concentration and normal gastric secretion were determined. A dramatic recovery after intramuscular injections with vitamin B12 was observed.