THE CASE OF JEUNE SYNDROME AMONG THE PRECARPATHIAN POPULATION.

Despite the fact that Jeune syndrome is rather rare, neonatologists and pediatricians need to be aware of this pathology. This will facilitate early diagnostics of the condition and aid in the choice of the most adequate algorithms for its monitoring and treatment. The aim: To describe the case of Jeune syndrome among the Precarpathian population. Infant patient with Jeune syndrome and relevant medical records. Methods used in the study: clinical-genealogical and syndromal analysis, general clinical examination, radiologic method, including computed tomography (CТ) scan with 3D image reconstruction, methods of ultrasound diagnostics. The study was conducted in accordance with the Declaration of Helsinki Ethical Principles. The newborn baby was diagnosed with asphyxiating thoracic dystrophy on the basis of personal observation and conducted complex examination. According to the literature, this syndrome is rarely diagnosed in this age group. The diagnosis was based on the clinical and phenotypic manifestations of the syndrome, primarily on the characteristic association of symptoms of specific chest deformity and severe respiratory failure with oxygen dependence in the patient. Skeletal and pulmonary changes on radiographs and computed tomography scans were rather indicative. Brief follow-up data on the patient at the age of nine months are given.

Blood calprotectin in children with juvenile idiopathic arthritis: relationship to flare development after discontinuation of treatment.

OBJECTIVES: The study aim was to prospectively evaluate the relationship between disease flare development in children with juvenile idiopathic arthritis (JIA) after discontinuation of treatment and serum calprotectin levels (MRP8/14). MATERIAL AND METHODS: Determination of blood serum level of calprotectin was performed in 54 patients with inactive JIA from various regions of Ukraine. The inclusion criterion was the existence of an inactive state of the disease in children with JIA for at least 6 months. During 1 week after blood sampling for determination of serum calprotectin (MRP8/14) level the patients were completely discontinued of all therapy. Determination of calprotectin level in blood serum was performed with reagents EK-MRP8/14 Buhlmann (MRP8/14; S100A8/9), Switzerland, using the ELISA method. RESULTS: The trial results showed that 3 months after discontinuation of treatment in patients with inactive JIA, the flares developed in 5 out of 54 patients (9.3%). The median calprotectin level before discontinuation of the treatment was 1,700 ng/ml in patients who developed a flare, and 1,500 ng/ml in other studied patients (not statistically significant). At 6 months, the flare had developed in an additional 3 out of 48 (6.3%) of patients, who continued to be followed up, while their median calprotectin serum levels were 1,300 ng/ml and 1,500 ng/ml respectively (not statistically significant). At 12 months, the flares had developed in 13 more out of 45 (28.9%) patients, who continued to be followed up, while the median calprotectin serum level in these patients before discontinuation of treatment was 1,100 ng/ml and 1,650 ng/ml respectively (not statistically significant). CONCLUSION: After discontinuation of treatment a flare over the next year of follow-up developed in 38.9% of patients. The study results did not reveal a significant difference in calprotectin level in patients with JIA prior to complete discontinuation of treatment who developed a flare and those without a flare after 3, 6 and 12 months.