FEATURES OF CLINICAL SYMPTOMS AND SIGNS, HEMATOLOGICAL AND BIOCHEMICAL PARAMETERS IN CHILDREN WITH JOINT HYPERMOBILITY IN A LATE PERIOD UPON THE CHORNOBYL NPP ACCIDENT. / OSOBLYVOSTI KLINIChNOÏ SYMPTOMATYKY, GEMATOLOGIChNYKh TA BIOKhIMIChNYKh POKAZNYKIV U DITEY̆ Z GIPERMOBIL'NISTIu SUGLOBIV U VIDDALENYY̆ PERIOD PISLIa AVARIÏ NA ChORNOBYL'S'KIY̆ AES.

OBJECTIVE: establishing the types and frequency of disembriogenetic stigma in children with joint hypermobility given the clinical and laboratory features, genetic component and endocrine regulation of these disorders in a late period upon the accident. MATERIALS AND METHODS: Children (n = 109) inhabiting the radiologically contaminated territories and having the connective tissue dysplasia (CTD) signs were involved in the study. Diseases in family history, ossalgia complaints, fractures in a personal history, bone disembriogenetic stigma, joint hypermobility, type of somatic diseases, blood serum biochemical parameters (namely calcium, alkaline phosphatase, total protein, cholesterol, creatinine, iron, ferritin content), serum cortisol, free thyroxine, pituitary thyroid-stimulating hormone (TSH) levels, free amino acid composition in urine and radiation dose were considered. RESULTS: Radiation doses in children having the CTD ranged from (0.37 ± 0.11) mSv to (0.56 ± 0.10) mSv with no difference from that in those without CTD. Joint hypermobility (JHM) correlated with cancer in family history (rs = 0.53) and lower extremity varicose vein disease (rs = 0.40) (p < 0.05). Incidence of ossalgia, easy fatigability, and bone fractures was higher in children with CTD. Anomalies of the dentofacial system were first in line (38.5 %) in these children. Proportion of children with grade II JHM and platypodia was lower (rs = 0.42), but with lower extremity deformations was higher (rs = 0.68) (p < 0.05) vs. in the control group. Iron and ferritin deficiencies both with lymphocytosis were more common in children with CTD than in the comparison group (p < 0.05). The increased content of oxyproline, lysine, proline both with glycine deficiency were detected in children having the CTD, i.e. an imbalance of amino acids from the collagen content was observed featuring a predominance of catabolic processes over anabolic ones. There was a direct correlation between the TSH level and the JHM grade (rs = 0.49), although the values of hormone concentration in these children did not exceed the reference range (maximum values were 3.3 µIU/ml). CONCLUSIONS: The revealed abnormalities in amino acid content, ferrokinetics, and thyroid function indices can affect the collagen formation, organic matrix structure of bone tissue and significantly deregulate the hemato- poiesis. The later can underlie the pathways of haematologic malignancy development.

Rationale treatment and preventive measures for correction of bone structure in children with acute leukemia, exposed to ionizing radiation due to the Chornobyl accident. / Ocinka likuval''no-profilaktychnyh zahodiv dlja korekcii' struktury kistkovoi' tkanyny u ditej z gostrymy lejkemijamy, jaki zaznaly vplyvu ionizujuchogo vyprominennja vnaslidok avarii' na ChAES.

Objective. To justify and evaluate the effectiveness of treatment and preventive measures taken to correct the bio-chemical and biophysical changes in the structure of bone in children exposed to ionizing radiation due to Chornobyl accident in the dynamics of leukemic process. Materials and methods. The study involved 144 patients with acute lymphoid and myeloid leukemia. Serum calcium, total protein, alkaline phosphatase, cortisol and thyroid-stimulating hormone were investigated, as well as calcium and phosphate excretion, amino acids composition in the urine, acid-base balance and buffer systems of blood, bone density. Results. A reduction of bone densitometry, calcium and cortisol levels and increase of serum alkaline phosphatase and urine oxyproline were found in children with acute leukemia in acute I phase and during the chemotherapy. In the period of remission the majority of patients did not differ from the comparison group. The signs of osteopenic syndrome that require monitoring of the bone structure status were revealed in 10.0 % of children of the comparison group. Radiation doses of leukemia patients were 3.66 ± 0.11 mSv. No relationship between radiation dose and indicators that reflect the state of the bone tissue structures has been established. The use of complex health care measures in children with acute leukemia in remission, contributed to the positive effect of 62.0 %. Worser effect was observed in patients with an unfavorable course of the disease. Conclusions. Results of the study of bone tissue metabolism prove fore a change in the organic and mineral matrix in patients with acute leukemia exposed to ionizing radiation as a result of the Chonobyl NPP accident, and persons without hematologic diseases, and the need for treatment and prevention.

Peculiarities of gastrointestinal disease concomitant to heart connective tissue dysplasia in children born from persons exposed to ionizing radiation in childhood after the ChNPP accident. / Osoblyvosti patologii' shlunkovo-kyshkovogo traktu u ditej iz syndromom dysplazii' spoluchnoi' tkanyny sercja, jaki narodylysja vid bat''kiv, oprominenyh v dytjachomu vici v rezul''tati avarii' na ChAES.

Study objective. The study examined the peculiarities of upper gastrointestinal tract diseases in children suffering from a syndrome of heart connective tissue dysplasia (HCTDS) born from persons exposed to ionizing radiation in a childhood after the ChNPP accident. Materials and methods. The study included 120 children suffering from HCTDS (group I) and 75 persons having no signs of the syndrome (Group II). Diagnosis of HCTDS was obtained from echocardiography in Band M-modes and Doppler echocardiography. Esophagogastroduodenoscopy was performed using a flexible endoscope "OLYMPUS" model GIF-P3. Abdominal diagnostic ultrasound was used to assay the structure of abdominal parenchymal viscera. Results. Upper gastrointestinal tract diseases (UGITD) manifest earlier in children suffering the HCTDS (beginning from a preschool age). Along with age the systemic pattern of organ and system injury becomes evident i.e. there were 6.8 clinical/nosological entities per one child in Group I and 4.9 in Group II. Clinical presentation of chronic UGITD diseases in children suffering concomitant HCTDS were characterized by more frequent exacerbation after the psychoemotional and physical strain, more frequent intestinal motor dysfunction, nervous system disorders (80 %), and chronic infections (62.2 %). Endoscopic pattern of chronic UGITD with concomitant HCTDS featured a combination of mucosal inflammation (a range of various forms of esophagitis, erythematous and hypertrophic gastroduodenopathy) with abnormal gastrointestinal motor function i.e. gastroesophageal and duodenogastric reflux (73.34 %). Destructive lesions at most of duodenal zone were found in 20 %. Conclusions. UGITD namely chronic gastroduodenitis with typically concomitant involvement of several parts of digestive tract are most prevalent within all digestive system diseases in remote period upon the Chornobyl disaster in children born from persons exposed to ionizing radiation in a childhood. Presentation of UGITD occurs earlier in children suffering HCTDS with evident systemic pattern of organ and system injury along with an age. Refluxes of various nature and localization are the leading factors for UGITD onset in children suffering HCTDS. Thus a range of multidirectional tools of reflux disease prophylaxis is required for inclusion to the disease management.

Lymphocytosis in children after the Chornobyl NPP accident.

OBJECTIVE: To establish the sources of lymphocytosis in children living in ecologically unfavorable conditions after the Chornobyl Nuclear Power Plant accident aiming the high risk group formation for oncohematological diseases among the children's population. MATERIALS AND METHODS: The 226 children with lymphocytosis were surveyed. The of child's life history and kind of somatic morbidity were considered. Quantitative and qualitative parameters of blood cells, biochemical indices including immunoglobulins (A, M, G), circulating immune complexes, phagocytosis indices were assayed. RESULTS: Children with lymphocytosis were more often born with a high bodyweight, they demonstrated manifestations of constitutional lymphatism, lymphadenopathy and more often had respiratory infections. Quantitative and qualitative changes of hemopoietic elements depended on a type of a disease. Activation of lipid peroxidation, dysimmunoglobulinemia and increased level of circulating immune complexes were revealed. Treatment and preventive actions promoted normalization of hemogram indices in 58% of children surveyed. CONCLUSIONS: The abnormalities in immune indices, activated lipid peroxidation in membranes of hemopoietic elements, and manifestations of granulocytopoietic disorders were established it children with lymphocytosis. Therefore these children can be attributed tot the group of increased risk on oncohematological disease.