Wilson disease with thrombocytopenia (case report).

We present an adolescent patient with WD accompanied with secondary amenorrhea, and thrombocytopenia. NK, a 14 year-old girl, had amenorrhea for 5 months despite having had regular menses for 2 years. An abdominal ultrasound scan revealed ascitis and some ovarian cysts. On physical examination: slight jaundice, edema of lower extremities, skin purpuric rash, enlarged abdomen, dry skin. She had no hepatomegaly and no splenomegaly. Breast and pubic hair development was concomitant with Tanner stage 4. There was performed laboratory and instrumental investigations. The patient was diagnosed as WD owing to the low level of ceruloplasmin, with increased level of copper in 24-hour urine excretion and in dry liver tissue. The needle biopsy of liver showed severe hepatocellular necrosis, inflammatory changes and fibrosis. The platelet count was found to be low with lack of increased number of megakaryocytes in the bone marrow aspiration suggesting the thrombocytopenia was not exclusively owing to hypersplenism. The absence of antithrombocyte and other autoimmune and viral antibodies excluded respectively the diagnosis of autoimmune thrombocytopenia, other autoimmune diseases and viral infections. Thus, we support the recommendation that adolescents with amenorrhea or children with thrombocytopenia without any obvious cause should be evaluated for WD, because the early detection and treatment of WD is capable of reversing described changes and restoring a normal liver function.

[Blood biochemical changes in maternally deprived children and their emotional status].

Environmental rearing conditions during the neonatal period are critical for the establishment of neurobiological factors controlling behavior and stress responsiveness. Early maternal deprivation in animals consisting of a single 24-h maternal deprivation episode during early neonatal life has been proposed as an animal model for certain psychopathologies including anxiety, depression and schizophrenic-related disorders. The aim of the present research was to show the mechanisms how the early maternal deprivation in humans influences the emotional status of children. To understand the effects of early deprivation on the regulation of the hypothalamic-pituitary-adrenal axis of children following social interactions, we examined the blood neurotransmitters levels (Norepinefrin NA; Dofamin DF; Serotonin SE) in a group of healthy institutionalized children at age from 6 to 36 months from Tbilisi Infant's House. A group of healthy children of the same age from Mother & child shelters formed the control group. Emotional status was assessed by Leusher's Color Test in a group of healthy institutionalized children at age from 3 to 6 years from Tskneti Orphanage. Institutionalized children showed elevations in blood NE and decreased SE and DF concentrations in comparison with non deprived children's blood. The higher was percentage of children with high level of anxiety in the group of children from orphanage than in the children with family care. The results suggest that early maternal deprivation may contribute to long-term regulatory problems of the stress-responsive system that may be resulted in altered emotionality and behavior in deprived children.

[Some indicators of children homeostasis, living in arsenic polluted environment].

Two groups of school children (7-16 years old) were studied. One child was from Lukhuni gorge, where arsenic was mined and processed for many years; 11 children were living far (>50 km) from the territory of arsenic mining. Pediatric monitoring revealed significant increase of general morbidity, particularly acute respiratory diseases. Cytogenetic, immunological and hematological investigations were conducted in 43 children (21 from group 1 and 22 from group 11). Simultaneously, the level of arsenic in blood and urine was defined. At all points the differences were revealed, that affirmed the impact of ecological factors on the organism and adaptative processes in children.

[Dependence of child health on the ecology].

The purpose of the work was to establish correlation between detected with the help of micronuclei in exfoliation cells infringements in the genetic disorder of Acute Respiratory Infection (ARI) at children living in Lukhuni gorge. Heavy metals having mutagenous trait can cause variety of diseases and first of all influence on the genetic apparatus, which damage often reflects chromosome change. The study of micronuclei in exfoliation cells is comparatively new, easily reachable and believable informative method. From Lukhuni gorge it is indicated true lifting amount of micronuclei. In a distance this indications where far less. It is investigated 150 children: 50 from Lukhuni gorge (I group) where the maintenance of arsenic is sharply raised in environment and 100 from the regions remote from Lukhuni gorge. Amount average quantity micronuclei in 1000 cells in I group was very high 22,38+/-1,26 in. In the second was 4,36+/-0,50 (t=12,82; p<0,0001). All children, under the anamnesis and the clinical data, have been divided on frequently were ill of Acute Respiratory Infection(in one year > 4-5 times) and seldom were ill of Acute Respiratory Infection (< 4-5 times). ARI most frequently were marked in this region. It is revealed correlation between amount of micronuclei in exfoliation cells and parameters of illness respiratory diseases at children living in territories, polluted with arsenic and his derivatives (R=0,789; p<0,000001).