ABSTRACT
Idiopathic thrombocytopenic purpura (ITP) of Childhood is a common hemorrhagic diathesis. The purpose of the present work is to summarize the results of 20-year observation over children suffering ITP, to record certain progress in diagnosing and treatment of this disease and to raise the problems still to be solved. The authors have been observing 181 ITP patients (girls 89, boys 92) at the age of 3 months up to 15 years. Childhood ITP mostly occurs at the age of 1 to 6 year i.e. in the so called "critical period" of immune system forming. Several peculiar cases were identified among infants and the children above 10 years old. Clinical manifestation of all the ITP patients was characterized with cutaneous hemorrhagic syndrome. 95% of childhood cases showed deep thrombocytopenia with the thrombocyte level <30X10 9/l. Most of the patients suffered acute form of ITP (87,9%). In the majority of cases illness took its normal predictable course after the glucocorticosteroid therapy. In heavy cases the combination of glucocorticosteroid and intravenous immunoglobulin is recommended. The indications of splenectomy were considered to be heavy, life -threatening, excessive bleeding, mainly in cases of chronic, steadily recurrent forms of ITP. Remissions were achieved in most of cases.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Immunoglobulins, Intravenous/therapeutic use , Purpura, Thrombocytopenic, Idiopathic/diagnosis , Purpura, Thrombocytopenic, Idiopathic/drug therapy , Adolescent , Child , Child, Preschool , Female , Georgia (Republic) , Humans , Infant , Male , Purpura, Thrombocytopenic, Idiopathic/etiology , Treatment OutcomeABSTRACT
Megaloblastic anemias are basically caused by vitamin B(12) and/or folic acid deficiency. Childhood vitamin B(12) deficiency is extremely rare. There are congenital and acquired forms of vitamin B(12)-deficiency anemias. The article captures findings of 10 year observation of 3 patients with Imerslund-Gräsbeck Syndrome (congenital chronic megaloblastic anemia with proteinuria), in which the diagnosis was established by us in early childhood and due to correct treatment and prevention complete clinical-laboratory remission is kept so far. We have also observed rare case of acquired megaloblastic anemia - 14 years old vegetarian patient, who was diagnosed with vitamin B(12)-deficiency anemia based on history, clinical and para-clinical data. It was caused by strict vegetarianism of the patient. Therefore first of all the diet was corrected. In 5 days of specific treatment with vitamin B(12) "reticulocyte crisis" was manifested (proving the correctness of diagnosis and treatment) and complete clinical-hematological remission was achieved in 2 weeks. The given cases are interesting as megaloblastic anemias in childhood are both rare and difficult to diagnose. In such cases timely diagnosis, treatment and prevention tactics should be based on cause-and-effect relation of disease.
Subject(s)
Anemia, Megaloblastic/diagnosis , Anemia, Megaloblastic/etiology , Vitamin B 12 Deficiency/complications , Vitamin B 12 Deficiency/diagnosis , Vitamin B 12/therapeutic use , Vitamin B Complex/therapeutic use , Adolescent , Anemia, Megaloblastic/drug therapy , Female , Humans , Vitamin B 12 Deficiency/drug therapyABSTRACT
beta-thalassemia is the most common monogenic hereditary blood disease in children. It is also considered to be the regional pathology for Georgia. The influence of iron metabolism disorder on metabolic processes taking place in erythrocyte membrane and their role in pathogenesis of beta-thalassemia, is very important until now. The aim of our research was to study the condition of oxidoreduction processes in RBC membranes on the background of iron metabolism disorder in children with beta-thalassemia. We observed 44 patients with beta-thalassemia aged 0.4-14 years. Iron, ferritin, malon-dialdehyde and catalase were evaluated. The carried out investigation revealed, that oxidoreduction processes in patients with beta-thalassemia, together with iron overload, is one of the factors in promoting further disorder of proliferation and differentiation processes in erythrone system and also in formation of ineffective erythropoiesis. The revealed changes in data of iron metabolism. malon-dialdehyde and catalase showed us the need for correction of this disorder. Pathogenetically there are good reasons to include in the combined treatment beta-thalassemia the membrano-protective preparations (vitamin E, acetylcysteine) together with the hemotransfusion and chelator therapy.
