[Congenital disorders of hemostasis in children with Perthes disease]. / Wrodzone zaburzenia ukladu hemostazy u dzieci z choroba Perthesa.

The level of selected parameters of the coagulation system and fibrynolysis (prothrombin time, partial thromboplastin time, fibrinogen level, albumin C system, V Leiden factor and III antithrombin level) in 25 children who had been treated with Perthes disease was evaluated. In three children prolonged prothrombin time occurred; in one child anomalous protein C system was noted. The remaining parameters were normal in all children. The investigation results reveal that congenital disturbances of the haemostasis system were not the cause of Perthes disease in 24 children.

[Femur head necrosis in haemophilia and after prolonged steroid therapy--description of two cases]. / Jalowa martwica glowy kosci udowej w przebiegu hemofilii oraz po leczeniu hormonami kory nadnercza--opis 2 przypadków.

Clinical and radiological course of femoral head necrosis of known aetiology (in a boy, aged 8, with haemophilia and in a girl, aged 13, after prolonged steroid therapy) was compared with the typical course in children with Perthes disease.

[Diagnostic and therapeutic management of essential thrombocythemia in children]. / Postepowanie diagnostyczno-lecznicze u dzieci z nadplytkowoscia samoistna.

BACKGROUND: Essential thrombocythemia is a myeloproliferative disease resulting from the clonal proliferation of a multipotent myeloid stem cell with an increased platelet count in peripheral blood. In 2% of cases the cause remains unknown. It is then called idiopathic essential thrombocythemia. THE AIM: The objective of this study was to present the diagnostic and therapeutic management of essential thrombocythemia in children. MATERIAL AND METHODS: The authors analysed complete medical records of 5 patients treated for essential thrombocythemia in the Department of Children Haematology and Oncology, Medical University Lublin during a 6 years (1996-2002) period. The investigated group consisted of 5 children: 3 boys and 2 girls with mean age of 7 years and 8 months (varying from 5 years and 3 months to 11 years and 8 months). RESULTS: The peripheral platelet count was above 1200 x 10(9)/L. Three patients developed functional symptoms of microvascular blood flow alteration (headaches, sensory disorders. paresthesiae). In 2 patients thrombocythemia was asymptomatic and the diagnosis was made incidentally. Diagnosis of essential thrombocythemia was made according to the criteria of the Polycythemia Vera Study Group and treatment with anagrelide hydrochloride was started. One patient due to bad drug tolerance was treated with hydroxyurea. Treatment of essential thrombocythemia in all patients resulted in: (1) decreased (500-600 x 10(9)/L) platelet count in peripheral blood; (2) resolving the symptoms of small vessels obturation (headaches, sensory disorders, paresthesiae); (3) low platelet separating megakaryocytes count in control myelograms (4) no transformation into acute neoplastic disease. CONCLUSIONS: In the treatment of children with essential thrombocythemia anagrelid was effective in four of five patients.

[Clinical course, diagnostic and therapeutic management of immune thrombocytopenic purpura in children]. / Przebieg kliniczny, diagnostyka i leczenie dzieci z maloplytkowoscia autoimmunologiczna -- analiza kliniczna.

PURPOSE: Immune thrombocytopenic purpura (ITP) is a common paediatric disease. Diagnosis of acute ITP is based on a platelet count less than 20 x 10(9)/L, with normal haemoglobin concentration and white blood cells count and absence of underlying conditions such as lymphoproliferative disorders or lupus erythematosus. The objective of this study was to present the clinical picture, the diagnostic procedures and therapeutic management of immune thrombocytopenic purpura in children treated in the Department of Paediatric Haematology and Oncology, Medical University, Lublin. PATIENTS AND METHODS: The authors analysed complete medical records of 107 patients treated for immune thrombocytopenic purpura. The investigated group consisted of 39 boys and 68 girls with age varying from 2 to 17 years and 7 months. In 44 patients the peripheral platelet count was less than 20 x 10(9)/L, in 24 children the platelet count ranged between 20-30 x 10(9)/L, in others it varied between 30-50 x 10(9)/L. In 48 patients the physical examination revealed splenomegaly. RESULTS: l. In 72 patients the cause of thrombocytopenia was established and included: in 24 patients -- upper respiratory tract infection, in 16 patients -- viral infections (varicella, rubella, mumps), in 19 patients -- CMV infection, in 7 patients -- EBV infection, in 6 patients -- contact with toxic substances; 2. In 5 patients thrombocytopenia episodes were diagnosed at least twice; 3. Typical presentation of acute ITP was observed in all patients; 4. Splenomegaly was confirmed in 48 patients by diagnostic ultrasound. 5. Thirty patients recovered spontaneously without treatment; 6. Treatment of 65 patients resulted in complete recovery; 7. In 11 patients after 3 to 6 months of observation, splenectomy was performed. CONCLUSIONS: Therapeutic management of immune thrombocytopenic purpura in children treated in the Department of Paediatric Haematology and Oncology, Medical University, Lublin are comparable to the reports of Intercontinental Childhood ITP Study Group.

Urinary excretion of N-acetyl-beta-D-glucosaminidase and alpha1-microglobulin in children with proliferative blood diseases.

The purpose of the study was to estimate the urinary excretion of NAG and alpha-1M among children who suffer from proliferative blood diseases. The group of the examined children included those who went through a viral hepatitis (VH) and who are or were treated by means of cytostatic drugs. The study comprised 73 children aged from 4 to 18 (average 11.7+/-3.5. There were 70 children with the diagnosis of leukemia and 3 with the diagnosis of non-Hodgkin lymphoma. The examined group was divided according to the stage of treatment of a basic disease. Group I--22 children who are treated currently or whose treatment has been completed recently. Group II--51 children whose treatment was completed over two years ago. In group II there were 4 subgroups distinguished depending on positive antigenemia HBs and the presence of HCV antibodies. There were no clinical or biochemical features of damage of renal function observed among any of the children. The testing group consisted of 70 healthy children who were selected regarding age and sex. The urinary excretion of NAG and alpha-1M was estimated in the second morning portion of urine and it was presented as NAG/creatinine and alpha-1M/creatinine ratio. The results of the research underwent the statistical analysis by means of a t-Student test. It was stated that the urinary excretion of NAG and alpha-1M was higher among children who currently are or were treated by means of cytostatics drugs. It was also stated that the urinary excretion of NAG was higher among the children who went through viral hepatitis C in comparison with HBs antigen carriers. Similarly, the urinary excretion of alpha-1M was higher among children with positive markers of viral hepatitis B and C markers in comparison with a group of HBs antigen carriers.