[Cytochemical study of alveolar macrophages].
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Biomedical subjects
Publications and source records attributed to P Cvetković.
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Anthracycline antibiotic (Rubidomycin and Adriamycin) are often used for treatment of acute leukemia and variety of solid tumors. The use of greater doses of these agents is mostly limited by the damage of the cardiac muscle and by heart failure. The Rubidomycin cardiac toxicity analysis of children with acute leukemia has been considered in this paper. The results were obtained by investigating 53 patients who received this drug. They were classified in subgroups in relation to the total dose administered. Acute or chronic myocardial damage appeared in 6 children. The subgroup incidence of damage is directly proportional to the total dose administered.
Acute leukemias in infants, including the congenital and neonatal leukemia, present a number of unfavourable features. Age of the infant, hyperleukocytosis, outstanding organomegaly, early onset of the CNS leukemia are some of the factors causing this group of acute leukemias of childhood to be those with highest risk. The poor prognosis of the illness is further worsened by frequent rejection of cytotoxic therapy, often failure in inducing remission and its shortness. Two patients with acute lymphoblastic leukemia, aged 4 months, are presented. Clinical the hematologic remission was achieved in both by the application of the current therapeutic methods. Recurrence and CNS leukemia appearing 5 months after the remission resulted in death of one patient. The second patient also developed CNS leukemia 5 months after remission. It was treated and another remission was achieved, but the child died due to interstitial pneumonia.
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Occurrence of familial leukaemia confirms the influence of genetic factors in the appearance of malignant diseases. Such cases were described in one generation, in distant relations of two and three generations and in monozygotic siblings. Either the same type of leukaemia was recorded or different types. This is thought to be caused by pathological gene transmission in interaction with environmental factors which increase the tendency to malignant diseases in these families. Two cases of familial type of leukaemia are presented in the paper; in one family the illness appeared among members of one generation, but in the other in two generations. In both families it was a question of acute lymphoblastic leukaemia. Other types of malignancy were not found, nor was any environmental factor confirmed to have influenced cancerogenesis. Cytogenetic studies of the patients did not show any deviation from the normal kariotype. The illness evolution was fatal. This is the second report of familial leukemia in Yugoslav literature.
The results of the erytropoietin level determination in serum and urine of patients with congenital anemias are presented and compared to the results obtained in children with acute aplastic anemias. Three patients with congenital hypoplastic anemia Diamond-Blackfan, two with Fanconi's anemia, one with congenital pancythopenia with hyperplastic marrow and five patients with acute aplastic anemia were studied. The increased serum erythropoietin level was found in every patient whose blood hemoglobin was less than 12g%. Erythropoietin was detected in nonconcentrated urine when serum erythropoietin level was higher than 0,5 units/ml. The statistically significant negative correlation between the serum erythropoietin level and blood hemoglobin concentration was found. In two patients suffering from congenital anemias, in whome the significantly increased erythropoietin level (about 1.0 units/ml) was detected, increased ammount of hemoglobin F in peripheral blood as well as increased MCV--signs of so called "stress" erythropoiesis-were noted. The results presented, together with the results obtained by other authors, indicate that congenital anemias studied here are not due to the disturbance in erythropoietin production.
Results of the investigation of 3 children aged from 2 to 7,5 with diagnosed aplastic anemia of Fanconi type are presented. Two children were females and one child was a male. Their illness symptoms appeared at the age of one, three and four respectively. All three patients had similar symptomes: palenes, tiredness, epistaxis, appearance of hemorrhagic syndrome and delayed growth. The following anomalies were also present: small size, microcephaly, mandibular hypoplasia, high palate and malformation of the urinary tract. In one child ductus Botalli persistens was also revealed. The laboratory findings showed presence of pancytopenia of the blood and increased level of both iron and erythropoietin in the serum. The karytype of two children revealed several cells with broken chromatin and with polyploid and tetraploid cells. The meiogram showed presence of all cells in bone marrow but in lowered number. Celularity I.
The congenital erythroid hypoplastic anemia is a rare disease of the early childhood and is manifested through isolated hypoplasia of the red blood cells. The authors present a case of a mother and her daughter with a classical picture of the congenital erythroid hypoplasia appearing jointly with some anomalies. The mother was found to have triphalangia of both thumbs while the child had hexodactylion, the simian scar and hypogammaglobulinemia; Both mother and child were of small size (below 3 PC). This rare syndrome of joint anomalies and congenital hypoplastic anemia may show different expressivity of particular signs which is evidenced through the presented patients.
Within the group of diseases of immunodeficiency Wiscott-Aldrich syndrome can be excepted as one with well definical picture. Here presented are two patients with allness onset in the earliest childhood. Their chinical picture was a typical one showing often infections, thrombocytopenia and later appearance of eczema. Beside the dicreased number of the thrombocytes both patients had eosynophilia, changed immunoglobulines and lack of isohemogglutinin. One of them also showed low response to phytohemagglutinin and low values of the T and B rosettes. One of the patients lived until four years of age while the other one is aged five now and is in relatively good condition.
In their work authors have analyzed some epidemiologic and etiologic data that can be taken as the elements of risk in leukemogenesis. The investigations have been done retrospectively in 212 children suffering from acute leukemia and treated in Pediatric Clinic in Beograd. Certain factors of risk are particularly studied as: repeated viral infections, repeated use of antibiotics, diagnostic and therapeutic irradiation of children, familiar data on congenital anomalies and cancer and same harmful prezygotic and prenatal influences of possible significance. The authors also presented the results of their cytogenetic investigations obtained from 32 children. It is especially pointed out the significance of detailed data taken from patients suffering from these diseases.
43 children suffering of acute leucaemia were treated in University Children's Hospital Belgrade during the period of 1969- april 1975. 39 patients were treated as acute lymphoblastic leucaemia (90,7%), and 4 patients as acute nonlymphoblastic leucaemia (9,3%). Complete remission of 34 patients treated as ALL by protocol PARIS 06 was established in 94,1%. All 5 patients suffering of ALL, treated by protocol PARIS 01 LA 72 had complete remission. 85,7 patients treated by protocol 06 AL 66 survived one year. 53,5% patients survived two years, 14,2% three years, and 3,5% survived four years. These facts are not final, because 35% patients are still alive. From 5 patients treated by protocol 01 AL 72 4 patinets are in complete remission, but this period is too short to per mit any conclusion. Meadle survival time for patients suffering of ANLL is 7,5 months. One patient is in complete remission for already 9 months.
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