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Biomedical subjects

R Simkó

Publications and source records attributed to R Simkó.

6 recordsLinked to original sources

[Polycytemia vera in an 11-year old girl].

The case of an 11 year old girl with three line type of polycythaemia vera, with 4 cm splenomegaly and a plethoric complexion is presented. Peripheral blood values were as follows: RBC: 7.75 x 10(12)/l, Hb: 18.8 g/l, WBC: 15.2 x 10(9)/l, platelets: 920 x 10(9)/l. Serum erythropoietin level: < 1 mU/ml. In vitro, erythroid colonies developed from the bone marrow in the absence of added erythropoetin. For three years the haematocrit value has been controlled by regular venesections. Since extreme thrombocytosis developed, the treatment was continued with interferon alpha. Different treatment protocols are discussed.

Age Factors↗

Interferon-alpha in childhood haematological malignancies.

The application of cytostatics has brought about a breakthrough in the treatment of childhood haematological malignancies in the past 20 years. Chemotherapy appears to be least successful in the rare, low and very high mitotic index diseases, which often have an enormous tumour-burden. The suitability of chemotherapy in minimal residual leukaemia is also of some doubt. In these situations a 'conservative' treatment may be more appropriate. Because interferon-alpha has a distinct mechanism of action, and a broad-spectrum haematopoietic inhibitory activity, it is relatively nontoxic and noncancerogenic, and it may have a role in the treatment of malignant haematological disorders, either as a mono- or combination therapy. The exact indications and dosages for interferon in childhood malignancies are far from clear. Up to now, it has proved to be most efficacious in small tumour masses, providing a theoretical basis for application in minimal residual disease. Controlled clinical data, however, are not yet available. It remains to be determined whether or not interferon can be added to current chemotherapy protocols without a significant reduction of dose. Hopefully, a deeper understanding of the activities of interferon will allow us to plan better trials with combination treatments.

Antineoplastic Agents↗

[Menkes disease].

The Menkes or kinky hair disease is a rare, sex-linked systemic disorder of the copper metabolism. It is lethal in the first three years due to cerebral and cerebellar degeneration. Apropos of their case the authors summarise the knowledge on the diagnosis, pathogenesis and therapy.

Brain↗

[Progression of cutaneous T cell lymphoma in leukemia].

Transition of a (probably primary) cutaneous non-Hodgkin's lymphoma to T-stem cell leukaemia was observed in a case. Unusual clinical features, histological and laboratory data hampered the diagnosis-making of this rare disease. Using the ALL-BFM 90 HRG protocoll complete remission was achieved. Under the treatment varicella-zoster virus encephalitis took place.

Child↗

[Nephropathy in patients treated with ifosfamide].

Between 1987-1992 in the Department of Pediatric Oncology of Child Health Center at Miskolc the appearance of nephropathy caused by high dose Ifosfamide was studied (using different protocols) in patients with childhood malignancy. From 7 patients in 5 children nephrotoxicity were developed. In consequence of Ifosfamide rickets 2 patients are given continuous bicarbonate and calcitriol substitution. The authors call attention to the frequency of Ifosfamide nephropathy, low serum bicarbonate and phosphate concentration with glucosuria, aminoaciduria and hypochloremic acidosis were the manifestations of the disturbances of renal tubular functions. Prospective monitoring of these serum and urinary abnormalities may lead to early detection of tubulopathy and allow early replacement therapy.

Acidosis, Renal Tubular↗

[Therapeutic problems in disseminated histiocytosis X].

The authors are dealing with the therapy problems of disseminated Langerhans cell histiocytosis on the basis of seven patients. In the treatment the combination of Vinblastine and Prednisolone was usually applied. The therapy was successful in four cases. The most significant prognostic factors were found to be the presence of organ dysfunction (mainly hepatic and pulmonary), the bone involvement and immunological parameters, respectively. Real therapy goals are stressed.

Female↗