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Biomedical subjects

L Timár

Publications and source records attributed to L Timár.

At least 19 recordsLinked to original sources
PubMed1998

Bone marrow transplantation in non-malignant disorders.

From January 1992 to December 1997, 21 consecutive patients (14 SAA, 3 SCID, 1 Fanconi anemia, 1 Diamond-Blackfan anemia, 1 mucolipidosis and 1 mucopolysaccharidosis type I.) were transplanted (16 HLA-id. family, 2 MUD and 3 haploidentical family donors) in a single center. The median follow up period is 41 months (range 7-76). The probability of 3.5 year overall disease free survival is 14/21 (67%), the transplant related mortality is 4/21 (19%). All the SCID patients are alive and disease free. 3 SAA patients had signs of fungal infection prior to transplant. They died in spite of intensive antifungal treatment resulting reduced DFS for SAA to 71%. Two patients with lysosomal storage disorders (mucolipidosis and MPS I.) rejected the haploidentical T-cell depleted graft 1 and 11 months after transplant, respectively. In 2 cases non-engraftment occured, both patients were retransplanted successfully.

Adolescent↗
PubMed1994

[Prophylactic and therapeutic use of immunoglobulins in patients with bone marrow transplantation].

Eleven children were transplanted in our BMT Unit. All but one received standard IVIG preparations in doses of 100 mg/kg b. w. regularly on days before and after transplantation -1, +14, +28, +60 and +90, respectively, and anti-CMV hyperimmune globulin (Cytotect) was given to six patients in the same doses, respectively. In spite of the severe immunodeficiency bacterial infections were verified only in four patients, and CMV infection in three. New infection only occurred in two of the three patients, who hadn't been given CMV prophylaxis, while in the group of six children having been given Cytotect prophylaxis only one became infected from endogenous reactivation of CMV. Therapeutic application of immunoglobulin compounds were used in four of our transplanted patients. Two of them suffered from sepsis during transplantation, one from protracted immunoneutropenia and one from CMV antigenaemia after the transplantation, respectively. Our conclusion is that the administration of immunoglobulins may contribute to the prevention of infections and to the treatment of some complications in BMT recipients. Anti-CMV immune globulin seems to be more effective than standard IVIG in the prevention of CMV infection.

Adolescent↗
PubMed1993

[Leukocyte adhesion defect--a rare form of congenital immune deficiency].

Leukocyte adhesion defect (LAD) is an inherited defect of phagocytic function. This disorder is characterised by delayed separation of the umbilical cord, severe recurrent bacterial infections, impaired formation of pus, and high leukocyte counts. The granulocytes have severe defect in their chemotactic mobility and endocytosis. The disease is attributed to the absence of the leukocyte adhesion molecules. (CD11/CD18), which can be verified with monoclonal antibodies. The authors describe the disease-process of the first patient diagnosed in Hungary. Perinatally the omphalitis, periumbilical abscess and periproctal abscess leading to rectovaginal fistula, in the first months the otitis, mastoiditis, and expressed leukocytosis referred to the impaired function of phagocytic cells, which was verified by laboratory tests as well. The decreased inflammation and cicatrization were also striking. This severe form of LAD can be cured only by bone marrow transplantation with preliminary sanitation of the foci of infection. It took about six months. Unfortunately, the patient died of sepsis immediately before transplantation.

Antibodies, Monoclonal↗
PubMed1991

The pattern of activation antigen expression on T-lymphocyte subpopulation in infectious mononucleosis.

The peripheral blood mononuclear cells of patients with infectious mononucleosis (IM) have been characterized by the determination of activation antigens using a panel of 11 monoclonal antibodies (MoAbs) belonging to 9 clusters. The activation of CD8+ peripheral blood mononuclear cells of IM patients was found to be determined by HLA-DR and CD45RO MoAbs. The antibodies of the CD30, CD40 and CD70 termed antigens also showed an increased expression as compared to the controls. In contrast, the CD25, CD69, CD71, and CD45 antigens were expressed at a low rate on the surface of the CD8+ cells. We found, on the other hand, a very low percentage of B lymphocytes in the peripheral blood, which reflects on the virus caused antigen modulation and on the effectivity of activated CD8 cells with the characteristic expression of the above outlined markers in IM. In six asymptomatic patients, the percentage of CD8+HLA-DR+, as well as of CD8+CD45RO+ cells proved to be lower than that in the active phase of the disease. The number of B cells showed normal value in the clinically asymptomatic cases.

Adolescent↗
PubMed1982

A prospective study on infectious mononucleosis in childhood--symptoms, serology, Epstein-Barr-Virus specific leukocyte migration inhibition.

The clinical course, serological changes and the development of the specific cell-mediated immune response to Epstein-Barr-Virus (EBV), measured in terms of leukocyte migration inhibition, were followed in 40 children suffering from an EBV infection. The patients were followed for between six and 24 months. Although the majority of the children were under six years of age, they presented a typical clinical course; heterophil antibodies could only be demonstrated in 60% of the cases. Anti-VAC-IgM and IgG antibodies were found in all patients during the acute phase, but no anti-EBNA could be demonstrated. In children under three years of age, no antibodies against the D component of the early antigen were found; this antibody was found in 50% of the adolescents. An antibody against the R component of the early antigen could be demonstrated in 73% of the children five to six months after the onset of the disease. Specific leukocyte migration inhibition was present only during convalescence or later. A relationship between the appearance of anti-EBNA and the development of specific leukocyte migration inhibition has been established.

Acute Disease↗