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Biomedical subjects

G Kriván

Publications and source records attributed to G Kriván.

12 recordsLinked to original sources
PubMed2006

Hungarian experience with Langerhans cell histiocytosis in childhood.

The Langerhans cell histiocytosis (LCH) in children is relatively rare and the long-term analysis of therapy results has not been done yet in Hungary. The aim of this study was to investigate the incidence, clinical features, prognostic risk factors, and treatment results of children's LCH in Hungary in a 20-year period. Children less than 18 years of age with newly diagnosed LCH in Hungary were entered in this study. Clinical data of all children with LCH were reported to the National Childhood Cancer Registry in Hungary from 1981 to 2000. The clinical files were collected and abstracted for information regarding age at diagnosis, gender, disease characteristics, treatment, and outcome of treatment. Median follow-up duration of surviving patients is 10.98 years. Between January 1981 and December 2000, 111 children under 18 years of age were newly diagnosed with LCH in Hungary. The annual incidence of LCH in children younger than 18 years of age was 2.24/million children. The male-female ratio was 1.36:1; the mean age was 4 years 11 months. Thirty-eight children had localized disease and in 73 cases systemic dissemination was found already at the time of diagnosis. Twenty-two patients were treated only by local surgery, 7 by surgery with local irradiation, and 5 children got only local irradiation. In 2 cases remission was achieved with local steroid administration. Seventy-five patients received chemotherapy. In the 20 years of the study 14 children died, 9 due to the progression of the disease. Sixteen patients had relapse with a mean of 2.16 +/- 1.29 years after the first diagnosis. Three patients with relapse got chemotherapy generally used in lymphoma and remission was achieved. The overall survival of all patients (n = 111) was 88.3 +/- 3.1% at 5 years and 87.3 +/- 3.2% at 10 and 20 years. Childhood LCH is a well-treatable disease and the survival rate is high. Even disseminated diseases have a quite good prognosis in childhood.

Adolescent↗
PubMed2005

Incidence of oropharyngeal candidosis in stem cell transplant (SCT) patients.

Eighty persons with haematological malignancies receiving stem cell transplantation (SCT) were examined over a 24 months period. Hyposalivation, a common complaint in patients treated by intensive chemotherapy and radiotherapy can predispose to oral candidal colonisation as well. This study was focused on correlation between the fungal colonisation of the oral cavity and the total unstimulated saliva flow rate of 80 patients with haematological malignancies before and after stem cell transplantation and in addition, on their oral health state. Despite the fact that prior to being involved in the transplantation programme, the patients were subjected to dental examination and decayed teeth were found in 20 out of 80 patients (25%). From the 2233 different oropharyngeal specimens fungi were isolated before conditioning from 16 patients (20%), and during aplasia from 19 patients (23.7%). Objective xerostomia (unstimulated total saliva flow rate < or = 0.1 ml/min) was detected in 28 patients (35%). Stem cell transplant patients with pretransplant mouth dryness had higher incidence of Candida albicans and other fungal colonisation than those with normal saliva secretion.

Adolescent↗
PubMed1998

Allogeneic bone marrow transplantation for acute leukemia in adults.

From September 1993 to August 1997, 24 consecutive adult acute leukemia patients (20 AML and 4 ALL) received allogeneic bone marrow transplant (21 sibling, 1 twin, 2 MUD). The probability of 3 year leukemia free survival is 19/24 (79%), the transplant related mortality is 2/24 (8%), the relapse rate is 3/24 (13%). The median follow up period is 34 months (range 7-51). Three AML patients with high probability of TRM received a special radiation-free conditioning regimen (mitobronitol/cytarabine/ cyclophosphamide) originally described by Kelemen et al in CML patients for decreasing transplant related complications. All the three patients are alive and disease free over 3 years.

Adolescent↗
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↗
PubMed1998

Autologous stem cell transplantation for malignant lymphomas.

From December 1995 to April 1998, 20 consecutive adult patients suffering from chemosensitive relapse of Hodgkin's or non-Hodgkin lymphoma (11 Hodgkin, 9 non-Hodgkin Lymphoma) received autologous stem cell transplantation. The median follow up period is 15 months (range 6-28). The overall survival is 18/20 (90 %), the event free survival is 13/20 (65%). None of the patients died of transplant related cause.

Adult↗
PubMed1997

[Chronic granulomatous disease (CGD): dysfunction of the neutrophil granulocyte NADPH-oxidase enzyme system].

Dysfunction of NADPH oxidase results in chronic granulomatous disease (CGD), a syndrome characterized by severe bacterial and fungal infections. Phagocytes of the patients are unable to kill ingested microorganisms which leads to the formation of granulomas and abscesses. Predominant pathogens are the catalase-positive bacteriae (Staphylococcus aureus) and some fungi (Aspergillus species). Infections of these patients should be treated by antimicrobial agents, which penetrate cells and kill pathogens inside. The aim of this study was to give a short description of the structure and function of the NADPH oxidase enzyme and to summarize the results obtained during the diagnostic of 10 patients with chronic granulomatous disease. Characterization of the disease was confirmed by mutation analyses.

Common Variable Immunodeficiency↗
PubMed1996

[The bone-marrow transplantation program in Hungary: report from the period 1990-1995].

The article summarises the statistical data of bone marrow transplantation (BMT) carried out in Hungary between 1990-1995 in yearly distribution. Since the first BMT up to the end of 1995, 168 BMT were performed. The number of transplantations since 1990 to our days was gradually increasing. As a result of this activity in the three transplantation centers (National Institute of Haematology and Immunology, St. László Hospital and County Hospital in Miskolc) 36 allogeneic and 12 autologous BMT were performed in 1995. Out of the allogeneic BMT cases, 14% of them were completed with unrelated, donors in the last three years. The most frequent indications for allogenic BMT are: chronic myeloid leukaemia (CML), acute lymphoid leukaemia (ALL), acute myeloid leukaemia (AML), myelodysplasia, severe aplastic anaemia. Child allogenic BMTs are carried out on pediatric patients in St. László Hospital in leukaemia, severe aplastic anaemia cases and children born with immunodeficiency. Autologous BMTs started in an organised way in 1995 for adult patients in cases of non-Hodgkin lymphoma, Hodgkin lymphoma and for children with solid tumour indication in the Miskolc Centre. BMT activity in Hungary compared with international data, especially within Europe, shows a significant drop behind. To calculate for ten million inhabitants, the optimal BMT activity should be between 100-200 transplantations (allogeneic and autologous BMT together) in 1994. Among the Central European countries Hungary and Poland fall most behind. Autologous BMTs in most countries of Europe are above of allogeneic BMTs in numbers as indication in cases of lymphoma and solid tumours (first of all mamma carcinoma) comes into focus. This summary emphasises the most important difficulties in connection with the development of the National BMT program.

Adolescent↗
PubMed1996

[Diagnostic value of C-reactive protein levels in children with bone marrow transplantation].

Serum quantitative C-reactive protein concentrations were measured in 16 bone marrow transplanted children at 202 occasions during and after the transplant period. Serum C-reactive protein concentrations were moderately increased in patients with viral and protozoon infections (5-67 mg/l). High values were measured in patients with bacterial and fungal infections. The C-reactive protein level was between 15-102 mg/l in Coag. neg. Staphylococcus sepsis, and 160-178 mg/l in Pseudomonas aeruginosa infection, when blood cultures were positive. Values of 154-358 mg/l was found with Candida sepsis. C-reactive protein levels were 10-17 mg/l in 7 acute GvHD episodes, only one of the patients had high level (325 mg/l) in GvHD. In these cases the condition was very severe and affected the total surface of the skin and the gastrointestinal tract also. C-reactive protein becomes a valuable aid as laboratory parameter in the diagnosis of bone marrow transplant recipients with suspected bacterial infection and in monitoring of therapeutic efficiency.

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

[Hyperimmunoglobulinemia E (Job) syndrome].

A patient with hyperimmunoglobulin E (Job's) syndrome is presented. The authors review the clinical and immunological characteristics of the disease and sum up the different explanations for the pathogenesis of the syndrome.

Anti-Bacterial Agents↗