PubMed Health⌕ Search

Biomedical subjects

G Kende

Publications and source records attributed to G Kende.

At least 19 recordsLinked to original sources
PubMed1994

Familial clustering of malignant germ cell tumors and Langerhans' histiocytosis.

Three sisters in a family with seven children whose grandmother had an ovarian embryonal carcinoma experienced development of malignant and a malignant-like situation in childhood. Two were diagnosed as having malignant germ cell tumors of the ovary, and the third was found to have Langerhans' histiocytosis. The two girls with germ cell tumor shared an identical human leukocyte antigen, whereas the sister with histiocytosis shared one identical haplotype with them. All three children have been treated successfully with chemotherapy and are doing well off of treatment.

Aged↗
PubMed1994

Congenital haemolytic anaemia associated with adenylate kinase deficiency.

Chronic haemolytic anaemia associated with adenylate kinase (AK) deficiency is very rare and only seven cases in five families have been described. We present six children of one family who are deficient of this enzyme and in three of them a combined G6PD deficiency was found. AK deficiency was transmitted by an autosomal recessive gene and heterozygous state was not accompanied by disease, whereas homozygously affected individuals present a congenital chronic non-spherocytic haemolytic anaemia with haemoglobin levels of 8-9 g/dl. Patients also deficient in G6PD suffer from a more severe haemolytic anaemia with haemoglobin levels around 6 g/dl. The AK-deficient children are also mentally retarded. Splenectomy performed in five of the six patients resulted in complete remission of the haemolytic process.

Adenylate Kinase↗
PubMed1994

Familial leukemia: description of two kindreds and a review of the genetic aspects of the disease.

We describe two kindreds of Arab ancestry characterized by multiple cases of acute lymphoblastic leukemia. Consanguinity and intermarriages were prevalent in the two families. Age, mode of presentation, characteristics of the leukemic cells, response to treatment and prognosis were remarkably similar among the patients. A short review of the literature on familial leukemia is given.

Child↗
PubMed1994

In vivo clonal evolution of pre-B to B-cell acute lymphoblastic leukemia in childhood.

Two cases are described that provide further evidence for clonal evolution in pre-B-cell acute lymphoblastic leukemia. Two infants, whose lymphoblasts at diagnosis were morphologically subtyped as L1 and immunophenotyped as HLA DR+, CD19+, CD10+/- and C mu-, were induced and maintained in remission. One child relapsed 6 months after initiation of therapy. This time his lymphoblasts had L3 morphology and immunophenotyping demonstrated the appearance of surface immunoglobulins. The second child relapsed 18 months after initiation of therapy with a lymphomatous picture. He also had peripheral and bone marrow blasts with L3 morphology and surface immunoglobulins. A lymph node biopsy showed diffuse small non-cleaved lymphoma with a 'starry sky' appearance compatible with Burkitt's lymphoma. Only one case with a similar clonal evolution has been reported in the literature, but no surface immunoglobulins were demonstrated. The significance of clonal evolution in these cases and its potential practical implications are discussed.

Clone Cells↗
PubMed1992

The management of pediatric brain tumors in a tertiary center.

Between January 1982 and January 1990, 120 newly diagnosed pediatric patients were treated at The Chaim Sheba Medical Center. Sixty three (52.5%) tumors arose in the posterior fossa and 57 (47.5%) appeared supratentorially. A wide variety of histologic subtypes was seen, each requiring tumor-specific treatment. The modern imaging techniques-CT and MRI-offered better planning of operation, treatment and follow up. All children with highly malignant tumors were treated with combination chemotherapy besides the 'conventional radiotherapy'. Since 1987 the "eight in one day" protocol has been used extensively pre- and post-irradiation. Five-year survival, varied significantly according to tumor type, location and stage. The average delay from presentation of symptoms to diagnosis was 6 months. A multidisciplinary approach has been used in the treatment, rehabilitation and follow-up of these children. In this study, the results of treatment are presented and the role of chemotherapy is discussed.

Adolescent↗
PubMed1989

Aplastic crisis due to human B19 parvovirus infection in red cell pyrimidine-5'-nucleotidase deficiency.

Two siblings with chronic hemolytic anemia due to red cell pyrimidine-5'-nucleotidase (P-5'-N) deficiency, presented within a few days of each other with a febrile illness and pancytopenia. The cause of the aplastic crisis was an acute infection with human B19 parvovirus (B19 HPV) as proven by immunoelectron microscopy and DNA hybridization. This is the first report on the association of B19-HPV-related aplastic crisis with P-5'-N deficiency.

5'-Nucleotidase↗
PubMed1988

Non-Hodgkin's lymphoma complicating acute lymphoblastic leukemia in remission.

Second malignancies occurring in patients cured of childhood acute lymphoblastic leukemia (ALL) are rare but increasingly recognized. There are only three reported cases of non-Hodgkin's lymphoma complicating ALL in remission. We report here another case of B-cell-type large-cell lymphoma, presenting in the uterus of a 24-year-old woman, 16.5 years after the diagnosis of ALL was made. Although immune markers of the ALL were not available, it was thought to be unlikely that the second malignancy was a relapse of the original tumor. Due to the long latency period and the minimal exposure to alkylating agents, the role of chemotherapy seems to be insignificant in this case. The development of a second malignancy could be a manifestation of the basic susceptibility to cancer.

Adult↗
PubMed1988

HLA-A11 is associated with poor prognosis in childhood acute lymphoblastic leukemia (ALL).

A possible association between HLA antigens, susceptibility or resistance to leukemia, and responsiveness to treatment has been studied in 144 patients with childhood acute lymphoblastic leukemia (ALL) and compared to other prognostic factors, i.e. white blood cell (WBC) counts, age at onset, sex, ethnic origin, and cell surface markers. All sequentially newly diagnosed children (97) comprised the group for the prospective study (PSG) and were followed for 6 years. The group included 37 patients classified as T-ALL, 41 as CALLA+, 27 as NULL, 12 as B and pre-B, and 27 unclassified patients, who were diagnosed before 1980. During the follow-up period, 45 patients of the PSG died. Forty-seven patients designated long-term survivors (LTS) have been followed 6-20 years after diagnosis, having completed a 3-5 year course of anti-leukemia therapy, and having remained disease free thereafter. High WBC counts at diagnosis and T-cell-surface markers were associated with poor prognosis, as were enthnic origin and specific HLA antigens. Thus, there was one (1) a significant increase in HLA-A30 and a decrease in HLA B-14 in the PSG Jewish patients; and (2) a complete absence of HLA-ALL in LTS while, in the PSG, 8 of 9 HLA-All-positive patients died during the follow-up period. This suggests that HLA-All is associated with poor prognosis in childhood ALL.

Adolescent↗
PubMed1987

PGI*3(Israel), a new, unstable allele in the phosphoglucose isomerase system.

Phosphoglucose isomerase (PGI) and 16 other biochemical genetic markers were studied in an Israeli-Arab family previously described for hereditary deficiency of adenylate kinase (AK) and glucose 6-phosphate dehydrogenase (G6PD). In this inbred family a rare PGI*3 allele was observed in 11 of 32 members tested, indicating an autosomal codominant inheritance. The electrophoretic mobility of this allele is similar to that of the PGI*3 allele found in Indian populations, but unlike the Indian allele, it has a very low specific activity and heat stability. This PGI*3 allele, designated PGI*3 (Israel), seems to be a different unstable mutation and along with AK and G6PD deficiencies seems to be associated with severe anaemia.

Alleles↗
PubMed1983

Results of treatment of high risk childhood acute lymphoblastic leukemia.

Sixteen children with high risk acute lymphoblastic leukemia (ALL) who had one or more of the following risk factors: white cell count over 50 X 10(9)/liter, mediastinal mass, age under 2 or over 10 years, extramedullary involvement, or T-cell markers, were treated by a new protocol. All attained complete remission and 11 are still in their continuous first remission for 6-53 months. High activity of adenosine deaminase (ADA) in the leukemic cells seems to be an independent risk factor, as in the high ADA level group, 4 out of 7 patients relapsed and died, while none of the 8 patients with low ADA levels relapsed or died.

Adenosine Deaminase↗
PubMed1982

Acute lymphoblastic leukemia subtypes in Israel: the Sheba medical center experience.

During the period from 1978 to 1981, 52 patients with ALL were diagnosed and treated at the Chaim Sheba Medical Center. Using standard cell markers to subtype the blasts, 49 of the patients could be classified: 16 were found to be T-cell ALL, 10 common ALL, five null ALL, four pre-B and 14 were partially characterized as non-B, non-T. Analysis of the series revealed two distinctive features: high prevalence (30%) of T-cell ALL among both Jews and Arabs and a high proportion, two-thirds, of high risk patients due to high initial WBC counts, unfavourable age or T-cell characteristics. The minimal incidence of ALL among the Gaza Strip Arab children during the study period is 4:100,000, which is close to the incidence in the Western world. During previous years the leukemia incidence in the Gaza Strip was very low while the most common lymphatic malignancies were Burkitt tumor and other non-Hodgkin lymphomas.

Adenosine Deaminase↗
PubMed1982

Pretreatment prognostic factors and hospitalization periods in childhood acute lymphoblastic leukemia.

Pretreatment prognostic factors and hospitalization periods were analyzed in 57 consecutive children who had acute lymphoblastic leukemia diagnosed between 1967 and 1977, and who were followed up for at least two years. We investigated possible correlations between white blood cell (WBC) count, organomegaly and mediastinal enlargement at diagnosis, as well as age and sex, with the length of first remission and survival. Children presenting with a combination of all of the following four risk factors--WBC count greater than or equal to 50,000/mm3, enlarged mediastinum, spleen and liver greater than or equal to 3 cm below the costal margin--comprised a poor-prognosis group, in which boys predominated. Age in this small group of patients had no correlation with prognosis. On the other hand, no specific risk factor was predictive for survival in the 48 children who had less than four of the risk factors. In this better-prognosis group, children less than 2 yr and greater than 10 yr had a higher relapse rate than those of intermediate ages, and girls had a significantly better prognosis than boys. These results indicate that age and sex are intercorrelated with the above risk factors, so that analysis of the effect of a single risk factor or even a combination of two factors can be misleading. In the light of these findings, an interpretation of the discrepancies in the literature is suggested. Of interest, too, is the poor prognosis and the high frequency of T-cell leukemia among Arab children. Finally, we stress the importance of day-care facilities that enable shorter hospitalization periods and improve the quality of life.

Antibiotics, Antineoplastic↗
PubMed1979

Blurring of vision: a previously undescribed complication of cyclophosphamide therapy.

Five children with cancer treated with high dose cyclophosphamide experienced blurring of vision. In three patients, the blurring lasted less than one hour and disappeared, abruptly, but in the other two, it was prolonged, lasting three and 14 days with gradual improvement. In all five complete restoration of normal vision occurred. We have not found other reports of this association in the literature.

Adolescent↗