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Biomedical subjects

G Kenet

Publications and source records attributed to G Kenet.

54 records · Page 3Linked to original sources

Two embryonal cancers after in vitro fertilization.

BACKGROUND: In vitro fertilization is not considered to be associated with an increased rate of pediatric malignancies, and only three have been reported in the literature. Two additional rare pediatric tumors in children conceived through this technique are reported. METHODS: Two children 12 and 18 months of age, developed hepatoblastoma and clear cell sarcoma of the kidney, respectively. They were both products of uneventful pregnancies induced by in vitro fertilization. No other environmental, prenatal, or family factor was found. RESULTS: The first child died after a failed remission induction with cisplatin, doxorubicin, and vincristine, whereas the second child is alive with no evidence of disease 18 months after diagnosis and treatment according to NWTS protocol. CONCLUSIONS: A possible association between in vitro fertilization and pediatric malignancies is suggested.

Antibiotics, Antineoplastic↗

Autologous umbilical cord blood transfusion.

The purpose of this study was to examine some aspects of umbilical cord blood collection for autologous transfusion in premature infants. All 120 microbacterial cultures (aerobic and anaerobic) of cord blood samples as well as 30 cultures of mycoplasma were treated. Cord prothrombin fragment (F 1 + 2) concentrations were quantified at one and 10 minutes after clamping of the cord. F 1 + 2 concentrations assessed on 25 newborn infants were similar and no linear association with time of clamping could be drawn. This means that cord blood thrombosis is not activated for at least 10 minutes following clamping of the cord. As far as is known, the first newborn infant to benefit from this method of transfusion is reported here. The premature infant received two portions of autologous blood (on days 5 and 7). No untoward effects were noted. Blood, collected from the umbilical cord, is a safe source for autotransfusion, provided that bacteriological testing has been carried out.

Blood Coagulation↗

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↗

Autologous cord blood transfusion.

Newborn piglets were exsanguinated (60% of blood volume) and retransfused 1 h later. One test group received adult pig red blood cells, the other piglet cord blood cells; controls were infused with plasma. While all controls died, satisfactory results were achieved in piglets transfused with either adult or foetal blood. The feasibility of collecting human cord blood for transfusion was assessed in 100 samples of human cord blood. Blood was collected aseptically and aerobic and anaerobic cultures set up. All samples of cord blood were sterile, and all were Mycoplasma negative. Coagulation parameters were analysed in eight cord plasma samples stored at -20 degrees C for 45 days. No significant abnormalities were found immediately after birth or after storage.

Animals↗

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↗

Lymphomatous T-cell leukemia in two Arab children. Is there a role for an environmental effect.

Two Arab children from the Gaza strip presented with fever, weakness, hepatosplenomegaly, lymphadenopathy and leukocytosis. The peripheral and bone marrow blasts had an immunophenotype compatable with T-cell acute lymphoblastic leukemia, and exhibited unusual markers (CD2+, CD3+, CD4-, CD8-). Cytogenetic studies revealed t(8;14)(q24;q11), possibly involving the alpha/delta locus of the T-cell receptor gene on chromosome 14 rather than the immunoglobulin heavy-chain locus usually involved in the t(8;14)(q24;q32), which is typical for Burkitt's leukemia/lymphoma. One of the children had a brother who died of T-cell acute lymphoblastic leukemia a few years later, however, his blasts showed deletion of chromosome 12. The possible role for environmental factors associated with low socioeconomic status, as well as of genetic factors in leukemogenesis are discussed.

Child, Preschool↗

Fatal Shigella sepsis in a neutropenic patient.

Bacteremia during infection with Shigella is relatively rare and usually self-limited. Bacteremia during shigellosis bearing a high fatality rate has been reported in young infants and in persons with malnutrition or with the acquired immunodeficiency syndrome. We report a case of Shigella sonnei septicemia in a severely neutropenic patient who had fever, abdominal pain, diarrhea, malnutrition, and dehydration. She died after five days despite intensive care. We emphasize that Shigella should be considered among the possible pathogens causing sepsis in neutropenic patients.

Adult↗

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↗

Fluorescent in situ hybridization for the detection of t(8:14) in Burkitt's lymphoma.

Burkitt's lymphoma cells exhibit the reciprocal balanced chromosomal translocation t(8:14) in 75% of patients. Cytogenetic analysis is time-consuming, requires in vitro culture with mitogens and enables to analyse a relatively small number of cells. We evaluated the role of multicolor fluorescent in situ hybridization (FISH) in the rapid detection of t(8:14) in Burkitt's lymphoma cells. FISH detected the JH/myc translocation in 100% of the cells of five cell lines carrying the classical t(8:14) and in fresh cells obtained from a newly diagnosed Burkitt's lymphoma patient. In contrast, the translocation was not detected in the Bajb cell line that does not carry t(8:14). We conclude that FISH is a rapid and reliable diagnostic tool for the detection of the JH/myc translocation in Burkitt's lymphoma patients.

Biopsy, Needle↗

Treatment of systemic lupus erythematosus by prolonged administration of high dose intravenous immunoglobulin: report of 2 cases.

Two patients with life threatening manifestations of systemic lupus erythematosus (SLE), unresponsive to corticosteroid and immunosuppressive therapy, were treated with high dose intravenous immunoglobulin (IVIG). Following IVIG therapy, lupus pneumonitis and encephalitis in the first patient, and lupus nephritis in the second patient, resolved. Continuous treatment with IVIG, every 4 weeks for up to 20 months induced a prolonged clinical and laboratory remission. Treatment with cytotoxic agents was stopped, and the dosage of corticosteroids lowered. Exacerbation of lupus nephritis occurred in the second patient after 10 months of IVIG therapy. We suggest that prolonged use of high dose IVIG may be a useful therapy for acute exacerbations of SLE and for inducing prolonged remissions.

Adult↗

Bromobenzene accelerates hepatocyte streaming in rats.

Sixty male adult rats weighing between 250 and 300 g were divided in two equal experimental groups. The first experiment was designed to evaluate hepatocyte streaming. Animals were injected with 0.5 microCi [3H]thymidine and 1 hour later received one intraperitoneal injection of bromobenzene (3.8 mmol/kg, dissolved in corn oil). The animals were then killed in groups of five at 1 hour and 2, 4, 7, 14, and 30 days. The aim of the second experiment was to evaluate labeling index changes with time. Animals received one intraperitoneal injection of bromobenzene and were killed in groups of five at 1, 2, 3, 4, 7, and 14 days. They received [3H]thymidine 1 hour before killing. Bromobenzene induced a necrosis in the third acinus zone that disappeared within a week. On day 0 the labeling index of hepatocytes and littoral cells was 0.3% +/- 0.04% and 0.4% +/- 0.05%, respectively. On the second day, it reached 14.6% +/- 2.6% and 10.1% +/- 3.1% and returned to its initial value after 1 week. Dead cells in the third zone were replaced by inflowing cells from the intact zones. Hepatocytes and littoral cells streamed at the same velocity of 6 +/- 0.5 micron/day, faster than in untreated animals with velocity of 3.2 microns/day. Parenchyma and stroma responded to injury in a coordinated fashion. From the functional point of view, hepatocytes and littoral cells operate as a unit that is called proliferon. The maximal proliferon life span was 57 days after bromobenzene treatment and 108 days in controls.

Animals↗

Infantile idiopathic thrombocytopenic purpura.

Idiopathic thrombocytopenic purpura (ITP) in childhood is a benign disease, as only 10% to 20% of the patients have a chronic course. A retrospective study of 57 ITP patients ranging in age from four months to two years revealed that 30% of them proceeded to chronicity. Unlike ITP in the general pediatric population, chronic infantile ITP was characterized by male predominance, a high frequency of preceding viral infections, and lack of responsiveness to any of the known modalities of treatment.

Child↗

The 1226G (N370S) Gaucher mutation among patients with Legg-Calve-Perthes disease.

Legg-Calve-Perthes disease (LCPD) is an avascular necrosis of the femoral head with an annual incidence of 5-15/100,000. The estimated incidence of Gaucher disease, a lysosomal recessive storage disease, is 1:850, with a carrier rate of 1:17.5 for the 1226G (N370S) mutation among Ashkenazi Jews in whom there is a predilection. Since clinical and radiological findings of avascular hip necrosis due to either Gaucher disease or LCPD may be indistinguishable, misdiagnosis may occur. The purpose of this study was to evaluate the incidence of 1226G Gaucher mutation in a cohort of radiologically confirmed LCPD patients (diagnosed 1986-2000) in Israel. Enzyme assay was performed for confirmation of affected versus carrier status in patients with the 1226G mutation. In all, 78 LCPD patients, 86% males, 51% with severe bone disease, were studied. Family history was negative for Gaucher disease. Ethnic origin was 39% Ashkenazi Jewish, 6% Arab, and 55% other ethnicities. One Ashkenazi Jewish LCPD patient was homozygous for the 1226G mutation, and 4 LCPD patients were carriers: 3 Ashkenazi Jewish and 1 Arab patient. The frequency of the 1226G mutation among the LCPD patients was increased relative to historical Ashkenazi Jewish Israeli controls (P = 0.01). Since Gaucher disease may be misdiagnosed as LCPD, glucocerebrosidase enzyme testing is recommended among Ashkenazi Jewish children diagnosed with LCPD.

Clinical Enzyme Tests↗

Chromosomal translocation (1:13) in a case of alveolar rhabdomyosarcoma.

PURPOSE: To describe a patient with a variant translocation (1;13)(p36;q14) in an alveolar rhabdomyosarcoma and compare the clinical course with four other cases. PATIENTS AND METHODS: A 10-year-old girl presented with multiple masses involving the thigh, abdomen, chest wall, and scalp with pleural effusion and edema of the lower extremities. RESULTS: A bone marrow biopsy, aspirate, and biopsy of the thigh mass all showed tumor invasion. Histopathology and cytogenetics of the thigh mass revealed an alveolar rhabdomyosarcoma with a t(1;13)(p36q14) variant. There was no response to aggressive therapy and the patient died within 3 weeks of admission. CONCLUSION: Variant t(1;13)(p36;q14) has now been described in 5 cases of rhabdomyosarcoma, and may define a subset of patients with extensive disease at diagnosis unresponsive to current therapeutic modalities.

Child↗

Helicobacter pylori-associated gastric lymphoma in a girl.

We present a case of a 14-year-old girl with gastric large cell lymphoma. The girl's lymphoma was characterized by the presence of mucosa-associated lymphoid tissue. Infection with Helicobacter pylori (HP) was ascertained at the time of diagnosis. The girl was successfully treated by a combination of chemotherapy (MACOP-B) and anti-HP drugs (omeprazole plus amoxicillin). Thrombus of the inferior vena cava, a rare complication, evolved during treatment.

Adolescent↗