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Biomedical subjects

R Zaizov

Publications and source records attributed to R Zaizov.

At least 127 records · Page 7Linked to original sources
PubMed1986

Successful non-neurotoxic therapy (without radiation) of a multifocal primary brain lymphoma with a methotrexate, vincristine, and BCNU protocol (DEMOB).

A 3.5-year-old boy with a multifocal primary lymphoma of the brain was treated successfully without neurotoxicity with a treatment regimen that did not include radiation. The protocol of Dexacort (dexamethasone), methotrexate, Oncovin (vincristine), and BCNU (carmustine) (DEMOB), which was developed with the use of MTX pharmacokinetic studies, was given over 7.5 months, and resulted in tumor disappearance on computerized tomography scans and marked improvement in clinical status. The patient remains in good health 3 years after diagnosis (March 1985).

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Effect of splenectomy on destructive bone changes in children with chronic (Type I) Gaucher disease.

The incidence and severity of osteolytic bone changes in patients with chronic (Type I) Gaucher disease splenectomized in the first decade of life were compared to those in patients of the same age group and similar degree of severity of the disease in whom the spleen remained intact at least until the second half of the second decade. The size of the spleen, measured by palpation, was used as an index of severity. In the splenectomized group osteolytic changes appeared within a few months following splenectomy in six out of eight cases. The changes were severe in five cases and moderate in one. In contrast, in the non-splenectomized group, evidence of bone destruction was found in two out of eight patients and classified as mild in both cases. Furthermore, in three patients in this group, who remained free of bone destruction until splenectomy in the second half of the second decade, osteolytic lesions appeared soon after the operation. Children with chronic Gaucher disease can be spared a great deal of suffering caused by bone disease, if splenectomy is avoided or postponed as far as possible.

Bone Resorption↗
PubMed1986

Long-term culture of infant leukemia cells: dependence upon stromal cells from the bone marrow and bilineage differentiation.

Infant leukemia cells with 46XY,t(11; 17)(q23; p13) karyotype and a hybrid pre B myeloid phenotype (HLA-DR, (Ia), B4 and My7-positive and CALLA and T11-negative) and immunoglobulin heavy chain gene rearrangement were maintained in long-term culture for over 10 months. The in-vitro survival and growth of the leukemia cells were strictly dependent upon the presence of their autologous marrow stromal cells. The latter could be replaced by the 14F1.1 clone of preadipocytes derived from mouse bone marrow. Neither heterologous human marrow or foreskin fibroblasts nor fibroblast or endothelial like cell lines from mouse stroma could mimic the effect of autologous stroma or 14F1.1 adipocytes. The leukemia cells maintained their original phenotype throughout the 10-month culture period with either their autologous stroma or the 14F1.1 adipocytes. They could be induced to differentiate in two distinct directions. Phorbol myristate acetate induced adherence of the leukemia cells and development of macrophage properties. In contrast, conditioned medium from a hybridoma producing B-cell growth factor caused aggregation of the leukemia cells and expression of CALLA antigen and surface IgM. This bipotency of the leukemia cells and their dependence upon marrow stroma are properties in common with stem cells.

Bone Marrow Cells↗
PubMed1986

Partial splenectomy in Gaucher's disease.

In 11 children with hypersplenism due to Gaucher's disease, partial splenectomy was planned with the aim to prevent the development of postsplenectomy sepsis and also to slow the advance of the disease in the rest of the reticuloendothelial system by permitting continuing accumulation of the beta-glucocerebroside in the remaining splenic tissue. In seven children, partial splenectomy was performed successfully, the weight of the splenic tissue removed ranging from 400 to 3,680 g. The postoperative course was uneventful and the average duration of hospitalization was 12 days. In subsequent follow-up, isotope scanning demonstrated continuing growth of the splenic remnant and there were no episodes of postsplenectomy sepsis nor evidence of increased accumulation of beta-glucocerebroside in the liver or bones. These children showed a marked improvement in the growth curve and dramatic improvement in the hematologic picture. Of the four remaining children, in two, partial splenectomy was followed by complete removal of the remaining spleen due to necrosis, whereas in two, total splenectomy was performed since the huge spleens were extensively infarcted. Our experience suggests that partial splenectomy is the treatment of choice in the management of young patients with hypersplenism due to Gaucher's disease.

Adolescent↗
PubMed1986

Genetic heterogeneity in Gaucher disease.

Considerable clinical variability occurs in adult Gaucher disease type I and three main subtypes may be delineated: a very mild form, a severe form, and a moderate form which itself presents various clinical manifestations. A study based on 25 families from our clinic and a review of published reports showed that when both parents were heterozygous and more than one child was affected with Gaucher disease type I, there was always intrafamilial similarity concerning the three subtypes. In families where one parent and at least one child were affected, variability in the clinical subtype of Gaucher disease type I might occur among the affected members of the family. We propose that the three different clinical subtypes of this disease reflect the genetic heterogeneity of two alleles, G1a and G1b and the three corresponding genotypes represent the three different subtypes of the disease.

Gaucher Disease↗
PubMed1986

Differential leukocyte count in infants of diabetic mothers. Increased band count associated with macrosomia.

The differential leukocyte count was studied within the first 24 hours of life in 115 infants of diabetic mothers (IDMs) appropriate for gestational age (AGA), 16 IDMs large for gestational age (LGA), 104 infants of non-diabetic mothers (INM's) AGA, and 22 INMs-LGA. A significant "shift to the left" was found in IDM's-LGA only. The usual cause of "shift to the left" such as maternal hypertension or fever, respiratory distress syndrome, meconium aspiration, neonatal asphyxia, sepsis, convulsions, or hypoglycemia could not explain this finding. It is hypothesized that increased glucocorticoid secretion may possibly play a role.

Adult↗
PubMed1986

Neonatal anti-Kell isoimmune hemolytic disease with spherocytes.

Two infants with hemolytic disease of the newborn due to Kell incompatibility are described. The peripheral blood smears revealed many spherocytes. This important hematologic sign in a neonate suggests not only ABO incompatibility, hereditary spherocytosis, or G6PD deficiency, but also the possibility of Kell incompatibility.

Blood Group Incompatibility↗
PubMed1986

In vitro restoration by interleukin-2 (IL-2) of the impaired natural killer cell activities, IL-2 receptor expression, and T cell proliferation in hemophilia.

Five of 22 hemophiliacs who were seropositive for human T cell leukemia virus III (HTLV III) and manifested severe impairment of immune parameters (both in vivo and in vitro) similar to those observed in patients with clinical symptoms of acquired immune deficiency syndrome (AIDS) were chosen for this study. Profound lymphopenia was observed in four of five patients with decreased and qualitatively impaired helper/inducer (T4) cells and increased T suppressor/cytotoxic (T8) cells. Observed in all patients was impaired endogenous production of interleukin-2 (IL-2), expression of the IL-2 receptor combined with diminished responses to mitogens, mixed leukocytes reaction (MLR), and natural killer (NK) reactivity. In vitro supplement of exogenous IL-2 markedly augmented T and NK cell functions, as well as the expression of activation antigens on both T4 and T8 cell in four of five patients. Our findings suggest that a substantial proportion of this cell-mediated immunologic defect in hemophiliacs stems from their inability to produce adequate amounts of IL-2. Interleukin-2 may therefore have the potential for therapy as an immune response modifier in patients with hemophilia by providing beneficial preventive therapy for patients at risk.

Acquired Immunodeficiency Syndrome↗
PubMed1985

Expression of extra class I major histocompatibility antigens on T-cell acute lymphoblastic leukemia (ALL) lymphoblasts.

The presence of extra HLA antigens has been demonstrated, serologically and biochemically, on the surface of lymphoblasts from a patient with acute lymphoblastic leukemia of the T-cell subtype (T-ALL). Family analysis of this patient revealed the presence of the expected antigens, plus an additional HLA antigen (A24) which could be demonstrated by cytotoxicity on the lymphoblasts. Absorption studies revealed that the lymphoblasts had the ability to remove cytotoxic antibodies from alloantisera; similarly, absorption of these alloantisera with normal cells removed the reaction against the extra antigen from the lymphoblasts. The extra HLA molecules were also demonstrated by one-dimensional IEF. Two heavy chain-like molecules, together with the beta 2m subunit, were obtained after removal of appropriate antigens from externally labeled leukemia cells by the use of monoclonal antibody W6/32, which detects a class I specific determinant. The pI of the one heavy chain was shown to be very similar to that of the serologically detected A24. Our data thus suggest that the extra antigens detected by serological reagents may have been due to activation of silent class I MHC gene(s) at the protein level.

Adolescent↗
PubMed1985

The use of the portal system for the transplantation of a neonate kidney graft in a child with Wilms' tumor.

A single an-encephalus neonate kidney graft was transplanted into the portal system of a 6-year-old recipient who had previously undergone removal of the right kidney and inferior vena cava because of Wilms tumor. The left kidney ceased to function shortly thereafter. The child was supported very poorly on hemodialysis, and showed repeated very high levels of cytotoxic antibodies in her serum. The first cross-negative kidney graft that was available harbored two main arteries and duplicate collecting system with two very thin ureters. These vascular anatomic and pathologic variations of both donor graft and recipient necessitated the use of the portal system for renal graft venous drainage and the aorta for the graft revascularization. The ureters that had pinpoint-like lumen were inserted together into the lumen of the native ureter stump and fixated. One year after the transplantation the serum creatinine level is 1.8 mg/dL.

Aorta↗
PubMed1984

The epidemiology of childhood acute lymphoblastic leukemia and non-Hodgkin's lymphoma in Israel between 1976 and 1981.

n epidemiologic survey of childhood acute lymphoblastic leukemia (ALL) and non-Hodgkin's lymphoma (NHL) occurring in Israel, Judea, Samaria and the Gaza Strip between the years 1976 and 1981, revealed 205 cases of ALL and 69 of NHL. The mean annual incidence of lymphatic malignancies was 3.1/10(5) in the Israeli Jews, 2.3/10(5) in the Israeli Arabs and 2.5/10(5) in the Gaza Strip. In the Jewish population there was a peak in the incidence of lymphatic malignancies at the 2-5 years age group, while in the Israeli Arabs this was less prominent. There were no significant differences in the incidence or type of lymphatic malignancies in the various Jewish or Arab groups but there was a trend for a high leukemia to lymphoma ratio (LLR) in the patients from the Gaza Strip. A relatively higher LLR was observed in families of a high socioeconomic status, but it did not reach statistical significance. T-cell ALL comprised about a third of the typed ALL cases. A high proportion of the patients with ALL belonged to the high-risk category: 46% of the Jewish children and 76% of the Gaza Strip children. White blood cell count above 100,000/mm3 were found at presentation in 36.7% of the Gaza Strip patients but only in 9.4% of the Jewish patients. In spite of that, the survival at 4 years of the Jewish and Arab patients was similar. However, the patients with T-cell ALL had a significantly worse survival than the standard risk or the non-T high-risk group: 43.3 +/- 9.7, 66.6 +/- 7.1 and 63.6 +/- 10.4%, respectively. Compared to a previous study conducted in this country in the sixties it appears that the epidemiologic differences that were observed at that time between the various Jewish ethnic groups have practically disappeared.

Adolescent↗
PubMed1984

Expression of HLA-DR alloantigens on acute lymphoblastic leukemia lymphoblasts.

We have described the expression of HLA-DR alloantigens on the surface of lymphoblasts from patients with acute lymphoblastic leukemia (ALL). The patient groups included 49 acute lymphoblastic leukemia (ALL) patients (15 common ALL [CALLA +]; 11 "Null" ALL [CALLA-]; 19 T-Cell ALL; 4 Pre-B ALL, and one patient with hairy cell leukemia (HCL). Thirty one of these patients, who exhibited Ia-like antigens demonstrable by monoclonal antibodies, expressed HLA-DR utilizing alloantisera to Class II histocompatibility alloantigens (25/26 non-T-ALL; 2/4 Pre-B ALL; 3/19 T-ALL, and one HCL). The expression of HLA-DR on lymphoblasts was confirmed by family studies of five patients, indicating that ALL lymphoblasts can be used to perform HLA-DR phenotyping or genotyping of such patients. Another important finding was the coexpression on T-cell ALL lymphoblasts of markers for T-helper, T-supressor/cytotoxic, and thymic differentiation marker T6, together with Ia-like and HLA-DR, in one patient, and markers for T-helper, T6, and CALLA in another patient.

Adolescent↗
PubMed1984

Impaired immune regulation in children and adolescents with hemophilia and thalassemia in Israel.

The immune function was assessed in 22 children, adolescents and young adults with asymptomatic hemophilia, and 15 with thalassemia, in Israel. Five patients with hemophilia and two with thalassemia were found to be severely abnormal, having cutaneous anergy, very low T-helper cells, elevated T-suppressor cells, inverted T-helper/suppressor ratio, reduced response to mitogens and antigens, and nonfunctional NK cells. Four of the five hemophilia patients exhibited profound lymphopenia also. Decreased T-helper and mildly elevated T-suppressor cells with inverted T4/T8 ratio were observed in the hemophiliacs as a group. In the severe group, the reduction in T-helpers and T4/T8 ratio was more pronounced. The thalassemics as a group were found to have increased numbers of T-suppressor cells with decreased T-helper cells in those with intact spleen only. Both groups studied were found to have elevated IgG levels and low natural killer (NK) activity and normal response to mitogens. Cutaneous anergy was found to be a reliable indication for severe T-cell dysfunction and may serve as an early indication of impending AIDS. These results indicate that patients with hemophilia and with heavily hypertransfused thalassemia may be at increased risk of AIDS as they approach adolescence.

Acquired Immunodeficiency Syndrome↗
PubMed1984

Brain sarcoma of meningeal origin after cranial irradiation in childhood acute lymphocytic leukemia. Case report.

The authors report their experience with an unusual case of intracerebral sarcoma of meningeal cell origin in an 8 1/2-year-old girl. This tumor occurred 6 1/2 years after cranial irradiation at relatively low dosage (2200 rads) had been delivered to the head in the course of a multimodality treatment for acute lymphocytic leukemia. The tumor recurred approximately 10 months after the first surgical intervention. Macroscopic total excision of the recurrent growth followed by whole-brain irradiation (4500 rads) failed to eradicate it completely and local recurrence prompted reoperation 18 months later. This complication of treatment in long-term childhood leukemia survivors is briefly discussed, as well as the pathology of meningeal sarcomas.

Brain Neoplasms↗
PubMed1983

Effect of polar organic compounds on leukemic cells. Butyrate-induced partial remission of acute myelogenous leukemia in a child.

Polar organic compounds, such as dimethylsulfoxide and butyric acid, are known to induce differentiation in Friend erythroleukemia cells as well as in other cell types. It has been found that many of the compounds that induce cellular differentiation, inhibit 3H-thymidine incorporation and induce cell damage when incubated with leukemic cells from patients with acute or chronic myelogenous or acute lymphocytic leukemia. These effects are time and dose dependent. Among the compounds tested, butyrate was the most potent. Parenteral administration of butyrate (500 mg/kg/day) for ten days to a child with acute myelogenous leukemia in relapse, and resistant to conventional therapy, resulted in elimination of myeloblasts from the peripheral blood, an increase in mature myeloid cells and a reduction in 3H-thymidine uptake by the patient's peripheral blood cells. Bone marrow myeloblasts were reduced from 70-80% to 20% following the course of intravenous butyrate. No impairment of liver or renal function and no coagulation abnormalities were observed during butyrate treatment. Organic agents that induce cell differentiation may provide additional reagents for the clinical management of selected cases of leukemia.

Adult↗