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Biomedical subjects

E Rosenthal

Publications and source records attributed to E Rosenthal.

At least 127 records · Page 7Linked to original sources
PubMed1995

Translocation (2;14)(p13;q32) in CD10+ ;CD13+ acute lymphatic leukemia.

The rare t(2;14)(p13;q32) was previously described in the three pediatric patients with acute lymphatic leukemia. In these cases this abnormality was found at diagnosis, manifested the sole chromosomal abnormality, and was associated with a favorable prognosis. We here describe three cases of leukemia where such translocations were found at relapse, were associated in two of the cases with additional known characteristic chromosomal aberration, and were associated with a grave prognosis. Interestingly enough, the malignant cells of all three patients shared the same surface antigens: CD34, HLA DR, CD10, CD20, and the myeloid marker CD13. The leukemic clone exhibiting t(2;14) probably evolved from a t(1;19)6q- pre-B acute lymphatic leukemia in one of the cases, and from a chronic phase Ph1 chromosome in another. The significance of the translocation and the coexistence of CD10 and CD13 on the same cell are discussed.

Adolescent↗
PubMed1995

Use of saliva in home monitoring of carbamazepine levels.

Total carbamazepine (CBZ) levels in serum of 61 epileptic children were compared with saliva levels. Both resting and stimulated saliva was analyzed. The salivary levels were 38.6% of serum CBZ levels. A highly significant correlation was noted (r = 0.89, p < 0.001). Stimulation had no effect on saliva CBZ levels (r = 0.97). Salivary and serum CBZ levels were not affected by storing the samples for 7 days at room temperature. The data indicate that salivary CBZ may provide a reliable alternative monitoring method to Tegretol therapy, especially in children, in whom blood sampling is difficult. Furthermore, the samples may be collected at home and delivered to the laboratory by mail.

Adolescent↗
PubMed1995

Successful long-term ventricular pacing via the coronary sinus after the Fontan operation.

A man with double inlet left ventricle and severe subpulmonary stenosis underwent a Fontan operation at the age of 29 years. Eight years later he developed atrial flutter with complete heart block. To avoid a further thoracotomy, a unipolar carbon tipped electrode was placed into the posterior cardiac vein via the coronary sinus. More than 8 years after implantation of the original lead, and after two generator changes, telemetric thresholds remain between 1.8-2.1 volts. Percutaneous transvenous ventricular pacing via the coronary sinus can produce an excellent long-term result and should be the initial approach of choice after a Fontan-type operation.

Adult↗
PubMed1994

CD10+ cell population in the bone marrow of patients with advanced neuroblastoma.

Immunocytologic analyses of bone marrow can provide clinically useful prognostic information in neuroblastoma. While analyzing the bone marrow with a panel of monoclonal antibodies, which detect neuroblasts and other defining B-, T-, and myloid lineage, we identified two infants with stage IV-S neuroblastoma whose bone marrow contained a large population of common acute lymphoblastic leukemia (ALL)-like cells. This population expressed HLA-DR, CD19(B1), CD10(CALLA), and occasionally CD20(B1). Since 1988, 17 additional patients with advanced neuroblastoma (IV-S, III, and IV) were studied by us. In 10 of the 19 patients, the bone marrow revealed an expanded CD10 population (20-70%). It appears that this group of patients has a better prognosis. Out of 9 patients who did not have an expanded CD10 population, 8 died within 9 months from diagnosis, whereas out of 10 patients with an expanded CD10 population only one died and the others are alive, 6-30 months from diagnosis (P < 0.001). An expanded CD10 population in the bone marrow of disseminated neuroblastoma patients may therefore serve as a prognostic factor. Apart from the prognostic value of this particular population in the single patient, its presence may shed light on the interrelationship between the immune system and the neuroendocrine compartment.

Adolescent↗
PubMed1994

Primary central nervous system Burkitt's lymphoma presenting as Guillain-Barré syndrome.

A rare case of CNS Burkitt's lymphoma presenting as acute Guillain-Barré syndrome is presented. A 6-year-old previously healthy female presented with acute onset of limb and truncal weakness, involvement of ocular and bulbar nerves, and areflexia. The clinical diagnosis of Guillain-Barré syndrome prompted treatment with intravenous gammaglobulin with no response. A lumbar puncture following revealed marked pleocytosis, elevated protein, and decreased glucose. Immunological, cytological, and molecular studies of these cells confirmed the diagnosis of Burkitt's lymphoma IgM, kappa with t(8;14) and rearrangement of the J and kappa immunoglobulin chains. Aggressive systemic and intrathecal chemotherapy were started and within 5 days remission was achieved. The child is in complete remission 2 years from diagnosis. Although very rare, CNS lymphoma should be taken into account in every patient presenting with the clinical features of acute polyneuropathy.

Acute Disease↗
PubMed1994

[Kaposi disease and sex hormones: apropos of a case, review of the literature].

The authors report one case of AIDS-related-like Kaposi's sarcoma (KS) in a 59-year-old bisexual man without HIV-1 and HIV-2 infection. KS developed while the patient was receiving both androgen and steroid therapy for aplastic anemia, and regressed after their simultaneous interruption, despite the persistence of aplastic anemia. The authors discuss the etiology of KS in the patient, with a special regard to a putative role of the androgen therapy. The authors examine the arguments of the literature, probably underestimated, that may suggest a role of sex hormones in the pathogenesis of KS.

Anemia, Aplastic↗
PubMed1994

Accuracy of central venous pressure measurements in the inferior vena cava in the ventilated child.

We compared central venous pressure measurements from three sites, superior vena cava, inferior vena cava and right atrium, in 12 children undergoing cardiac catheterization whilst on assisted ventilation. There was no significant difference between the mean pressures in the superior vena cava and the inferior vena cava. Furthermore, there was also no difference between the mean pressures in the inferior vena cava and the right atrium. Because of the greater safety of the placement of pressure monitoring lines in the inferior vena cava via the femoral vein, this approach should be the preferred route for central venous pressure monitoring in children.

Blood Pressure Determination↗
PubMed1994

Cholestatic pruritus: effect of phototherapy on pruritus and excretion of bile acids in urine.

Pruritus associated with hepatic cholestasis may cause significant morbidity and its correlation to retention of bile acids in skin is inconsistent. Available treatment modalities are only partially effective and can have several adverse effects. Phototherapy has recently been reported to improve cholestatic pruritus, but has not been evaluated previously in children, and its mechanism is still unclear. We report the outcome of multiple Daylite phototherapy treatments over two years in a seven-year-old child with chronic hepatic cholestasis that was resistant to other therapeutic modalities. Bile acid levels in urine were used as markers of effectiveness in parallel with clinical response. Night phototherapy alone increased the bile acids/creatinine ratio in urine from 1.54 +/- 0.04 mumol/mg at baseline to 2.07 +/- 0.29 mumol/mg. Continuous phototherapy combined with night diuresis raised the ratio further to 2.28 +/- 0.55 mumol/mg. Night diuresis alone had no effect. Continuous phototherapy combined with night diuresis raised the bile acids/creatinine ratio by 44% on the first day and by 61% on the second day, but declined to baseline on the third day of treatment. A marked clinical improvement was noted for one week following two days of phototherapy. This schedule has been repeatedly effective in improving pruritus for approximately one year and may be due to the ability of phototherapy to enhance excretion of bile acids and other possible pruritogens into urine.

Bile Acids and Salts↗
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↗
PubMed1993

Identification of homologues to beta-catenin/plakoglobin/armadillo in two invertebrates, Urechis caupo and Tripneustes gratilla.

beta-Catenin and plakoglobin are intracellular proteins that participate in cell-cell adhesion, probably through interaction with the cadherin family of transmembrane adhesion proteins. They are also homologous to the segment polarity gene, armadillo, from Drosophila. I have cloned and sequenced armadillo/beta-catenin/plakoglobin homologues from two other invertebrates, Urechis caupo and Tripneustes gratilla, and shown that the mRNA is present in oocytes, eggs and early embryos. In Urechis, the mRNA is particularly abundant in oocytes, but is not translated until after fertilization. These results provide further indications that cell adhesion proteins play a key role during embryogenesis.

Amino Acid Sequence↗