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Biomedical subjects

E Rosenthal

Publications and source records attributed to E Rosenthal.

At least 19 recordsLinked to original sources

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

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

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

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

[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

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

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

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

Sequence analysis of translationally controlled maternal mRNAs from Urechis caupo.

Fertilization of Urechis caupo oocytes stimulates dramatic changes in the pattern of protein synthesis. This shift is brought about entirely through selective translation of the large pool of maternal mRNAs synthesized and stored during oogenesis. My laboratory has identified cDNA clones to more than 20 different Urechis maternal mRNAs. These have been used to determine whether the complementary mRNAs are translated in oocytes or embryos, and to analyze the polyadenylation status of the mRNAs at different stages. For 14 of the mRNAs, multiple, overlapping cDNA clones were isolated, and the complete sequence of the mRNA molecule was determined. Of these 14 mRNAs, half are from the subset that is translated in growing and full-grown oocytes, but not in embryos. These 7 mRNAs have poly(A) tails before fertilization. The other 7 are from the subset that is not translated at any time before fertilization, and has very short poly(A) tails in oocytes. After fertilization these mRNAs are recruited onto polysomes and extensively polyadenylated. The sequence data from the two classes of maternal mRNAs was compared in an attempt to identify consensus sequences that could regulate translation directly, or indirectly, by controlling polyadenylation or secondary structure formation. Two features of the sequences correlate very well with the translation and polyadenylation of the different mRNAs--the identity of the base immediately preceding the AUG start codon, and the presence of the sequences UUUUA and UUUUUA in the 3' untranslated region.

Animals

[Local antibiotic administration in osteomyelitis treatment--a comparative study with two different carrier substances].

Two antibiotic carriers (collagen sponge/PMMA-beads--loaded with gentamicin) for the treatment of osteomyelitis of long bones are compared in a randomised prospective study of 20 patients in respect of efficacy, safety and biocompatibility. In addition, the concentration of gentamicin in serum, urine and wound exudate was measured. This revealed marked differences. Gentamicin was released rapidly from the collagen sponge, leading to high levels in wound exudate and urine within the first 48 hours, with measurable but non-toxic concentrations in the serum. The liberation of gentamicin from the PMMA beads was much slower. This resulted in low steady levels in wound exudate and urine, and no detectable concentrations in the serum. The clinical outcome showed no differences between both methods of application. In 80% of the collagen sponge group and in 90% of the PMMA-beads group the osteomyelitis was completely allayed with disappearance of all infectious parameters. The number of re-operations was significantly higher in the PMMA group. It is recommended to use collagen sponges for the local treatment of osteomyelitis in cases that can probably be eradicated in a single surgical procedure, whereas PMMA beads should be applied in larger defects requiring a space-occupying function for further reconstructive operations.

Adolescent

[Acute Staphylococcus aureus myocarditis in AIDS. 2 cases].

The authors report two cases of acute myocarditis due to Staphylococcus aureus in patients with AIDS. There was no history of opportunist infections in either case but the CD4 lymphocyte levels were very low. The myocarditis caused acute cardiac failure and death. Histological examination showed microabscesses filled with Gram positive cocci throughout the myocardium. Bacteriological studies identified the Staphylococcus aureus. Staphylococcus aureus myocarditis without endocardial or pericardial involvement is very rare. It is the result of septic emboli in the cardiac microcirculation. Bacterial myocarditis has rarely been diagnosed in HIV positive patients. Both our cases featured severe cell-mediated immunodeficiency without associated neutropaenia. The decreased bactericidal activity of the neutrophil polynuclears and/or a deficit in the immunity mediated by the B-cell lymphocytes in AIDS could explain the lethal septic complications observed in our two cases.

AIDS-Related Opportunistic Infections