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PURPOSE: The aims of this study were to evaluate the response of oral iron treatment in children with iron deficiency anemia (IDA) fed whole cow's milk (WCM) or soy formula; to compare the incidence of fecal blood loss in infants fed WCM and soy formula; and to evaluate the incidence and relation of protein-losing enteropathy (PLE) and IDA by testing serum albumin, fecal blood loss, and fecal alpha1-antitrypsin (alpha1AT). METHODS: Twenty-four children with nutritional IDA were randomly assigned to receive either 16 oz WCM or soy formula daily. Both groups were treated with daily therapeutic oral iron during 12 weeks. Stool specimens for hemoglobin losses were collected at weeks 0, 3, 6, and 12. Levels of serum albumin and fecal alpha1AT were tested at diagnosis and when IDA was corrected. RESULTS: Anemia was corrected in 21 of the 24 children by week 6 or 12. Median fecal hemoglobin losses were not increased in either group at diagnosis or during treatment. Seven of 24 children had PLE at diagnosis with elevated fecal alpha1AT levels of 72 to 381 mg/dL that returned to normal after correction of IDA. Their initial fecal alpha1AT levels averaged 170 mg/ dL at diagnosis and 21 mg/dL after the IDA was corrected. Excessive WCM intake of 30 oz/day or more was present in 63% of the infants. CONCLUSIONS: Treatment of nutritional IDA with oral iron was just as effective with a limited quantity of either WCM or soy formula. Fecal hemoglobin losses were uncommon and did not differ in children at diagnosis or during treatment of IDA. PLE associated with IDA resolves when the IDA is corrected, but differences between children fed WCM or soy formula could not be detected.

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Over 50% of patients with newly diagnosed rhabdomyosarcoma (RMS) are in the 'intermediate risk' group with a 3-year progression-free survival of approximately 65%. This group consists of stage 1, group III, non-orbit tumours; stage 2, group II and III; and all stage 3 patients utilising the Intergroup Rhabdomyosarcoma Study (IRS) staging system. The role of doxorubicin in the treatment of RMS has been controversial. Ifosfamide, both alone and in combination with etoposide, has significant activity in patients with RMS. The aim of this pilot study was to examine the efficacy and toxicity of a chemotherapy regimen of alternating cycles of vincristine/doxorubicin/cyclophosphamide and etoposide/ifosfamide for intermediate risk RMS. 30 patients with intermediate risk RMS or undifferentiated sarcoma (US) were treated with alternating cycles of vincristine/doxorubicin/cyclophosphamide (VDC) and etoposide/ifosfamide (EI) at planned intervals of 3 weeks. Local treatment of the tumour in most cases was performed after four cycles of chemotherapy, followed by an additional 10 cycles of chemotherapy. At a median follow-up of 37.5 months, the Kaplan-Meier estimate of 3-year event-free survival was 85% (95% confidence interval 72-99%). The overall survival at 3 years was 91% (95% confidence interval 80-100%). No patient died from toxicity. The most common toxicity was febrile neutropenia in 35% of VDC and 26% of EI cycles. No nephrotoxicity or cardiac toxicity was seen. No patient progressed prior to week 12 local therapy. Alternating cycles of VDC and EI are an effective treatment for patients with intermediate risk RMS and US. Toxicity is tolerable. Delaying local treatment until week 12 does not compromise outcome.

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PURPOSE: The aim of the therapeutic trials was to optimize the treatment of severe aplastic anemia (SAA) and moderate aplastic anemia in children who lack a suitable bone marrow donor, using immunosuppressive therapy in the most effective combination and dose. PATIENTS AND METHODS: Two sequential therapeutic trials for the treatment of severe and moderate aplastic anemia in children were conducted by 10 institutions. The treatment protocols included antithymocyte globulin (ATG), prednisone, and cyclosporine A (CSA); patients entered on the first protocol, 0190 (ATG X 2), were given two courses of ATG, and those enrolled on the second protocol, 0190B (ATG X 1), were given only one course of ATG. Ten patients were evaluable on ATG X 2. All patients had SAA; three had hepatitis-induced severe aplastic anemia (HI-SAA). Twelve patients were evaluable on ATG X 1; all had SAA, one of whom had HI-SAA. RESULTS: Seven of 10 patients on ATG X 2 responded, and eight of 12 patients treated on ATG X 1 responded. CONCLUSION: Treatment with immunosuppressive therapy using ATG, CSA, and prednisone was very well tolerated. The response rates in both protocols were similar, and results compare favorably with those of previous therapeutic trials, suggesting that a second course of ATG is not necessary.

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Three national US agencies report on work-related fatal injuries, and one uses the "injury at work" designation on the death certificate to identify and characterize these fatalities. The accuracy of the "injury at work" notation has not been validated. The authors used selected external causes of death (from the International Classification of Diseases, Ninth Revision, Clinical Modification) that are highly likely to be work-related or not work-related as a standard to compare with the California death certificate "at work" designation for the years 1979-1989. Data from the National Center for Health Statistics for the years 1979-1984 were used to measure prevalence for purposes of determining the predictive value of a positive or negative work-related notation on the death certificate. The sensitivity of the "at work" designation was 77.6%, with a specificity of over 99%. Sensitivity but not specificity varied by age, sex, and specific external cause of death. The predictive value positive of the "at work" designation was about 60%, which suggests caution in using it for some epidemiologic purposes.

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The Epstein-Barr virus (EBV)-induced diseases of males with X-linked lymphoproliferative disease (XLP) include fatal infectious mononucleosis (IM), non-Hodgkin lymphoma (ML), agammaglobulinemia, and aplastic anemia. These phenotypes also occur as sporadic cases in families, and EBV seronegative males in these families must be considered at risk for XLP until they seroconvert normally to EBV. Given that 50% of males inheriting the defective XLP gene die following primary EBV infection, it is vital that they be identified pre-EBV infection. Here we report results using molecular genetic techniques to provide information as to the relative risks of EBV negative males and potential carrier females in ten families wherein a single male had died of IM.

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A multimedia communication campaign was conducted between 1988 and 1989 to promote family planning among men in Zimbabwe. The campaign consisted of a 52-episode semiweekly radio soap opera, about 60 motivational talks, and two pamphlets about contraceptive methods. Changes over time were measured by comparing a subset of a follow-up survey conducted from October to December 1989 to a baseline survey conducted from April to June 1988. Men exposed to the campaign were also compared to men who were not exposed. The follow-up survey revealed that the campaign reached 52 percent of men aged 18 to 55. Among married Shona-speaking men, use of modern contraceptive methods increased from about 56 percent to 59 percent during the campaign. Condom use increased from about 5 percent to 10 percent. Awareness and current use of modern contraceptives was also higher among men exposed to the campaign, primarily because of their greater awareness of condoms. Men exposed to the campaign were significantly more likely than other men to make the decision to use family planning and to say that both spouses should decide how many children to have.

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Hyponatremia and malignant hypertension are rare manifestations of Wilms' tumor. Hyponatremia associated with malignant hypertension of any cause is not explained. We present a patient with hyponatremia, malignant hypertension, Wilms' tumor, and an elevated atrial natriuretic factor (ANF). We believe the elevated ANF caused the hyponatremia.

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Reproducible models for examining early stages of human B cell differentiation are poorly developed. We now describe the establishment and characterization of a novel human leukemic cell line that recapitulates the pre-B to B cell stage of differentiation. This cell line, designated BLIN-1, was initially established in tissue culture medium containing low m.w. B cell growth factor, and consistently shows a dependency on this cytokine for optimal growth at low density. BLIN-1 cells have a 9p chromosomal abnormality, identical to the abnormality present in the leukemic blasts from the patient's original bone marrow aspirate. The immunologic phenotype of BLIN-1 is consistent with a cell arrested at the pre-B cell stage of development. Analysis of Ig gene rearrangement and Ig expression in a series of BLIN-1 subclones show that the cells spontaneously rearrange kappa light chain genes, leading to the differentiation of surface kappa-negative pre-B cells into surface kappa-positive B cells. The BLIN-1 cell line is, to our knowledge, the first defined human model for examining this critical developmental stage in human B cell ontogeny. As such, it offers a unique resource for examining variables influencing onset of kappa L chain gene rearrangement and expression.

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Two children with hepatoblastoma, marked thrombocytosis, and extremely elevated alpha fetoproteins are reported. It is not clear from review of the literature whether hepatoblastomas or hepatocarcinomas are associated with thrombocytosis. Elevated thrombopoietin levels were found in liver tumor extract from one patient.

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