The two-edged sword of curing childhood cancer.
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Biomedical subjects
Publications and source records attributed to Philip M Rosoff.
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Genetic determinism labels the morally problematical claim that some socially significant traits, traits we care about, such as sexual orientation, gender roles, violence, alcoholism, mental illness, intelligence, are largely the results of the operation of genes and not much alterable by environment, learning or other human intervention. Genetic determinism does not require that genes literally fix these socially significant traits, but rather that they constrain them within narrow channels beyond human intervention. In this essay we analyze genetic determinism in light of what is now known about the inborn error of metabolism phenylketonuria (PKU), which has for so long been the poster child 'simple' argument in favor of some form of genetic determinism. We demonstrate that this case proves the exact opposite of what it has been proposed to support and provides a strong refutation of genetic determinism in all its guises.
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The risk of second malignancies after successful treatment for Hodgkin disease can be considerable. The most common malignancies are solid tumors arising in irradiated sites, such as the breast and thyroid gland after mantle field radiation. Sarcomas and other musculoskeletal tumors are also seen. We describe a young woman who developed an intra-abdominal desmoid tumor more than 4 years after completing therapy for Stage IIB Hodgkin disease, treated with combination chemotherapy (ABVD) and mantle irradiation. The tumor did not occur at either a surgical site or within a radiation field. She did not carry a mutation for familial adenomatosis polypoli.
BACKGROUND: Survivors of childhood cancer are at increased risk for osteoporosis, cardiovascular disease, and second malignancies-conditions for which modifiable risk factors are recognized and lifestyle interventions have shown benefit. Although some data regarding health behaviors of this population exist, receptivity to health promotion is largely unknown. METHODS: A survey was mailed to 380 survivors (age range, 11-33 years) of childhood leukemia, lymphoma, or central nervous system carcinomas (and guardians of survivors < 18 years old) to elicit data on exercise, dietary intake of calcium, fat, and fruits and vegetables, smoking status, readiness to pursue lifestyle change, quality of life, and interest in various health interventions. RESULTS: Responses from 209 survivors (a 55% response rate) suggested that most did not meet guidelines for fruit and vegetable consumption (79%), calcium intake (68%), or exercise (52%), 42% were overweight/obese, and 84% consumed > 30% of calories from fat. Older (> 18 years) compared with younger (< 18 years) survivors were more likely to smoke (17% vs. 1%), to be obese (21.6% vs. 14.6%), and to have suboptimal calcium intakes (75.6% vs. 57.6%). No differences in lifestyle behaviors were observed between cancer groups. Compared with interventions aimed at weight control, improving self-esteem, or smoking cessation, the highest levels of interest were found consistently for interventions aimed at getting in shape and eating healthy. Survivors preferred mailed interventions to those delivered in-person, by telephone counselors, or via computers. CONCLUSIONS: Survivors of childhood cancer practiced several suboptimal health behaviors. Health promotion interventions aimed at areas of interest and delivered through acceptable channels have the potential to improve long-term health and function of this vulnerable population.
OBJECTIVE: Mailed surveys are widely used to collect epidemiologic and health service data. Given that nonresponse can threaten the validity of surveys, modest incentives are often used to increase response rates. A study was undertaken among childhood cancer survivors and their parents to determine if response rate to a mailed survey differed with provision of immediate versus delayed incentives. DESIGN: A self-administered survey designed to ascertain health behaviors was mailed to 397 childhood cancer survivors (and their parents if the survivor was <18 years of age). Subjects were randomized into two groups based on gender, age, race, and cancer type. One group received a 10 US dollars incentive with their blank survey (unconditional incentive), whereas the other group received the incentive upon receipt of their completed survey (conditional incentive). If children were minors, both the parent and the child received incentives. RESULTS: No significant differences in response rates were observed with respect to gender, age, race, or cancer type. However, significant differences in response rates were observed between incentive groups, with unconditional incentives yielding significantly higher response rates than conditional incentives for child survivors who were > or =18 years (64.4% versus 49.0%), as well as younger child survivors (62.5% versus 43.6%) and their parents (64.8% versus 41.5%; all P < 0.05). CONCLUSIONS: The provision of an immediate incentive generated significantly higher response rates to this mailed health survey among childhood cancer survivors and their parents. Given that survey studies are commonly conducted across various pediatric populations, these findings may help inform the design of future pediatric survey research.
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It is currently thought that approximately 1% of children with Down syndrome will develop a "premalignant" syndrome known as transient myeloproliferative disorder (TMD). Prospective, population-based studies of the incidence of TMD in Down syndrome infants is lacking. Although most cases of TMD resolve by 1 year of age, data suggest that 10% to 20% of Down syndrome patients with TMD develop AML-M7 (megakaryoblastic leukemia). To identify the true incidence of TMD in the Down syndrome population, a sensitive, rapid, and cost-effective method of quantifying circulating megakaryoblasts in large numbers of patients was needed. In this pilot study, the authors tested the hypothesis that there are fewer than 1% megakaryoblasts of nucleated cells circulating in the blood of normosomic infants. Four-antigen flow cytometry was used to establish the percentage of megakaryoblasts present in each of 100 cord blood samples collected blindly from "normosomic" live births. There was a mean percentage of 0.017% megakaryoblasts in 100 cord blood samples from normosomic infants. Flow cytometry proved to be a sensitive, rapid, and reproducible method for the quantification of megakaryoblasts. Less than 1% of circulating nucleated cells in the blood of newborn infants are megakaryoblasts, providing a comparison population for the authors' larger proposed incidence study.
PURPOSE: Adult survivors of childhood cancer have been an underserved and understudied population. Few clinics are available to take care of them, unlike the numerous ones that exist for children. The authors established a clinic that would take care of all survivors diagnosed before the age of 25 years, ensuring the participation of a significant number of adults. The authors observed that many adult patients came to their annual visits accompanied by one or both of their parents. The rate was almost three times as high compared with parents in either a primary care or subspecialty internal medicine clinic. METHODS: The authors investigated this phenomenon by asking parents of adult survivors to fill out a questionnaire that collected demographic information as well as reasons for parents accompanying their adult children to doctors' appointments. Open-ended comments were also solicited. RESULTS: Most parents who came with their adult survivor children did not accompany their other children to doctor visits and commented that they felt there was a unique bond created by the cancer experience that did not diminish with increasing age of their children. The rate of parental attendance was independent of diagnosis or demographic indicators. Many parents stated that they continued to be concerned about their child's diagnosis, overall health, and risk for cancer recurrence. DISCUSSION: Parents of adult survivors of childhood cancer may harbor deep feelings of protectiveness that continue well beyond the initial treatment and off-treatment periods when the threat of primary cancer recurrence is a realistic concern. This can be manifested in them by accompanying their adult children to doctors' appointments and deserves further study.
IgA deficiency is a relatively common congenital immunodeficiency in children. It can either be asymptomatic or lead to frequent infections, most often of the sinuses and lungs. Intensive chemotherapy for acute leukemia is also profoundly immunosuppressive and can be complicated with life-threatening infections, usually associated with neutropenia and prolonged lymphopenia in the post-bone marrow transplant setting. Isolated, acquired immunoglobulin deficiency that occurs during treatment has been described but is usually transient. In this report, the authors describe a patient with infant acute myelogenous leukemia with acquired, persistent IgA deficiency.
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Explore the source record for details and available documents.
Explore the source record for details and available documents.