Towards environment and health promoting South African schools.
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Biomedical subjects
Publications and source records attributed to J Byrne.
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Retinoblastoma (Rb) is a rare childhood tumor of the eye. In the heritable form, tumors are often bilateral and survivors have a greatly increased risk both for a second malignancy and for having children with Rb. Familial patterns of both cancer and birth defects are poorly understood in families with a heritable cancer, and little is known of the ways that a heritable cancer affects the lives of long-term survivors. To find out more about these and other issues in the lives of long-term survivors of childhood and adolescent cancer, we interviewed 56 adult survivors of retinoblastoma (15 with the heritable form) and 84 brothers and sisters as controls, who formed part of a large retrospective cohort study. Rb survivors were interviewed between 1980 and 1983, when they were 30 years old on average. Types of employment and health problems did not differ between survivors and controls, regardless of sight, but the income of blind survivors was considerably less than that of partially sighted survivors. Despite similar marriage rates, fewer survivors than controls reported a pregnancy (RR = 0.45; 95% CI; 0.24-0.83 for both sexes combined). Parents of children with heritable Rb seemed more likely to have had cancer than parents in families with nonheritable Rb (P = 0.06), and mothers were more likely than fathers to be affected (P = 0.01). This small series suggests that having retinoblastoma may have many long-term consequences, reaching beyond genetic and physical effects to touch family life and income attainment and the health of other family members. Follow-up of more modern cohorts and the use of molecular tools will clarify the long-term consequences of more recent therapies, and patterns of familiar cancer.
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Sydenham's chorea has not previously been associated with abnormal findings on CT scan, but there have been reports of abnormal MRI in affected patients. The authors describe a case of Sydenham's chorea in which striatal abnormalities were found on both CT and MRI. Repeat MRI when the patient's illness had resolved was virtually normal. This suggests that abnormal imaging is entirely compatible with a diagnosis of Sydenham's chorea in a patient with an appropriate clinical history. Resolution of such abnormalities on repeat scanning provides further support for the diagnosis.
Our understanding of the renin-angiotensin-aldosterone system (RAAS) has advanced considerably in recent years. The RAAS plays a central role in the control of salt and water balance, and in the regulation of blood pressure and cardiovascular homeostasis. The haemodynamic changes in the period after myocardial infarction stimulate intense activation of both the circulating and the local RAAS. This acts through its end-products, angiotensin II and aldosterone, to promote sodium and fluid retention, and to increase cardiac contractility and systemic vascular tone. There is increasing evidence that, in the long term, this apparently adaptive response may be harmful, and might contribute to the development of some of the complications seen after infarction. Angiotensin II is capable of inducing coronary as well as systemic vasoconstriction, and may therefore prolong the duration of ischaemia. The response of the RAAS after infarction can be modified pharmacologically. Angiotensin converting enzyme (ACE) inhibitor drugs are already the mainstay of treatment in heart failure, and have now been shown to have a crucial role in the prevention of ventricular remodelling after myocardial infarction. Although the precise mechanism of this benefit is unclear, it provides further incentives to develop more effective strategies capable of suppressing neurohumoral activity following infarction.
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Cytogenetic techniques were used to study the tissue involved in neural tube defects. Eighteen patients have been evaluated and no specific alterations have been detected. We conclude that, whatever are the mechanisms that lead to neural tube defect, their origins must be searched for at the molecular level.
OBJECTIVE: To determine the impact of treatment on scholastic performance in the first cohort of survivors of childhood acute lymphoblastic leukemia who are old enough to have completed their educational experience. DESIGN: Retrospective cohort study. SETTING: Twenty-three institutions in the Childrens Cancer Group. SUBJECTS: A total of 593 adult survivors of childhood acute lymphoblastic leukemia and 409 sibling controls. OUTCOME MEASURES: Enrollment in special programs, grades during high school, graduation from high school, college admission, and college graduation. RESULTS: After diagnosis, survivors were more likely than their sibling controls to enter a special education (relative risk [RR] = 3.4; P < .01) or a learning disabled (RR = 3.6; P < .01) program, while just as likely to enter gifted and talented programs (RR = 1.0). The risk associated with special education and learning disabled programs increased with increasing dose of cranial radiotherapy. Despite these problems, survivors generally had the same probability as their siblings of finishing high school, entering college, and earning a bachelor's degree. However, survivors treated with 24 Gy and those diagnosed before 6 years of age were less likely to enter college (RR = 0.67 and 0.6, respectively; P < .01). CONCLUSIONS: This large study demonstrates that childhood acute lymphoblastic leukemia survivors have a greater likelihood of being placed in special education or learning disabled programs than their siblings, but that most are able to overcome these problems. Dose of cranial radiotherapy and age at diagnosis are the most important education-related risk factors.
BACKGROUND: Therapeutic advances have extended survival for most children and adolescents with cancer beyond 5 years from diagnosis. However, excess mortality continues beyond 5 years, and a significant portion results from causes other than the primary cancer. Risk factors for these deaths are not currently known. Thus, the authors studied mortality in a cohort of adult survivors of childhood and adolescent cancer to determine whether survivor characteristics were associated with increased relative risk of death from other causes. METHODS: Using 3255 siblings as control subjects, the authors studied survival in a retrospective cohort study of 2319 adults who were at least 5-year survivors of cancer diagnosed before reaching 20 years of age and between 1945 and 1974 (the NCI Five Center Study). Follow-up occurred between 1980 and 1983 at a mean survivor age of 32 years (range, 21-55 years). RESULTS: Between cohort entry and follow-up, 292 (13%) survivors and 50 (2%) controls died. One-third of the deaths in survivors were from causes other than the primary malignancy. Compared with control subjects, between ages 21 and 40 years, survivors had a more than threefold risk of death from other causes. The relative risk (RR) for death from other causes was greatest for survivors treated with radiation and alkylating agents (RR = 6.1; 95% confidence interval [CI], 3.0-12.4) and for those treated with radiation alone (RR = 3.8; 95% CI, 2.3-6.2; Cox regression analysis containing terms for treatment and cancer diagnosis). CONCLUSIONS: Adult survivors of childhood and adolescent cancer had a higher death rate than their siblings, even after removing the effect of primary cancer as the direct cause. Moreover, death from other causes was most strongly associated with increasing intensity of therapy, and this excess risk did not diminish with increasing age and duration of cancer survival. Because contemporary anticancer therapy is, in general, even more intensive than that received by the survivors described in this study, medical surveillance of cancer survivors is increasingly important to diagnose and treat potentially life-threatening complications that may occur decades after therapy has ceased.
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We describe a mother and her 3 children with variable scalp defects and limb defects consistent with a diagnosis of Adams Oliver syndrome also presenting with additional anomalies including congenital heart disease, microcephaly, epilepsy, mental retardation, arrhinencephaly, hydrocephaly, anatomic bronchial anomalies, and renal anomalies. The clinical variation between the individuals is more pronounced than in previously reported families.
In the complete absence of APCs staphylococcal superantigens induced IL-2, IL-4, IL-5, IFN-gamma, and IL-2R gene transcripts in both highly purified human T cells and FACs sorted CD4+ memory (CD45RA-) T cells. Secretion of IL-2, IL-4, and IFN-gamma, as well as DNA synthesis, on the other hand, required the presence of monocytes. At cytokine gene transcript level, three patterns of expression were noted after superantigen activation of T cells in the presence vs the absence of APC. mRNA levels for IL-2 were markedly up-regulated in the presence of monocytes, IL-4 and IFN-gamma transcripts increased only modestly, and IL-5 and IL-2R mRNA levels were unaffected. Blocking mAbs against LFA-1 and LFA-3 added to staphylococcal enterotoxin B (SEB)-activated cultures of T cells and autologous monocytes, reproducibly decreased both T cell proliferation and genetic expression of IL-2, IL-4, IL-5, and IL-2R, although having little or no effect on IFN-gamma transcripts. Further, under those conditions of blocking, secretion of IL-2 and IL-4 was dramatically decreased, whereas IFN-gamma secretion remained essentially unchanged. In contrast, LFA-1 and LFA-3 mAbs completely abrogated IFN-gamma secretion from PHA-activated T cell-monocyte mixtures, although having no inhibitory effect on T cell proliferation. These results indicate a characteristic and differential involvement of adhesion molecule-mediated signals in superantigen-induced T cell proliferation, differential cytokine gene expression, and cytokine secretion.
Jen Byrne and Vicki Bruce conduct Parenting Education for expectant parents, a private practice of childbirth education in Adelaide. How the practice evolved and what goals were set, the time lines, commitment to the cause and taking risks to achieve results all required careful thought. There were barriers too, in setting up this practice. This paper discusses the particular issues involved in setting up a private practice in childbirth education. Midwives and nurses are new to the area of private practice and our education has not prepared us for this expansion of our role. Issues such as the legal aspects, financial survival, professional support and marketing are discussed.
Twelve patients were involved in a 3 month stimulation induced cycling programme at the Royal Perth Rehabilitation Hospital. A number of the patients were less than 1 year post injury, all except one had an incomplete injury, and most were receiving physiotherapy. All patients who completed the programme increased their time of cycling and, in all but one case, the exercise load, indicative of a local training effect. Significant improvements were found in voluntary isometric strength, stimulated isometric strength and stimulated isometric endurance of the quadriceps, muscle grading of the quadriceps and biceps femoris and the cross-sectional areas of the quadriceps and total thigh muscle. No change was found in voluntary isokinetic strength of the quadriceps. All patients with incomplete injuries reported improvements in the activities of daily living (ADL) after the programme. Bone mineral density (BMD) was examined in two patients, one less than 1 year post injury, and one greater than 4 years post injury. The programme of cycling did not restore BMD in the latter patient. However, while the former patient still displayed a reduced BMD after the programme, it is unknown whether this loss of bone was retarded. This needs further investigation. This study demonstrates the effectiveness of a combined physiotherapy/cycling programme in the rehabilitation of people with spinal injuries. To be successful this type of programme has to be incorporated into the rehabilitation process, as has been done at the Sir George Bedbrook Spinal Unit.
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The reported success of treatment for children with medulloblastoma must be balanced against the effect that treatment has on the quality of life of long-term survivors. The outcome of long-term survivors reported in previous studies has been conflicting. The authors evaluate the mental and behavioral skills of a group of medulloblastoma survivors from their institution, all of whom had survived for more than 5 years postdiagnosis. A review of the institutional records yielded 32 patients. Twenty-three families were interviewed by telephone and, of these, 13 subjects came to the hospital for detailed neuropsychological and neurological evaluations. Intelligence quotient (IQ) was less than 90 for all participants tested, and patients diagnosed before the age of 3 years had lower IQ scores on average than those diagnosed later. Mean IQ and achievement test scores in reading, spelling, and mathematics were all higher in survivors who had undergone shunting. Achievement test results were often not in accord with intellectual potential, and individual intellectual skills varied widely. Perceptual-motor task performance was below average in more than 50% of the participants, but motor dexterity was more severely affected than perception. Problems in learning and a delay in both physical growth and development were seen in a majority of participants. This study directs attention to the serious difficulties faced by long-term survivors of medulloblastoma and their families, and underscores the importance of routine neuropsychological testing. Moreover, the study provides further impetus to seek alternatives to irradiation in the treatment of malignant brain tumors.
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