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Background: The maternal and neonatal risks associated with pregnancy in women with heart disease receiving comprehensive prenatal care have not been well defined., Methods and Results: We prospectively enrolled 562 consecutive pregnant women with heart disease and determined the outcomes of 599 pregnancies not ending in miscarriage. Pulmonary edema, arrhythmia, stroke, or cardiac death complicated 13% of pregnancies. Prior cardiac events or arrhythmia, poor functional class or cyanosis, left heart obstruction, and left ventricular systolic dysfunction independently predicted maternal cardiac complications; the cardiac event rate can be predicted using a risk index incorporating these predictors. Neonatal complications (20% of pregnancies) were associated with poor functional class or cyanosis, left heart obstruction, anticoagulation, smoking, and multiple gestations., Conclusions: Pregnancy in women with heart disease is associated with significant cardiac and neonatal complications, despite state-of-the-art obstetric and cardiac care. Maternal cardiac risk can be predicted with the use of a risk index.

The high-temperature thermal degradation of four alternative automotive fuels (methanol, ethanol, natural gas, and liquefied petroleum (LP) gas) have been examined as a function of fuel-oxygen equivalence ratio and exposure temperature using fused silica flow reactor instrumentation coupled to in-line GC-TCD and GC-MS detection. Organic speciation for methanol, natural gas, and LP gas were consistent with previous measurements. However, several previously undetected organic by-products were observed from ethanol oxidation and pyrolysis. Organic speciation was found to vary significantly between methanol and ethanol and less so between natural gas and LP gas. Non-methane organic gases (NMOG) and specific reactivities of the respective fuels were measured, and trends with respect to proposed reactivity adjustment factors are discussed. A qualitative comparison of NMOG quantified in the flow reactor tests with the results of recent vehicle tests is also reported. The most significant differences in the comparisons were observed for toxic compounds, including the lack of detection of acetalde-hyde, 1,3-butadiene, and benzene from flow reactor experiments of methanol degradation, and the lack of detection of 1,3-butadiene from flow reactor experiments of ethanol combustion. Possible sources for the formation of these compounds in vehicle tests are discussed.

Control strategies for urban ozone traditionally have been based on mass reductions in volatile organic compounds (VOCs). Studies show, however, that some organic gas species (such as alkanes and alcohols) form an order of magnitude less ozone than equal mass emissions of others (such as alkenes and aldehydes). Chemically detailed photochemical models are used to assess uncertainty and variability in reactivity quantification. VOC control strategies based on relative reactivity appear to be robust with respect to nationwide variations in environmental conditions and uncertainties in the atmospheric chemistry. Control of selective organic gas species on the basis of reactivity can offer cost savings over traditional strategies.

Objectives: This study was undertaken to determine the results of repair of partial atrioventricular (AV) canal in patients > or = 40 years old., Background: Although postoperative outcomes in younger patients have been well documented, the fate of older patients with repaired partial AV canal is less clear., Methods: From 1958 to 1990, 31 patients 40 to 71 years old (mean age 51) had repair of partial AV canal. Twenty-three patients had repair of the cleft mitral valve; two had mitral valve replacements; and six needed no mitral valve operation., Results: Early mortality was 6%. One patient was lost to follow-up. Nine of the early survivors are known to have died. There is a small but significant development over the long term of atrial arrhythmias, complete heart block, subaortic stenosis, recurrent mitral regurgitation and, rarely, mitral stenosis. Three of the 28 patients available for follow-up had mitral valve reoperation and subaortic stenosis developed in 2. Nineteen patients were alive in 1991 (mean follow-up 14 years). Seven patients were in New York Heart Association functional class I, eight were in class II, and four were in class III. Fifteen of the 19 patients reported sustained postoperative improvement., Conclusions: Patients > or = 40 years old can have partial AV canal repair with low risk. Long-term survival is good, with subjective improvement in symptoms. Late complications occur but are uncommon, suggesting that long-term follow-up is warranted.

Technetium-99m bone and Gallium-67 scintigraphy has been widely used in the management of children with solid tumors. A retrospective review of all patients with rhabdomyosarcoma (RMS) from January 1980 to December 1989 was undertaken in order to determine the sensitivity and specificity of scintigraphy in detection of metastatic disease and to determine optimum frequency of serial scans in follow up. Over the 10-year period, 40 patients were diagnosed and treated for RMS: 22 were newly diagnosed from 1980-1984 and of these 16 had a minimum 5-year disease-free period whilst 6 died of the disease, giving a cure rate of 73%. A total of 271 bone and 236 Gallium-67 scans were performed. With respect to detection of metastatic disease in all tissues, Gallium-67 scans had a sensitivity of 84%, specificity 95% and bone scans had a sensitivity of 70% and specificity 95%. Bone scan sensitivity and specificity for skeletal metastases were 100% and 95%, respectively. Considering only patients with Gallium avid primary tumors, the Gallium-67 scan sensitivity was 94%. Ten patients developed new metastatic disease or primary recurrence after starting therapy, 8/10 within 12 months of diagnosis. Frequent surveillance scanning in the treatment phase, 2 years post-diagnosis, is of value in the early detection of metastases and monitoring disease response to therapy. Surveillance scanning after completion of treatment is more difficult to justify.

Thirty-five children (aged 0-9 yr) who had presented with Stage IV neuroblastoma were studied to see if avidity for 67Ga or 99mTc-methylene diphosphonate (MDP) uptake in both primary and secondary sites at diagnosis conferred any prognostic significance. Twenty-three percent of the patients were disease free and off treatment at the time of study. Crude survival did not differ between groups. Duration of survival and the likelihood of completing treatment were related to the scintigraphic appearance at the time of diagnosis, after adjustment for potential confounding effects, using Cox's proportional hazards regression and multiple logistic regression. After adjustment for confounding influences, neither 67Ga avidity nor uptake of 99mTc-MDP was associated with a significantly worse prognosis, both in terms of adjusted survival and likelihood of completing treatment. Patients with 67Ga-avid scans at diagnosis did not demonstrate significantly worse survival (HR 1.47, 95% CI 0.43-5.11) than those without 67Ga avidity. They were somewhat less likely to complete treatment (OR 0.23, 95% CI 0.03-1.63), but this did not reach statistical significance. Similarly, although patients with 99mTc-MDP positive scans demonstrated somewhat worse survival (HR 2.47, 95% CI 0.45-13.54), this result did not reach statistical significance, nor were they less likely to complete treatment (OR 0.69, 95% CI 0.07-6.67) than those with 99mTc-MDP negative scans. Uptake of 99mTc-MDP into extraosseous sites was also not associated with worse survival (HR 1.45, 95% CI 0.58-3.62) nor with decreased likelihood of completing treatment (OR 0.78, 95% CI 0.12-5.09). Other than indicating disease stage, these results do not support the hypothesis that the scintigraphic appearance at diagnosis confers prognostic information in children with advanced neuroblastoma.

Three of 38 children given high-dose cytosine arabinoside therapy developed a previously undescribed complication. Neurological problems are a frequent occurrence in patients given this therapy, particularly cerebellar ataxia, but the development of bulbar and pseudobulbar palsy has not been reported. In two of these cases, it was sufficiently marked for the course of treatment to be curtailed and occurred at a relatively low cumulative dose of the drug. Neurotoxicity can occur at any time using high-dose cytosine therapy.

To determine the timing of pubertal development and the frequency of gonadal dysfunction in children who survive acute lymphoblastic leukemia, we assessed pubertal status and the plasma levels of sex steroids, gonadotropin, and inhibin in 45 children (20 girls and 25 boys) who had received combination chemotherapy along with 24 Gy of irradiation to the cranium (modified LSA2L2 protocol). We also reexamined testicular biopsy specimens, obtained at the time of the cessation of chemotherapy, for the presence of germ cells. Germ-cell damage, indicated by marked elevations in the plasma level of follicle-stimulating hormone (P less than 0.001 for the comparison with normal children), was evident in both sexes and was confirmed in the boys by the absence of germ cells in the testicular biopsy specimens and by the small size of the testes for pubic-hair stage. Only 44 percent of the pubertal girls had measurable plasma inhibin levels, as compared with more than 93 percent of normal pubertal girls. Although plasma sex-steroid levels were normal, the secretion of luteinizing hormone in response to stimulation with gonadotropin-releasing hormone was elevated in the pubertal children (P less than 0.01 for the comparison with normal controls)--a finding that suggests compensation for decreased gonadal function. Despite clear evidence of gonadal damage, girls had early menarche at a mean age (+/- SD) of 11.95 +/- 0.91 years, as compared with the Australian standard of 12.98 +/- 1.11 years (P less than 0.01). Thus, in girls, puberty was early despite primary gonadal damage. Thirteen of 23 boys reached puberty at a mean age of 12.36 +/- 0.73 years. We conclude that treatment for acute lymphoblastic leukemia may lead to primary gonadal damage in both sexes, regardless of the age at treatment, but that the secondary characteristics of puberty develop at a normal age or, in girls, relatively early.

Murine parietal yolk sac carcinoma cells were examined by scanning and transmission electron microscopy to determine the ultrastructural changes resulting from growth, in vitro, in media containing different serum concentrations. Cells grown in medium supplemented with 10% fetal bovine serum (FBS) formed spherical bodies, were gradually oval with numerous surface microvilli, well-organized microtubules, abundant free polysomes and a well-developed Golgi apparatus. By contrast, cells grown in 1% FBS failed to form multicellular spheres, were generally flattened over the growth surface and lacked the surface and intracellular features demonstrated when cells were grown in 10% serum. These differences could explain the alterations in the glycosylation of secreted glycoprotein associated with culture in the presence of low serum.

In a study of 77 children who had been treated for acute lymphoblastic leukaemia (ALL) with an LSA2L2 (Memorial Sloan-Kettering) chemotherapy protocol plus radiotherapy (24 Gy) as cranial prophylaxis, growth was examined 3.0-9.5 years after diagnosis. The children's growth slowed and they crossed height percentiles towards the end of or after treatment. The Z-score, which reflects the deviation of height measurements from the population mean, was used to assess height change. The mean Z-score was 0.16 at diagnosis, -0.30 2 years later, -0.71 4 years later, and -1.37 6 years later. Height for age had fallen by more than 1 standard deviation of the population mean in 32% of survivors 4 years after diagnosis and in 71% 6 years after diagnosis. Younger children and those tall for age at diagnosis were more severely affected. Growth-hormone (GH) response to standard provocation tests was measured in 46 patients; 30 had partial or complete GH deficiency. Mean pulsatile GH secretion was low in the 34 patients tested. Cranial irradiation is probably the most important causative factor in the development of GH deficiency in survivors of ALL.

Rhabdomyosarcoma of the heart is a rare tumor, especially in childhood and particularly in the left ventricle. A primary tumor of this type was successfully resected after echocardiographic assessment alone in a young girl exposed prenatally to diphenylhydantoin. The patient is well and free of recurrence 2 1/2 years later.

Sixteen episodes of a distinctive, papular rash occurred in eight patients following withdrawal of 6-mercaptopurine (6MP) and methotrexate (MTX) used as maintenance therapy for acute lymphoblastic leukemia (ALL). The rash also developed in one of the eight patients when only 6MP was discontinued. The eruption occurred mainly on the face, and in this site resembled the perioral dermatitis seen following withdrawal of topical fluorinated steroids. The rash generally began within 3 weeks of stopping 6MP and lasted 3 to 4 weeks. It failed to improve with the use of topical corticosteroid. We conclude that this rash is caused by the withdrawal of oral 6MP.

Thirteen cases of leukemia, 12 of them acute, occurred in 3 generations of a family comprising 293 members. Individual cases could not be linked to the possession of any of a range of genetic markers. Cytogenetic studies showed no constitutional chromosome abnormalities. Preliminary results of virologic studies suggested the presence of oncornaviruses in at least 1 leukemic individual in this family. This aggregation of leukemia cases likely resulted from a genetic, probably polygenic, predisposition, in association with the activity of leukemogenic factors whose nature remains to be clearly defined.