High levels of education and employment among women with Turner syndrome.

BACKGROUND: Turner Syndrome (TS) is due to X chromosome monosomy and affects ~1 per 2500 females at birth. The major features are short stature and primary ovarian failure. Short stature and monosomy for a maternal X chromosome have been implicated in impaired functionality in adult life; however, data on adult outcomes in TS are limited. In this study we evaluated the influence of adult height and parental origin of the single X chromosome on education, employment, and marital outcomes among women with TS. METHODS: This was a cross-sectional study of 240 women (25-67 years old) with TS participating in an intramural National Institutes of Health (NIH) study. Parental origin of the single X chromosome was determined by genotyping proband and parental genomic DNA. Information on education, employment, and family status was self reported. Normative data was obtained from the U.S. Bureaus of Census and Labor and Statistics. RESULTS: Seventy percent of the TS group had a baccalaureate degree or higher, compared with 30% of U.S. women (p<0.0001). Eighty percent of the TS group was employed compared with 70% of the U.S. female population. Approximately 50% of the TS group had ever married, compared with 78% of the general female population (p<0.0001). Height and parental origin of the single normal X chromosome had no association with education, employment, or marital status. CONCLUSION: Women with TS currently achieve education and employment levels higher than the female U.S. population but are less likely to marry. Neither adult height nor parental origin of the single X chromosome influenced outcomes in education, employment, or marriage.

Serious aortic complications in a patient with Turner syndrome.

An asymptomatic young woman was discovered to have life-threatening aneurysms and dissection of the thoracic aorta during routine evaluation in a Turner syndrome (TS) study. The presence of a heart murmur and hypertension had led to diagnosis and surgical repair of an atrial septal defect at age 5 and of aortic coarctation at age 12. The diagnosis of TS was made at 16 years of age due to short stature and delayed pubertal development. She was treated with growth hormone from age 16 to 18 and with atenolol, thyroid hormone, and estrogen. She discontinued her medications and was lost to medical follow-up at age 20. Upon presenting here at age 26, she reported a very active lifestyle, including vigorous exercise and an acting career, with no symptoms of chest or back pain or shortness of breath. Cardiovascular imaging revealed aortic regurgitation, an unsuspected dissection of a severely dilated ascending aorta, and a large descending aortic aneurysm. She required surgical replacement of her aortic valve and ascending aorta, followed by endovascular repair of the descending aortic aneurysm. This patient illustrates the importance of considering the diagnosis of TS in girls with congenital aortic defects and the absolute necessity for close, expert follow-up of these patients who are at high risk for complications after surgical repair due to an underlying aortopathy, hypertension, and metabolic disorders. This patient also emphasizes the need to publicize and follow screening guidelines as there is an increasing number of patients with congenital defects who need transition to adult care.

Attempted validation of ICRP 30 and ICRP 66 respiratory models.

The validation of human biological models for inhaled radionuclides is nearly impossible. Requirements for validation are: (1) the measurement of the relevant human tissue data and (2) valid exposure measurements over the interval known to apply to tissue uptake. Two lung models, ICRP 30(1) and ICRP 66(2), are widely used to estimate lung doses following acute occupational or environmental exposure. Both ICRP 30 and 66 lung models are structured to estimate acute rather than chronic exposure. Two sets of human tissue measurements are available: (210)Po accumulated in tissue from inhaled cigarettes and ingested in diet and airborne global fallout (239,240)Pu accumulated in the lungs from inhalation. The human tissue measurements include pulmonary and bronchial tissue in smokers, ex-smokers and non-smokers analysed radiochemically for (210)Po, and pulmonary, bronchial and lymph nodes analysed for (239,240)Pu in lung tissue collected by the New York City Medical Examiner from 1972 to 1974. Both ICRP 30 and 66 models were included in a programme to accommodate chronic uptake. Neither lung model accurately described the estimated tissue concentrations but was within a factor of 2 from measurements. ICRP 66 was the exception and consistently overestimated the bronchial concentrations probably because of its assumption of an overly long 23-d clearance half-time in the bronchi and bronchioles.

Outcomes of spontaneous and assisted pregnancies in Turner syndrome: the U.S. National Institutes of Health experience.

OBJECTIVE: To assess fetal and maternal outcomes of pregnancies in women with Turner syndrome (TS). DESIGN: Retrospective case series. SETTING: Clinical research center. PATIENT(S): 276 adults with cytogenetically proven TS participating in an intramural natural history protocol. INTERVENTION(S): None. MAIN OUTCOME MEASURE(S): Menstrual and obstetric histories, 50-cell karyotypes, and cardiovascular evaluation including aortic diameter measurements. RESULT(S): Our cohort included five women with spontaneous pregnancies and five with pregnancies using assisted reproduction (ART). All five women with spontaneous pregnancies had spontaneous puberty, despite 45,X in ≥90% of their 50-cell karyotype. Participants had a total of 13 pregnancies and 14 live births. One child had cerebral palsy; the others were chromosomally and developmentally normal. Delivery was by cesarean section in four out of seven spontaneous and six out of six ART-related pregnancies. One mother experienced preeclampsia in an ART-related twin pregnancy that required a preterm delivery; she has marked but stable aortic dilation years later. CONCLUSION(S): Approximately 2% of our study cohort experienced spontaneous pregnancies despite high-grade X monosomy, and a similar number achieved pregnancy via oocyte donation and ART. The potential for life-threatening cardiovascular complications warrants comprehensive screening before conception or single-embryo transfer, and caution regarding unintentional pregnancies for TS women.

Measurement of the indoor and outdoor (220)Rn (thoron) equilibrium factor: application to lung dose.

A miniature four-chamber alpha track detector was developed that measures both (222)Rn (radon) and (220)Rn (thoron), in duplicate. Using this detector and the previous long-term measurements of the (220)Rn decay products (212)Pb, and (212)Bi, an equilibrium factor, F(eq), is derived for both outdoor and indoor (220)Rn environments (0.004±0.001 outdoors and 0.04±0.01 indoors). The lung airway dose can then be calculated from a dose factor from UNSCEAR that requires the equilibrium equivalent thoron concentration (EEC), i.e. the product of F(eq) and the (220)Rn gas concentration. The lung dose from thoron in domestic or occupational surveys is often overlooked. The values of F(eq) for thoron in several published studies are in general agreement with the values reported here. Thus, a long-term alpha track measurement of thoron multiplied by an appropriate indoor or outdoor equilibrium factor yields the EEC, which can be used to assess bronchial lung dose.

Thoron: its metrology, health effects and implications for radon epidemiology: a summary of roundtable discussions.

A roundtable discussion was made at the end of the workshop. All the presentations were summarised in this discussion. It involved measurement techniques, quality assurance and dose assessment and health effects of thoron and its progeny. In particular, major epidemiological studies may be affected by thoron interference in radon measurements. Since their data are not sufficient when compared with that of radon, further efforts in thoron studies will be needed.

[Progeny of Palmistichus elaeisis Delvare & LaSalle (Hymenoptera: Eulophidae) parasitising pupae of Bombyx mori L. (Lepidoptera: Bombycidae) of different ages]. / Progênie de Palmistichus elaeisis Delvare & LaSalle (Hymenoptera: Eulophidae) parasitando pupas de Bombyx mori L. (Lepidoptera: Bombycidae) de diferentes idades.

Palmistichus elaeisis Delvare & LaSalle is a natural pupal parasitoid of eucalyptus defoliator lepidopterans and is considered a promising biocontrol agent. However, the development of efficient rearing techniques for this natural enemy are first required before it can be used in biocontrol programs. Bombyx mori L. pupae are potential alternative hosts for this parasitoid mass rearing, and they are easy to rear. Therefore, we investigated the most suitable host age and the effects of parasitoid age on progeny production of P. elaeisis. B. mori pupae, 24 h-, 48 h-, 72 h- or 96 h-old were exposed to P. elaeisis females of similar age. The duration of the life cycle (egg-adult) of P. elaeisis was not affected by the age of the parasitizing female; however, the host age affected parasitoid development. The best parasitization was obtained for 72 h- to 96 h-old parasitoid females when offered to 48 h- to 72 h-old host pupae, allowing the synchronized rearing of a large number of P. elaeisis offspring.

Progênie de Palmistichus elaeisis Delvare & LaSalle (Hymenoptera: Eulophidae) parasitando pupas de Bombyx mori L. (Lepidoptera: Bombycidae) de diferentes idades / Progeny of Palmistichus elaeisis Delvare & LaSalle (Hymenoptera: Eulophidae) parasitising pupae of Bombyx mori L. (Lepidoptera: Bombycidae) of different ages

Palmistichus elaeisis Delvare & LaSalle é um parasitóide pupal natural de pupas de lepidópteros desfolhadores de eucalipto e é considerado um promissor agente de controle biológico. No entanto, o desenvolvimento de técnicas de criação eficientes desse inimigo natural é inicialmente necessário para que ele possa ser utilizado em programas de controle biológico. Pupas de Bombyx mori L. são hospedeiras alternativas em potencial para criação massal de P. elaeisis. Por isto, nós avaliamos a idade mais suscetível do hospedeiro e os efeitos da idade do parasitóide na produção de progênie de P. elaeisis. Pupas de B. mori com 24, 48, 72 ou 96h de idade foram expostas a fêmeas de P. elaeisis de mesmas idades. A duração do ciclo de vida (ovo-adulto) de P. elaeisis não foi afetada pela idade das fêmeas, entretanto, a idade do hospedeiro afetou o desenvolvimento do parasitóide. O melhor parasitismo foi obtido com fêmeas do parasitóide com 72h a 96h e pupas de B. mori com 48h a 72h de idade, que permitiram a elevação da progênie de P. elaeisis.

Palmistichus elaeisis Delvare & LaSalle is a natural pupal parasitoid of eucalyptus defoliator lepidopterans and is considered a promising biocontrol agent. However, the development of efficient rearing techniques for this natural enemy are first required before it can be used in biocontrol programs. Bombyx mori L. pupae are potential alternative hosts for this parasitoid mass rearing, and they are easy to rear. Therefore, we investigated the most suitable host age and the effects of parasitoid age on progeny production of P. elaeisis. B. mori pupae, 24h-, 48h-, 72h- or 96h-old were exposed to P. elaeisis females of similar age. The duration of the life cycle (egg-adult) of P. elaeisis was not affected by the age of the parasitizing female; however, the host age affected parasitoid development. The best parasitization was obtained for 72h- to 96h-old parasitoid females when offered to 48h- to 72h-old host pupae, allowing the synchronized rearing of a large number of P. elaeisis offspring.

An unusual complication of thrombolysis--bilateral retro-orbital haematomata.

We present a case of bilateral retro-orbital haematomata following thrombolysis of pulmonary embolism in a severely haemodynamically compromised 29-year-old female. The haematomata developed two days post-thrombolysis with marked visual impairment. Visual acuity was restored following bilateral drainage of the superior aspects of the retro-orbital space.

Efeito da disponibilidade de hospedeiro e de alimento nas características biológicas de Trichogramma galloi Zucchi (Hymenoptera, Trichogrammatidae) / Efect of host and food availability on the biological characteristics of Trichogramma galloi Zucchi (Hymenoptera, Trichogrammatidae)

Características biológicas de Trichogramma galloi Zucchi, 1988 foram avaliadas em laboratório onde esses parasitóides foram criados com ovos de Diatraea saccharalis (Lepidoptera, Pyralidae), com ou sem mel, e expostos a ovos do hospedeiro após 0, 6, 12, 24, 36, 48, 60, 72 e 84 horas da emergência. As taxas de parasitismo e de viabilidade mostraram-se elevadas para indivíduos que receberam alimento. A razão sexual não foi influenciada pelo alimento. O número de indivíduos por ovo somente mostrou diferença para aqueles adultos que não receberam alimento e permaneceram 6 horas sem ovos do hospedeiro. Conferindo o efeito da disponibilidade de ovos, somente a razão sexual, com ou sem mel, não mostrou diferenças. Os resultados mostram que T. galloi necessita de um suprimento de carboidratos e que o tempo pode influenciar a capacidade reprodutiva.

Calculation of the air concentrations of serially decaying nuclides--a new method applied to radon progeny.

A new calculation method is presented for determining the air concentrations of any serially decaying nuclides. This new method is easily incorporated into a Basic or spreadsheet program that is used to record the decay data. Specifically, the measurements were designed to illustrate radon progeny from a filter sample that is gross-alpha counted. The method is based on regressing the decay data, recorded in 1-min intervals, on the time after the end of sampling with an equation that describes the alpha activity as a function of the decay exponentials, e-lambdat. The results from both generated data and experimental filter samples were compared to the results obtained by two other well-known methods. All the methods give comparable estimates for the progeny air concentrations; non-linear regression and weighting by variances produces the smallest error estimates with the method presented here.

Plasma granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor levels in critical illness including sepsis and septic shock: relation to disease severity, multiple organ dysfunction, and mortality.

OBJECTIVE: To define the circulating levels of granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF) during critical illness and to determine their relationship to the severity of illness as measured by the Acute Physiology and Chronic Health Evaluation (APACHE) II score, the development of multiple organ dysfunction, or mortality. DESIGN: Prospective cohort study. SETTING: University hospital intensive care unit. PATIENTS: A total of 82 critically ill adult patients in four clinically defined groups, namely septic shock (n = 29), sepsis without shock (n = 17), shock without sepsis (n = 22), and nonseptic, nonshock controls (n = 14). INTERVENTIONS: None. MEASUREMENT AND MAIN RESULTS: During day 1 of septic shock, peak plasma levels of G-CSF, interleukin (IL)-6, and leukemia inhibitory factor (LIF), but not GM-CSF, were greater than in sepsis or shock alone (p < .001), and were correlated among themselves (rs = 0.44-0.77; p < .02) and with the APACHE II score (rs = 0.25-0.40; p = .03 to .18). G-CSF, IL-6, and UF, and sepsis, shock, septic shock, and APACHE II scores were strongly associated with organ dysfunction or 5-day mortality by univariate analysis. However, multiple logistic regression analysis showed that only septic shock remained significantly associated with organ dysfunction and only APACHE II scores and shock with 5-day mortality. Similarly, peak G-CSF, IL-6, and LIF were poorly predictive of 30-day mortality. CONCLUSIONS: Plasma levels of G-CSF, IL-6, and LIF are greatly elevated in critical illness, including septic shock, and are correlated with one another and with the severity of illness. However, they are not independently predictive of mortality, or the development of multiple organ dysfunction. GM-CSF was rarely elevated, suggesting different roles for G-CSF and GM-CSF in human septic shock.

The 1999 Lauriston S. Taylor lecture--back to background: natural radiation and radioactivity exposed.

I am profoundly grateful for being chosen as the twenty-third presenter of the Lauriston S. Taylor Lecture, and I share this honor with a list of distinguished scientists, including my husband, who pay tribute to the premier leader in radiation protection. In 1938, Laurie was working for the National Bureau of Standards and chaired the Advisory Committee on X-ray and Radium Protection, a group of 8 persons, who set the pace for all forthcoming radiation standards. NBS had, since 1913, been standardizing essentially all of the radium offered for sale in the U.S., and the problem arose to revise the handbook on proper conditions for handling radium based on the then current knowledge. This resulted in Handbook 23 (1938) superseding the 1934 work. At the time Laurie was a scientist working in the measurements side of radiation and though he contributed much to that field, his name is inseparably linked with guidance in radiation protection. Today we pay tribute for his leadership that he carried out with intelligence, grace, and personal warmth. My talk today deals mostly with measured data for naturally occurring internal radiation emitters and how these data can be used for predictive purposes in estimating the dose and risk from internal body contamination. This stresses the "and Measurements" part of the Council's title. The topic of this year's NCRP Annual Meeting is "Radiation Protection in Medicine: Contemporary Issues." I believe that physicians and State and Federal agencies will have to cope with complaints following various exposure situations resulting from the cleanup of background radionuclides during closure at nuclear facilities, military use of radioactivity, and occurrences of high natural background in some locations. They will find comfort in the knowledge that existing background radiation data can be the basis for predictions of realistic dose and risk in most situations.

A comparison between the localization of lung tumors in uranium miners and in nonminers from 1947 to 1991.

BACKGROUND: Lung cancer was noted to be increased in cigarette smoking miners and nonminers. Carcinogen particulates deposit differentially in the central, middle, and peripheral zones of the bronchial tree depending on the size of the particle. The object of this study was to evaluate the incidence of tumors; their cell types; and the relationship of particulate size to their position in the bronchial tree. METHODS: Tumor position in the bronchial tree was studied for a cohort of 467 uranium miners and 311 nonminers with lung cancer. RESULTS: An examination of all histologic subtypes showed that the proportion of lung cancers in the central zone was significantly greater in miners than in nonminers presumably due to the deposition of radon decay products attached to the silica dust particles. The higher percentage of central tumors in the miners was primarily due to the distribution of a greater proportion of squamous cell and small-cell tumors. The ratio of 0.75 for the central to middle and peripheral location for adenocarcinomas was much lower than for squamous cell and small-cell carcinomas with ratios of 1.4 and 7.3, respectively. In the mining cohort, there were ten times as many small-cell tumors in the central area as in the middle and peripheral regions, whereas, for the nonminers there were only five times as as many centrally located small-cell tumors as middle and peripheral (chi square is 7.0 degrees, P < 0.01). These data suggest that radon may be deposited preferentially to the central region of the lungs in uranium miners. CONCLUSIONS: Based on our observations of the differential positions of lung tumors in the bronchial tree for miners and nonminers and previous studies by others regarding size-dependent deposition of particulates in the bronchial tree, it is concluded that inhaled dust, radon, and cigarette smoke combine to form large particulates that deposit in the central bronchial tree. Filtered cigarette smoke or other small carcinogens from smaller particulates that deposit more peripherally.

Pennsylvania takes lead in drug education venture.

The Omnibus Budget Reconciliation Act (OBRA) of 1990 required all states to implement drug utilization review programs for outpatient medications covered by Medical Assistance. The State Society is taking a lead role in its response to the retrospective portion of the program. This article explains that role and overviews how OBRA requirements will affect Pennsylvania physicians.

Validation of a personal radon monitor for use in residential 22Rn exposure studies.

A personal radon monitor (PRM) has been developed and validated for use in studies of residential 222Rn exposure. A pilot study was performed in six New Jersey homes with elevated Rn concentrations. Occupants wore PRMs, and identical monitors were placed in many rooms of the home. Extensive assessment of PRM performance has been done. A simple model for personal Rn exposure has been tested and measured occupant exposures have been compared with stationary Rn measurements. The PRM works well, with precision limited by the counting error in the exposure range examined (2-100 kBq m-3 h). The PRM sensitivity is 2.2 kBq m-3 h, and its efficiency is 2.6 tracks (kBq m-3 h)-1 per 81 mm2 film.

Personal and home 222Rn and gamma-ray exposure measured in 52 dwellings.

A personal 222Rn and gamma-ray detector has been developed. The detector precision is limited only by the Poisson counting error and has a lower limit of detection in this study of 4.5 kBq m-3 h (4 pCi L-1 day). The detector was used in a study of 52 homes in Illinois to measure the personal exposure vs. the simultaneous exposure on all levels of the home. The ratio of personal exposure to basement 222Rn concentration averaged 0.22, with a high degree of scatter (R2 = 31%). The ratio of personal exposure to first floor 222Rn concentration was 0.71, with good correlation (R2 = 85%). In the absence of personal monitoring data, the best estimate of personal exposure appears to be from measurements in the first-floor living space of the home.