Subendocardial blood flow in children with congenital aortic stenosis

Subendocardial blood flow may be estimated from the ratio of flow to the subendocardium to myocardial oxygen consumption. The first may be estimated from the diastolic pressure time index (area between aortic and left ventricular (LV) pressure during diastole) and the latter by the tension time index (integral of LV pressure during systolic ejection). Subendocardial flow index (SEFI) averaged 1.27 (0.96-1.78) in 13 children with normal aortic valves. SEFI averaged 0.88 (0.43-1.65) in asymptomatic children with congenital aortic stenosis and was never greater than 0.9 in symptomatic children. Aortic valve area and systolic pressure difference did not correlate well with symptoms. SEFI and aortic valve area increased in 26 of 28 patients after surgery. However, 23 of 28 had varying degrees of aortic regurgitation following valvotomy. Since calculation of SEFI is not affected by aortic regurgitation, it would appear to be a more useful measure of surgical success than aortic valve area.     

Effect of divergent selection for immune responsiveness and of major histocompatibility complex on resistance to Marek's disease in chickens

Lines of chickens selected for nine generations for high (H) or low (L) antibody response to SRBC, a randombred control (C) line, and an F1 cross between H and L lines were challenged for resistance to Marek's disease (MD). Hens only were challenged at day-old by contact with virulent MD Strain K. Birds were serologically typed for MHC erythrocyte antigens. Chicks from the L and H lines died earlier and later, respectively, than the C chicks, whereas time of death did not differ between F1 birds and the L chicks. Mortality in the L line (70.1%) was higher than in the C line (42.8%), but mortality in the H line (40.9%) was not lower than in the C line or the F1 cross (47.5%). Effects of MHC genotypes and haplotypes on mortality from MD were estimated within lines with a logistic regression model. Effect of MHC was moderate in the H line (P < .10) and highly significant in the C line (P < .005). Effects of MHC genotypes were similar in the H and C line but differed in the L and F1. Heritability of mortality from MD estimated with a threshold model including relationships between individuals was .40 when all lines were grouped together, whereas heritability estimated for each line separately was .45, .51, and .78 in the H, C, and L lines, respectively. Correlations between estimated breeding values for antibody response to SRBC and mortality from MD varied between lines and sexes. Correlations also were affected by whether or not the MHC effect was taken into account.     

Further delineation of spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type, with emphasis on diagnostic features

Further delineation of a generalized bone dysplasia which we call spondylo-meta-epiphyseal dysplasia, short limb-abnormal calcification type is presented. This dwarfing condition has several serious complications, with the most common cause of death being spinal cord damage secondary to atlantoaxial instability. It is a heritable condition with an autosomal recessive mode of transmission. Radiologic diagnostic criteria are developed on the basis of studies in 8 patients with the oldest being between 4 and 5 years old. The condition is clinically and radiographically apparent neonatally or in early infancy, and it is probable that all or almost all affected individuals will come to medical attention in the age range screened by this study.     

Determinants of children's health care use: an investigation of psychosocial factors

Factors related to the amount of health care used by 5- to 11-year-old children in a health maintenance organization (HMO) were investigated using a comprehensive multivariate model that assessed the contribution of child health need, mental health, and social functioning; maternal mental health, social support and health care utilization; and family functioning and life events. Mothers reported on the 450 participating children. Health care visits for a two-year retrospective period were obtained from the computerized encounter system. Child health need and maternal patterns of health care use were powerful predictors of the overall amount of health care used, and these factors discriminated high users from low users of care. Family conflict was associated with a higher volume of care, while children's depressive symptoms and non-white race were related to lower use. Maternal social support, mental health, and life events were not predictive of use in either full multivariate model. Enabling factors were held relatively constant by participation of all families in a prepaid HMO. The multiple regression model explained 33% of the variance in use, slightly more than in previous studies of children's health care use. When included in a comprehensive analysis, child and family psychosocial characteristics help to explain children's health care use beyond what is possible using simple health and illness variables. The implications of these findings in the development of further research and to the practice of routine pediatric care are discussed.     

Association of Sj?gren's syndrome with HLA-B8

HLA antigens were examined by microdroplet lymphocyte cytotoxicity in 31 patients with Sj?grens syndrome. Fifty percent of 22 Causcasian patients (7 of 17 females and 4 of 5 males) had HLA-B8, as compared to 21% of 1205 controls (P=0.002). The presence of HLA-B8 was not correlated with parotid salivary flow, keratoconjunctivitis sicca, or focus score. Because of its association with a number of autoimmune diseases, including Sj?gren's syndrome, it is suggested that HLA-B8 is genetically liked to an immune response gene(s) that predisposes the individual to autoimmune phenomena. The expression of autoimmunity may be determined by infectious or environmental factors.     

Thermal conductivity and Lorenz number of the heavy rare-earth metals

A detailed investigation of the thermal conductivity of pure, heavy rare-earth metals has been made in the range of temperatures 0.3–4.2 K. The results obtained on the 10 samples investigated (Gd, Dy, Ho, Er, Yb, and Tb) show that the thermal conductivity can be readily separated into a linear and a quadratic term in temperature. The linear term, when related to the residual electrical resistivity, gives Lorenz numbers which are remarkably close to the theoretical value and this indicates an electronic linear contribution to the thermal conductivity; the experimental evidence available suggests that the quadratic term is due to a phonon contribution. Interesting information on the scattering mechanisms for electrons and phonons is also obtained.This work was in part supported by the Calouste Gulbenkian Foundation and Instituto de Alta Cultura, Portugal (J.B.S.) and a Rhodes Scholarship (R.R.).     

Radiology in primary hyperaldosteronism

Autonomous hypersecretion of aldosterone (primary hyperaldosteronism) is caused by either hyperplasia (usually bilateral) or an adenoma (frequently unilateral) of the adrenal cortex. Systemic hypertension due to an aldosteronoma is a potentially curable condition through surgical extirpation of the offending organ. In our experience with 37 patients clinically suspected to have primary hyperaldosteronism, radiological methods contributed significantly in preoperative diagnosis. These included (1) selective bilateral adrenal vein catheterization and blood sample collection, (2) adrenal venography, and (3) radioisotope adrenal scan. Unilateral hyperfunction could be accurately detected by the aldosterone assays from the collected samples. When adrenal venography was technically satisfactory, a nodule or aggregate of nodules measuring at least 7 mm and located on the margin of the gland or 1.5 cm or more in diameter when located in the center of the gland were readily identified. Enlarged adrenal gland on venography, in itself, was not a dependable index of a hyperfunctioning gland. Presence of a higher uptake on one side on the radioisotope adrenal scan did not always indicate the hyperfunctioning gland, but lack of lateralization of adrenal hyperfunction was more accurately predicted on the radioisotope scan than by venography. Four histopathological patterns were recognized in the surgically removed adrenal glands, but no correlation between these patterns and clinical behavior or postoperative course was found.     

Inhibition of sodium transport by prostaglandin E2 across the isolated, perfused rabbit collecting tubule

This study was designed to examine whether prostaglandin E2 can directly affect sodium transport across isolated perfused rabbit renal collecting tubules. Changes in transepithelial potential and isotopic sodium fluxes in response to peritubular prostaglandin E2 were measured. In addition, changes in transepithelial potential of the outer medullary collecting tubule in response to prostaglandin E2 were also measured. With few exceptions, all rabbits received 5 mg/day desoxycorticosterone acetate for 4-11 days before experimentation. The results of the experiments show that: (a) prostaglandin E2 inhibits the negative transepithelial potential in the cortical collecting tubule as well as the outer medullary collecting tubule; (b) prostaglandin E2 inhibits net sodium transport out of the lumen by inhibiting efflux while backflux is unaffected; (c) prostaglandin E2 produces this inhibition within 15 min, and the effects are dose dependent and reversible. These results suggest that prostaglandin E2 may modulate sodium transport in vivo and may contribute to the final regulation of sodium excretion.