Showing papers by "Medical University of South Carolina published in 1982"

Widespread periodic intrinsic connections in the tree shrew visual cortex

Abstract: Intrinsic connections within the tree shrew (Tupaia glis) visual cortex (area 17) are organized in periodic stripelike patterns within layers I, II, and III. This anatomical network resembles the regularly organized stripes of 2-deoxyglucose accumulation seen after stimulation of alert animals with uniformly oriented lines. Such connections imply that widespread lateral interactions are superimposed on the retinotopic organization of area 17 and suggest alternative interpretations of cortical columns.

The Effect of Treatment on Mortality in Mild Hypertension: Results of the Hypertension Detection and Follow-up Program

Abstract: In the Hypertension Detection and Follow-up Program, 7825 (71.5 per cent) of the 10,940 participants had diastolic blood pressures averaging between 90 and 104 mm Hg on entry into the study and were designated Stratum 1. Half were referred to their usual source of care in the community (the referred-care group), and half were treated intensively in special clinics (the stepped-care group). Five-year mortality in the Stratum 1 patients given stepped care was 20.3 per cent lower than in those given referred care (P less than 0.01). Particularly noteworthy was the beneficial effect of stepped-care treatment on persons with diastolic pressures of 90 to 104 mm Hg who had no evidence of end-organ damage and were not receiving antihypertensive medication when they entered the study. This subgroup had 28.6 per cent fewer deaths at five years among those treated with stepped care than among those treated with referred care (P less than 0.01). These findings support a recommendation that in patients with mild hypertension, treatment should be considered early, before damage to end organs occurs.

A stochastic model of self-renewal and commitment to differentiation of the primitive hemopoietic stem cells in culture.

Abstract: We recently identified a murine hemopoietic stem cell colony which consists of undifferentiated (blast) cells and appears to be more primitive than CFU-GEMM in the stem cell hierarchy. The progenitors for the colony which we termed "stem cell colony" possess an extensive self-renewal capacity and the ability to generate many secondary multipotential hemopoietic colonies in culture. We replated a total of 68 stem cell colonies from cultures of murine spleen cells and analyzed the number of stem cell--and granulocyte(neutrophil)-erythrocyte-macrophage-megakaryocyte (GEMM) colony-forming cells in individual stem cell colonies. Of the 68 stem cell colonies, 35 contained progenitors (abbreviated as "S"-cells) for stem cell colonies. The distributions of S-cells and CFU-GEMM in individual stem cell colonies were extremely heterogeneous. Neither the frequency distributions of S-cells nor CFU-GEMM in stem cell colonies could be fitted well by Poisson distribution. Rather, the frequency distribution of the s-cells could be approximated by a geometric distribution and that of CFU-GEMM by an exponential distribution, both of which are variates of the gamma distribution. Our observations are in agreement with those on the distributions of CFU-S in individual spleen colonies and provided support for a stochastic model for stem cell self-renewal and commitment in culture. Application of the theory of the branching process to the distribution of S-cells revealed a distributional parameter "p" of 0.589 which is also in agreement with the earlier report on the p value for reproduction of CFU-S.

Anatomical banding of intrinsic connections in striate cortex of tree shrews (Tupaia glis)

Abstract: The intrinsic connectivity of striate cortex was investigated by injecting horseradish peroxidase (HRP) into this area in tree shrews. Such HRP injections demonstrated periodically organized, stripelike connections within area 17. These stripes occur in layers I-IIIA and consist of a small number or retrogradely filled neurons, some clearly pyramidal, together with HRP-labeled axon terminals. HRP-filled axons trunks run between labeled stripes, interconnecting adjacent and distant regions of the stripe pattern. Correlation with Golgi-stained tissue suggests that these stripes are horizontally interconnected by pyramidal neurons with long intracortical axon collaterals (followed for distances over 1 mm from the soma). The HRP-labeled strips measure about 230 micrometers in width, with a center-to-center repeat distance of 450--500 micrometers. They have been mapped over an 8 mm2 area of striate cortex and would thus seem capable of effecting lateral interactions over considerable portions of the retinotopic map. In their dimensions and overall pattern, these anatomical stripes resemble the 2-deoxyglucose (2-DG) bands resulting from visual stimulation of trees shrews with stripes of a single orientation. While the functional role of the HRP-labeled stripes is unclear, their similarities with the 2-DG pattern raise the intriguing possibility that they may be related to orientation selectivity. The striking regularity of these extensive lateral interconnections emphasizes the importance of horizontal intralaminar connections within the cortex.

Fibroblast selection in scleroderma. An alternative model of fibrosis.

Abstract: Human preputial skin fibroblasts were cloned in vitro, and studies in 21 clones were shown to exhibit a 3-fold difference in collagen synthesis; this heterogeneity of phenotypic expression was conserved over multiple population doublings. When the clones were exposed to sera from 10 scleroderma patients, selective growth of high-collagen-producing fibroblasts was observed. Prostaglandin E2 abrogated the selective effect of scleroderma serum on high-collagen-producing fibroblast clones. Besides enhanced collagen synthesis, these clones of normal fibroblasts shared an insensitivity to feeding schedule with fibroblasts from scleroderma skin. These data raise the possibility that scleroderma represents a process of selection of fibroblasts programmed to produce increased amounts of collagen.

Physician Communication with Children and Parents

Abstract: The way physicians communicate with patients has been shown to affect physiologic measurements, adherence to therapeutic regimens, and satisfaction with medical care. The purpose of this study was to document the content of medical interviews in routine pediatric visits and to identify demographic and situational characteristics that influenced the extent of communication between doctor and child. One hundred fifteen office visits to 49 physicians were videotaped and analyzed. Children studied were 4 to 14 years old with a mean age of 8.5 years. Verbal transactions were coded according to direction of communication, transaction type, and content category. Coder reliability for this system was 0.84. A considerable amount of the total communication, 45.5%, was between doctor and child. Doctors interacted differently with parents and children. More information about the current problem was obtained from children; physicians provided feedback primarily to parents. Parents received 4.4. times as much information as children about the nature and prognosis of a condition. The extent to which doctors talked to children in "substantive" areas was primarily associated with a child's age (r = .52, P less than .001) but was partly influenced by family size (r = .20, P less than .05) and family utilization (r = .22, P less than .02). Race, socioeconomic status, type of problem, and previous encounter with the examining physician did not alter communication patterns. Boys were given more information than girls (6.5% vs 4.0%, P less than .01). We suggest a theoretical framework for future investigation and teaching that identifies the child as an active participant in the medical process.

Malignant lymphoma. 1. The histology and staging of 473 patients at the National Cancer Institute.

Abstract: A retrospective review was performed of 473 consecutive patients with malignant lymphoma referred to the National Cancer Institute. All patients had their clinical and pathologic material reviewed and where necessary reclassified. Using a modification of the Rappaport system, 180 (38.1%) patients had a nodular lymphoma, 293 (61.9%) had a diffuse lymphoma. Nodular lymphoma patients usually presented with lymphadenopathy; diffuse lymphoma patients often presented with extranodal disease, particularly those patients with poorly differentiated lymphocytic, "histiocytic", and Burkitt's lymphoma. Median age for all patients was 46 years, but was lower for diffuse poorly differentiated lymphocytic, diffuse undifferentiated, and Burkitt's lymphoma patients. Women were more likely to have a nodular rather than diffuse lymphoma. P less than 0.05. Analyzed by clinical staging, most nodular lymphoma patients had CS III (66.1%) disease, while most diffuse lymphoma patients had either CS III (31.1%) or CS IV (38.9%) disease. Analyzed by pathologic staging, most nodular lymphoma patients had PS III (34.8%) or PS IV (49.4%) disease, most diffuse lymphoma patients had PS IV (56.3%) disease. Only 7% of all lymphoma patients had PS I disease, only 14.9% were PS II. Systemic symptoms were more likely to be found in diffuse lymphoma and/or advanced stage patients.

Clonal origin of murine hemopoietic colonies with apparent restriction to granuclocyte-macrophage-megakaryocyte (GMM) differentiation.

Abstract: We characterized murine hemopoietic colonies consisting of granulocytes, macrophages, megakaryocytes, and blast cells and yet lacking erythroid elements. Mouse marrow or spleen cells were cultured in methylcellulose media in the presence of 10% (v/v) pokeweek mitogen-stimulated spleen cell-conditioned medium (PWM-SCM) and 2 units/ml erythropoietin for 8 days. Granulocyte-macrophage-megakaryocyte (GMM) colonies could be distinguished from granulocyte-erythrocyte-macrophage-megakaryocyte (GEMM) colonies because the former lacked the typical appearance of bursts with red color. Analysis of Y-chromosomes in mixing experiments with male and female marrow cells confirmed the clonal nature of the GMM colonies. Differential counts of GMM colonies revealed varying, but significant, numbers of blast cells in all of the day-8 and day-12 colonies and in seven out of ten day-14 GMM colonies. In general, the percentages of blast cells were inversely related to the length of incubation in culture. Replating experiments confirmed the absence of late erythroid precursors such as CFU-E and normoblasts in all of the 50 day-8 GMM colonies. However, six out of the 50 GMM colonies contained early progenitors capable of erythroid expression, such as BFU-E, CFU-EM, CFU-GEM, and CFU-GEMM. In contrast, the three day-14 GMM colonies which did not reveal blast cells failed to produce secondary colonies. Thus, while the progenitors for the latter colonies are restricted to only granulocyte-macrophage-megakaryocyte differentiation, some of the apparent GMM colonies containing blast cells may have originated in early progenitors close to pluripotent stem cells. Detailed cytological analyses and replating experiments are necessary for characterization of true differentiation potentials of mixed colonies in culture.

Enterobacter cloacae: Bacteremia, Epidemiology, and Antibiotic Resistance

Abstract: From the clinical viewpoint, Enterobacter cloacae and Enterobacter aerogenes are the two most important species of the genus Enterobacter. E. cloacae lacks lysine and arginine decarboxylases, whereas E. aerogenes has these enzymes. Enterobacter currently accounts for 40-12%?o of all cases of gram-negative bacteremia although it is not a cause of bacteremia at some hospitals. This study showed that E. cloacae has been endemic at a medical center hospital for at least five years and caused 4.5% of all cases of bacteremia in 1978. In May 1977 and July 1979 outbreaks of bacteremia due to E. cloacae occurred in the burn ward of the hospital. Most infected patients had multiple sites (urine, wound, and sputum) colonized before bacteremia occurred. No environmental or personnel sources of E. cloacae in the burn ward were discovered. Isolates of E. cloacae from the second outbreak were resistant to tobramycin, amikacin, and silver nitrate. The plasmid content of these strains differed from those of the first outbreak. For a five-year period, most strains of E. cloacae were resistant to cephalothin and ampicillin; however, resistance to aminoglycosides was rare. A literature review disclosed that E. cloacae has become prominent in burn units, but that it also has caused serious infections in nonburned patients. E. cloacae tends to contaminate various medicinal, iv, and other hospital products. Therefore, bacteremia due to E. cloacae, should prompt an investigation of exposures to potentially contaminated materials, especially those introduced into the bloodstream. Therapy of E. cloacae infections remains problematic and is complicated by the emergence of multiresistant strains. Aminoglycosides are the most active agents against E. cloacae, but new agents such as cefotaxime, cefaperazone, moxalactam, and thienamycin are active and eventually may be valuable in single-drug therapy of infection due to E. cloacae.

Modification of visual callosal projections in rats.

Abstract: The effects of a variety of developmental manipulations on the distribution of the callosal pathway to visual cortex were examined by using the Fink Heimer technique in adult rats. First, the callosal projections in albino and pigmented rats were compared and found to be similar. The callosal pathway was limited in area 17 to a region adjoining its lateral border with area 18a. Second, dark-reared rats were found to have normal callosal projections. Third, rats bilaterally enucleated at birth had expanded callosal inputs within area 17. Fourth, monocular enucleation at birth produced an expanded callosal pathway to area 17 contralateral to the enucleation and normal callosal projections to the opposite hemisphere. The expanded callosal inputs after enucleation showed a patchy distribution and usually avoided the most medial part of area 17. Fifth, a reduction in the callosal projections to the area 17/18a border was found after neonatal unilateral optic tract lesions. Sixth, expanded callosal inputs to area 17 were found following unilateral thalamic lesions at birth. The abnormal projection occupied mainly layers IV and III. The results of the different experiments indicate that the detailed distribution of the visual callosal projection within area 17 depends heavily on the organization of the retinogeniculocortical pathways to each hemisphere.

Evidence for similar conformational changes in α2-macroglobulin on reaction with primary amines or proteolytic enzymes

Abstract: Reactions of alpha(2)-macroglobulin (alpha(2)M) with primary amines (ammonium chloride, methylammonium chloride and ethylammonium chloride) or proteolytic enzymes (trypsin, chymotrypsin and thrombin) resulted in changes of the absorption, fluorescence and circular-dichroism spectra and of the sedimentation coefficient of the inhibitor. All physico-chemical changes caused by the inactivation of alpha(2)M by the amines were identical with, or highly similar to, those induced by the formation of the enzyme-inhibitor complexes. This suggests that similar conformational changes of the inhibitor occur in the two types of reactions. The frictional ratio, calculated from the increase in sedimentation coefficient, decreased from 1.67 for untreated alpha(2)M to 1.57 for the amine- or proteinase-treated inhibitor. This change is due to a decrease in either asymmetry or hydration of the protein, resulting in a slightly smaller hydrodynamic volume. The circular-dichroism analyses indicated that the reaction of alpha(2)M with either amines or proteinases is accompanied by a loss of the small amount (about 5%) of alpha-helix of the untreated protein. The changes of u.v. absorption and fluorescence suggested that about one out of the eight to ten tryptophan residues of each alpha(2)M subunit is buried as a result of the conformational change. All spectroscopic and hydrodynamic changes that were observed are compatible with a spatial rearrangement of the subunits of alpha(2)M, as implicated by the ;trap' hypothesis for the mechanism of inhibition of proteinases. However, a conformational change involving a decrease in the hydrodynamic volume of each subunit cannot be excluded.

Surface binding, internalization and degradation by cultured human fibroblasts of low density lipoproteins isolated from type 1 (insulin-dependent) diabetic patients: changes with metabolic control.

Abstract: A previous study of low density lipoprotein metabolism by cultured cells focused on the metabolism of normal lipoproteins in vitro by fibroblasts isolated from diabetic patients. No abnormalities were found. We have followed the opposite approach. Using normal human fibroblasts as test cells we compared the metabolism in vitro of low density lipoproteins isolated from diabetic patients before and after metabolic control. We found a significant decrease (p<0.02) in internalization and degradation of low density lipoproteins isolated from diabetic patients before metabolic control when compared with those isolated from normal control subjects or from the same patients after metabolic control. The observed changes were mainly apparent in intracellular degradation. To evaluate whether the observed differences in low density lipoprotein behaviour were correlated with lipid or apolipoprotein composition, we measured cholesterol, triglyceride, apolipoprotein B and total protein levels in the low density lipoproteins tested. A significant decrease (p<0.05) of the triglyceride/protein ratio was found in post-control low density lipoproteins suggesting that a high triglyceride content may interfere with low density lipoprotein metabolism. The present study represents the first observation that metabolic control in diabetes mellitus can alter low density lipoprotein-cell interaction and suggests a possible mechanism for the enhanced incidence of atherosclerosis in diabetic patients.

Retinotopic and temporal organization of the optic nerve and tracts in the adult goldfish.

Abstract: In order to investigate the role of the different factors controlling the pathways and termination sites of growing axons, selected optic fibers were traced from the eye to the tectum in adult goldfish either by filling them with HRP, or by severing a group of fibers and tracing their degeneration in 2 micrometers plastic sections stained with toluidine blue. Some fish received more than one lesion and others received both lesions and HRP applications. Two major rearrangements of the optic fibers were identified, one at the exit from the eye, the other within the optic tracts. Near the eye the optic fibers appear to be guided by the conformation of the underlying tissue planes that they encounter. The most recently added fibers, from the peripheral retina, grow over the vitread surface of the older fibers toward the blood vessel in the center of the optic nerve head. Behind the eye the fibers follow this blood vessel until it leaves the side of the optic nerve, and the fibers from peripheral retina are left as a single group on the ventral edge of the optic nerve cross section. As a consequence of this pattern of fiber growth the fibers form an orderly temporal sequence in the optic nerve, with the oldest fibers from the central retina on one side of the nerve and the youngest from peripheral retina on the other. In addition, the fibers are ordered topographically at right angles to this central-to-peripheral axis, with fibers from ventral retina on each edge of the nerve, dorsal fibers in the center, and nasal and temporal fibers in between. This arrangement of the optic fibers continues with only a little loss of precision up to the optic tracts. A more radical fiber rearrangement, seemingly incompatible with the fibers simply following tissue planes occurs within the optic tracts. Each newly arriving set of fibers grows over the surface of the optic tracts so that the older fibers come to lie deepest in the tracts. This segregation of fibers of different ages ensures that the rearrangement is limited to each layer of fibers. The abrupt reorganization of the fibers occurs as the tracts split around the nucleus rotundus to form the brachia of the optic tracts. The fibers are then arranged with temporal fibers nearest the nucleus rotundus and nasal fibers on the opposite edges of the brachia. From this point the fibers grow out over the tectal surface to their termination sites with only minimal rearrangements. Therefore the optic fiber rearrangements show evidence of several different sorts of constraints acting on the fibers at separate points in the optic pathway, each contributing to the final orderly arrangement of the fibers on the optic tectum.

Transient retinofugal pathways in the developing chick.

Abstract: The central retinal projections have been examined in normal chick embryos early in development to determine if a transient ipsilateral projection is present. Anterograde transport of HRP identified using tetramethyl benzidine as a chromogen and degeneration techniques were used on embryos of successive ages between 6 and 16 days of incubation to show the central distribution of the retinal axons from one eye. Besides the anticipated contralateral projection, a small projection was identified to primary visual nuclei on the side of the brain ipsilateral to the injected or lesioned eye in embryos between days 6 and 12 of incubation. A projection from the injected eye into the contralateral optic nerve was also identified in a number of embyos. By embryonic day 15 the retinal projection to the ipsilateral side of the brain and into the contralateral optic nerve had disappeared. This loss of the anomalous projections coincides with a period of substantial cell death in the retinal ganglion cell layer. It appears, therefore, that in the chick, like the rat, the ipsilateral retinofugal projection resulting from an embryonic enucleation may in part be due to retention of a normal ipsilateral projection.

Malignant lymphoma. II Prognostic factors and response to treatment of 473 patients at the National Cancer Institute.

Abstract: Treatment results were reviewed in 473 consecutively staged and treated patients at the National Cancer Institute over a 22-year period from 1953 to 1975. Responses correlated with histologic pattern and stage of disease. Complete responses to radiotherapy were frequent in CS I (86%) or PS I (91%). CS II (70%) or PS II (69%) nodular lymphoma patients. Similar treatment regimens were less effective in diffuse lymphoma patients, CS I (53%) or PS I (57%) and CS II (50%) or PS II (51%). Using chemotherapy or combined modality approaches, complete responses were obtained in a high proportion of advanced nodular disease patients, CS III (51%) or PS (59%), CS IV (44%), or PS IV (46%). With the introduction of combination chemotherapy and/or modern radiotherapeutic techniques, 52% CS III and 63% PS III, and 47% CS IV and 46% PS IV patients achieved a complete response. Patients with nodular lymphoma tend to have higher complete response rates and longer survivals than their counterparts with diffuse histologic types (P less than 0.05). Patients with nodular lymphocytic lymphoma had a better survival than those with mixed or "histiocytic" histologic types (P less than 0.03). Patients with diffuse well differentiated lymphocytic lymphoma survived significantly longer than patients with other diffuse histologic types (P less than 0.05). Percentage and prominence of nodularity were not of prognostic significance in those patients with combined nodular and diffuse patterns of disease. When compared by histologic type, patient sex did not appear to be an important prognostic factor. The presence of B-symptoms was associated with a poorer survival in patients with nodular disease (P less than 0.05) and in patients with diffuse disease (P less than 0.001). Over the years of this study, survival appears to have improved in each histologic subtype except DPDL.

Antibodies to collagen in scleroderma.

Abstract: Using the enzyme-linked immunosorbent assay (ELISA), we detected antibodies to interstitial (type I) and basement membrane (type IV) collagens in the sera of patients with scleroderma (systemic sclerosis) Antibodies against type IV collagen were found in significant levels in these patients and correlated with the presence of abnormal pulmonary diffusion capacity Levels of antibodies to type I collagens also correlated significantly with pulmonary diffusion capacity Absorption of sera with type I or type IV collagens before analysis in the ELISA eliminated reactivity in an antigen-specific pattern, indicating that these antibodies reacted with determinants specific for either type I or type IV collagens The removal of immune complexes by ultracentrifugation had no effect on serum antibody levels Autoantibodies to basement membrane and interstitial collagens may participate in the pathogenesis of scleroderma

Degradation of cytoskeletal proteins in experimental spinal cord injury

Abstract: Spinal cord injury was produced in rats by dropping a 10 g weight from 30 cm upon dura-invested exposed spinal cord. Examination of the fine structure of the traumatic lesion (15 min to 30 min) revealed granular degeneration of axons and occasional loosening of myelin lamellae. Older lesions (4 to 72 hours) showed degeneration of axons and vesiculation of myelin. At 15 minutes there is more loss of neurofilament proteins than of myelin proteins. Substantial decreases in the neurofilament and myelin proteins were observed at 30 minutes and the losses were even greater 2–72 hours after injury. This indicates that degeneration of axons may precede degradation of the myelin sheath and also that increased proteinase(s) activity, possibly activated by calcium, mediates the traumatic axonolysis and myelinolysis in experimental spinal cord trauma.

Ultrastructural visualization of galactosyl residues in various alimentary epithelial cells with the peanut lectin-horseradish peroxidase procedure

Abstract: A conjugate of peanut lectin with horseradish peroxidase (PL-HRP) has been employed for ultrastructural localization of glycoprotein with presumed terminal galactose residues in mouse alimentary epithelial cells. The PL-HRP conjugate imparted electron opacity in sites that stain at the light microscopic level, as for example, Golgi cisternae in surface epithelial cells of the stomach and in superficial and deep crypt cells and goblet cells of the large intestine. Ultrastructural staining revealed that Golgi cisternae intermediate between the trans and cis faces stained selectively in these sites. Secretion stored in secretory granules or Golgi vesicles in the cells lacked affinity for PL-HRP conjugate, however. Selective staining of intermediate Golgi cisternae in cells with unreactive secretory product is interpreted as indicating the site of galactosyl transferase activity and a location where galactose occurs transitorily as the terminal sugar in the glycoprotein side chains. The luminal aspect of the surface epithelial cells in the stomach and columnar cells in the colon also stained, but with some variability. Staining of these surfaces was considered possibly attributable to PL affinity of some of the secretory glycoprotein which, after absorbing to the cell surface, lost terminal sialic acid through action of luminal enzyme. PL-HRP conjugate stained granules in pancreatic zymogen cells near the block surface but not in other cells, presumably because of limited penetration of reagent. Secretion on the surface of pancreatic acinar cells or in the lumen also exhibited affinity for PL-HRP complex as did the luminal surface of gastric chief cells. Staining of secretion in the pancreatic zymogen cells and gastric chief cells for galactose appeared inconsistent with lack of evidence for presence of glycoprotein in these sites which failed to stain with the periodic acid-Schiff or periodic acid-thiocarbohydrazide-silver proteinate methods for demonstrating glycoprotein at the light and electron microscopic levels. This discrepancy points to possible selective binding of PL-HRP conjugate to a moiety other than terminal galactose of glycoprotein in a few histologic sites. These results demonstrate the applicability of the PL-HRP technique at the ultrastructural level and provide information concerning the chemical structure of epithelial cell glycoproteins and their biosynthesis.

Modulation of the Immune Response by Plasma Protease Inhibitors

Abstract: In two different cytolytic assays, dose-dependent inhibition of antibody-dependent cellmediated cytotoxicity and natural killing by alpha2-macroglobulin (α2m) and its subunits was observed. α2m subunits were approximately four times more inhibitory in both assays than the native 2m molecule on a molar basis. Analysis of trypsin protein esterase activity demonstrated that α2m subunits were four times more active than the parent molecule.

Acute alcohol intoxication and female orgasmic response

Abstract: This study tested the hypothesis that increasing levels of acute alcohol intoxication are related to systematic changes in female orgasmic experience reflected by physiological, behavioral, and cognitive indices. Using a repeated measures design with monthly experimental sessions, each of 18 university women were sustained at four different blood alcohol concentrations (BAC) in counterbalanced order prior to viewing sexually explicit videotapes and engaging in masturbation to orgasm. Measures of vaginal blood volume obtained by means of a vaginal photoplethysmograph and complemented by a behavioral latency measure showed a progressive and systematic depressant effect of alcohol on orgasmic responding. Higher BACs were associated with longer orgasmic latencies and decreased subjective intensity of orgasm, while, paradoxically, women reported significantly greater sexual arousal and orgasmic pleasurability under conditions of moderate and high alcohol intoxication. Results have implications for tre...

The Prenatal Diagnosis of Adrenoleukodystrophy. Demonstration of Increased Hexacosanoic Acid Levels in Cultured Amniocytes and Fetal Adrenal Gland

Abstract: Amniocentesis was performed in two women heterozygous for adrenoleukodystrophy (ALD). One fetus was male, and the hexacosanoic acid (C26) level in the cultured amniotic cells was 0.808 microgram per mg of protein, compared to 0.104 +/- 0.069 (S.D.) in controls. Pregnancy was interrupted at 22 wk gestation. The fetal adrenal cortex showed the ultrastructural inclusions characteristic of ALD and C26 accounted for 35% of the fatty acids in the cholesterol esters extracted from this tissue, more than one thousand times control. The second amniocentesis was performed in a woman who was also heterozygous for an electrophoretic variant of glucose 6- phosphate dehydrogenase (G6PD), and a member of a kindred showing genetic linkage of loci for ALD and G6PD. The fetus was female and the C26 level in cultured amniotic cells was 0.577. Pregnancy was interrupted at 11 wk for reasons unrelated to ALD. Study of C26 level and G6PD type in cultured fetal tissues confirmed heterozygosity for ALD.