Two patients with hereditary homozygous C2 deficiency are described. They showed many similar clinical features. Both had discoid-like skin rashes and serologic abnormalities consistent with SLE, but neither had evidence of involvement of any organ other than skin. Although biopsy of these lesions revealed expected changes by light microscopy, typical immunofluorescent findings were not present. The presence of the HL-A haplotype 10, W18 was seen in both families and was associated with the C2 deficiency gene. The 2 patients were mutually nonreactive in MLC tests and both were found to be homozygous for the LD7a type.

Adult↗

PubMedDate not supplied

Systemic lupus erythematosus associated with klinefelter's syndrome.

Studies in estrogen metabolism were carried out in 2 males with systemic lupus erythematosus (SLE) who also had Klinefelter's syndrome. Although these studies revealed elevated levels of urinary estrogens in 1 patient, abnormalities of estradiol metabolism suggesting persistent estrogen stimulation were detected in both. This report notes an association between SLE and Klinefelter's syndrome and suggests that chronic estrogenic stimulation may be significant in the development of SLE in these 2 patients.

Adult↗

PubMedDate not supplied

Collagen production by rat hepatocytes and sinusoidal cells in primary monolayer culture.

The cellular sources of collagen in normal rat liver have been examined. Hepatocytes and nonparenchymal (sinusoidal) cells were isolated and established in primary monolayer culture. These cells were incubated with radiolabeled proline in the presence of l-ascorbate and beta-aminopropionitrile. Nondialyzable material was prepared from the cell layer and the medium from each type of culture. The level of collagen accumulation was determined by measuring labeled hydroxyproline and sensitivity to purified bacterial collagenase. In hepatocytes, collagen represented 0.2% of both secreted and cell-associated labeled protein. In sinusoidal cells, collagen was 3.2% of secreted and 1.1% of cell-associated proteins. The total secreted labeled collagen, expressed per microgram of DNA, was similar in hepatocytes and sinusoidal cells. However cell-associated collagen in hepatocyte culture was approximately 10-fold that present in sinusoidal cells. These findings indicate that, while collagen formation is a relatively important function of sinusoidal cells, in normal liver the contribution of hepatocytes to total hepatic collagen accumulation may be substantial.

Animals↗

PubMedDate not supplied

Types of collagen synthesized by normal rat liver hepatocytes in primary culture.

Collagen formation is an important function of liver parenchymal cells that may be relevant to the pathogenesis of hepatic fibrosis. The types of collagen synthesized by cultured normal rat liver hepatocytes were examined. Cells isolated from adult rats by enzymatic dispersion of the liver were established in primary monolayer culture. Cells were then incubated with radiolabeled proline for 20 hr in the presence of ascorbate and the lathrogen beta-aminopropionitrile. Collagen secreted into the cell media was assessed separately from that in the cell layer. The greater proportion of newly synthesized collagen was associated with the cell layer. Collagen types were identified by ion exchange chromatography and by polyacrylamide gel electrophoresis. Types I, III, IV, and V collagen were present in both media and cell layer. Types III and V were the predominant types found. Very little Type I collagen was synthesized by these cultured normal hepatocytes. The percentages of Types I, III, IV, and V collagens, combining media and cell layer, were 6, 38, 19, and 36, respectively.

Animals↗

PubMedDate not supplied

Viewing chronic mental illness: a conceptual framework.

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Chronic Disease↗

PubMedDate not supplied

Measurement of the velocity of ultrasound in human cortical bone and its potential clinical importance. An in vivo preliminary study.

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Adult↗

PubMedDate not supplied

Histologic study of artificial skin used in the treatment of full-thickness thermal injury.

Integra artificial skin is an effective means of treatment for full-thickness burns. In extensive burn injury the use of such skin substitutes may become the treatment of choice. The artificial skin consists of a dermal substitute of bovine collagen and chondroitin-6-sulfate and an epidermal layer of synthetic polysiloxane polymer (Silastic). Serial biopsy specimens were obtained from 131 patients during a period of 7 days to 2 years after application. In this histologic study, six sequential phases of repair were discerned. In addition, there were occasional unusual histologic features, eosinophilic infiltration, and/or macrophage-derived giant cell formation in the wound area; however, such findings did not clinically correlate with a negative response to Integra artificial skin. Good repair was obtained, with rare exceptions. An intact dermis was achieved as well as definitive closure of a complete epidermal layer with a minimum of scarring.

Artificial Organs↗

PubMedDate not supplied

Development of Bragg diffraction imaging for medical use.

Experience gained in developing a real-time acoustical imaging system using Bragg diffraction is presented. Particular emphasis has been placed on medical applications. We have studied the difficulties in assembling a system capable of producing high-quality images. Engineering data on the optical mounting and on the design of the transducer along with representative images are presented and discussed.

Bone and Bones↗

PubMedDate not supplied

Measurement of the velocity of ultrasound in the human femur in vivo.

A pulse-echo technique for measuring the velocity of ultrasound in the cortex of the femur in vivo is presented. The average result for 27 asymptomatic volunteers is 3250 +/- 190 m/s. The results show no significant differences between proximal and distal femoral sites, right and left femora, males and females. There is also no significant difference between the velocity of sound in the femur and in the radius in normal volunteers. The reproducibility of the time of flight measurement for three volunteers is found to be about 2%, and the total uncertainty of the femoral velocity of ultrasound measurement is determined to be 4% for proximal sites and 7% for distal sites. As the femur is a load-bearing bone, the site of frequent fracture both in the elderly and in the osteoporotic, the measurement of the velocity of sound at this new site may be of great value in investigating these conditions and may ultimately be helpful in assessing "quality" of the femoral cortex in those patients undergoing total hip or knee replacement.

Adult↗

PubMedDate not supplied

The measurement of trabecular bone mineral density using coherent and Compton scattered photons in vitro.

A photon scattering method for measuring the trabecular bone mineral density (TBMD) in vitro is described. This method involves the measurement of the ratio of coherent to Compton 90 degrees scattered photons from Am-241 by using a narrow beam geometry with an intrinsic germanium detector. The feasibility of using smaller scattering angles for better counting efficiency and the associated problems in their application for in-vivo measurements were investigated. Calibration of the system with fresh trabecular bone samples showed a linear relationship between the coherent to Compton ratio R of the detected counts and the TBMD (r = 0.94). The effect of the overlying soft tissue on the R ratio was significant while the effect of self-attenuation by the trabecular bone itself and the cortical layer was negligible. It was found that the marrow fat content could alter the value of the R ratio. Our results show that for a 10% increase in the fat content in the interstices of the trabecular bone there is a 2.5% decrease in the R ratio. This technique together with soft tissue corrections will enable us to measure the TBMD of the calcaneum in vivo, assuming a small variation in the trabecular fat content. The estimated absorbed dose to the bone marrow is about 139 mrad.

Americium↗