A 79-year-old Armenian-born woman with stable, long-term familial Mediterranean fever had progression of chronic renal failure concurrently with two types of skin lesions. One lesion resembled erysipelas, which is quite common in familial Mediterranean fever, whereas the other was panniculitis, only occasionally described in familial Mediterranean fever. The unique histopathologic features of the latter are presented. The onset of acute cutaneous disease in this patient coincided with worsening renal disease and preceded a flare of disease activity in other sites after a 14-year period of quiescence. The severe and unremitting pain from the skin lesions and their tendency to form nonhealing ulcers were substantial causes of morbidity in this patient with familial Mediterranean fever.

Aged↗

PubMed1987

Heritable disorders of hair.

Changes in the quality and quantity of hair occur in a large number of hereditary syndromes. Because of their rarity and our lack of knowledge about their pathophysiology, no entirely satisfactory classification of these hair disorders exists. This review arbitrarily groups the heritable disorders of the hair into five classes: primary hair shaft disorders, alopecia without associated defects, ectodermal dysplasias, changes in hair color, and ectopic hair.

Alopecia↗

PubMed1986

Alopecia: common and uncommon.

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Administration, Topical↗

PubMed1986

Intermittent hair follicle dystrophy. Report of a new disorder.

A new disorder of the hair follicle is described that leads to increased fragility of the shaft. Light microscopic studies of hair follicles revealed disturbances of keratinization in the shafts that are very likely responsible for the breakage. The most consistent change seen by transmission electron microscopy was intercellular edema of the outer root sheath, while scanning electron microscopy of the hair revealed disturbances of the surface and broken ends. Specific biochemical abnormalities of the hair proteins were not found. Possible mechanisms for the disease are discussed.

Alopecia↗

PubMed1985

Cost containment: freestanding emergency centers and the emergency department.

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Ambulatory Care Facilities↗

PubMed1982

Modification of decision-making behavior of third-year medical students.

Modification of physician behavior has been suggested as one approach to controlling health care expenditures. Third-year students participated in an experimental program which attempted both to construct an educational approach that included aspects of such expenditures and to measure the effectiveness of the approach. Students were divided between experimental and control groups, with the former receiving information relative to the appropriate use and costs of diagnostic procedures. Both groups were presented with case studies dealing with specific disease processes discussed in the program. Members of the experimental group scored significantly better in their ability to determine diagnoses, make patient-management decisions, and choose essential diagnostic procedures. Average charges generated by the experimental student group were half the amount generated by the control group. Future studies will be required to determine whether the behavior modification carries forward in practice and to provide a standardized methodology for use by members of the faculty and house staff in educating future medical students.

Clinical Laboratory Techniques↗

PubMed1979

Liquid diffraction analysis of the model membrane system. Egg lecithin + myelin protein (N-2).

The scattering from the model membrane system, egg lecithin + myelin protein (N-2), has been analyzed by liquid diffraction methods. It is found that by manipulating the protein-lipid ratio, the scattering domains of the protein and lipid can be identified. The multilayer contribution can also be identified by its position and concentration behavior in both the intensity pattern and its Fourier transform. When the multilayer and protein components are subtracted, the phospholipid scattering and an interaction term are left: these two can be resolved by a reasonable assessment of their relative magnitude in the boundary region where they overlap. The interaction term can then be used to determine the most probably position of the protein in the membrane. The deconvoluted protein and lipid transforms can then be combined in the proper way to obtain the electron density profiles. The resolution of the interaction term is not yet complete, but a method for accomplishing this is discussed.

Egg Yolk↗

PubMed1979

The model membrane system. Egg lecithin + myelin protein (N-2). Effect of solvent density variation on the X-ray scattering.

The electron density contrast method has been applied to the membrane system egg lecithin + myelin protein (N-2). The case treated here is for a low protein concentration. As theory predicts, the scattering from the different regions of the membrane (protein, hydrocarbon, and polar head group regions) are modulated differently by changing the contrast. It is then possible to separate out the electron pair correlation functions for the different regions, and from these to determine the membrane election density distribution for an external electron density p0 = 0.

Egg Yolk↗

PubMed1977

The effect of centrifugal force on glycosaminoglycan production by aortic smooth muscle cells in culture.

Cultured smooth muscle cells from pig aorta subjected to centrifugation (48 h at 45 g over a 72-h period) increased their production of glycosaminoglycans by approximately 50%. The sulphated components, heparan sulphate, dermatan sulphate and chondroitin sulphate, showed a relatively greater increase than hyaluronic acid (66-34%). The results are consistent with the hypothesis that mechanical stress, such as hypertension, leads to increased accumulation of glycosaminoglycans in the aortic wall and that secondary trapping of lipoproteins by sulphated glycosaminoglycans produces atherosclerotic plaques.

Animals↗