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Biomedical subjects

S R Kohn

Publications and source records attributed to S R Kohn.

At least 19 recordsLinked to original sources
PubMed1995

Muir-Torre syndrome.

The Muir-Torre syndrome is an autosomal dominantly inherited genodermatosis with malignant potential that is characterized by the presence of at least one sebaceous gland tumor (adenoma, epithelioma, or carcinoma) and a minimum of one internal malignancy. The syndrome has been documented in 147 individuals. Associated features in some of the Muir-Torre syndrome patients are colorectal carcinomas and genitourinary neoplasms. More than half of the 292 visceral cancers described in Muir-Torre syndrome patients were colorectal carcinomas; nearly 60% of these tumors were located at or proximal to the splenic flexure. The presence of even a single Muir-Torre syndrome-associated sebaceous tumor warrants serious consideration for further evaluation of that individual for the syndrome. Therefore, initial and periodic examination for internal malignancy should be performed in individuals with such tumors and patients with the syndrome. Also, family members of Muir-Torre syndrome patients should be screened for Muir-Torre syndrome-associated cutaneous lesions and visceral cancers.

Adenoma↗
PubMed1994

Cutaneous manifestations of diabetes mellitus.

Diabetes mellitus (DM) is a heterogeneous group of disorders characterized by a high serum glucose level and by disturbances of carbohydrate and lipid metabolism. It is estimated that 11 million persons in the United States have DM, 90% of whom have non-insulin-dependent DM. At least 30% of persons with diabetes have some type of cutaneous involvement during the course of their chronic disease. This review classifies the cutaneous findings in DM into four categories: (1) skin diseases with strong to weak association with DM; (2) cutaneous infections; (3) cutaneous manifestations of diabetic complications; and (4) skin reactions to diabetic treatment. Each of these categories is reviewed as well as the pathophysiology of the normal and diabetic basement membrane for a better understanding of the cutaneous manifestations of DM.

Basement Membrane↗
PubMed1994

Atrophia maculosa varioliformis cutis. Report of two cases and review of the literature.

Atrophia maculosa varioliformis cutis was initially described in 1918 as an entity in which both linear and punctate scars appeared spontaneously on normal facial skin. To the best of our knowledge, only five additional cases have been described. We describe two patients, 14 and 20 years of age, whose histories and clinical lesions fit the description of atrophia maculosa varioliformis cutis. The histologic findings are also described, and the literature to date is reviewed.

Adolescent↗
PubMed1993

Systemic sclerosis.

Systemic sclerosis affects the connective tissue of various organs. It is characterized by alteration of the microvasculature with a dense inflammatory cellular infiltrate, followed by massive deposition of collagen. This review discusses diagnostic criteria, clinical subsets, survival data, clinical evaluation, epidemiology, pathogenesis, immunobiology, genetics, and therapy for systemic sclerosis in adults. Because the diagnosis of systemic sclerosis is most dependent on its cutaneous involvement, knowledge of this is essential in the evaluation and management of patients with this disorder.

Humans↗
PubMed1991

Altered beta adrenergic receptor function in subjects with symptomatic mitral valve prolapse.

Individuals with mitral valve prolapse (MVP) frequently show symptoms of a hyperadrenergic state. beta adrenergic receptor characteristics were compared in the lymphocytes of subjects with symptomatic MVP and control subjects during rest and exercise. At rest, the proportion of receptors binding agonist with high affinity, as determined from isoproterenol competition for (-)[125I]-iodopindolol binding sites, was greater in MVP subjects than in controls. With exercise, the proportion of high-affinity receptors in MVP subjects decreased to control levels. Isoproterenol stimulation of lymphocyte 3',5'-cyclic adenosine monophosphate (cyclic AMP) also was greater in MVP subjects than in controls at rest, but not during exercise. Plasma catecholamine concentrations in MVP subjects were normal during both rest and exercise. Unlike exercise, isoproterenol infusion elicited clinical manifestations of increased adrenergic responsiveness in MVP subjects. The beta receptor in exercised MVP subjects exhibited unusually high affinity agonist binding (i.e. a lower dissociation constant KH than in either the same subjects at rest or exercised controls) and also abnormal coupling to the stimulatory guanine nucleotide-binding regulatory protein (GS) of adenylate cyclase, as reflected by the inability of guanine nucleotide to convert the receptor to a low-affinity state. These findings suggest that functional alteration of the beta adrenergic receptor, in the absence of abnormal plasma catecholamine levels, might contribute to the hyperadrenergic state of MVP subjects at rest. However, desensitization of high affinity beta receptors or altered receptor-GS coupling might preserve normal adrenergic responsiveness during exercise.

Adult↗
PubMed1991

A brief history of trying: man, NASA, and medicine.

The development of the V2 rocket during World War II raised the possibility of manned spaceflight to the level of "serious consideration." Concepts of aviation medicine led to the specialty of space medicine. Projects Mercury, Gemini, Apollo, Skylab, and Apollo-Soyuz, and the Space Transportation System have helped us gain important biomedical information, including but not limited to, microgravity and its effects on red cell mass, orthostatic tolerance, exercise capacity, bone density, and muscle nitrogen levels. Problems of "space motion sickness" and micro-organisms in space have become major concerns. This analytic review covers these topics and relevant others.

Aerospace Medicine↗
PubMed1991

The word is out.

Explore the source record for details and available documents.

Dermatology↗
PubMed1991

Association of sebaceous gland tumors and internal malignancy: the Muir-Torre syndrome.

The Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis characterized by: (1) at least a single sebaceous gland tumor (either an adenoma, an epithelioma, or a carcinoma) and (2) a minimum of one internal malignancy. To date, 120 patients with MTS have been reported. The most commonly associated neoplasms were colorectal (51%) and genitourinary (25%). Unlike colorectal neoplasms in the general population, the majority (58%) of these tumors in MTS patients occurred proximal to or at the splenic flexure. Nearly half of the MTS patients had more than one primary malignancy. Cutaneous lesions occurred before or concurrent with the diagnosis of the initial cancer in 41% of these patients. The median age for the appearance of the skin lesions was 53 years (range, 23 to 89 years); the median age for the detection of the initial visceral neoplasm was 50 years (range, 23 to 81 years). The cancers appear to have an indolent course in many of the MTS patients; the median survival has not been reached and the median follow-up is 10+ years. Patients with an MTS-associated cutaneous lesion should have a complete evaluation for gastrointestinal or genitourinary cancers. Although the penetrance of this disease is variable, its autosomal dominant inheritance suggests that relatives should be examined for sebaceous gland tumors and internal malignancy.

Adult↗
PubMed1990

Noduloulcerative or "malignant" syphilis occurring in an otherwise healthy woman: report and review of a dramatic dermatosis.

We present the case of an otherwise healthy woman with noduloulcerative ("malignant") syphilis, the first female patient reported with this dermatosis in fifty years. This rare form of secondary syphilis is characterized by noduloulcerative lesions with rupioid crusts that dramatically involve the face while usually sparing the palms and soles. The aggression and destruction of the skin lesions may mimic that of other granulomatous and infiltrative dermatoses. Since the incidence of syphilis is increasing, physicians should be aware of this variant that is so disfiguring it has historically been referred to as lues maligna.

Facial Dermatoses↗
PubMed1990

Vitiligo and pernicious anemia presenting as congestive heart failure.

The skin often provides diagnostic clues to systemic disorders. Vitiligo is an acquired disease characterized by depigmentation of the skin due to destruction of melanocytes. Vitiligo may be an autoimmune disease and is associated with other disorders that may also arise due to autoimmune mechanisms. We present the unusual case of vitiligo associated with pernicious anemia in a patient who presented to the hospital because of hyperdynamic congestive heart failure.

Adult↗