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4 results on '"Helfrich, David J."'

1. Normotensive renal failure in systemic sclerosis

Author

Helfrich, David J., Banner, Barbara, Steen, Virginia D., and Medsger, Thomas A., Jr.

Subjects
Acute renal failure -- Causes of, Scleroderma (Disease) -- Care and treatment, Hypertension -- Complications, Scleroderma (Disease) -- Complications, Adrenocortical hormones -- Health aspects, Health
Abstract

Systemic sclerosis, also known as scleroderma, is a rare autoimmune disease affecting connective tissue and characterized by thickening of the skin due to deposits of collagen, a fibrous protein. One of the most serious complications resulting from scleroderma is kidney or renal failure, which usually occurs in patients who also have high blood pressure or hypertension. However, a number of studies describe scleroderma patients having renal failure without hypertension. Over a 33 year period 131 patients who experienced scleroderma related kidney failure were followed. Eleven percent of this group had normal blood pressure or were normotensive. Researchers attempted to isolate factors that would cause a normotensive scleroderma patient to develop renal failure. Their analysis revealed that these patients had significantly higher incidences than the hypertensive patients of various diseases: asthma (80 percent versus 33 percent of hypertensives); small blood vessel diseases (90 percent versus 38 percent); and an abnormal decrease in blood platelets or thrombocytopenia (83 percent versus 21 percent). Another significant finding in the medical histories of the normotensive scleroderma patients who developed renal failure was the fact that 64 percent of them had been administered maximum doses of corticosteroids during the two months prior to the onset of kidney failure, compared to only 16 percent of the hypertensive scleroderma patients. Since both scleroderma and corticosteroid medications can cause injury to circulatory tissue or vascular endothelium, it seems likely that high doses of corticosteroids were a contributing factor to the onset of kidney failure. It is recommended that only low doses of corticosteroids be prescribed for patients with scleroderma who are at high risk for developing kidney failure.

Published
1989

4. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease.

Author

Scalapino K, Arkachaisri T, Lucas M, Fertig N, Helfrich DJ, Londino AV Jr, Steen VD, and Medsger TA Jr

Subjects
Adult, Age of Onset, Autoantibodies blood, Autoantigens blood, Autoantigens immunology, DNA Topoisomerases, Type I immunology, Dermatomyositis physiopathology, Exoribonucleases, Exosome Multienzyme Ribonuclease Complex, Female, Heart Diseases physiopathology, Humans, Male, Middle Aged, Muscle, Skeletal physiopathology, Prognosis, RNA Polymerase III immunology, Scleroderma, Systemic mortality, Scleroderma, Systemic physiopathology, Severity of Illness Index, Survival Rate, Outcome Assessment, Health Care, Scleroderma, Systemic classification, Scleroderma, Systemic epidemiology
Abstract

Objective: To describe the differences between patients with systemic sclerosis (SSc) having childhood versus adult onset evaluated at a single medical center., Methods: Patients were divided into those with childhood onset (first SSc symptom or finding before age 16 yrs) and those with early adult and late adult onset. The 3 groups were compared with respect to disease classification, clinical, laboratory and serologic data, and survival., Results: One hundred eleven childhood onset SSc cases seen between 1960 and 2003 were compared with 2559 adult onset SSc cases (1087 with onset age 16-40 and 1472 with onset after age 40 yrs) first evaluated between 1972 and 2001. Age distribution at onset was unimodal, suggesting that childhood disease is part of the spectrum of adult onset SSc. A significantly greater proportion of childhood onset patients had overlap syndromes, most frequently with polymyositis-dermatomyositis (PM-DM), and skeletal muscle involvement. Children with diffuse cutaneous (dc) SSc had significantly lower maximum mean total skin thickness scores than adult patients with dcSSc. Renal involvement was uncommon in childhood onset cases, and the frequency increased with age of onset. Serum anti-PM-Scl and anti-U1RNP antibodies were detected significantly more frequently in childhood than in adult onset cases. In contrast, anti-RNA polymerase III and anticentromere antibodies were found significantly more frequently in adults. Survival was significantly better among childhood than all adult onset cases combined, but similar to survival in young adult onset SSc cases. Scleroderma heart disease was a frequent cause of death among children with SSc., Conclusion: Patients with juvenile onset SSc more frequently have an overlap syndrome with PM-DM, higher frequency of skeletal muscle involvement, serum anti-PM-Scl and anti-U1RNP antibody, fatal cardiac disease, and improved survival compared with adult onset SSc cases.

Published
2006

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