Biomedical subjects

D J Helfrich

Publications and source records attributed to D J Helfrich.

5 recordsLinked to original sources

PubMed1989

Clinical and radiographic improvement of bone of the second lumbar vertebra in Paget's disease following therapy with etidronate disodium: a case report.

Paget's disease of bone is most commonly seen in the spine; it may either be asymptomatic or may cause pain and neurologic impairment. We describe a patient with back pain due to Paget's disease of the second lumbar vertebra and spinal stenosis. The patient had a dramatic clinical and radiographic response to 2 courses of etidronate disodium, which was evidenced by resolution of the back pain and improvement in bony spinal stenosis. Etidronate may be effective in reversing bony spinal stenosis due to Paget's disease.

Etidronic Acid↗

PubMed1989

Normotensive renal failure in systemic sclerosis.

Of 140 patients with "scleroderma renal crisis" encountered during a 33-year period, 15 of 131 (11%) whose blood pressures were recorded were normotensive during this complication. In comparison with 116 patients with hypertension, the normotensive patients significantly more often had microangiopathic hemolytic anemia (90% versus 38%) and thrombocytopenia (83% versus 21%). Pulmonary hemorrhage occurred in 6 normotensive patients. More normotensive patients had received high doses of corticosteroids (prednisone greater than or equal to 30 mg/day) during the 2 months immediately preceding renal crisis (64% versus 16%). A role for corticosteroids in precipitating renal crisis is suggested. The 12-month survival was significantly reduced in the normotensive patients (13% versus 35%).

Acute Kidney Injury↗

PubMed1989

Scleromyxedema serum increases proliferation but not the glycosaminoglycan synthesis of dermal fibroblasts.

In comparison to normal fibroblasts cultured in parallel, scleromyxedema fibroblasts grew less well, synthesized increased amounts of glycosaminoglycans (GAG) in vitro and had ultrastructural abnormalities. Serum obtained from a patient with scleromyxedema increased in vitro fibroblast proliferation but not the GAG synthesis per cell. Serum obtained after therapy, at the time when clinical improvement was observed, continued to stimulate fibroblast proliferation. Thus the serum factor stimulating the fibroblast proliferation did not modulate their GAG synthesis and had no direct relationship to disease activity.

Female↗

PubMed1988

Scleromyxedema myopathy: case report and review of the literature.

We describe a patient who had scleromyxedema associated with proximal myopathy. The histopathologic and electron microscopic features are presented. In addition, we review the clinical and pathologic features of all 9 previously reported patients with documented scleromyxedema and myopathy. Proximal (pharyngeal) or distal dysphagia (7 of 7 patients), elevated creatine kinase (5 of 8), elevated aldolase (3 of 3), and electromyographically demonstrated myopathy (7 of 7) were usual features. Four patients had muscle biopsies that showed myofibril vacuolar changes, but inflammation was infrequent (2 cases). Our patient responded to oral prednisone and weekly intravenous methotrexate with improvement of the erythroderma, papular rash, and muscle strength.

Arm↗

PubMed1988

Giant cell arteritis of the tongue presenting as macroglossia.

While the tongue has been noted to be involved in giant cell arteritis, there are no recorded instances in which the disease has been confined to the tongue. We describe a patient who presented with macroglossia and was found to have a necrotizing vasculitis with giant cells on lingual biopsy. No other evidence for systemic vasculitis was detected. This is the first case report of an isolated vasculitis of the tongue.

Deglutition Disorders↗