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Biomedical subjects

J I Sebes

Publications and source records attributed to J I Sebes.

At least 19 recordsLinked to original sources
PubMed2000

Hereditary arthro-ophthalmopathy (Stickler syndrome).

We report a 34-year-old woman with hereditary arthro-ophthalmopathy (HAO; Stickler syndrome). This disorder is a rare inherited generalized connective tissue disorder involving the eyes, facial structures and the skeleton, resulting from defective type II collagen formation. There is premature degenerative arthritis and osteopenia with increased incidence of fractures. The differential diagnosis and treatment for bone loss are discussed.

Adult↗
PubMed1999

Effect of calcitriol and pamidronate in multiple myeloma.

Addition of bisphosphonates to standard treatment of multiple myeloma (MM) decreases bone pain and skeletal events without influencing bone healing. Calcitriol, besides its established effects on bone remodeling and calcium metabolism, has both immunoregulatory and cell differentiating effects in vitro and in vivo. Moreover, low serum calcitriol has been reported in MM. We tested the effects of supportive treatment with calcitriol and pamidronate on bone disease in two stage-III-B MM patients with diffuse bone involvement, normal serum calcium, and low serum calcitriol. Complete blood counts, serum calcium, creatinine, quantitative serum and urine immunoglobulins, and biochemical indices of bone turnover, serum calcidiol, calcitriol, parathyroid hormone, skeletal radiographs, and bone mineral density by dual x-ray absorbtiometry were measured every 1-6 months for 16 months in the first patient and 7 months in the second patient. Both patients showed a dramatic improvement of MM activity and in bone disease documented by serial radiographs in the first patient and by increased bone mineral density (approximately 15%) in the second. The reduced serum calcitriol in both patients and the elevated parathyroid hormone observed in the first patient before treatment returned to normal. Supportive treatment with pamidronate does not induce bone healing in MM. Therefore, the results observed with the addition of calcitriol suggest that this hormone may have contributed to the apparent arrest of the progression of MM and caused stimulation of bone healing.

Antineoplastic Agents↗
PubMed1995

Treatment of calcinosis with diltiazem.

OBJECTIVE: To test the hypothesis that the calcium antagonist diltiazem is effective in the treatment of calcinosis. METHODS: Diltiazem, 240-480 mg/day, was given to 4 patients with idiopathic or CREST-related (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) calcinosis for 1-12 years. Serial radiographs of the affected areas, using identical technique, and clinical evaluations were obtained. A fifth patient, who did not tolerate diltiazem, received verapamil, 120 mg/day for 18 months. RESULTS: All patients taking diltiazem had a reduction or disappearance of the calcific lesions, with striking clinical improvement. One patient's case was followed for 12 years. The response to diltiazem during the first 5 years of treatment has been previously reported in detail; however, over 7 years of additional treatment, there was further reduction of the lesions. One patient developed a large calcific lesion while receiving verapamil for hypertension, and after verapamil was replaced with diltiazem, there was a dramatic response. Verapamil was ineffective in the fifth patient, who did not tolerate diltiazem. CONCLUSION: Long-term treatment with diltiazem, but not verapamil, is effective in calcinosis.

Adult↗
PubMed1995

Hypoparathyroidism counteracts risk factors for osteoporosis.

Although increased bone density has been reported in patients with hypoparathyroidism, it is not known whether hypoparathyroidism can overcome the influence of risk factors for osteoporosis and whether the increased bone density is uniform throughout the entire skeleton or greater in certain regions depending on the bone composition and location. In the current study, bone density was measured in patients with postsurgical hypoparathyroidism and risk factors for osteoporosis. Bone mineral density was determined in eight patients with postsurgical hypoparathyroidism, one with idiopathic hypoparathyroidism, and two with pseudohypoparathyroidism in eight different areas of the skeleton using well established methods: single photon absorptiometry of the radius, dual energy x-ray absorptiometry of the spine, hip, and the whole skeleton, and quantitative computed tomography of the spine. Risk factors for osteoporosis were documented in each subject. The data showed that despite the presence of 1-4 risk factors for osteoporosis, patients with postsurgical hypoparathyroidism had bone mineral density above the normal mean in most locations. The locations with the highest increment were the Ward's triangle and the trochanter area of the proximal femur (dual energy x-ray absorptiometry) (Ward's: Z score + 1.59 +/- 0.57, P < 0.03; trochanter 1.31 +/- 0.42, P < 0.02). The elevation of bone density was not observed in one patient with idiopathic hypoparathyroidism, and variable results were observed in two patients with pseudohypoparathyroidism. Based on these findings, in postsurgical hypoparathyroidism, the bone mineral density is above the normal mean despite the presence of risk factors for osteoporosis, and both cortical and trabecular bone are affected.

Adolescent↗
PubMed1993

Bone scintigraphy and costochondritis.

Four patients with infectious costochondritis were studied with computed tomography (CT) and bone scintigraphy. In all four patients the bone scan detected and accurately localized the sites of involvement. CT did not image bilateral involvement in one patient. Three of the four patients were intravenous drug abusers; in two of these patients, a species of Aspergillus was the offending organism. Therefore, in patients with signs and symptoms of chest wall inflammation, scintigraphy is the most direct route to the diagnosis of costochondritis. This entity is apparently occurring more frequently because of the prevalence of intravenous drug abuse. Bone scintigraphy delineates both the extent of individual costochondral lesions and their multiplicity.

Adult↗
PubMed1993

Osteonecrosis of the humeral head in sickle cell disease.

The prevalence and incidence of osteonecrosis (ON) of the humeral head in sickle cell disease was determined by a study of 2524 patients who were entered into a prospective study and followed for an average of 5.6 years. At entry, 5.6% had roentgenographic evidence of ON in one or both shoulders. There was little difference in age-adjusted prevalence among genotypes, but there were striking differences in age-specific rates. Observed at ages ranging from five to 24 years, 3.25% of sickle cell anemia (S/S) patients, but only 1.1% of sickle cell disease (S/C) patients, had ON. No S/beta+ thalassemia patients younger than 25 years of age had ON on entry. The highest age-adjusted incidence rate was found in S/S patients with concomitant alpha-thalassemia (4.85 per hundred patient-years), followed by S/beta zero-thalassemia (4.84 per hundred patient-years), S/beta+ thalassemia (2.61 per hundred patient-years), S/S without alpha-thalassemia (2.54 per hundred patient-years), and S/C (1.66 per hundred patient-years). Only 20.9% of patients reported pain or had limited range of movement at the time of diagnosis. Sickle cell disease is a frequent cause of ON of the humeral head, especially in children and young adults.

Adolescent↗
PubMed1992

Geographic distribution of rheumatoid arthritis in ancient North America: implications for pathogenesis.

The fifth centennial of Columbus stimulates renewed interest in New World origins of disease. The earliest documentation of rheumatoid arthritis was in the New World. Subsequent study of its distribution in the New World in antiquity defines localization to a very specific geographic region. The absence of rheumatoid arthritis in 63 archaeological sites surrounding the original "catchment area" and in five Old World sites, with documented spread over time, suggests that it is a vector (microorganism or allergen)-transmitted disease.

Arthritis, Rheumatoid↗
PubMed1991

Sickle cell disease as a cause of osteonecrosis of the femoral head.

BACKGROUND AND METHODS: Osteonecrosis of the femoral head is an important complication of sickle cell disease. We studied 2590 patients who were over 5 years of age at entry and followed them for an average of 5.6 years. Patients were examined twice a year, and radiographs of the hips were taken at least twice: at study entry and approximately three years later. RESULTS: At study entry, 9.8 percent of patients were found to have osteonecrosis of one or both femoral heads. On follow-up, patients with the hemoglobin SS genotype and alpha-thalassemia were at the greatest risk for osteonecrosis (age-adjusted incidence rate, 4.5 cases per 100 patient-years, as compared with 2.4 in patients with the hemoglobin SS genotype without alpha-thalassemia and 1.9 in those with the hemoglobin SC genotype). Although the rate of osteonecrosis in patients with the hemoglobin SC genotype did not differ significantly from that in patients with the hemoglobin SS genotype without alpha-thalassemia, osteonecrosis tended to develop in these patients later in life. Intermediate rates of osteonecrosis were observed among patients with the hemoglobin S-beta zero-thalassemia and the hemoglobin S-beta(+)-thalassemia genotypes (3.6 and 3.3 cases per 100 patient-years, respectively). Osteonecrosis was found in patients as young as five years old (1.8 cases per 100 patient-years for all genotypes). The frequency of painful crises and the hematocrit were positively associated with osteonecrosis. The mean corpuscular volume and serum aspartate aminotransferase level were negatively associated. Twenty-seven patients had hip arthroplasty during the study; 10 were under 25 years of age. Five of the 27 required reoperation 11 to 53 months after the initial operation. CONCLUSIONS: Osteonecrosis of the femoral head is common in patients with sickle cell disease, with an incidence ranging from about 2 to 4.5 cases per 100 patient-years. Patients with the hemoglobin SS genotype and alpha-thalassemia and those with frequent painful crises are at highest risk. The overall prevalence is about 10 percent. The results of hip arthroplasty are poor.

Adolescent↗
PubMed1991

Noninvasive diagnostic imaging in hemoglobinopathies.

We have provided a global review of the current applications of newer modalities, particularly MR imaging, as applicable to the major manifestations of sickle cell disease, the most common hemoglobinopathy. There is a need for well-designed prospective longitudinal studies utilizing MR imaging to gain further insight into the pathophysiology of this intriguing disease. Additionally, MR imaging, because of lack of ionizing radiation, is ideally suited for long-term sequential studies and may thus provide an objective means for monitoring response to therapy.

Adolescent↗
PubMed1990

The effect of diltiazem on calcinosis in a patient with the CREST syndrome.

We describe a patient with a 23-year history of progressive calcinosis and features of the CREST syndrome (calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasias) who was treated with diltiazem, 240 mg/day, for 5 years. No clinical exacerbation of calcinosis occurred during treatment. Radiographs showed no new lesions, and there was reduction in the size of the existing lesions. Bone scans revealed a progressive decrease in the uptake of the radionuclide by soft tissue foci. We propose that diltiazem may stop the progression of calcinosis by reducing the cellular calcium influx in affected tissues.

Administration, Oral↗