Sweet's syndrome and monarthritis in a human immunodeficiency virus-positive patient.
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Biomedical subjects
Publications and source records attributed to C Cormier.
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A group of 16 infants, 2 weeks to 11 months old, with malignant osteopetrosis were investigated to examine their vitamin D metabolism and parathyroid function. Bone biopsies from 6 children were studied by light microscopic histomorphometry and by electron microscopy. Considerable heterogeneity existed among the patients with respect to the parameters reflecting mineral metabolism and with respect to the histological manifestations of the disease. The most constant findings were as follows. Immunoreactive parathyroid hormone (iPTH) was elevated in all children, except in 1 patient who had tubular acidosis, and plasma calcium was low or normal, suggesting skeletal resistance to PTH. Plasma 1,25-dihydroxyvitamin D [1,25-(OH)2D] was not constantly elevated and appeared to depend on plasma phosphorus, as both parameters were negatively correlated (r = 0.704, p less than 0.01). Osteoblast activity, as evaluated by circulating alkaline phosphatase and osteocalcin and osteoblast number, measured for 6 children by bone histology, were not increased, despite hyperparathyroidism, suggesting PTH resistance or defective osteoblasts. Osteoclasts could be detected in 5 of the 6 children who had a biopsy. Osteoclast number (5.7-13.3% of bone surface) was normal or mildly increased, and marrow spaces were relatively well developed in 4 patients, whereas 1 child had markedly increased osteoclast number (28.3% of bone surface) and reduced marrow cavities. These 5 children received transplants, and engraftment occurred in all, except in the "hyperosteoclastic" patient. Further studies are necessary to establish the prognostic significance of this histologic feature.
Most of the research on osteoporosis has concentrated on the etiology of the decrease in bone mass. One of several important factors is bone fragility. Genetic and racial factors are important but are modified by environmental factors such as dietary calcium, body weight, and physical activity. Methods of bone mass measurement currently available show some overlap between bone mass in osteoporotic patients who have sustained fractures and normal persons without fractures. However, these methods do have a predictive value in determining a person's risk of future fractures (in the spine or elsewhere).
Osteoporosis occurs in patients with rheumatoid arthritis, acromegaly, anorexia nervosa, chronic liver disease, sickle cell hemoglobinopathies, and mastocytosis. Osteomalacia occurs in patients with renal tubular acidosis, and with tumors.
A 49 year old white woman with systemic lupus erythematosus and bronchiolitis obliterans was treated with prednisone (1 mg/kg daily), which led to a transitory improvement in pulmonary status. Cyclophosphamide was then added--4 mg/kg daily intravenously for five days, then 2 mg/kg daily orally--and this was followed by a dramatic and prolonged improvement.
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Danazol, an inhibitor of pituitary gonadotropin, has been proposed in the treatment of systemic lupus erythematosus (SLE). We report the case of a female patient with SLE in whom a hepatocellular carcinoma was discovered after 4 years of treatment with danazol. Except for 3 days of hypochondrium pain, there were neither clinical signs of liver tumor nor biological abnormalities. An ultrasonography showed 2 tumors of the liver. At histological examination after surgery, one of the tumors was found to be a benign adenoma, while the other was a well differentiated hepatocellular carcinoma. When longterm danazol therapy is required, ultrasonography may be useful for early tumor detection.
The retrospective study of the first 67 patients treated by chemonucleolysis, since December 1980, shows a good overall result, in 69 p. cent of the cases (40 patients) and a failure in 31 p. cent of the cases (18 patients), with a 42 months follow-up. The radiculalgia is cured or quite improved in one month in 90 p. cent of the cases and in 2 years in 88 p. cent. Lumbalgia is absent or moderate after 1 month in 58 p. cent of the cases and after 2 years in 81 p. cent. It persists, most of the time, in women, and this is statistically significant. 15 immediate failures and 3 recurrent sciaticas at the same level are noticed after a 22 months free interval. The possibility of severe complications requires a perfect technique and well-defined indications. The study confirms the good results of nucleolysis with a 42 months follow-up.
A case of proliferative myositis, an unusual inflammatory pseudotumour of skeletal muscle, is reported. This case was particular in that the lesion was located in the popliteal fossa, developed over a long period and above all, was revealed by compression of the sciatic nerve. The diagnosis was confirmed by peripheral biopsy of the mass which showed normal muscular tissue associated with numerous fibroblasts and with characteristic basophilic giant cells. The clinical, histological and pathogenetic features of the disease are discussed with emphasis on the importance of a thorough pathological examination to exclude with certainty a possible sarcoma.