Musculoskeletal manifestations in cystic fibrosis.

Although bone and joint manifestations are common in children with cystic fibrosis (CF), they have received little attention in adults. As compared to healthy individuals, bone mineral density is low, even with calcium intakes greater than 1500 mg/d. Nevertheless, calcium and phosphate levels in blood and urine are often normal, and vitamin D levels vary. Short stature with a low body mass index and central hypogonadism are the rule in these patients. Fractures and kyphosis are often reported. CF arthropathy occurs in 2-8.5% of patients. Arthritis develops, and there may be skin eruptions. Non-steroidal antiinflammatory drug therapy is effective. Hypertrophic osteoarthropathy associated with respiratory failure is present in 2-7% of patients. Rheumatoid arthritis, spondyloarthropathies, sarcoidosis, and amyloidosis have been reported in association with CF. Knee pain due to patellofemoral syndrome, quinolone-induced arthropathy, and mechanical back pain have been described. Rheumatoid factor titers are higher than in healthy controls, particularly in patients with episodic arthritis. No data are available on antiperinuclear factor or antikeratin antibody titers. Tests for antinuclear antibody are usually negative. Circulating immune complex levels and antibodies to heat shock proteins may be elevated. Antineutrophil cytoplasmic antibody of the bactericidal/permeability-increasing protein (BPI) or azurocidin (AZ) type has been reported, often in high titers (up to 40%).

Post-fracture osteolysis of the pubic bone simulating a malignancy: report of a case.

Post-fracture osteolysis of the pubic bone is rare. We report a case of a 70-year-old woman with osteoporosis and a history of radiation therapy 2 years earlier. At presentation, she was found to have a bilateral sacral fracture and fractures of both pubic rami on one side. The pain persisted, and follow-up radiographs showed osteolysis of the pubic rami suggestive of metastatic disease. The development of a bony callus within 8 months established the diagnosis of benign osteolysis. About 50 cases of osteolysis at fracture sites have been reported to date, of which about a dozen occurred after radiation therapy. All the patients were elderly women with post-menopausal osteoporosis. Radiation therapy probably further increases the risk in this setting. The possibility of osteolysis at fracture sites in patients with osteoporosis should be borne in mind to avoid unnecessary and burdensome investigations that are costly and cause undue anxiety to the patients. Rest is the only effective treatment.

Cementoplasty for painful bone metastases: a series of 42 cases.

The aim of this study is to assess the interest of cementoplasty in the management of bone metastases, based on a retrospective study involving 42 cementoplasty procedures. The median age of the patients is 59 years, with 24 women (57%) and 18 men, all of them presenting with an advanced disease. The cementoplasty was situated in long bones (ten patients), dorsal or lumbar vertebrae (13) and flat bones (20). The principal indication was pain, sometimes with consolidation. The intensity of pain decreases between the day before and the month following the treatment (P = 0.04) among the 25 patients who had pain assessment before and after the procedure. We note 57% good results (24 patients), 9.5% failures (4 patients), and 31% with partial results (13 patients), that is, a total of 88% with "partial or full" alleviation. 16 patients out of 22 (73%) who were no longer able to walk (because of the pain or risk of fracture) were able to resume walking. We observed in this series 40.5% of complications, all of them benign. Cementoplasty improves the quality of life of patients with bone metastases, by decreasing the pain in 88% of cases and allowing the resumption of walking in almost three-quarters of the patients who had lost the ability to walk.

Symptomatic treatment with lanreotide microparticles in inoperable bowel obstruction resulting from peritoneal carcinomatosis: a randomized, double-blind, placebo-controlled phase III study.

PURPOSE: To investigate the somatostatin analog lanreotide as symptomatic treatment for inoperable bowel obstruction due to peritoneal carcinomatosis. PATIENTS AND METHODS: In all, 80 patients with peritoneal carcinomatosis, inoperable malignant digestive obstruction, and two or more vomiting episodes per day or nasogastric tube (NGT) who were previously treated with intravenous corticosteroids and proton pump inhibitors were randomly assigned to one 30-mg injection of lanreotide microparticles (n = 43) or placebo (n = 37) in a 10-day, double-blind, parallel-group phase. The primary end point was the proportion of patients responding on day 7 (one or fewer episodes of vomiting per day or no vomiting recurrence after NGT removal [for ≥ 3 consecutive days in both cases]). Vomiting frequency/NGT secretion volumes, nausea, abdominal pain, well-being, and safety were also assessed. Patients could then enter an open-label lanreotide-only phase. The study was conducted at 22 European hospitals. RESULTS: More patients receiving lanreotide than placebo were responders; this difference was not statistically significant for the intent-to-treat (ITT) population on the basis of diary cards (primary analysis; 41.9% [18 of 43] v 29.7% [11 of 37], respectively; odds ratio, 1.75; 95% CI, 0.68 to 4.49; P = .24) but was statistically significant for the corresponding supportive per protocol analysis (57.7% [15 of 26] v 30.4% [seven of 23]; P < .05) and ITT analysis, on the basis of investigators' assessments (50.0% [19 of 38] v 28.6% [10 of 35]; P < .05). Improvements in well-being were significantly greater with lanreotide on days 3, 6, and 7. No significant differences were observed for other secondary end points. Only two (mild/moderate) treatment-emergent adverse events were considered related to lanreotide. CONCLUSION: These results show that lanreotide has some efficacy and is safe in the symptomatic treatment of patients with inoperable bowel obstruction due to peritoneal carcinomatosis.

Investigations for bone metastasis from an unknown primary.

OBJECTIVES: To evaluate the respective contributions of various investigations used to identify the primary tumor in a cohort of patients referred for diagnostic evaluation of one or more bone metastases. METHODS: A single-center retrospective study was conducted in a cohort of patients admitted between October 1990 and January 2000 for evaluation of one or more bone metastases with no known primary. All patients underwent radionuclide bone scanning, a chest radiograph, and an abdominal ultrasound scan. Computed tomography (CT) of the chest and abdomen, CT of the brain, and tumor marker assays were performed as clinically indicated. Using the final diagnosis as the reference standard, we evaluated the diagnostic usefulness of each investigation. RESULTS: The 152 patients (104 men and 48 women) had a mean age of 63.5+/-12.5 years. The primary was located in the lung in 37 patients, prostate in 26, breast or female genital tract in 24, urinary system in 11, gastrointestinal tract in 11, head and neck in 6, and other organs in 4. In 33 patients, no primary was identified. The extraskeletal metastases were located chiefly in the liver (20.4%), lung (17.1%), pleura (13.2%), and brain (7.2%). Bone biopsies were performed in 107 patients: 84 had a single bone biopsy, 16 had two bone biopsies, and 7 had three bone biopsies. The first bone biopsy was taken in the rheumatology department with or without fluoroscopic guidance in 62 patients, in the radiology department under CT guidance in 6 patients, and in the surgery department in 32 patients; this information was unavailable for the 7 remaining patients. The first bone biopsy was taken from the iliac bone in 48 patients, spine in 32, sacrum in 10, rib in 3, and other sites in 7. The histological biopsy findings indicated adenocarcinoma in 58 cases, epidermoid carcinoma in 28 cases, undifferentiated carcinoma in 2 cases, and other histological patterns in 9 cases. In 80 patients, another metastatic site was easier to access than the bone metastasis. Tumor marker assays were of limited value for determining the site of the primary, with the exception of prostate-specific antigen. CONCLUSION: Bone biopsies performed by rheumatologists, generally under fluoroscopic guidance, usually indicate the site of the primary or at least the histological type. Tumor markers are often positive but are of limited usefulness for identifying the primary.