SAPHO syndrome masquerading as metastatic bone disease

A 46‐year‐old woman who had had a right mastectomy for breast carcinoma a month before underwent bone scintigraphy. The examination revealed multiple pelvic, vertebral and sternal hot spots suggestive of bone metastases. Standard X‐rays and CT confirmed the presence of bony lesions but they were not typical of bone metastases. As the radiographic appearance was reminiscent of SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, osteitis), bone biopsies were performed. Histology showed fibrosis and hyperostosis but no tumour cells. On further questioning, the patient revealed she had had palmar pustulosis and sacroiliitis some years earlier. The purpose of the case report is to show that accurate diagnosis of SAPHO syndrome requires careful clinical and radiological examinations.     

Idiopathic osteosclerosis mimicry of a tooth: case report

This report presents two cases of idiopathic osteosclerosis involving the maxilla and mandible which were identified as a buccally impacted canine and a retained root, respectively, on clinical and plain radiographical examinations. Both patients were females who presented with hypodontia. Radiographic evaluation revealed solitary well-defined radiopaque masses with thickened cortical border. Both patients were undergoing orthodontic treatment and one was planned for a surgical traction of unerupted tooth prior to cone-beam CT assessment. In this report, we reviewed the clinical findings and explained the radiographic appearance of idiopathic osteosclerosis through plain radiographs and cone-beam CT to facilitate its identification among general dentists and oral and maxillofacial radiologists.     

The metabolic syndrome is frequent in Klinefelter's syndrome and is associated with abdominal obesity and hypogonadism

OBJECTIVE: Klinefelter's syndrome is associated with an increased prevalence of diabetes, but the pathogenesis is unknown. Accordingly, the aim of this study was to investigate measures of insulin sensitivity, the metabolic syndrome, and sex hormones in patients with Klinefelter's syndrome and an age-matched control group. RESEARCH DESIGN AN METHODS: In a cross-sectional study, we examined 71 patients with Klinefelter's syndrome, of whom 35 received testosterone treatment, and 71 control subjects. Body composition was evaluated using dual-energy X-ray absorptiometry scans. Fasting blood samples were analyzed for sex hormones, plasma glucose, insulin, C-reactive protein (CRP), and adipocytokines. We analyzed differences between patients with untreated Klinefelter's syndrome and control subjects and subsequently analyzed differences between testosterone-treated and untreated Klinefelter's syndrome patients. RESULTS: Of the patients with Klinefelter's syndrome, 44% had metabolic syndrome (according to National Cholesterol Education Program/Adult Treatment Panel III criteria) compared with 10% of control subjects. Insulin sensitivity (assessed by homeostasis model assessment 2 modeling), androgen, and HDL cholesterol levels were significantly decreased, whereas total fat mass and LDL cholesterol, triglyceride, CRP, leptin, and fructosamine levels were significantly increased in untreated Klinefelter's syndrome patients. In treated Klinefelter's syndrome patients, LDL cholesterol and adiponectin were significantly decreased, whereas no difference in body composition was found in comparison with untreated Klinefelter's syndrome patients. Multivariate analyses showed that truncal fat was the major determinant of metabolic syndrome and insulin sensitivity. CONCLUSIONS: The prevalence of metabolic syndrome was greatly increased, whereas insulin sensitivity was decreased in Klinefelter's syndrome. Both correlated with truncal obesity. Hypogonadism in Klinefelter's syndrome may cause an unfavorable change in body composition, primarily through increased truncal fat and decreased muscle mass. Testosterone treatment in Klinefelter's syndrome only partly corrected the unfavorable changes observed in untreated Klinefelter's syndrome, perhaps due to insufficient testosterone doses.     

The prognostic significance of facial lymphoedema in HIV-seropositive subjects with Kaposi sarcoma

Background

Kaposi Sarcoma (KS) is a multifocal angioproliferative neoplasm characterized by inflammation, oedema, neoangiogenesis and spindle cell proliferation. The pathogenesis of human immunodeficiency virus (HIV)-associated KS (HIV-KS) is multifactorial. HHV-8 is an essential factor but not in itself sufficient to cause HIV-KS, the development of which is influenced by HIV, by increased production of cytokines and by growth factors. Whether HIV-KS is a true malignancy or a reactive hyperplastic inflammatory condition is debatable.

Results and Conclusion

Oedema of the face, legs and hands is a prominent feature of HIV-KS and is probably caused by lymphoedema related to the HIV-KS lesions. The cases of two HIV-seropositive subjects with KS-associated facial lymphoedema are reported. Extensive oral HIV-KS in association with facial oedema in the absence of anti-retroviral treatment appears to be an indication of a poor prognosis.     

依替膦酸二钠对卵巢切除妇女骨密度降低的预防作用

目的 探讨双膦酸盐对切除双侧卵巢的妇女骨密度减低的预防作用。方法 18例早期恶性肿瘤行双侧卵巢切除的患者使用骨吸收抑制剂依替膦酸二钠,400 mg/d×14d,每3个月间断用药一次,持续1年,并以20例因其它病因而实施去势手术的妇女作为对照,研究骨吸收抑制剂依替膦酸二钠对早期恶性肿瘤去势术后妇女骨量的保护作用。结果使用依替膦酸二钠1年时,患者的骨量虽然未能保持在术前水平,但可以显著减少骨量的丢失,与对照组相比差异有显著性(P<0.05),并且骨密度的变化与BALP呈负相关性(P<0.05)。投药1年时肿瘤患者无1例复发。结论依替膦酸二钠可以减少去势手术妇女的骨量丢失,对预防去势手术妇女的骨质疏松有积极的作用。     

Bone and collagen turnover during treatment with inhaled dry powder budesonide and beclomethasone dipropionate

OBJECTIVES--To assess bone and collagen turnover in asthmatic children treated with dry powder budesonide from the Turbuhaler and dry powder beclomethasone dipropionate from the Diskhaler in a dose of 800 micrograms/day. SUBJECTS--Thirteen prepubertal children with asthma. DESIGN--Open crossover study with two treatment periods and treatment free run-in and wash-out periods. All periods were of two weeks' duration. At day 14 in each period blood samples were taken for assessment of serum osteocalcin, the carboxyterminal propeptide of type I collagen (PICP), and the aminoterminal propeptide of type III collagen (PIIINP). At the same time urine was collected for assessment of creatinine corrected pyridinoline (uPYR/cr) and deoxypyridinoline (udPYR/cr) crosslinks. RESULTS--Osteocalcin concentrations were not influenced by any of the treatments. During budesonide treatment mean (SEM) PICP was reduced by 18% (8%) (p = 0.03), PIIINP by 24% (3%) (p = 0.0002), uPYR/cr by 16% (6%) (p = 0.03), and udPYR/cr by 21% (13%) (p = 0.12). During treatment with beclomethasone dipropionate mean (SEM) PICP was reduced by 20% (6%) (p = 0.01), PIIINP by 36% (3%) (p = 0.0002), uPYR/cr by 18% (4%) (p = 0.004), and udPYR by 13% (5%) (p = 0.02). The suppressive effect of beclomethasone dipropionate on PIIINP was more marked than that of budesonide (p = 0.001). CONCLUSION--Treatment with dry powder budesonide and beclomethasone dipropionate 800 micrograms/day is associated with suppression of bone and collagen turnover. The suppression seems to be more marked during treatment with beclomethasone dipropionate. Long term effects and effects of lower doses of budesonide and beclomethasone dipropionate on bone and collagen markers needs further study.