Thrombosed synthetic hemodialysis access fistulas: failure of fibrinolytic therapy

Seven episodes of acute thrombosis occurring in five patients with polytetrafluoroethylene dialysis fistulas were treated with local infusions of low-dose streptokinase. Bleeding from previous dialysis puncture sites necessitated stopping the infusion in six out of seven patients, although in one of these six, the graft reopened. The seventh patient had never been dialyzed through the graft and thrombolysis was achieved without incident. Surgery was avoided in only one patient. The authors contend that in these patients the risks of fibrinolytic therapy outweigh the benefits. Surgical thrombectomy, coupled with intraoperative angiography and possible angioplasty, is the preferred method of treating these patients. Venography prior to the creation of the fistula helps the surgeon avoid diseased vessels and may avert early failure of the fistula.     

Percutaneous extraction of urinary calculi: use of the intercostal approach

The authors achieved successful percutaneous extraction of urinary calculi via an intercostal approach in 24 patients. In one patient, a large hydrothorax developed and thoracentesis was required; 2 patients had moderate and 6 minimal pleural fluid collections which did not require treatment. No patient had pneumothorax. Intercostal puncture provides direct access to the upper and middle poles of the kidney when they lie above the twelfth rib and subcostal angulation is not feasible. Such an approach is advantageous for stones in the ureter, as well as renal stones which are inaccessible from the lower pole. Fluoroscopy should be performed when planning the puncture in order to avoid the lung, and a working sheath is recommended.     

Patients with sclerosteosis and disease carriers: Human models of the effect of sclerostin on bone turnover

Sclerosteosis is a rare bone sclerosing dysplasia, caused by loss‐of‐function mutations in the SOST gene, encoding sclerostin, a negative regulator of bone formation. The purpose of this study was to determine how the lack of sclerostin affects bone turnover in patients with sclerosteosis and to assess whether sclerostin synthesis is decreased in carriers of the SOST mutation and, if so, to what extent this would affect their phenotype and bone formation. We measured sclerostin, procollagen type 1 amino‐terminal propeptide (P1NP), and cross‐linked C‐telopeptide (CTX) in serum of 19 patients with sclerosteosis, 26 heterozygous carriers of the C69T SOST mutation, and 77 healthy controls. Chips of compact bone discarded during routine surgery were also examined from 6 patients and 4 controls. Sclerostin was undetectable in serum of patients but was measurable in all carriers (mean 15.5 pg/mL; 95% confidence interval [CI] 13.7 to 17.2 pg/mL), in whom it was significantly lower than in healthy controls (mean 40.0 pg/mL; 95% CI 36.9 to 42.7 pg/mL; p < 0.001). P1NP levels were highest in patients (mean 153.7 ng/mL; 95% CI 100.5 to 206.9 ng/mL; p = 0.01 versus carriers, p = 0.002 versus controls), but carriers also had significantly higher P1NP levels (mean 58.3 ng/mL; 95% CI 47.0 to 69.6 ng/mL) than controls (mean 37.8 ng/mL; 95% CI 34.9 to 42.0 ng/mL; p = 0.006). In patients and carriers, P1NP levels declined with age, reaching a plateau after the age of 20 years. Serum sclerostin and P1NP were negatively correlated in carriers and age‐ and gender‐matched controls (r = 0.40, p = 0.008). Mean CTX levels were well within the normal range and did not differ between patients and disease carriers after adjusting for age (p = 0.22). Our results provide in vivo evidence of increased bone formation caused by the absence or decreased synthesis of sclerostin in humans. They also suggest that inhibition of sclerostin can be titrated because the decreased sclerostin levels in disease carriers did not lead to any of the symptoms or complications of the disease but had a positive effect on bone mass. Further studies are needed to clarify the role of sclerostin on bone resorption. © 2011 American Society for Bone and Mineral Research     

急性淋巴细胞白血病(上)

急性淋巴细胞白血病是淋巴前体细胞异常引起的恶性疾病,儿童与成人均可能发生。儿童发病高峰2～5岁。有效治疗的稳步进展使本病在儿童中的治愈率80％以上,同时为新的治疗方案提供了良机,新方案将保留我们在白血病无病生存病例中获得的治疗经验,同时减轻当前强化治疗方案中的毒副作用。