Hemolytic uremic syndrome with hypocomplementemia, serum C3NeF, and glomerular deposits of C3

A 4-year-old boy had hemolytic uremic syndrome (HUS) associated with depression of serum C3 level, a B-hemolytic streptococcal throat infection, and an elevated level of antistreptolysin O titer. In addition to the characteristic histologic changes associated with this syndrome, substantial infiltration of polymorphonuclear leukocytes and nodular deposits of C3 globulin were seen in the glomeruli of the first biopsy specimen. Two months after clinical remission, he had a recurrence of hemolytic anemia, thrombocytopenia, and acute renal failure. The serum C3 concentration had decreased again, and serum C3NeF was detected in the serum. The typical changes associated with HUS were still present on electron microscopy. Bilateral nephrectomy and renal transplantation were done because of the development of uncontrollable severe hypertension and increasing azotemia. This patient had three manifestations of HUS, but because of several differences, such as hypocomplementemia, serum C3NeF, a recurrence, and persistent glomerular deposits of C3 globulin, he appears to have had a different form of the syndrome.     

Osteosarcomatosis

A review of the 690 cases of osteosarcoma in the radiographic file of the Armed Forces Institute of Pathology revealed 29 cases of "osteosarcomatosis" (multiple skeletal sites of osteosarcoma). Fifteen of these patients were 18 years old and under and manifested rapidly appearing, usually symmetric, sclerotic metaphyseal lesions. The remaining 14 patients were more than 18 years old and had fewer, asymmetric sclerotic lesions. In most patients (28 of 29), a radiographically dominant skeletal tumor was seen. Pulmonary metastases occurred in the majority of patients and were detected at the same time as the bone lesions. These 29 patients were studied with regard to demographic data and skeletal distribution and radiographic appearance of their lesions. As a result of the findings, a metastatic origin from a primary dominant osteosarcoma is favored over a multifocal origin as the basis for osteosarcomatosis. Osteosarcomatosis is more commonly encountered in the mature skeleton than has been previously recognized.     

Methodological issues in economic assessments of new therapies. The case of colony-stimulating factors

Pharmacoeconomic evaluations estimate the value of medical interventions by comparing their clinical consequences and costs. Economic analyses, based on resource use data collected during 3 clinical trials of lenograstim, were performed as part of a lenograstim economic evaluation programme. Undertaking economic evaluations alongside clinical trials presents a number of methodological challenges, since the trials may be performed in atypical settings, have inappropriate follow-up, or use end-points that are not useful for economic evaluation. This paper reports on how these challenges were met in the lenograstim economic evaluation programme. In particular, it was decided that the evaluations would be based on an intent-to-treat perspective, with the same period of follow-up (for costs) for both lenograstim and vehicle groups.     

Pretreatment bone scan in SCFE: a predictor of ischemia and avascular necrosis

Sixty-two consecutive patients with 73 slipped capital femoral epiphyses (SCFEs) underwent pretreatment planar technetium bone scan to identify the presence of ischemia of the femoral head. The relationship of pretreatment ischemia and the development of avascular necrosis (AVN) was evaluated after a minimum follow-up of 12 months. All patients were treated with spica casting or pinning across the physis. No forceful manipulation was performed. None of the 63 stable SCFEs demonstrated ischemia by bone scan, and none developed AVN. Of the 10 unstable SCFEs, six demonstrated ischemia by bone scan, and five of the six developed AVN. None of the four unstable SCFEs without ischemia on bone scan developed AVN. Pretreatment bone scan is a sensitive predictor for development of AVN in unstable SCFEs and may provide a method for evaluating interventional treatments for AVN associated with SCFE.