INTRODUCTIONApproximately 5-7% of Wilms tumor (WT) patients present with bilateral disease [1]. Modern WT management consist of ample chemotherapy, nephron-sparing surgery, and, when indicated, radiotherapy. The focus of treatment is survival, and especially in this group of patients the preservation of good longterm renal function is of utmost importance. Nevertheless, the surviving individual may develop renal failure or late effects due to anticancer treatment. The aim of both European and American study groups has been to use preoperative chemotherapy to allow tumor shrinkage and increase the chance for NSS. With the changing methodology in the treatment over time, the overall renal failure has decreased from 16.4% in NWTS 1-2, and 9.9% in NWTS-3 to 3.9% in NWTS-4, although with varying follow-up periods [2]. The incidence of end-stage renal failure (ESRF) was 0.6% for unilateral tumors, 11.5% for bilateral tumors, and >50% for Denys-Drash syndrome (DDS)/WAGR syndrome [1]. Within the group including bilateral tumors, metachronous tumors have a higher incidence of ESRF as compared to synchronous tumors (18 vs. 9%) [2]. Causes were bilateral nephrectomy for persistent or recurrent tumor (74%), DDS, radiation nephritis, chemotherapy toxicity, and surgical complications. Of these, DDS and radiation nephritis were the most significant causes [2].The aim of this study was to analyze the long-term outcome (follow-up >5 years) of patients treated at our center with respect to survival, renal function, and late effects (secondary tumors). PATIENTS AND METHODSBetween 1967 and 2007, 344 patients were treated for WT at the Emma Children's Hospital AMC. Of this cohort, 41 (11.9%) patients (23 females) with a median age of 2.25 years (range 4 months-8 yrs), were diagnosed with Bilateral WT (BWT). Twenty-eight (8%) were synchronous and 13 (3.8%) were metachronous. Seven presented with distant metastasis at diagnosis, five with lung metastasis, one with liver metastasis, and one with both lung and liver. Almost all were treated according to International Society of Pediatric Oncology (SIOP) protocol recommendations, with a combination of chemotherapy, surgery, and radiotherapy if indicated. However, the first SIOP study started in 1971, and in the first protocols no recommendations were given and patients were treated according to center decisions.Data were retrospectively collected from the patient records, operative reports, office notes, and long-term follow-up clinic notes. Four patients had to be excluded for lack of data, because they lived in another country, or because our center was only involved in the diagnostic process or saw the child for a second opinion, after which they returned to their own regional hospital for further treatment and follow-up. Ten patients died of disease, nine within 5 years after diagnosis. Long-term follow-up was defined as a follow-up >5 years by which 25/28 children could be included in the analysis (Fig. 1). The following data were collected: age at diagnosis, gender, tumor type (met...
BackgroundAlthough only 39 % of patients with wrist trauma have sustained a fracture, the majority of patients is routinely referred for radiography. The purpose of this study was to derive and externally validate a clinical decision rule that selects patients with acute wrist trauma in the Emergency Department (ED) for radiography.MethodsThis multicenter prospective study consisted of three components: (1) derivation of a clinical prediction model for detecting wrist fractures in patients following wrist trauma; (2) external validation of this model; and (3) design of a clinical decision rule. The study was conducted in the EDs of five Dutch hospitals: one academic hospital (derivation cohort) and four regional hospitals (external validation cohort). We included all adult patients with acute wrist trauma. The main outcome was fracture of the wrist (distal radius, distal ulna or carpal bones) diagnosed on conventional X-rays.ResultsA total of 882 patients were analyzed; 487 in the derivation cohort and 395 in the validation cohort. We derived a clinical prediction model with eight variables: age; sex, swelling of the wrist; swelling of the anatomical snuffbox, visible deformation; distal radius tender to palpation; pain on radial deviation and painful axial compression of the thumb. The Area Under the Curve at external validation of this model was 0.81 (95 % CI: 0.77–0.85). The sensitivity and specificity of the Amsterdam Wrist Rules (AWR) in the external validation cohort were 98 % (95 % CI: 95–99 %) and 21 % (95 % CI: 15 %–28). The negative predictive value was 90 % (95 % CI: 81–99 %).ConclusionsThe Amsterdam Wrist Rules is a clinical prediction rule with a high sensitivity and negative predictive value for fractures of the wrist. Although external validation showed low specificity and 100 % sensitivity could not be achieved, the Amsterdam Wrist Rules can provide physicians in the Emergency Department with a useful screening tool to select patients with acute wrist trauma for radiography. The upcoming implementation study will further reveal the impact of the Amsterdam Wrist Rules on the anticipated reduction of X-rays requested, missed fractures, Emergency Department waiting times and health care costs.Trial registrationThis study was registered in the Dutch Trial Registry, reference number NTR2544 on October 1st, 2010.Electronic supplementary materialThe online version of this article (doi:10.1186/s12891-015-0829-2) contains supplementary material, which is available to authorized users.
In patients with a suspected pancreatic or periampullary tumor, the tumor size, weight loss, and jaundice are key predictors of metastasis at exploration. SL might be beneficial in patients with a tumor ≥3 cm and severe weight loss and in those with a tumor ≥4 cm and moderate weight loss.
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