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It has recently been suggested that peripheral nerve or plexus blocks performed with the use of a nerve stimulator at low currents (

Purpose Unilateral mandibular distraction osteogenesis (DO) has been shown to cause gross changes in the mandibular condyle and articular disc. The purpose of this study was to correlate histologic findings with these gross changes in a minipig distraction model. Materials and Methods Semiburied distractors were placed via submandibular incisions in 15 minipigs. Two unoperated animals served as controls. The protocol consisted of 0-day latency and rates of 1, 2, or 4 mm/day for a 12-mm gap. After the minipigs were killed (at 0, 24, or 90 days), ipsilateral and contralateral condyles and discs were harvested, decalcified, prepared for standard paraffin embedding, and evaluated to determine changes in 1 ) morphology and thickness of the articular cartilage and subchondral bone and 2 ) morphology of the disc. Results In control animals, there were no degenerative changes in the articular cartilage and underlying condylar bone; there were no significant differences in the mean articular cartilage thickness. The temporomandibular joint discs were normal. In experimental animals, distracted condyles showed increasing degenerative changes and mean articular cartilage thickness as the DO rate increased. The discs were thinner. These changes were present, but to a lesser degree, in the contralateral condyles. After 90 days, degenerative changes in the condyles and discs were reduced, after remodeling, except in the 4 mm/day DO group. Conclusions Histologic changes in the condyles and temporomandibular joint discs in response to mandibular DO correlated with previously reported gross changes. These changes were greater at higher distraction rates and remodeling back to normal occurred in mandibular condyles distracted at 1 mm/day.

Increased attention has been directed at the quality of randomized controlled trials (RCTs) and how they are being reported. We examined leading anesthesiology journals to identify if there were specific areas for improvement in the design and analysis of published clinical studies. All RCTs that appeared between January 2000 and December 2000 in leading anesthesiology journals (Anesthesiology,Anesthesia & Analgesia,Anaesthesia, and Canadian Journal of Anaesthesia) were retrieved by a MEDLINE search. We used a previously validated assessment tool, including 14 items associated with study quality, to determine a quality score for each article. The overall mean weighted quality score was 44% +/- 16%. Overall average scores were relatively high for appropriate controls (77% +/- 7%) and discussions of side effects (67% +/- 6%). Scores were very low for randomization blinding (5% +/- 2%), blinding observers to results (1% +/- 1%), and post-beta estimates (16% +/- 13%). Important pretreatment clinical predictors were absent in 32% of all studies. Significant improvement in the reporting and conduct of RCTs is required and should focus on randomization methodology, the blinding of investigators, and sample size estimates. Repeat assessments of the literature may improve the adoption of guidelines for the improvement of the quality of randomized controlled trials.

Multiple gastrointestinal stromal tumors typically occur in familial form associated with KIT receptor tyrosine kinase or platelet-derived growth factor receptor-alpha (PDGFRA) germline mutations, but may also develop in the setting of type 1 neurofibromatosis. The molecular abnormalities of gastrointestinal stromal tumors arising in neurofibromatosis have not been extensively studied. We identified three patients with type 1 neuro-fibromatosis and multiple small intestinal stromal tumors. Immunostains for CD117, CD34, desmin, actins, S-100 protein, and keratins were performed on all of the tumors. DNA was extracted from representative paraffin blocks from separate tumor nodules in each case and subjected to a nested polymerase chain reaction, using primers for KIT exons 9, 11, 13, and 17 and PDGFRA exons 12 and 18, followed by direct sequencing. The mean patient age was 56 years (range: 37-86 years, male/female ratio: 2/1). One patient had three tumors, one had five, and one had greater than 10 tumor nodules, all of which demonstrated histologic features characteristic of gastrointestinal stromal tumors and stained strongly for CD117 and CD34. One patient died of disease at 35 months, one was disease free at 12 months and one was lost to follow-up. DNA extracts from 10 gastrointestinal stromal tumors (three from each of two patients and four from one patient) were subjected to polymerase chain reactions and assessed for mutations. All of the tumors were wild type for KIT exons 9, 13, and 17 and PDGFRA exons 12 and 18. Three tumors from one patient had identical point mutations in KIT exon 11, whereas the other tumors were wild type at this locus. We conclude that, although most patients with type 1 neurofibromatosis and gastrointestinal stromal tumors do not have KIT or PDGFRA mutations, KIT germline mutations might be implicated in the pathogenesis of gastrointestinal stromal tumors in some patients.

Bone marrow transplant nephropathy (BMTN) classically presents more than 100 days after transplantation as an acute nephritis with hypertension, azotaemia and anemia that usually results in end stage renal failure (ESRF). The risk of developing BMTN may be greater with the use of more intensive chemotherapy and higher total body and tumor bed irradiation. Cis-retinoic acid (RA) may further increase the risk of developing BMTN. Here, we report the cases of two children who developed typical clinical and biochemical features of BMTN. They were both treated for stage IV neuroblastoma with chemotherapy, bone marrow transplant (BMT) conditioning that included total body irradiation and RA therapy after BMT, although the patient in case 1 had established renal insufficiency prior to the commencement of RA. Renal biopsy of these children showed classical BMTN histology, and the renal manifestations progressed quickly; the patient in case 1 became dialysis dependent by 1 year post-bone marrow transplant. Recently, RA has been added to the post-BMT therapy in children with stage IV neuroblastoma. The occurrence of BMTN in two children treated with RA in our unit is unlikely to be coincidental. Although RA has been shown to confer a significant survival advantage in this disease, animal studies and a previous case report have suggested it could increase the toxic effects of chemotherapy and renal irradiation. It is likely that RA contributed to the deterioration in renal function in these patients.

During the past 30 years, the orthopaedic oncology group at the Massachusetts General Hospital has treated 648 patients with osteosarcoma centrally located in the bone. Using records maintained in a specifically designed computer system, a study was done to assess the factors that seemed to influence the survival outcome. The overall survival for the entire series was 68% at an average followup of 6 +/- 4 years. Death occurred at a mean of 3 +/- 3 years. Patient gender had no effect, but age of the patient was correlated with survival data, with the poorest survival for the older patients. Surgical treatment had no effect on outcome, but the Musculoskeletal Tumor Society stage of the lesion, the presence of metastases or local recurrence, and the chemotherapeutic treatment (very dependent on the drugs available and adjuvant versus neoadjuvant administration at various decades) all had a profound effect. In addition, anatomic location, size of the tumor, and percentage of tumor cells killed after neoadjuvant chemotherapy all had an effect on outcome.

BK virus (BKV) is recognized as a significant cause of renal allograft dysfunction in adults, and there is growing awareness of its importance in the pediatric population. Eighteen pediatric renal transplant recipients and 18 age-matched controls were prospectively studied. Anti-BKV immunoglobulin G (IgG) and IgM titres were assayed in all subjects at entry to the study. Polymerase chain reaction (PCR) for BKV DNA was performed on urine and serum at entry, and prospectively tested again at 4, 8 and 12 months. Mean age +/- s.d. of transplant recipients and controls was 14.6 +/- 3.3 and 13.9 +/- 0.33 yr respectively [not significant (NS)]. Transplant patients were studied at a mean time of 5.6 +/- 4.2 yr post-transplant. 56% of transplant patients and 39% of controls were seropositive (+ve BKV IgG) (NS). Plasma BKV PCR was positive in one transplant patient (who also had positive urine PCR) and in none of the controls. The prevalence of positive urine PCR in transplant patients was greater than in controls (33% vs. 0%, p = 0.02). Positive urine BKV PCR was more commonly found in patients treated with mycophenolate than azathioprine (p = 0.04). We conclude that the prevalence of BKV seropositivity and viral activation in this Australian pediatric renal transplant population is similar to that reported in adult and pediatric populations in other countries. BK viruria was more common in children with greater immunosuppression, suggesting that this group is at higher risk of BKV induced nephropathy.

External anatomic landmarks have traditionally been used to approximate the location of the neck blood vessels to optimize central venous cannulation of the internal jugular vein (IJV) while avoiding the common carotid artery (CCA). Head rotation affects vessel orientation, but most landmark techniques do not specify its optimal degree. We simulated catheter insertion via both an anterior and central approach to the right IJV using an ultrasound probe held in the manner of a syringe and needle in 49 volunteers. Increased head rotation from 0 degrees, 15 degrees, 30 degrees, 45 degrees, and 60 degrees to the left of midline was associated with higher probability of a simulated needle contacting the IJV and the CCA. For both approaches, the risk of CCA contact was10% for head rotations ofor=45 degrees. Increased body surface area (BSA) and body mass index (BMI) were associated with more CCA contact at head rotations of 45 degrees or 60 degrees. To optimize IJV contact while reducing the likelihood of inadvertent contact with the CCA, the head should be rotated no more than 30 degrees in patients with high BMI or BSA, but it may be turned to 60 degrees if BMI or BSA is low.

The viral disease, smallpox, was well known through the end of the 20th Century. Because it has been eradicated from natural populations, the present clinical experience with managing the disease is limited. Similarly, research in the pathophysiology, treatment, and prevention of the disease has recently become a priority. Concerns regarding smallpox as a weapon of bioterrorism have led to the implementation of a new prophylactic vaccine program, a renewal in variola vaccine research, and treatment regimens against variola infection.

Aneurysmal bone cyst (ABC) is a locally aggressive osseous lesion that typically occurs during the first two decades of life. ABC was regarded historically as a nonneoplastic process, but recent cytogenetic data have shown clonal rearrangements of chromosomal bands 16q22 and 17p13, indicating a neoplastic basis in at least some ABCs. Herein we show that a recurring ABC chromosomal translocation t(16;17)(q22;p13) creates a fusion gene in which the osteoblast cadherin 11 gene (CDH11) promoter region on 16q22 is juxtaposed to the entire ubiquitin-specific protease USP6 (Tre2) coding sequence on 17p13. CDH11-USP6 fusion transcripts were demonstrated only in ABC with t(16;17) but other ABCs had CDH11 or USP6 rearrangements resulting from alternate cytogenetic mechanisms. CDH11 is expressed strongly in bone, and our findings implicate a novel oncogenic mechanism in which deregulated USP6 transcription results from juxtaposition to the highly active CDH11 promoter.

Oncogenic osteomalacia (OO) is a rare paraneoplastic syndrome of osteomalacia due to phosphate wasting. The phosphaturic mesenchymal tumor (mixed connective tissue variant) (PMTMCT) is an extremely rare, distinctive tumor that is frequently associated with OO. Despite its association with OO, many PMTMCTs go unrecognized because they are erroneously diagnosed as other mesenchymal tumors. Expression of fibroblast growth factor-23 (FGF-23), a recently described protein putatively implicated in renal tubular phosphate loss, has been shown in a small number of mesenchymal tumors with known OO. The clinicopathological features of 32 mesenchymal tumors either with known OO (29) or with features suggestive of PMTMCT (3) were studied. Immunohistochemistry for cytokeratin, S-100, actin, desmin, CD34, and FGF-23 was performed. The patients (13 male, 19 female) ranged from 9 to 80 years in age (median 53 years). A long history of OO was common. The cases had been originally diagnosed as PMTMCT (15), hemangiopericytoma (HPC) (3), osteosarcoma (3), giant cell tumor (2), and other (9). The tumors occurred in a variety of soft tissue (21) and bone sites (11) and ranged from 1.7 to 14 cm. Twenty-four cases were classic PMTMCT with low cellularity, myxoid change, bland spindled cells, distinctive "grungy" calcified matrix, fat, HPC-like vessels, microcysts, hemorrhage, osteoclasts, and an incomplete rim of membranous ossification. Four of these benign-appearing PMTMCTs contained osteoid-like matrix. Three other PMTMCTs were hypercellular and cytologically atypical and were considered malignant. The 3 cases without known OO were histologically identical to the typical PMTMCT. Four cases did not resemble PMTMCT: 2 sinonasal HPC, 1 conventional HPC, and 1 sclerosing osteosarcoma. Three cases expressed actin; all other markers were negative. Expression of FGF-23 was seen in 17 of 21 cases by immunohistochemistry and in 2 of 2 cases by RT-PCR. Follow-up (25 cases, 6-348 months) indicated the following: 21 alive with no evidence of disease and with normal serum chemistry, 4 alive with disease (1 malignant PMTMCT with lung metastases). We conclude that most cases of mesenchymal tumor-associated OO, both in the present series and in the reported literature, are due to PMTMCT. Improved recognition of their histologic spectrum, including the presence of bone or osteoid-like matrix in otherwise typical cases and the existence of malignant forms, should allow distinction from other mesenchymal tumors. Recognition of PMTMCT is critical, as complete resection cures intractable OO. Immunohistochemistry and RT-PCR for FGF-23 confirm the role of this protein in PMTMCT-associated OO.

Summary: Although relatively common, soft tissue tumors frequently present diagnostic problems for practicing pathologists. Immunohistochemistry has facilitated the diagnosis of many mesenchymal tumors; however, there is considerable overlap in the staining profiles among cells demonstrating fibroblastic and myofibroblastic differentiation. It has been our experience that soft tissue tumors associated with abundant extracellular collagen deposition commonly cause problems in classification. One reason for this is that tumors displaying this morphology include representatives from different histogenetic families. Specifically, tumors exhibiting fibroblastic, myofibroblastic and even lipomatous differentiation may manifest as a densely collagenous mass. It is the purpose of this review to highlight these collagen-rich soft tissue tumors.

Presentation of Case A nine-year-old girl was admitted to the hospital because of hepatosplenomegaly and pain in the thigh. The patient had been well until three years earlier, when she began to have frequent episodes of epistaxis. Two years before admission, mild abdominal distention developed. Periodic examinations by her physician were reported to have revealed no abnormalities. Two months before admission, the abdominal distention worsened but was not accompanied by abdominal pain. Physical examination revealed hepatosplenomegaly. An abdominal ultrasonographic study confirmed the presence of hepatosplenomegaly, without evidence of portal-vein thrombosis (Figure 1). The results of liver-function tests were normal or . . .

Although the majority of mesenchymal lesions of the gastrointestinal tract are neoplastic in nature, nonneoplastic reactive processes may involve the gastrointestinal tract and mesentery, causing diagnostic confusion with more aggressive neoplasms, such as fibromatosis or gastrointestinal stromal tumors. In this study, we report a series of fibroinflammatory lesions of the gastrointestinal tract that we think represent a relatively cohesive group of tumors and describe the clinical and pathologic features of this entity, which we have termed "reactive nodular fibrous pseudotumor." The tumors affected five patients (four male and one female patient) who ranged in age from 48 to 71 years (mean 56 years). Two patients presented with acute abdominal pain without a significant past medical history, two had incidental lesions discovered during evaluation for other medical conditions, and one was found to have an abdominal mass. Three patients had a history of abdominal surgery. The tumors were multiple in three patients and solitary in two patients. In four cases, at least one of the tumors involved the small intestine or colon, and the lesion was confined to the peripancreatic soft tissue in one case. The tumors were firm, tan-white, ranged in size from 4.3 to 6.5 cm in greatest dimension, and were grossly well circumscribed. All of the lesions were of low to moderate cellularity and composed of stellate or spindled fibroblasts arranged haphazardly or in intersecting fascicles. Three cases had microscopically infiltrative borders. The stroma was rich in collagen, which was wire-like, keloidal, or hyalinized. Intralesional mononuclear cells were sparse but were more numerous peripherally and frequently arranged in lymphoid aggregates. Immunohistochemical stains demonstrated that all of the tumors stained for vimentin, 80% stained for CD117 or muscle specific actin, 60% stained for smooth muscle actin or desmin, and none of the tumors stained for CD34, S-100 protein, or anaplastic lymphoma kinase-1. Follow-up information was available in all cases: four patients had no residual disease following surgical resection (mean follow-up 16.3 months) and one patient who had an incomplete surgical resection had stable disease at 26 months. In summary, we report a series of distinct intraabdominal fibroinflammatory pseudotumors that we have collectively termed "reactive nodular fibrous pseudotumors." These lesions are uncommon and may infiltrate the bowel wall, thereby mimicking primary bowel neoplasms or intraabdominal fibromatosis. Recognition of these nonneoplastic lesions is important, as they pursue a benign clinical course, but may be confused with other mesenchymal neoplasms that require more aggressive treatment.

The objective of this prospective study was to determine the prevalence of hyperlipidemia in our pediatric renal transplant patients and to treat those with persistently elevated cholesterol and/or low-density lipoprotein (LDL) levels. All patients with a functioning renal allograft for greater than 6 months were studied (n = 18). Patients with cholesterol and/or LDL levels greater than the 95th percentile (n = 9) were commenced on an HMG-CoA reductase inhibitor, Atorvastatin and monitoring was performed for efficacy and adverse effects. Total serum cholesterol was elevated in 11 of 18 (61%) and triglyceride (TG) was elevated in 12 of 18 (67%) patients. Atorvastatin treatment was effective with a mean percentage reduction of total cholesterol of 41 +/- 10% (p < 0.01 vs. before treatment), LDL 57 +/- 7% (p < 0.01 vs. before treatment) and TG 44 +/-25% (p = 0.05 vs. before treatment). No adverse effects on allograft function or cyclosporin levels were experienced. Hyperlipidemia is a common problem and Atorvastatin is a safe and effective treatment in pediatric renal transplant recipients.

Purpose: Distraction osteogenesis (DO) is a commonly used technique for mandibular lengthening, but changes in the temporomandibular joint have not been well documented. The purpose of this study was to evaluate the effect of DO, at varying rates, on the mandibular condyle and articular disc. Materials and Methods: Semiburied distractors were placed via submandibular incisions in 15 minipigs. Two unoperated animals served as controls. The protocol consisted of 0 day latency and rates of 1, 2, or 4 mm/d for a 12-mm gap. After the animals were killed (0, 24, or 90 days), ipsilateral and contralateral condyles and discs were harvested and evaluated to determine changes in 1) condylar form and size, 2) condylar surface, and 3) the articular disc. Results: Articular surfaces of the condyles in control animals were smooth, with no irregularities or erosions. In animals undergoing distraction, ipsilateral condyles showed increasing changes in morphology and AP dimension, and surface contour irregularities as the DO rate increased. These changes were present, but to a lesser degree, in the contralateral condyles. Articular discs of both ipsilateral and contralateral sides showed variable thinning at the medial aspect at end DO. After 90 days, changes in the condyles and discs were reduced by remodeling except in the 4 mm/d DO groups. Conclusions: Results of this preliminary study indicate that gross changes occur in condyles and discs after unilateral mandibular DO. These changes are more severe at faster distraction rates (4 mm/d) and tend to resolve during neutral fixation when a rate of 1 mm/d is used. © 2002 American Association of Oral and Maxillofacial Surgeons J Oral Maxillofac Surg 60:1327-1333, 2002

Giant cell tumors (GCT) of the base of skull are rare neoplasms. This report reviews the treatment of four pediatric patients presenting with aggressive giant cell tumor, using fractionated and combined, conformal proton and photon radiation therapy at Massachusetts General Hospital and Harvard Cyclotron Laboratory.Three female patients and one adolescent male, ages 10-15 years, had undergone prior, extensive surgical resection(s) and were treated for either primary (two patients) or recurrent (two patients) disease. Gross residual tumor was evident in three patients and microscopic disease suspected in one patient. Combined proton and photon radiation therapy was based on three-dimensional (3-D) planning, consisting of fractionated treatment, one fraction per day at 1.8 CGE (cobalt-gray equivalent) to total target doses of 57.6, 57.6, 59.4, and 61.2 Gy/CGE.With observation times of 3.1 years, 3.3, 5.3, and 5.8 years, all four patients were alive and well and remained locally controlled without evidence of recurrent disease. Except for one patient with partial pituitary insufficiency following radiotherapy for sellar recurrent disease, thus far no late effects attributable to radiation therapy have been observed.3-D-conformal radiation therapy offers a realistic chance of tumor control for aggressive giant cell tumor in the skull base, either postoperatively or at time of recurrence. Conformal treatment techniques allow the safe delivery of relatively high radiation doses in the pediatric patient without apparent increase of side effects.

Since 1982, the orthopaedic research laboratories at the authors' hospital has done flow cytometric and more recently cytofluorometric deoxyribonucleic ploidic analyses of samples of bone and soft tissue tumors. The current authors attempt to define the value of such studies in distinguishing benign from malignant tumors, in conforming to stage of the tumors, and in helping to predict metastasis and death. The series consists of 1134 patients in whom the disease was verified and the survival data were available as a result of a questionnaire study. Statistically, the ploidic analyses were of remarkable value in defining malignancy and in correlating with the stage of the lesion. They were of less value in predicting survival, particularly for patients with osteosarcoma and chondrosarcoma, but seemed to predict survival effectively for patients with soft tissue sarcomas.

We describe five primary soft tissue tumors that had histologic features identical to intraosseous aneurysmal bone cyst. The tumors occurred in three females and two males, who ranged in age from 8 to 37 years (median 28 years). They arose in the deep soft tissue of the upper extremities, thigh, and groin region and typically presented as a rapidly growing mass; no involvement of the adjacent bones was identified. The tumors ranged in size from 2.5 to 9 cm (median 4 cm). Grossly, they were surrounded by a thin rim of bone and on sectioning had hemorrhagic cystic spaces delineated by fibrous septa. Histologically, the cystic spaces were filled with blood and the fibrous septa were composed of fibroblasts, osteoclast-type giant cells, and woven bone. Cytogenetic analysis of one tumor showed 46,XY,t(17;17)(p13;q12), a result similar to that which has been reported for intraosseous aneurysmal bone cyst. The differential diagnosis includes a variety of bone-forming and giant cell-containing tumors, the most important being extraskeletal osteosarcoma. Follow-up showed that four patients are free of disease 16 months to 10 years after surgery; one tumor regrew after incomplete initial excision, but the patient has been free of disease 16 months after a second operation. Soft tissue aneurysmal bone cyst is an uncommon benign tumor that can be treated by simple excision, and it should be distinguished from a variety of other reactive and neoplastic processes.

Case: A 64-year-old woman had undergone bilateral total knee arthroplasty in 1998. In 2010, she presented with a large, painless, diaphyseal soft-tissue mass of the lower leg. She indicated that she had no history of knee pain, trauma, or infection. Ultimately, the mass was found to be a synovial fluid-filled cyst that communicated with the knee joint, which was a result of severe osteolysis. Conclusion: Large diaphyseal tibial masses in the presence of total knee arthroplasty should raise a high index of suspicion not only for tumors and infections, but also for severe osteolysis. Knowledge of the various ways that osteolysis can present as well as an appropriate workup will help to guide diagnosis and management.

Cortical tubers are the most common brain lesions of the tuberous sclerosis complex (TSC).1 They vary widely in size, location, and appearance, may have a cystic or a calcified component, and are often epileptogenic.2,3 Histologically, tubers are hypomyelinated hamartomas, which are characterized by abnormal cortical lamination and are typically composed of giant or balloon cells, dysplastic neurons, and reactive astrocytes.4 We report the radiologic and pathologic findings of a resected calcified tuber identified as an epileptogenic focus. ### Case report. The patient is a 19-year-old right-handed woman with a known spontaneous TSC1 mutation. She was the product of a normal pregnancy. Seizure onset occurred at the age of 9 months and led to a diagnosis of TSC. Neuropathologic features include numerous cortical tubers and subependymal giant cell tumors (SGCT). The patient has experienced daily seizures since their onset. Multiple antiepileptic medications and other anticonvulsant treatments were tried without significant seizure control. The patient has a history of global developmental impairment. As part of the presurgical evaluation for neurosurgical intervention, an MRI showed SGCT, multiple tubers located in bilateral frontal, parietal, and occipital lobes, and a large (22 mm) T2 hypointense calcified lesion deep to the left superior frontal sulcus (figure). Review of prior scans revealed that the calcified component of this …

Meningiomas are one of the most common tumors that arise within the central nervous system; they represent up to 30% of all primary intracranial tumors. Extradural meningiomas are rare (

Mesenchymal tumors of the vulva are relatively uncommon. The majority is benign and composed of cells that demonstrate fibroblastic and myofibroblastic differentiation admixed with other connective tissue stromal elements. Many of the tumors are restricted to the distal superficial portion of the gynecological tract and have distinctive morphological features. Immunohistochemistry and molecular analyses are helpful in accurately identifying these tumors in select circumstances. The correct diagnosis is of importance as some of these various tumors have very different clinical outcomes.

Objectives The objectives of this paper are to prospectively determine the incidence of paediatric systemic lupus erythematosus (pSLE) in Australia as well as describe the demographics, clinical presentation and one-year outcome. Study design Newly diagnosed cases of pSLE were ascertained prospectively from October 2009 to October 2011 through the Australian Paediatric Surveillance Unit (a national monthly surveillance scheme for notification of childhood rare diseases) as well as national subspecialty groups. Questionnaires were sent to notifying physicians at presentation and at one year. Results The annual incidence rate was 0.32 per 105 children aged less than 16 years. The incidence was significantly higher in children of Asian or Australian Aboriginal and Torres Strait Islander parents. Approximately one-third of children underwent a renal biopsy at presentation and 7% required dialysis initially although only one child had end-stage kidney disease (ESKD) at one-year follow-up. Conclusion The incidence of pSLE in Australia is comparable to that worldwide with a significantly higher incidence seen in children of Asian and Australian Aboriginal and Torres Strait Islander backgrounds. Renal involvement is common but progression to ESKD, at least in the short term, is rare.

An estimated 290,000 surgical site infections occur after a procedure in the United States annually, accounting for 22% of all healthcare associated infections [1]. Deep surgical site infections (SSI) after arthroplasty or spine fusion surgery complicate up to 2% of cases, and result in revision surgery and prolonged antibiotic use [2, 3]. The patient morbidity and healthcare system cost is tremendous, with an estimated $566 million spent annually in hospital treatment costs for arthroplasty SSI alone [4]. Staphylococcus aureus is a frequent and feared cause of these infections, given its unique pathogenicity and ability to adhere to prosthetic material [5, 6]. Studies indicate S. aureus colonization prior to surgery is a risk of subsequent infection, with the nasal mucosa serving as a reservoir for S. aureus colonization and a source of secondary transmission to other body sites [7, 8]. Prevention of SSI by treatment of S. aureus colonization with intranasal topical mupirocin has been studied. A short-term suppression rate of 83% after multiple doses of nasal mupirocin was achieved in one randomized, placebo-controlled trial of 891 S. aureus colonized patients, resulting in a statistically significant reduction of invasive S. aureus infection [9]. Several controlled trials suggest a reduction in SSI with the use of pre-operative topical antiseptics [10, 11]. When nasal mupirocin was combined with use of chlorhexidine soap in a randomized, double-blind, placebo-controlled trial including 808 S. aureus colonized surgical patients, a significant reduction in deep S. aureus SSI was realized [12]. To reduce the risk of SSI after arthroplasty and spine fusion surgery at our institution, we historically provided a prescription for brand mupirocin ointment specifically formulated for application on intranasal mucosal surfaces twice a day for the five days prior to surgery, and instructions for the use of chlorhexidine soap the evening before surgery. After implementation of this protocol, we conducted an anonymous patient survey to measure compliance. Although 94% of patients used the chlorhexidine soap, only 86% applied the mupirocin ointment and 8% of patients stated they found it hard or very hard to purchase the mupirocin due to cost [13]. The brand nasal mupirocin ointment specifically produced for application on intranasal mucosal surfaces is only formulation currently available; although generic mupirocin ointment for topical use on skin is available at less cost, application of this formulation on mucosal surfaces may cause irritation. Our survey results, plus reports of emerging mupirocin resistance, led us to search for alternatives [14–19]. Povidone-iodine solution is a broad-spectrum antiseptic suitable for suppression of S. aureus in nasal secretions [20]. In contrast to the application of nasal mupirocin antibiotic ointment to eradicate S. aureus in the nares before surgery, the application of povidone iodine is intended to transiently suppress S. aureus in the nares during surgery. Our hypothesis was a one-time application of nasal povidone iodine just prior to surgery would be as effective as twice daily applications of nasal mupirocin during the five days before surgery in preventing SSI, and provide a more convenient option for patients at lower cost.

Sarcoma developing in association with a metallic orthopaedic prosthesis or hardware is an uncommon, but well recognized complication. We review 12 cases of sarcomas arising in bone or soft tissue at the site of orthopaedic hardware or a prosthetic joint. Nine patients were male, and three were female. Their ages ranged from 18 to 85 (mean 55) years at the time of diagnosis of the malignancy. Five patients had undergone hip arthroplasty for degenerative joint disease, four had been treated with intramedullary nail placement for fracture, two had staples placed for fixation of osteotomy, and one had hardware placed for fracture fixation followed years later by a hip arthroplasty. The time interval between the placement of hardware and diagnosis of sarcoma was known in 11 cases and ranged from 2.5 to 33 (mean 11) years. The patients presented with pain, swelling, or loosening of hardware and were found to have a destructive bone or soft tissue mass on radiography. Two sarcomas were located primarily in the soft tissue and 10 in bone. Seven patients developed osteosarcoma, four malignant fibrous histiocytoma, and one a malignant peripheral nerve sheath tumor. All sarcomas were high grade. Three patients had metastatic disease at the time of diagnosis. Follow-up was available on eight patients: five patients died of disease 2 months to 8 years (mean 26 months) after diagnosis; two patients died without evidence of disease 7 and 30 months after diagnosis; and one patient is alive and free of disease 8 years after diagnosis. Sarcomas that occur adjacent to orthopaedic prostheses or hardware are of varied types, but are usually osteosarcoma or malignant fibrous histiocytoma. They behave aggressively and frequently metastasize. Clinically, they should be distinguished from non-neoplastic reactions associated with implants, such as infection and a reaction to prosthetic wear debris.

The fascia iliaca nerve block provides excellent postoperative pain relief after knee surgery. It is easy to perform, needle insertion is not directly next to nerves or vessels, and it is associated with minimal side effects. Instructive case reports as well as a description and discussion of the technique are presented.

Soft tissue perineurioma is a relatively recently characterized, uncommon tumor composed of perineurial cells exhibiting immunoreactivity for epithelial membrane antigen (EMA). These lesions occur preferentially in adults and may arise in a wide variety of anatomic sites. We report the clinicopathologic, immunohistochemical, and ultrastructural features of six cases of a poorly recognized morphologic variant of soft tissue perineurioma, characterized by a highly distinctive reticular growth pattern. Four of the patients were women, two were men (age range, 34-61 yrs; median, 43 yrs). Four of the cases arose in the subcutis of the upper extremity; three were located distally (thumb, finger, palm), whereas one was situated more proximally near the elbow region. One case each was located in the gingiva and subcutaneous tissue of the inguinal region, respectively. In those cases in which clinical information was available (n = 5), the lesions were asymptomatic and had been present from 4 months to 10 years before resection. Tumor size ranged from 1.5 cm to 10 cm (median size, 4.25 cm). Microscopically the lesions demonstrated a predominantly lace-like or reticular growth pattern composed of anastomosing cords of fusiform cells with bipolar cytoplasmic processes and palely eosinophilic cytoplasm. Nuclei were centrally placed, ovoid to fusiform in shape, and no mitoses were seen. Transition to more cellular areas was focally present in all cases. The stroma was variably collagenous to myxoid. Immunohistochemically all six cases stained positively for EMA but not for S-100 protein. Two cases demonstrated focal positive cytoplasmic staining for cytokeratin, whereas one case was focally desmin positive. Ultrastructural examination of two tumors showed typical features of perineurial cells. Follow up (available in only two cases) showed no evidence of recurrence. Reticular perineurioma of soft tissue represents an unusual morphologic variant within the perineurioma group, which should be distinguished from myoepithelial tumors, extraskeletal myxoid chondrosarcoma, and myxoid synovial sarcoma.

Objective To describe the clinical characteristics and outcomes of patients with acquired immunodeficiency syndrome (AIDS) admitted to the intensive care unit (ICU). Design An observational cohort study with retrospective chart review. Setting ICU of an urban university medical center. Patients Consecutive ICU admissions of patients with AIDS at an urban university medical center between December 1993 and June 1996. Interventions None. Measurements and Main Results For each patient, we recorded ICU admission diagnosis, clinical characteristics, and outcome. Among 129 ICU admissions of patients with AIDS, 102 (79%) were admitted for infections, of which (45%) had infections caused by bacteria. Pseudomonas aeruginosa, Staphylococcus aureus, and other enteric pathogens were the most frequent isolates. Pneumonia accounted for 65% of 102 admissions for infections. Overall hospital mortality was 54%, but mortality was higher (68%) for patients with bacterial sepsis. Neutropenia was associated with differences in unadjusted survival rates, whereas CD4 counts were not. Independent predictors of hospital mortality included increasing acute physiology scores and severity of sepsis. Conclusions In our ICU, among patients with AIDS, sepsis resulting from bacterial infection is now a more frequent cause of admission than Pneumocystis carinii pneumonia. Severity of illness and the presence of severe sepsis were the clinical predictors most associated with increased mortality. Patients who are not receiving or responding to highly active antiretroviral therapy may become as likely to be admitted to an ICU with a treatable bacterial infection as with classic opportunistic infections. Therefore, broad-spectrum empirical antibacterial therapy is particularly important when the etiology of infection is uncertain.

The authors describe a tumor that had the histologic and ultrastructural features and immunohistochemical profile of an axial chordoma, but arose in the distal ulna. A skeletal survey failed to show any other site of involvement. The tumor was resected, and the patient remains free of disease 2 1/2 years later. Rare tumors with the histologic features of chordoma have been reported in appendicular locations. Chordoma periphericum, a tumor that has the potential to metastasize, needs to be distinguished from parachordoma because no classic parachordoma has been reported to disseminate.

Postsurgical evaluation of histologic changes of tumors after preoperative chemotherapy and/or radiotherapy has been a routine clinical practice of pathologists and oncologists. There appears to be secure evidence that the extent of tumor necrosis vs. viable tumor cells postchemotherapy is a clinically useful predictor of outcome. The significance of histologic tumor necrosis after radiotherapy, however, has not been clearly established and deserves further investigation. We investigated the correlation between histological extent of tumor necrosis, survival of tumor transplants, and radiation doses in an experimental model using three human tumor xenografts. Three human tumor cell lines were investigated: STS-26, SCC-21, and HGL-21. Tumors were grown subcutaneously in athymic nude mice and received external beam radiation of different doses. Tumors were excised 2 weeks postirradiation. One-half of the tumor was divided into 1-mm(3) fragments and transplanted to naive mice. The other half was examined for histologic tumor necrosis. Transplant survival was strongly correlated with radiation dose, TCD(p) (radiation dose that results in local tumor control in proportion, p, to irradiated tumors). In contrast, there was no clear association between transplant survival rate and the extent of tumor necrosis. The experimental model demonstrated a strong inverse correlation between radiation doses and tumor transplant survival. Histologic tumor necrosis did not correlate well with radiation doses or transplant survival rates. Despite common practices in histologic examination of tumors posttherapy, clinical interpretations and implications of histologic tumor necrosis after radiotherapy should be considered with caution.

Complex regional pain syndromes (CRPS) are challenging neuropathic pain states quite difficult to comprehend and treat. Although not yet fully understood, advances are being made in the knowledge of the mechanisms involved with CRPS. Patients often present with incapacitating pain and loss of function. Patients suffering from these disorders need to have treatment plans tailored to their individual problems. A comprehensive diagnostic evaluation and early and aggressive therapeutic interventions are imperative. The therapeutic approach often calls for a combination of treatments. Medications such as antiepileptics, opioids, antidepressants, and topical agents along with a rehabilitation medicine program can help a major portion of patients suffering from these disorders. Implantable devices can aid those patients with CRPS. While progress is being made in treating patients with CRPS, it is important to remember that the goals of care are always to: 1) perform a comprehensive diagnostic evaluation, 2) be prompt and aggressive in treatment interventions, 3) assess and reassess the patient's clinical and psychological status, 4) be consistently supportive, and 5) strive for the maximal amount of pain relief and functional improvement. In this review article, the current knowledge of the epidemiology, pathophysiology, diagnostic, and treatment methodologies of CRPS are discussed to provide the pain practitioner with essential and up-to-date guidelines for the management of CRPS.

Objective: To compare case-mix adjusted intensive care unit (ICU) length of stay for critically ill patients with a variety of medical and surgical diagnoses during a 5-yr interval. Design: Nonrandomized cohort study. Setting: A total of 42 ICUs at 40 US hospitals during 1988-1990 and 285 ICUs at 161 US hospitals during 1993-1996. Patients: A total of 17,105 consecutive ICU admissions during 1988-1990 and 38,888 consecutive ICU admissions during 1993-1996. Measurements and Main Results: We used patient demographic and clinical characteristics to compare observed and predicted ICU length of stay and hospital mortality. Outcomes for patients studied during 1993-1996 were predicted using multivariable models that were developed and cross-validated using the 1988-1990 database. The mean observed hospital length of stay decreased by 3 days (from 14.8 days during 1988-1990 to 11.8 days during 1993-1996), but the mean observed ICU length of stay remained similar (4.70 vs. 4.53 days). After adjusting for patient and institutional differences, the mean predicted 1993-1996 ICU stay was 4.64 days. Thus, the mean-adjusted ICU stay decreased by 0.11 days during this 5-yr interval (T-statistic, 4.35; p

Study objective To evaluate the causes, risk factors, and mortality rates associated with unexpected readmission to medical and surgical ICUs. Data sources MEDLINE citation review of primary articles focusing on ICU readmission or ICU outcomes from January 1966 to June 1999, and contact with authors of primary studies. Study selection Eight primary studies of ICU readmission and eight multi-institutional ICU outcome studies that reported ICU readmission rates were included. Data extraction We abstracted data on the methodology and design of the primary studies, overall rates, causes, predictors, outcomes, and measures of quality of care associated with ICU readmission. Data synthesis The average ICU readmission rate of 7% (range, 4 to 14%) has remained relatively unchanged in both North America and Europe. Respiratory and cardiac conditions were the most common (30 to 70%) precipitating cause of ICU readmission. Patients readmitted to ICUs had average hospital stays at least twice as long as nonreadmitted patients. Hospital death rates were 2- to 10-times higher for readmitted patients than for those who survived an ICU admission and were never readmitted. Predictors of ICU readmission have been neither well studied nor reproducible. Unstable vital signs, especially respiratory and heart rate abnormalities, and the presence of poor pulmonary function at time of ICU discharge appear to be the most consistent predictors of ICU readmission. There were no consistent data supporting the use of readmission rates as a measure of quality of care. Conclusions ICU readmission is associated with dramatically higher hospital mortality. Unstable vital signs at the time of ICU discharge are the most consistent predictor of ICU readmission. Further studies focusing on processes of ICU and hospital care are needed to determine if ICU readmission rates are a measure of quality of care.

Purpose: The major aim of treating vesicoureteral reflux in children is the prevention of renal scars. Dimercapto-succinic acid (DMSA) is the modality of choice for detecting renal scars. We documented the incidence of new renal scarring and measured changes in differential renal function after ureteral reimplantation using DMSA studies.Materials and Methods: We evaluated 45 boys and 98 girls with a median age of 2 years who had vesicoureteral reflux and underwent ureteral reimplantation. DMSA scans were done preoperatively and at a median of 3.4 years postoperatively. Maximal reflux grade was III in 84 children (59%), IV in 27 (19%) and V in 6 (4%).Results: Preoperatively DMSA studies showed scarred or contracted kidneys in 106 of the 143 patients (74%). After reimplantation mean change in differential function was 2.5%. New scars developed in 3 children (2%). We noted greater than 6% decrease in relative differential function without new scarring in 7 cases (5%).Conclusions: The incidence of new renal s...

I the current era of evidence-based medicine, urologists are increasingly called on to apply a greater scientific rigor to their daily practice. To do so, they must remain current with the new advances being reported in published studies and at scientific meetings. Furthermore, given the increasing volume of published clinical research, urologists need to discern between reports providing a high level of evidence for a causal effect and those that do not. A necessary tool for interpreting these articles is an understanding of clinical research designs. Although a variety of study designs are used in clinical research, they all possess a common purpose: to explore the associations and outcomes for a given condition or intervention. Ultimately, the goal is to determine the strength of evidence for a causal effect provided by a study and how best to apply these results to patient care. This review begins with a discussion of several basic concepts necessary for understanding clinical study designs. We then review the essential components of the most common clinical study designs: the case report/series, cross-sectional, case-control, cohort, pre-post experimental, nonrandomized clinical, and randomized clinical trial. In particular, their strengths, weaknesses, and threats to validity are discussed using examples drawn from urologic published reports.

Eight expert orthopedic pathologists voluntarily reviewed the clinicoradiologic and histologic data from 12 selected cases coded as osteosarcoma over the past seven years (procured from a total of 40 cases) from the files of the University of North Carolina and Wake Forest University. The study sample was chosen to include a full spectrum of histologic grades and subtypes including conventional, low-grade central, chondroblastoma-like, osteoblastoma-like, small cell, telangiectatic, parosteal, and periosteal variants. Participants were asked to render a diagnosis, provide a histologic grade (if applicable), and offer an opinion as to whether the patient could benefit from chemotherapy. Uniform agreement regarding the diagnosis of osteosarcoma was observed in 7 cases (58 hr %), including the most common, conventional subtypes, and the telangiectatic, parosteal, and periosteal variants. Among the remaining 5 cases, the consensus majority (> 4) also was osteosarcoma. However, additional diagnoses ranged from...

We studied whether histologic criteria for grading sarcomas could be applied to fine-needle aspiration biopsy (FNAB) specimens of adult spindle cell sarcomas, without knowledge of the sarcoma subtype, by reviewing 36 specimens. Grade 1 was assigned for minimal nuclear atypia and overlap, no necrosis, and rare mitotic figures, and grade 2 for moderate nuclear atypia, at least moderate nuclear overlap, appreciable mitotic figures, and necrosis. Severe nuclear atypia distinguished grade 3 from grade 2. A major noncorrelation between FNAB and histologic grades was defined as a misclassification of grade 1 vs grade 2 or 3. FNAB grades assigned were grade 1, 1; grade 2, 25; and grade 3, 10. There was 1 major noncorrelation due to a probable FNAB interpretation error. In 15 of 16 FNAB specimens of grade 2 or 3 sarcomas lacking mitotic figures, necrosis, or both, the nuclear atypia reflected the grade. In the remaining case, the degree of nuclear overlap and necrosis determined the grade. The histologic grading of sarcomas can be applied accurately to most FNAB specimens of spindle cell sarcomas without knowledge of the sarcoma subtype.

Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. Bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should raise suspicion of cholangitis, even in the absence of typical clinical findings.