Age and sex-specific risk in fractures with Down syndrome in a retrospective case-control study from Germany.

BACKGROUND: The increasing life expectancy of individuals with Down syndrome has led to a growing awareness of mid- and late-life conditions. METHODS: Based on the Disease Analyser database (IQVIA), this retrospective cohort study compared adults ≥18 years of age with Down syndrome (ICD-10: Q90) in general practices in Germany with a propensity score-matched cohort without Down syndrome. The outcome was the first diagnosis of a fracture within 5 years of the index date. The cumulative incidence of fractures over a 5-year period was presented using Kaplan-Meier curves. Univariable Cox regression analyses by age group and sex were performed to assess the association between Down syndrome and fractures. RESULTS: A total of 2547 individuals with Down syndrome and 12 735 individuals without Down syndrome were included in the study. A significantly higher cumulative fracture incidence within 5 years was observed in the age group 51-60 years (9.3% Down syndrome vs. 4.8% without Down syndrome, P = 0.003) as well as in the age group >60 years (20.3% Down syndrome vs. 8.6% without Down syndrome, P < 0.001) compared with the cohort without Down syndrome. Regression analysis showed a significant association between Down syndrome and fracture risk in women with Down syndrome aged 51-60 years (hazard ratio [HR] = 1.60; 95% confidence interval [CI]: 1.13-2.26), and in those aged 51-60 years (HR = 2.08; 95% CI: 1.27-3.41) and >60 years (HR = 2.98; 95% CI: 1.87-4.73)), but not in men. When comparing fractures in individuals with and without Down syndrome, shoulder and arm fractures were most common in the Down syndrome cohort. CONCLUSION: The results of our study indicate a positive association between individuals with Down syndrome and subsequent fractures in women and those aged >50 years. Prevention of falls appears to be particularly important in these populations. However, future studies should clarify the extent to which socio-economic factors, such as housing, play a role in this context.

Mesopancreatic excision for pancreatic ductal adenocarcinoma improves local disease control and survival.

BACKGROUND: Survival in ductal adenocarcinoma of the pancreatic head (hPDAC) is poor. After implementation of the circumferential resection margin (CRM) into standard histopathological evaluation, the margin negative resection rate has drastically dropped. However, the impact of surgical radicality on survival and the influence of malignant infiltration of the mesopancreatic fat remains unclear. At our institution, a standardized dissection of the mesopancreatic lamina and peri-pancreatic vessels are obligatory components of radical pancreatoduodenectomy. The aim of our study was to histopathologically analyze mesopancreatic tumor infiltration and the influence of CRM-evaluated resection margin on relapse-free and overall survival. METHOD: Clinicopathological and survival parameters of 264 consecutive patients who underwent surgery for hPDAC were evaluated. RESULTS: The rate of R0 resection R0(CRM-) was 48.5%, after the implementation of CRM. Mesopancreatic fat infiltration was evident in 78.4% of all consecutively treated patients. Patients with mesopancreatic fat infiltration were prone to lymphatic metastases (N1 and N2) and had a higher rate of positive resection margin (R1/R0(CRM+)). In multivariate analysis, only R0 resection was shown to be an independent prognostic parameter. Local recurrence was diagnosed in only 21.1% and was significantly lower in patients with R0(CRM-) resected hPDACs (10.9%, p < 0.001). CONCLUSION: Mesopancreatic excision is justified, since mesopancreatic fat invasion was evident in the majority of our patients. It is associated with a significantly improved local tumor control as well as longer relapse-free and overall survival.

Surgical margin clearance and extended chemotherapy defines survival for synchronous oligometastatic liver lesions of the ductal adenocarcinoma of the pancreas.

BACKGROUND: The role of surgery for circumscribed synchronous hepatic lesions of the pancreatic ductal adenocarcinoma (PDAC) remains controversial. Thus, the aim of our study was to compare survival outcome (OS) after surgery of patients with hepatic metastases (M1surg) to patients with only localized disease. METHODS: Correlation analysis of clinicopathological data and OS after resection of M1surg patients and patients with localized PDACs (M0) was performed. Patients were included for survival analysis only if a complete staging including perineural, venous and lymphatic invasion was available. RESULTS: Out of the study collective, 35 patients received extended surgery (M1surg), whereas 131 patients received standardized surgery for localized disease (M0). Length of hospitalization and mortality was similar in both groups. FOLFIRNOX as an adjuvant treatment regime was administered in ~ 23 and ~ 8% of M1surg and M0 patients, respectively. In subgroup analysis of R0 resected patients and in multivariate analysis of the total cohort, there was no difference in overall survival between both groups. Only the resection status (R1 vs R0) and venous invasion (V1) were identified as independent prognostic factors. Site of recurrence in R0 resected M1surg patients and in M0 patients were homogenously distributed. CONCLUSION: This is the first study demonstrating a survival benefit after extended surgery for synchronously hepatic-metastasized PDACs. We found no difference in survival outcome of metastasized patients when compared to patients with localized disease. FOLFIRINOX as an adjuvant treatment regime for resected M1surg presumably is worthwhile. Larger multicenter studies are still needed to validate our results.

Meta-analysis of totally extraperitoneal inguinal hernia repair in patients with previous lower abdominal surgery.

BACKGROUND: Previous lower abdominal surgery is considered a relative contraindication to laparoscopic totally extraperitoneal (TEP) inguinal hernia repair. This was a meta-analysis of studies comparing the feasibility and safety of TEP repair between patients with (PS), and without (NS) a history of lower abdominal surgery. METHODS: A systematic literature search was undertaken for studies comparing the outcome of TEP inguinal hernia repair in patients with, and without previous lower abdominal surgery. Data on postoperative outcomes were extracted and compared by meta-analysis. Odds ratios (ORs) and mean differences with 95 per cent confidence intervals were calculated. RESULTS: Seven comparative cohort studies were identified, involving a total of 1657 procedures (PS 326, NS 1331). There was a statistically significant difference between PS and NS favouring the NS group with regard to both primary outcomes: intraoperative morbidity (OR 2·85, 95 per cent c.i. 1·19 to 6·80; P = 0·02; 7 studies; I2 = 33 per cent), and postoperative morbidity in the multiport subgroup (OR 2·14, 1·28 to 3·58; P = 0·004; 5 studies; I2 = 0 per cent). For the secondary endpoints conversion rate, peritoneal tears, major intraoperative bleeding, postoperative haematoseroma and delay in return to normal activities, there was a statistically significant difference favouring the NS group. CONCLUSION: This study suggests that patients with previous lower abdominal surgery who need hernia repair get less benefit from TEP repair than those with no history of surgery.

[Slipped capital femoral epiphysis-etiology and pathogenesis]. / Epiphyseolysis capitis femoris ­ Ätiologie und Pathogenese.

BACKGROUND: Slipped capital femoral epiphysis (SCFE) is the most significant hip disease in adolescence. Because of its typical occurrence at the time of the pubertal growth spurt and some particular clinical symptoms, it has been a topic of special interest in orthopedic research for a long time. A large number of etiological factors have been described, however their meaning in detail is still controversial. OBJECTIVES: After a short summary of the structure and function of the epiphysis, an overview of concepts in the pathogenesis of SCFE is given. MATERIALS AND METHODS: A selective review of literature was performed. RESULTS: Etiology of SCFE includes genetic, endocrinologic, histochemical and biomechanical factors. During puberty, obesity together with reduced femoral antetorsion are the main risk factors for the disease. The rarer atypical SCFE is closely related to metabolic disorders or other chronical diseases. The pathogenesis is characterized by the disproportion between the load-bearing capacity of the epiphysis and the acting forces on the proximal femur. CONCLUSIONS: The concept of the multifactorial etiology of SCFE is convincing. The susceptibility of the femoral epiphysis is explained considering the remarkable biomechanic local forces. The initial damage can be shown on the histological and the histochemical level. Important new aspects refer to the leptin-metabolism. However, in the majority of cases, biomechanical factors seem to be more significant than endocrinological phenomena. A complete understanding has not succeeded to date.

[Extracorporeal irradiation : Reimplantation of bone segments in the treatment of malignant bone tumours]. / Die extrakorporale Bestrahlung : Replantation von Knochensegmenten bei malignen Knochentumoren.

BACKGROUND: Malignant bone tumors themselves and the wide resection required because of them may cause huge bone defects in the bone segment involved. Autologous bone grafts are a reliable option to cover these defects in many cases but their availability is limited. Besides common alternative reconstruction methods, including the use of allografts and/or prostheses, especially extracoroporeal irradiation (ECI) and reimplantation of the bone segment involved is attracting increasingly more attention nowadays. DISCUSSION: In the following, we report on indications/contraindications, details of the operative technique, as well as the recommended rehabilitation regime of ECI. Furthermore, we compare our own results with those published in the recent literature. Especially the advantages and disadvantages of this method, the risks and the complications are illustrated and critically discussed. CONCLUSION: Extracorporeal irradiation of a tumor bearing bone segment is a valuable alternative reconstruction technique following tumor resections of the pelvis, femur and tibia, with encouraging results with respect to local control, complication risks and functional outcome.

Impact of Single Center Treatment on Ewing Sarcoma 10-Year Long Term Survival Rates.

PURPOSE OF THE STUDY Ewing sarcomas (ES) are the second most common solid malignant bone tumors in both, children and adolescents, and systemic chemotherapy protocols were established during the last 3 decades which proved to be a successful approach in addition to local treatment. The purpose of the present study is (i) to provide survival rates and prognostic factors for patients with ES which received treatment in a single center and (ii) to compare data with results of multicenter studies. MATERIALS AND METHODS Patients (n = 38) were treated by the same surgeon whereas surgery was combined with radiotherapy in 55.3% of the patients (n = 21). Median age at diagnosis was 17.5 years (4.7-60) and the median follow-up time for all patients was 8.2 years (9.8 years for survivors, 3.2 years for non-survivors). RESULTS The survival rate for metastasis free sarcoma decreases from 90.5% to 50% for patients diagnosed with disseminated disease stage. Patients with a good response to chemotherapy survived in 83.3% of the cases. In addition, a higher OS was found for patients younger than 15 years (82.4%) when compared to patients older than 15 years (73.3%). In contrast, multicenter studies reported lower survival rates for metastasis free (~60%) and metastasis stages (< 40%). DISCUSSION The survival rates in the present single center study are higher than the rates reported from multi-center studies although same chemotherapy protocols were used and no substantially difference are apparent for patient population. CONCLUSIONS Based on the present data we re-emphasize that patients with Ewing sarcoma receive appropriate treatment in a large and qualified center particularly considering the survival rates. In addition, our data underline that a close collaboration between the oncological team and the experienced surgeon is crucial for patient's care. Key words: Ewing sarcoma, survival rate, single center, prognostic factors, chemotherapy, surgery, multi center, single center.

[Acetabuloplasty - The Dega and Pemberton technique]. / Beckenosteotomie nach Dega und Pemberton.

BACKGROUND: Hip dysplasia is the most common congenital deformity requiring surgical correction osteotomy in order to prevent early onset of secondary hip arthrosis. The shape of the acetabulum can be modified by Dega or Pemberton osteotomy and is indicated for hip dysplasia and luxation with irregularities of the socket for children aged between 2 and 12 when the y­physis is still open. METHOD: We will describe indication, contra-indication, preoperative planning as well as details of the Pemberton technique. In addition, we will provide practical advice based on our long-standing experience. RESULTS: We present long-term results from the literature and also from our department. In addition, we will explain and critically discuss our own experiences and the risks and complications of surgical techniques. Good long-term results are reported for acetabuloplasties and Salter osteotomy which are preferred for surgical treatment of hip dysplasia in early life. Advantages and disadvantages of both surgical techniques will be compared in the discussion section. CONCLUSION: Dega and Pemberton acetabuloplasty shows good long-term results regarding prevention of a secondary coxarthrosis. However, correct indication is crucial since this surgical technique is more difficult compared to Salter osteotomy but is also associated with a higher correction potential and a lower complication rate.

[Prognostic Value of Preoperative Anaemia and Perioperative Blood Transfusion in Surgical Treatment of Rectal Cancer]. / Prognostischer Wert präoperativer Anämie und perioperativer Fremdbluttransfusion in der chirurgischen Therapie des Rektumkarzinoms.

INTRODUCTION: There is evidence for the prognostic value of perioperative blood transfusion in the surgical treatment of patients with rectal cancer in the current literature. Also preoperative anaemia seems to have an impact on the outcome of these patients. The aim of this study was to evaluate the impact of preoperative anaemia and perioperative blood transfusion in patients with rectal cancer treated in our hospital. PATIENTS AND METHODS: 208 patients (81 females, 127 males; median age, 67 years) with rectal cancer were included in this retrospective study. All patients received surgical treatment. In 75 % of the patients an anterior rectum resection was performed while 25 % received an abdominoperineal rectum exstirpation. Patients with neoadjuvant treatment were included and statistical analyses were performed. RESULTS: 107 (51.4 %) patients exhibited preoperative anaemia. Patients with neoadjuvant treatment presented with significantly lower preoperative Hb (haemoglobin) values than patients without neoadjuvant treatment (p = 0.022). Patients with preoperative anaemia received significantly more blood transfusions (p = 0.001), had significantly longer hospital stays (p = 0.023) and significantly lower 5-years overall survival (p = 0.005). Blood transfusion was necessary in 82 patients (39.4 %). These patients presented with a significantly higher rate of perioperative complications (p = 0.01) and a lower 5-years overall survival (p = 0.002). In multivariate analyses neither preoperative anaemia nor perioperative transfusion was a significant prognostic factor. CONCLUSION: In our study preoperative anaemia and perioperative blood transfusion seems to have an impact on outcome of surgical treatment of patients with rectal cancer. However, in multivariate analyses neither preoperative anaemia nor perioperative transfusion was a significant prognostic factor.

Encapsulation status of papillary thyroid microcarcinomas is associated with the risk of lymph node metastases and tumor multifocality.

The management of papillary microcarcinoma (PMC) of the thyroid is controversial, especially after partial thyroid resection for benign thyroid disease. In order to detect prognostic factors for PMC, we analyzed 116 patients with PMC for encapsulation status and lymph node metastases. Between 10/1992 and 12/2010, 116 patients with PMC have been operated in our department (87 females, 29 males, median age 49 years). Eighty per cent of PMCs were diagnosed postoperatively. Seventy-six patients (66%) received a more extended resection with either thyroidectomy, near total thyroidectomy, or Dunhill operation either primarily or after completion operation, whereas 40 patients (34%) had only partial resection. Fifty patients (43%) received radioiodine (RIA) ablation. Lymph node metastases were found in 21 patients (18%). Univariate analysis showed four risk factors to be significantly associated with the risk of lymph node metastasis (p<0.05): male gender, younger age, age group<50 years and nonencapsulation of the tumor. Multivariate analysis demonstrated statistical significance for gender and tumor capsulation status. The tumor capsulation status also correlated with tumor multifocality. Our data show that the risk of lymph node metastases is significantly higher in partially or nonencapsulated PMC than in encapsulated specimens. We therefore suggest that the WHO classification should be extended to a compulsory notification of the encapsulation status in PMC.

[Stem cell-induced liver regeneration]. / Stammzell-induzierte Leberregeneration.

BACKGROUND: The liver has an excellent regenerative capacity after resection. However, below a critical level of future liver remnant volume (FLRV), partial hepatectomy is accompanied by a significant increase of postoperative liver failure. There is accumulating evidence for the contribution of bone marrow stem cells (BMSC) to participate in liver regeneration. Here we report our experience with portal vein embolisation (PVE) and CD133+ BMSC administration to the liver, compared with PVE alone, to augment hepatic regeneration in patients with critically low FLRV or impaired liver function. PATIENTS AND METHODS: Eleven patients underwent PVE of liver segments I and IV-VIII to stimulate hepatic regeneration prior to extended right hepatectomy. In these 11 patients with a FLRV below 25% and/or limited quality of hepatic parenchyma, PVE alone did not promise adequate proliferation. These patients underwent additional BMSC administration to segments II and III. Two radiologists blinded to patients' identity and each other's results measured liver and tumour volumes with helical computed tomography. Absolute, relative and daily FLRV gains were compared with a group of patients that underwent PVE alone. RESULTS: The increase of the mean absolute FLRV after PVE with BMSC application from 239.3 mL±103.5 (standard deviation) to 417.1 mL±150.4 was significantly higher than that from 286.3 mL±77.1 to 395.9 mL±94.1 after PVE alone (p<0.05). Also the relative gain of FLRV in this group (77.3%±38.2%) was significantly higher than that after PVE alone (39.1%±20.4%) (P=0.039). In addition, the daily hepatic growth rate after PVE and BMSC application (9.5±4.3 mL/d) was significantly superior to that after PVE alone (4.1±1.9 mL/d) (p=0.03). Time to surgery was 27 days±11 in this group and 45 days±21 after PVE alone (p=0.02). Short- and long-term survival were not negatively influenced by the shorter waiting period. CONCLUSION: In patients with malignant liver lesions, the combination of PVE with CD133+ BMSC administration substantially increased hepatic regeneration compared with PVE alone. This procedure bears the potential to allow the safe resection of patients with a curative intention that would otherwise carry the risk post-operative liver failure.

Global alteration in gene expression profiles of deciduas from women with idiopathic recurrent pregnancy loss.

Recurrent pregnancy loss (RPL) occurs in ∼5% of women. However, the etiology is still poorly understood. Defects in decidualization of the endometrium during early pregnancy contribute to several pregnancy complications, such as pre-eclampsia and intrauterine growth restriction (IUGR), and are believed to be important in the pathogenesis of idiopathic RPL. We performed microarray analysis to identify gene expression alterations in the deciduas of idiopathic RPL patients. Control patients had one antecedent term delivery, but were undergoing dilation and curettage for current aneuploid miscarriage. Gene expression differences were evaluated using both pathway and gene ontology (GO) analysis. Selected genes were validated using quantitative reverse transcription-polymerase chain reaction (qRT-PCR). A total of 155 genes were found to be significantly dysregulated in the deciduas of RPL patients (>2-fold change, P < 0.05), with 22 genes up-regulated and 133 genes down-regulated. GO analysis linked a large percentage of genes to discrete biological functions, including immune response (23%), cell signaling (18%) and cell invasion (17.1%), and pathway analysis revealed consistent changes in both the interleukin 1 (IL-1) and IL-8 pathways. All genes in the IL-8 pathway were up-regulated while genes in the IL-1 pathway were down-regulated. Although both pathways can promote inflammation, IL-1 pathway activity is important for normal implantation. Additionally, genes known to be critical for degradation of the extracellular matrix, including matrix metalloproteinase 26 and serine peptidase inhibitor Kazal-type 1, were also highly up-regulated. In this first microarray approach to decidual gene expression in RPL patients, our data suggest that dysregulation of genes associated with cell invasion and immunity may contribute significantly to idiopathic recurrent miscarriage.

Synovial sarcomas usually metastasize after >5 years: a multicenter retrospective analysis with minimum follow-up of 10 years for survivors.

BACKGROUND: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. PATIENTS AND METHODS: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years. RESULTS: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. CONCLUSIONS: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.

[Intramedullary motorized nail for equalization of posttraumatic leg length discrepancies]. / Ausgleich posttraumatischer Beinverkürzungen mit einem motorisierten intramedullären Nagel.

BACKGROUND: Fractures in childhood may result in a significant leg length discrepancy (LLD). The common correction method of LLD and deformities is callotasis with external fixation. This is often associated with pain, pin site infection, muscle tethering and reduced range of movement (ROM). PATIENTS AND METHODS: Between 2006 and 2008 a total of 11 cases of posttraumatic LLD (range 2.4-4.3 cm) were treated with a fully implanted motorized lengthening device (Fitbone®). Hospitalization time, leg equalization, rehabilitation time and complications compared to external fixation were recorded. RESULTS: Leg lengthening was successfully performed in all cases, in five combined with angular and/or rotatory corrections. The mean distraction index was 1.03 mm/day (range 0.6-1.2 mm/day) and the mean consolidation index was 40 days/cm (range 25.2-50.9 days/cm). The average hospital stay was 9.8 days (range 8-20 days). Bone or soft tissue infections were not observed, nor were the complications commonly associated with external fixation. Functional results were excellent as the preoperative knee ROM was regained in all cases and with improved ROM in three cases. CONCLUSION: The Fitbone® nail is a valuable alternative to conventional methods which reduces complications commonly associated with external fixation.

Preferential nuclear compartmentalization of endogenous mink cell focus-forming-related retroviral transcripts.

Endogenous mink cell focus-forming (MCF)-like retroviral sequences in the murine genome are stable, inherited sequences analogous to other chromosomal genes. As such, it is thought that they are transcribed and translated in a manner analogous to other genes. However, when the SL12.4 CD4-, CD8- thymoma cell line was studied for nuclear/cytoplasmic distribution of endogenous MCF-related transcripts, there was a nuclear predominance. The great majority of full-length 8.4-kb endogenous MCF-related transcripts were nuclear. Even the smaller, spliced 3.0-kb transcripts were at least as prominent in the nucleus as the cytoplasm, whereas cellular RNA was 80% cytoplasmic and other cellular transcripts were represented in the cytoplasm to a much greater extent than the nucleus. Size cannot fully account for the nuclear presence of MCF-related endogenous transcripts, because the 3.0-kb MCF transcripts occurred in the nucleus to a much greater relative extent than 3.8-kb c-myb transcripts. These studies point to retroviral-like structures of these transcripts as influencing their intracellular compartmentalization.

CpG oligodeoxynucleotides act as adjuvants that switch on T helper 1 (Th1) immunity.

Synthetic oligodeoxynucleotides (ODN) that contain unmethylated CpG motifs (CpG ODN) induce macrophages to secrete IL-12, which induces interferon (IFN)-gamma secretion by natural killer (NK) cells. Since these cytokines can induce T helper 1 (Th1) differentiation, we examined the effects of coadministered CpG ODN on the differentiation of Th responses to hen egg lysozyme (HEL). In both BALB/c (Th2-biased) and B10.D2 (Th1-biased) mice, immunization with HEL in incomplete Freund's adjuvant (IFA) resulted in Th2-dominated immune responses characterized by HEL-specific secretion of IL-5 but not IFN-gamma. In contrast, immunization with IFA-HEL plus CpG ODN switched the immune response to a Th1-dominated cytokine pattern, with high levels of HEL-specific IFN-gamma secretion and decreased HEL-specific IL-5 production. IFA-HEL plus CpG ODN also induced anti-HEL IgG2a (a Th1-associated isotype), which was not induced by IFA-HEL alone. Control non-CpG ODN did not induce IFN-gamma or IgG2a, excepting lesser increases in B10.D2 (Th1-biased) mice. Thus, CpG ODN provide a signal to switch on Th1-dominated responses to coadministered antigen and are potential adjuvants for human vaccines to elicit protective Th1 immunity.

Quantitative signal intensity ratios to distinguish between subfascial lipoma and atypical lipomatous tumor/well-differentiated liposarcoma using short-tau inversion recovery (STIR) MRI.

PURPOSE: To establish simple quantitative variables at short-tau inversion recovery (STIR) magnetic resonance imaging (MRI) to identify lipomas with high specificity in patients with indeterminate subfascial lipomatous tumors. MATERIALS AND METHODS: The MRI examinations of 26 patients (14 men, 12 women; mean age 63±12.5 [SD] years; range: 40-84years) with histopathologically proven subfascial atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLs) and those of 68 patients (32 men, 36 women; mean age, 56±13.5 [SD] years; range: 21-83years) with lipomas were retrospectively reviewed. Ratios derived from region of interest based signal intensity (SI) measurements of tumors and adjacent fat on STIR images were calculated and maximum tumor diameters were noted. Diagnostic parameter capabilities were assessed using ROC curve analysis. Interreader agreement was evaluated by calculation of intraclass correlation coefficients (ICC). RESULTS: Using a cut-off value of 1.18, STIR-SI ratios allowed discriminating between lipoma and ALT/WDL (AUC=0.88; P<0.001) yielding 93% specificity (95% CI: 77-99%) and 74% sensitivity (95% CI: 61-84%) for the diagnosis of lipoma. Interreader agreement was excellent (ICC=0.93). A significant difference in maximum tumor diameter was found between ALT/WDLs (mean: 18.1±6.0 [SD] cm; range: 5.6-33.1cm) and lipomas (mean: 9.7±5.0 [SD] cm; range: 2.9-29.1cm) (P<0.001). Using a cut-off of 11cm, maximum tumor diameter allowed discriminating between lipoma and ALT/WDLs with 92% specificity (95% CI: 75-99%) and 69% sensitivity (95% CI: 57-80%). The combination of a STIR-SI ratio<1.4 and maximum tumor diameter<11cm yielded 100% specificity (95% CI: 87-100%) and 65% sensitivity (95% CI: 54-77%) for the diagnosis of lipoma. CONCLUSION: The combination of STIR-SI ratio and maximum diameter allows discriminating between lipoma and ALT/WDL in initially indeterminate lipomatous tumors.

A new technique for correction of leg length discrepancies in combination with complex axis deformities of the lower limb using a lengthening nail and a locking plate.

PURPOSE: Intramedullary lengthening nails are an accepted alternative to external fixators but are limited by anatomical preconditions. Therefore, to date the use of external fixators is sometimes inevitable. We report on a new technique for correction of combined limb length discrepancies and complex axis deformities using solely internal devices - a lengthening nail and a locking plate. METHODS: Between October 2008 and November 2011 five patients (two femora, three tibias) with a mean leg length discrepancy of 36 mm (25 to 50) and a complex angular deformity were treated with a fully implantable motorized lengthening nail (Fitbone) and a locking plate. All patients were evaluated with regards to the pre- and postoperative leg length as well as axis alignment, functional outcome, lengthening indices and complications. RESULTS: A successful leg length equalization was achieved in all cases and physiological joint orientation angles in all but one case. The mean distraction index was 1.2 mm/day, the maturation index 24 days/cm and the consolidation index 35 days/cm. The functional outcome was very encouraging in all cases with bilateral free range of movement. In total, two complications were observed, one nonunion and one loss of leg length after an early locking bolt removal in a peripheral hospital. CONCLUSION: The combination of a fully implantable motorized lengthening nail and a locking plate is a valuable alternative option for treating selected cases with limb length discrepancies in combination with a complex deformity of the lower leg. However, the reported technique puts high demands on the preoperative planning, operative technique as well as surgeon's skills. LEVEL OF EVIDENCE: IV (retrospective series).

Analysis of thymic endogenous retroviral expression in murine lupus. Genetic and immune studies.

Inbred mouse genomes contain two subclasses of proviruses related to mink cell focus-forming (MCF) retroviruses: polytropic (Pmv), and modified polytropic (Mpmv). To determine whether one of these subclasses is associated with murine lupus, oligonucleotide probes specific for Pmv or Mpmv sequences were used in Northern analyses. Thymus 8.4 kb Mpmv RNA was expressed in five of five lupus-prone strains and crosses and this expression was not affected by genes that retard or accelerate development of lupus. Two of four leukemia-prone strains expressed low levels of such thymic transcripts, but none of 11 control strains did. 8.4 kb Mpmv RNA expression was not induced in thymuses of control mice by the lpr/lpr or gld/gld genotypes (which cause polyclonal immune activation) nor by treatment with mitogens. In contrast to Mpmv, thymic 8.4 kb Pmv expression was poorly associated with autoimmunity: it was easily detected in nearly all strains, and was increased by polyclonal activation in control mice. These studies indicate that the organ-specific thymic 8.4 kb Mpmv expression (a) is characteristic of several genetic backgrounds which predispose to murine lupus, (b) precedes and does not correlate with disease development, (c) is not due to polyclonal activation, and (d) is regulated independently of 8.4 kb Pmv expression.