Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease) and oligoarthritis

A 24-year-old woman who had sinus histiocytosis with massive lymphadenopathy (SHML, Rosai-Dorfman disease) also had oligoarthritis. We found only four previously reported cases of SHML with clinical joint disease. The clinical picture may suggest rheumatoid arthritis or a spondylarthropathy with peripheral joint involvement. SHML should be considered routinely among the differential diagnoses in young patients with arthritis and large lymphadenopathies. There is no consensus regarding the treatment. In our patient, conventional disease-modifying antirheumatic drugs followed by 3 months of adalimumab then 3 months of etanercept had no effect on the symptoms.     

Ureterosciatic hernia: a rare cause of ureteral obstruction visualized by multislice helical computed tomography

Ureterosciatic herniation is an extremely rare cause of ureteral obstruction, of which few cases have been published. We describe a case revealed by pyelonephritis with acute renal failure in an 81-year-old woman. After percutaneous nephrostomy tube placement and antibiotic therapy, urography and multiplanar computed tomography reconstructions of the pelvis confirmed the diagnosis. The symptoms resolved, and the hernia was then corrected surgically.     

Genetic basis for systemic sclerosis

Among the connective tissue diseases, systemic sclerosis is an orphan disease in which diffuse connective tissue alterations lead to multi-organ involvement. Environmental factors implicated in triggering this multifactorial disease include crystalline silica, chlorine solvents, welding vapors, and various other solvents. Clustering within families indicates a role for genetic factors. Although concordance for the disease among identical twins is low, concordance for autoantibodies associated with systemic sclerosis and for fibroblast gene expression profiles is higher. Because multiplex families are rare, association and candidate gene strategies are the most appropriate methods for investigating the genetics of systemic sclerosis. The most consistent data relate to the involvement of fibrosis genes, most notably the TGF-beta regulation pathway, secreted protein acid and rich in cysteine (SPARC) genes, and the fibrillin-1 gene (FBN1). Several variants of genes for cytokines or their receptors may be involved. Data on the vasculopathy characteristic of systemic sclerosis are somewhat conflicting. Investigations into the genetics of systemic sclerosis may shed light on the complex pathophysiology of this disease, help to identify factors that predict organ involvement, and suggest new treatment strategies.     

Correlation between groin pain and cup design of hip-resurfacing implants: a prospective study

Purpose

Cup design has been incriminated as the source of groin pain after hip resurfacing but has not been well described; thus, it was assessed in a prospective study looking at three implant types.

Methods

A group-match was done between three groups of hip resurfacing devices according to age, sex, body mass index, activity level, osteoarthritis aetiology and pre-operative scores.

Results

The global groin pain rate was 5.7 % at six months and 2.7 % at last follow-up. Groin pain rate was significantly different between the three groups (p = 0.004) and had a strong influence on the subjective results (p = 0.04). No groin pain emerged between six months and last follow-up. No clinical differences were noted in Harris hip score and Merle d’Aubigné-Postel score at last follow-up. However, the Oxford hip score and Devane activity score were significantly lower for cups with macrostructures.

Conclusion

The low groin pain rate in this prospective cohort was probably secondary to the specific surgical technique used and seems to be correlated with cup design. Macrostructures on the external part of the cup could be significantly harmful.     

Nephron-sparing surgery is superior to radical nephrectomy in preserving renal function benefit even when expanding indications beyond the traditional 4-cm cutoff

ObjectivesTo analyze to what extent partial nephrectomy (PN) is superior to radical nephrectomy (RN) in preserving renal function outcome in relation to tumor size indication.Methods and materialsClinical data from 973 patients operated at 9 academic institutions were retrospectively analyzed. Glomerular filtration rate (GFR) before and after surgery was calculated with the abbreviated Modification of the Diet in Renal Disease equation. For a fair comparison between the 2 techniques, all imperative indications for PN were excluded. A shift to a less favorable GFR group following surgery was considered clinically significant.ResultsMedian age at diagnosis was 60 years (19–91). Tumor size was smaller than 4 cm in 665 (68.3%) cases and larger than 4 cm in 308 (31.7%) cases. PN and RN were performed in 663 (68.1%) and 310 (31.9%) patients, respectively. In univariate analysis, patients undergoing PN had a smaller risk for developing significant GFR change following surgery than those undergoing RN did. This was true for tumors≤4 cm (P = 0.0001) and for tumors>4 cm (P = 0.0001). In multivariate analysis, the following criteria were independent predictive factors for developing significant postoperative GFR loss: the use of RN (P = 0.0001), preoperative GFR<60 ml/min (P = 0.0001), tumor size≥4 cm (P = 0.0001), and older age at diagnosis (P = 0.0001).ConclusionsThe renal function benefit carried out by elective PN over RN persists even when expanding nephron-sparing surgery indications beyond the traditional 4-cm cutoff.     

Protection Against Type 1 Diabetes Upon Coxsackievirus B4 Infection and iNKT-Cell Stimulation: Role of Suppressive Macrophages

Invariant natural killer T (iNKT) cells belong to the innate immune system and exercise a dual role as potent regulators of autoimmunity and participate in responses against different pathogens. They have been shown to prevent type 1 diabetes development and to promote antiviral responses. Many studies in the implication of environmental factors on the etiology of type 1 diabetes have suggested a link between enteroviral infections and the development of this disease. This study of the pancreatropic enterovirus Coxsackievirus B4 (CVB4) shows that although infection accelerated type 1 diabetes development in a subset of proinsulin 2–deficient NOD mice, the activation of iNKT cells by a specific agonist, α-galactosylceramide, at the time of infection inhibited the disease. Diabetes development was associated with the infiltration of pancreatic islets by inflammatory macrophages, producing high levels of interleukin (IL)-1β, IL-6, and tumor necrosis factor-α and activation of anti-islet T cells. On the contrary, macrophages infiltrating the islets after CVB4 infection and iNKT-cell stimulation expressed a number of suppressive enzymes, among which indoleamine 2,3-dioxygenase was sufficient to inhibit anti-islet T-cell response and to prevent diabetes. This study highlights the critical interaction between virus and the immune system in the acceleration or prevention of type 1 diabetes.Type 1 diabetes is characterized by the destruction of pancreatic islet β-cells by autoreactive CD4 and CD8 T cells, leading to low insulin production and incapacity to regulate blood glucose levels (1). Despite numerous studies, the etiology of type 1 diabetes remains elusive. Besides genetics (2–4), environmental factors such as viral infections have been suggested as triggers of type 1 diabetes (5–7). Most striking of these infections are the type B Coxsackieviruses belonging to the enterovirus genus whose genome and anti-Coxsackievirus antibodies were detected more frequently in the blood of recently diagnosed patients compared with healthy controls (8,9). Besides, enteroviral RNA or enteroviral particles were directly detected in the pancreas of type 1 diabetic patients, whereas they were undetectable in the pancreas of healthy donors (9,10). In a mouse model of type 1 diabetes, Serreze et al. (11) showed that diabetes can develop rapidly after Coxsackievirus B4 (CVB4) infection if mice had an advanced age and sufficient insulitis. Others have reported that inefficient islet β-cell response, viral dose, and replication rate as well as a lack of islet neogenesis could also promote accelerated diabetes development after CVB4 infection (12–14).Natural killer T (NKT) cells are CD1d-restricted, nonconventional T cells recognizing self and exogenous glycolipids. Most NKT cells express an invariant T-cell receptor α chain, Vα14-Jα18 (Vα14) in mice and Vα24-Jα18 in humans, and are named invariant NKT (iNKT) cells. They can promptly secrete copious amounts of interferon-γ (IFN-γ) and interleukin (IL)-4 and provide maturation signals to dendritic cells (DCs) and lymphocytes, thereby contributing to both innate and acquired immunity (15,16). iNKT cells are potent regulatory cells that can inhibit autoimmunity and promote immune responses against pathogens (1,17). Diabetes can be prevented in NOD mice by increasing iNKT cell numbers and by iNKT-cell stimulation with exogenous ligands such as α-galactosylceramide (αGalCer) (15,18,19). NOD mice protected from diabetes by iNKT cells have weak T helper 1 anti-islet β-cell responses (20). Indeed, iNKT cells can impair the differentiation of anti-islet CD4 and CD8 T cells, which become hyporesponsive or anergic (21). Contrary to their suppressive role in type 1 diabetes, iNKT cells can enhance immune responses to pathogens such as parasites, bacteria, and viruses (22,23).Our previous studies conducted in a murine model of type 1 diabetes with lymphocytic choriomeningitis virus infection revealed that iNKT cells could promote systemic antiviral CD8 T-cell responses while inhibiting deleterious anti-islet T-cell responses, thereby preventing type 1 diabetes (24,25). In the present study, we investigated the role of iNKT cells after CVB4 infection, revealing that diabetes development following CVB4 infection is associated with the infiltration of inflammatory macrophages into the pancreatic islets with subsequent activation of anti-islet T cells. However, the activation of iNKT cells during CVB4 infection results in the infiltration of suppressive macrophages into pancreatic islets. Indoleamine 2,3-dioxygenase (IDO) expressed by these macrophages was critical for the inhibition of diabetes development.     

Comparison of oncological outcomes after segmental ureterectomy or radical nephroureterectomy in urothelial carcinomas of the upper urinary tract: results from a large French multicentre study

Study Type – Therapy (multi‐centre retrospective cohort) Level of Evidence 2b What's known on the subject? and What does the study add? Upper urinary tract urothelial carcinomas (UUT‐UCs) are rare tumours. Because of the aggressive pattern of UC, radical nephroureterectomy (RNU) with bladder cuff removal remains the ‘gold‐standard’ treatment. However, conservative strategies, such as segmental ureterectomy (SU) or endourological management, have also been developed in patients with imperative indications. Some teams are now advocating the use of conservative management more commonly in cases of elective indications of UUT‐UCs. Due to the paucity of cases of UUT‐UC, only limited data are available on the oncological outcomes afforded by conservative management. We retrospectively investigated the oncological outcomes after SU and RNU in a large multi‐institutional database. Overall, 52 patients were treated with SU and 416 with RNU. There was no statistical difference between the RNU and SU groups for the 5‐year probability of cancer‐specific survival, recurrence‐free survival and metastasis‐free survival. The type of surgery was not a significant prognostic factor in univariate analysis. The results were the same in a subgroup analysis of only unifocal tumours of the distal ureter with a diameter of <2 cm and of low stage (≤T2). Our results suggest that oncological outcomes after conservative treatment with SU are comparable to RNU for the management of UUT‐UC in select cases.

OBJECTIVE

• ? To compare recurrence‐free survival (RFS), metastasis‐free survival (MFS) and cancer‐specific survival (CSS) after segmental ureterectomy (SU) vs radical nephroureterectomy (RNU) for urothelial carcinoma (UC) of the upper urinary tract (UUT‐UC) located in the ureter.

PATIENTS AND METHODS

• ? We performed a multi‐institutional retrospective review of patients with UUT‐UC who had undergone RNU or SU between 1995 and 2010.
• ? Type of surgery, Tumour‐Node‐Metastasis status, tumour grade, lymphovascular invasion and positive surgical margin were tested as prognostic factors for survival.

RESULTS

• ? In all, 52 patients were treated with SU and 416 with RNU. The median (range) follow‐up was 26 (10–48) months.
• ? The 5‐year probability of CSS, RFS and MFS for SU and RNU were 87.9% and 86.3%, respectively (P= 0.99); 37% and 47.9%, respectively (P= 0.48); 81.9% and 85.4%, respectively (P= 0.51).
• ? In univariable analysis, type of surgery (SU vs RNU) failed to affect CSS, RFS and MFS (P= 0.94, 0.42 and 0.53, respectively).
• ? In multivariable analyses, pT stage and pN stage achieved independent predictor status for CSS (P= 0.005 and 0.007, respectively); the positive surgical margin and pT stage were independent prognostic factors of RFS and MFS (P= 0.001, 0.04, 0.009 and 0.001, respectively).
• ? The main limitation of the study is its retrospective design, which is due to the rarity of the disease.

CONCLUSIONS

• ? Short‐term oncological outcomes after conservative treatment with SU are comparable to RNU for the management of UUT‐UC in select cases and should be considered an option.
• ? In every other case, RNU still represents the ‘gold standard’ for the treatment of UUT‐UC.

     

Recurrence of Dislocation Following Total Hip Arthroplasty Revision Using Dual Mobility Cups Was Rare in 180 Hips Followed Over 7?Years

Background

Dual mobility (DM) cups of mobile polyethylene were introduced to prevent total hip arthroplasty (THA) dislocation, but no large series with this design to treat recurrent instability have been reported.

Purpose

Our retrospective investigation ascertained the efficiency of DM cups in correction of recurrent dislocation and assessed any adverse effects.

Methods

One hundred eighty THAs with recurrent instability were revised to DM cups in 180 patients (mean age, 67.4 ± 11.7 years; range, 19 to 92 years). Thirty-one patients (17.2%) underwent at least one earlier THA revision, and 15 (10.3%) incurred non-union of the greater trochanter. Of the initial group in 2009, 145 patients had completed evaluations which included assessment of the Harris Hip Score and a radiographic assessment at a mean follow-up of 7.7 ± 2.2 years (range, 4 to 14 years). The rate of survival was calculated considering any reason for revision as failure.

Results

At follow-up, Harris hip score was 83.9 ± 16.1 (range, 21 to 100). Dislocation of the large articulation occurred in seven hips (4.8%), and only two recurred (1.4%) (one requiring additional revision). In addition, two intra-prosthetic dislocations of the small articulation (1.4%) were observed and needed revision surgery. The large number of earlier surgeries and non-union of the greater trochanter were related to recurrent instability. Two cups (1.4%) showed signs of definite loosening; six (4.1%) presented signs of possible loosening. Twenty-nine hips manifested femoral or acetabular osteolysis (20%), but only three were severe. Eight-year survival rate considering revision for any reason was 92.6% (95% CI, 85.5–96.4%).

Conclusions

This series indicates that DM cups are a viable option to treat recurrent THA instability. Their design provides a low risk of recurrent instability without increasing mechanical complications.     

Assessment of Risk Factors for Complications of Laparoscopic Partial Nephrectomy

Objective

Laparoscopic partial nephrectomy (LPN) is a technique that is emerging as an attractive option for the treatment of renal tumors ≤4 cm. We retrospectively analyzed our experience with LPN to identify patient and tumor features that correlate with a higher risk of complications.

Material and methods

From January 2001 to May 2007, 90 patients underwent LPN at our institution for a clinically localized renal tumor. A retrospective chart review was carried out. Clinical and pathological information were collected for each patient, including patient age and body mass index, tumor size, location and pattern of growth (cortical vs. corticomedullar), surgical approach (transperitoneal vs. retroperitoneal), warm ischemia time, technique that was used to achieve hemostasis, maximum thickness of the margin of resection, and histology. Statistical analysis (chi-square test, Fisher exact test, Mann-Whitney U test, linear regression model) was performed to test the correlation between the above-mentioned variables and the occurrence of complications.

Results

Twenty-two patients (24.4%) had surgical and/or medical complications in our series. The only variable that was found to significantly correlate with a higher number of complications was a corticomedullar tumor growth pattern as opposed to a cortical growth pattern (p = 0.02).

Conclusions

LPN is an attractive alternative to open partial nephrectomy for the treatment of small renal tumors. On the basis of our experience, the selection of patients with cortical renal lesions seems to be required to reduce the risk of complications and therefore maximize the advantages of this minimally invasive but challenging procedure.