Chronic recurrent multifocal osteomyelitis

Chronic recurrent multifocal osteomyelitis (CRMO), also known as chronic nonbacterial osteomyelitis, is an orphan disease that manifests as recurrent flares of inflammatory bone pain with or without a fever. The pain is related to one or more foci of nonbacterial osteomyelitis. To distinguish unifocal CRMO from a tumor or an infection, a bone biopsy is required in nearly all patients and a trial of antibiotic therapy in many. CRMO is now considered the pediatric equivalent of SAPHO syndrome, and recent data suggest that CRMO should be classified among the autoinflammatory diseases. The treatment of CRMO is not standardized. Although no randomized placebo-controlled trials are available, there is general agreement that nonsteroidal antiinflammatory drugs constitute the best first-line treatment and that bisphosphonates and biotherapies such as TNFα antagonists are effective in the most severe forms. Although CRMO is considered a benign disease, recent data suggest an up to 50% rate of residual impairments despite optimal management.     

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.     

Bilateral dissection of external iliac artery

External iliac artery (EIA) dissection and especially bilateral involvement is very rare. We report the case of a 49-year-old male intense bicyclist who had presented a dissection of the left EIA responsible for claudication. He underwent an iliofemoral vein graft bypass. The histopathologic examination showed a dissection of the EIA with an otherwise normal arterial wall. Two years after he resumed his sporting activity, a dissection of the right EIA occurred with the onset of claudication. The patient underwent a right iliofemoral vein graft bypass. Histopathologic examination showed the same lesions as on the left side. Bilateral involvement of EIA dissection is possible especially when the mechanism leading to dissection is persistent. An attentive follow-up is thus to consider.     

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.     

Network of Contacts between Cattle Herds in a French Area Affected by Bovine Tuberculosis in 2010

France attained ‘Officially Tuberculosis‐Free’ status in 2000. However, the Côte d’Or department (a French administrative unit) has since seen an increase in bovine tuberculosis (bTB) cases, with 35% of cases attributed to neighbourhood contamination. The aim of this study was to investigate the characteristics of neighbourhood contacts in an area affected by bTB in 2010, through the use of social network methods. We carried out a survey to determine the frequency and distribution of between‐herd contacts in an area containing 22 farms. Contacts were weighted, as not all types of contact carried the same risk of bTB transmission. Cattle movement was considered to be associated with the highest risk, but was not observed within the studied area during the study period. Contact with wild boars was the most frequent type of contact, but was associated with a very low risk. Direct cattle‐to‐cattle contacts in pasture and contacts with badger latrines were less frequent, but entailed a greater risk of M. bovis transmission. Centrality values were heterogeneous in these two networks. This would enable the disease to spread more rapidly at the start of epidemics than in a perfect randomly mixed population. However, this situation should also result in the total number of infected herds being smaller. We attributed 95% of the contacts to direct contact in pasture or contact with wild boars or badger latrines. Other kinds of contact occurred less frequently (equipment sharing, cattle straying) or did not occur at all (attendance at a show). Most of the contact types were correlated, but none was sufficient in itself to account for all contacts between one particular farm and its neighbours. Contacts with neighbours therefore represent a challenge for the implementation or improvement of control measures.