[High anti-topoisomerase-1 autoantibodies levels are associated with the extension of skin fibrosis and vascular progression in patients with systemic sclerosis]. / Des taux élevés d'auto-anticorps anti-topo-isomérase-1 sont associés à l'extension de la fibrose cutanée et à la progression vasculaire chez les patients atteints de sclérodermie systémique.

BACKGROUND: Anti-centromere antibodies, anti-topoisomerase-1 antibodies (ATA), and anti-RNA-polymerase III antibodies are three Systemic Sclerosis (SSc)-specific autoantibodies. Their detection is helpful in determining the prognosis. We aimed to evaluate whether ATA levels were associated with disease severity at diagnosis or disease progression during follow-up in ATA positive patients. METHODS: We conducted a single-centre French retrospective observational study, between 2014 and 2021. ATA positive patients fulfilling the ACR/EULAR 2013 classification criteria for SSc with a minimal follow-up of 1 year and 2 ATA dosages were included. SSc patients with high IgG ATA levels at baseline (>240IU/mL) were compared with SSc patients with low levels (≤240IU/mL), at inclusion and at 1 and 3 years. A variation of at least 30 % of ATA levels was considered significant. RESULTS: Fifty-nine SSc patients were included and analysed. There was a predominance of women and of patients with diffuse interstitial lung disease. Patients with high ATA levels exhibited a higher skin sclerosis assessed by the modified Rodnan skin score (P=0.0480). They had a lower carbon monoxide transfer coefficient (P=0.0457), a lower forced vital capacity (FVC) (P=0.0427) and more frequently had a FVC under 80 %, when compared to patients with low ATA levels (P=0.0423). Initial high ATA levels were associated with vascular progression at one year (21.95 % vs. 0 %; P=0.0495). CONCLUSION: ATA levels are associated with skin sclerosis and vascular progression in SSc. Beyond the detection of ATA, quantifying this autoantibody might be of interest in predicting disease severity and prognosis in SSc.

Prevalence of radiographic findings in chronic osteomyelitis.

BACKGROUND: Simple radiography in conjunction with pertinent medical history and a comprehensive physical examination is typically adequate for diagnosing chronic osteomyelitis (CO). However, radiographic manifestations of CO lack specificity; therefore, the concordance among specialists in this regard has not been systematically assessed. This study aimed to compare and evaluate the proficiency of orthopedic surgeons and radiologists in identifying radiographic indicators present in simple radiographs for diagnosing CO. METHODS: This cross-sectional study was a correlational investigation utilizing plain radiographs obtained from a cohort of 60 patients diagnosed with CO. Comprehensive assessments of the demographic and clinical characteristics, comorbidities, and microbiological parameters were conducted. Additional variables included the anatomical location of the CO, existence of fistulas, disease duration, and presence of pseudoarthrosis. This study meticulously documented the presence or absence of six specific findings: bone destruction, which incorporates erosion and radiolucencies around implants; bone sclerosis; cortical thinning concomitant with erosion; cortical thickening; sequestrum formation; and soft-tissue swelling. RESULTS: Most patients were men (75%), with a mean age of 45.1 years. Hematogenous etiology of CO represented 23%. Bone sclerosis (71.3%) and cortical thickening (67.7%) were the most common radiographic findings, followed by soft-tissue swelling (51.3%), sequestration (47.3%), bone destruction (33.3%), and cortical erosion (30.3%). The mean agreement was 74.2%, showing a marked disagreement rate of 25.8% among all radiographic findings. The presence or absence of soft tissue edema, a prominent radiographic finding that was more important than the other findings, showed the greatest disagreement. CONCLUSIONS: Radiographic findings in CO were universally observed in all patients, demonstrating a high degree of concordance among specialists, with the exception of soft tissue swelling.

FIB-4 liver fibrosis index correlates with aortic valve sclerosis in non-alcoholic population.

AIM: Hepatic fibrosis, a progressive scarring of liver tissue, is commonly caused by non-alcoholic fatty liver disease (NAFLD), which increases the risk of cardiovascular disease. The Fibrosis-4 (FIB-4) index is a non-invasive tool used to assess liver fibrosis in patients with NAFLD. Aortic valve sclerosis (AVS), a degenerative disorder characterized by thickening and calcification of valve leaflets, is prevalent in the elderly and associated with increased cardiovascular morbidity and mortality. Recent studies have suggested that AVS may also be linked to other systemic diseases such as liver fibrosis. This study aimed to investigate the relationship between the FIB-4 index and AVS in a non-alcoholic population, with the hypothesis that the FIB-4 index could serve as a potential marker for AVS. METHOD: A total of 92 patients were included in this study. AVS was detected using transthoracic echocardiography, and patients were divided into groups according to the presence of AVS. The FIB-4 index was calculated for all patients and compared between the groups. RESULTS: A total of 17 (18.4%) patients were diagnosed AVS. Patients with AVS had higher rates of diabetes mellitus, older age, hypertension, angiotensin-converting enzyme inhibitor use, higher systolic blood pressure (BP) and diastolic BP in the office, coronary artery disease prevalence, left atrial volume index (LAVI), left ventricular mass index (LVMI), and late diastolic peak flow velocity (A) compared to those without AVS. Moreover, AVS patients had significantly higher creatinine levels and lower estimated glomerular filtration rate. Remarkably, the FIB-4 index was significantly higher in patients with AVS. In univariate and multivariate analyses, higher systolic BP in the office (OR, 1.044; 95% CI 1.002-1.080, p = .024) and higher FIB-4 index (1.46 ± .6 vs. .91 ± .46, p < .001) were independently associated with AVS. CONCLUSION: Our findings suggest that the FIB-4 index is associated with AVS in non-alcoholic individuals. Our results highlight the potential utility of the FIB-4 index as a non-invasive tool for identifying individuals at an increased risk of developing AVS.

Weekly intraperitoneal steroid infusion to treat recurrent ascites in a patient with early stage of peritoneal dialysis-related encapsulating peritoneal sclerosis: A case report.

Encapsulating peritoneal sclerosis (EPS) is a rare and potentially fatal complication of long-term peritoneal dialysis (PD). EPS-induced large volume and recurrent ascites represents a challenging condition. We report a 51-year-old man with kidney failure treated with PD for 13 years who eventually developed early stage of EPS accompanied with poor intake and recurrent ascites. After management including discontinuing PD and switching to haemodialysis, as well as oral steroids and tamoxifen administration, the patient had refractory ascites. An intervention of weekly intraperitoneal steroid infusion with methylprednisolone was implemented for a year. Gradually, we observed a reduction in ascites drainage, an improvement of clinical symptoms and the patient's nutritional status. The PD catheter was successfully removed as there was no recurrence of ascites. Intraperitoneal corticosteroid administration represents a new intervention for patients with early stage of EPS and recurrent ascites after PD cessation.

Sclerosis rim volume ratio and Japanese Investigation Committee classification as predictive factors for femoral head collapse progression after non-vascularized fibular grafting in osteonecrosis patients.

OBJECTIVE: The purpose of this retrospective cohort study was to determine the relationship between sclerosis rim volume ratio (SVR) and the progression of femoral head collapse after non-vascularized fibular grafting (NVFG) surgery in patients with osteonecrosis of the femoral head (ONFH), investigating risk factors associated with femoral head collapse progression and establishing a predictive model to enhance clinical decision-making. METHODS: ONFH patients who underwent NVFG between January 2008 and December 2021 were analyzed retrospectively to assess the risk of post-operative collapse progression (collapse >2 mm). A logistic regression model was used to evaluate the independent risk factors associated with collapse progression, including age, sex, etiology, affected side, Japanese Investigation Committee classification (JIC), and the sclerosis rim volume ratio (SVR). SVR values was collected from three weight-bearing columns, namely SVR1, SVR2, and SVR3, respectively. RESULTS: 57 patients with 64 hips who had undergone NVFG and were followed up for at least one year were included. During the follow-up, collapse＞2 mm occurred in 30 hips (46.88%). Multivariable analysis revealed that JIC (p =0.037) and SVR1 (p = 0.04) were independent risk factors for collapse progression after NVFG. The results of the receiver operating characteristic (ROC) analysis indicated that the aforementioned indices provided a satisfactory prediction of early femoral head collapse progression in ONFH patients after NVFG. The regression model using the above two indicators as a composite index showed satisfactory performance in predicting early postoperative femoral head collapse progression, with an area under the curve (AUC) of 84.6%. CONCLUSIONS: SVR is significant predictor of post-operative collapse progression following NVFG, and the composite index provides an optimal predictive value for femoral head collapse progression after surgery.

Prediction of surgical outcomes in severe encapsulating peritoneal sclerosis using a computed tomography scoring system.

BACKGROUND/PURPOSE: Encapsulating peritoneal sclerosis (EPS) is a rare and potential lethal complication of peritoneal dialysis characterized by bowel obstruction. Surgical enterolysis is the only curative therapy. Currently, there are no tools for predicting postsurgical prognosis. This study aimed to identify a computed tomography (CT) scoring system that could predict mortality after surgery in patients with severe EPS. METHODS: This retrospective study enrolled patients with severe EPS who underwent surgical enterolysis in a tertiary referral medical center. The association of CT score with surgical outcomes including mortality, blood loss, and bowel perforation was analyzed. RESULTS: Thirty-four patients who underwent 37 procedures were recruited and divided into a survivor and non-survivor group. The survivor group had higher body mass indices (BMIs, 18.1 vs. 16.7 kg/m2, p = 0.035) and lower CT scores (11 vs. 17, p < 0.001) than the non-survivor group. The receiver operating characteristic curve revealed that a CT score of ≥15 could be considered a cutoff point to predict surgical mortality, with an area under the curve of 0.93, sensitivity of 88.9%, and specificity of 82.1%. Compared with the group with CT scores of <15, the group with CT scores of ≥15 had a lower BMI (19.7 vs. 16.2 kg/m2, p = 0.004), higher mortality (4.2% vs. 61.5%, p < 0.001), greater blood loss (50 vs. 400 mL, p = 0.007), and higher incidence of bowel perforation (12.5% vs. 61.5%, p = 0.006). CONCLUSION: The CT scoring system could be useful in predicting surgical risk in patients with severe EPS receiving enterolysis.

Post-transplant recurrence of focal segmental glomerular sclerosis: consensus statements.

Focal segmental glomerular sclerosis (FSGS) is 1 of the primary causes of nephrotic syndrome in both pediatric and adult patients, which can lead to end-stage kidney disease. Recurrence of FSGS after kidney transplantation significantly increases allograft loss, leading to morbidity and mortality. Currently, there are no consensus guidelines for identifying those patients who are at risk for recurrence or for the management of recurrent FSGS. Our work group performed a literature search on PubMed/Medline, Embase, and Cochrane, and recommendations were proposed and graded for strength of evidence. Of the 614 initially identified studies, 221 were found suitable to formulate consensus guidelines for recurrent FSGS. These guidelines focus on the definition, epidemiology, risk factors, pathogenesis, and management of recurrent FSGS. We conclude that additional studies are required to strengthen the recommendations proposed in this review.

[Histological evaluation of esophageal stricture in children with caustic burn]. / Patogistologicheskaya kharakteristika striktury pishchevoda pri kausticheskom ozhoge u detei.

OBJECTIVE: To analyze histological features of esophageal strictures in children with chemical burn following ingestion of household products containing sodium (potassium) hypochlorite, sodium (potassium) hydroxide. MATERIAL AND METHODS: We analyzed 3 children with complicated caustic esophageal burns. Children at the time of swallowing the caustic were 26.3±4.1 months. Multiple dilatations of esophageal stricture were ineffective. Therefore, extirpation or subtotal resection of the esophagus and esophagocoloplasty were performed. We stained specimens using hematoxylin and eosin, as well as Masson's trichrome. RESULTS: Severe esophageal burns caused by sodium (potassium) hydroxide and/or sodium hypochlorite are followed by irreversible tissue lesions and non-dilatable stricture. Strictures are localized in the areas of physiological narrowing of the esophagus. The longest stricture follows ingestion of liquid substance. Histological properties include atrophy of glands and mucous membrane, muscle layer substitution by connective tissue and diffuse sclerosis of esophageal wall. CONCLUSION: Non-dilatable esophageal stricture following caustic burn in children is due to irreversible morphological lesion of esophageal wall with mucous layer atrophy and sclerosis of all layers.

[The use of a urethral catheter with an ultrasound-induced biopolymer drug coating for the prevention of recurrent bladder neck sclerosis in patients after endoscopic treatment of benign prostate hyperplasia].

Recurrent bladder neck sclerosis is one of the common complications of endoscopic treatment of benign prostate hyperplasia, which often leads to multiple re-operations, including complex open and laparoscopic reconstructive procedures. One of the most promising minimally invasive methods for preventing recurrence of bladder neck sclerosis is balloon dilatation under transrectal ultrasound guidance. To improve the results of using this technique, a urethral catheter with a biopolymer coating, capable of depositing a drug and eluting it under the influence of diagnostic ultrasound, was proposed.

Sclerosing encapsulating peritonitis causing fatal bowel obstruction.

Sclerosing encapsulating peritonitis (SEP) is a rare condition characterised by a fibrotic peritoneal membrane encasing loops of bowel often resulting in obstruction. We present a case of SEP complicated by non-resolving small bowel obstruction in the context of prior malignancies and surgical complications. A literature review on SEP was performed to outline potential aetiologies, diagnostic investigations and treatment strategies that may be considered in the management of this disease.

Hepatitis C-associated Osteosclerosis (HCAO): Long-Term Follow-Up of a New Case Recovered After Antiviral Treatment.

Hepatitis C-associated osteosclerosis (HCAO) is a very rare condition that can be observed in a small number of patients with Hepatitis C Virus (HCV) infection. HCAO is usually characterized by widespread bone sclerosis, associated with severe bone pain, and increased levels of bone turnover markers, especially alkaline phosphatase (ALP). In this report, we present the case of a 55-year-old woman who was affected by HCV and came to our attention for severe and diffuse bone pain. Radiological studies showed bone sclerosis, and bone mineral density (BMD) was markedly increased, as well as serum ALP levels. The patient was initially treated with intravenous pamidronate, which provided only a transient benefit on clinical symptoms. Then antiviral therapy for HCV (interferon-alfa and ribavirin) was started and it was effective in making the viral load undetectable. After a long follow-up period, we observed a persistent remission of bone pain, a reduction in BMD together with a progressive trend toward the normalization of bone turnover markers. In conclusion, HCAO, although rare, should be considered among the potential causes of increased bone mass in patients with HCV infection, and treatment for the underlying infection may be effective in controlling the manifestations of this disease.

Bilateral ictal EEG is associated with better memory outcome after hippocampal sclerosis surgery.

OBJECTIVE: To compare memory outcomes after surgery for unilateral hippocampal sclerosis (HS)-associated epilepsy in patients with unilateral and bilateral ictal electrographic involvement. METHODS: We prospectively evaluated HS patients, aged 18-55 years and IQ ≥70. Left (L) and right (R) surgical groups underwent noninvasive video-EEG monitoring and Wada test. We classified patients as Ipsilateral if ictal EEG was restricted to the HS side, or Bilateral, if at least one seizure onset occurred contralaterally to the HS, or if ictal discharge evolved to the opposite temporal region. Patients who declined surgery served as controls. Memory was evaluated on two occasions with Rey Auditory-Verbal Learning Test and Rey Visual-Design Learning Test. Baseline neuropsychological test scores were compared between groups. Pre- and postoperative scores were compared within each group. Reliable change index Z-scores (RCI) were obtained using controls as references, and compared between surgical groups. RESULTS: We evaluated 64 patients. Patients were classified as: L-Ipsilateral (9), L-Bilateral (15), L-Control (9), R-Ipsilateral (10), R-Bilateral (9), and R-Control (12). On preoperative evaluation, memory performance did not differ among surgical groups. Right HS patients did not present postoperative memory decline. L-Ipsilateral group presented postoperative decline on immediate (P = 0.036) and delayed verbal recall (P = 0.011), while L-Bilateral did not decline. L-Ipsilateral had lower RCI Z-scores, indicating delayed verbal memory decline compared to L-Bilateral (P = 0.012). SIGNIFICANCE: Dominant HS patients with bilateral ictal involvement presented less pronounced postoperative verbal memory decline compared to patients with exclusive ipsilateral ictal activity. Surgery was indicated in these patients regardless of memory impairment on neuropsychological testing, since resection of the left sclerotic hippocampus could result in cessation of contralateral epileptiform activity, and, therefore, improved memory function.

Postoperative seizure and memory outcome of temporal lobe epilepsy with hippocampal sclerosis: A systematic review.

We conducted a systematic review and meta-analysis to evaluate postoperative seizure and memory outcomes of temporal lobe epilepsy with different hippocampal sclerosis (HS) subtypes classified by International League Against Epilepsy (ILAE) Consensus Guidelines in 2013. Following the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) and MOOSE (Meta-Analysis of Observational Studies in Epidemiology) guidelines, we searched PubMed, Embase, Web of Science, and Cochrane Library from January 1, 2013 to August 6, 2023. Observational studies reporting seizure and memory outcomes among different HS subtypes were included. We used the Newcastle-Ottawa scale to assess the risk of bias and the Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach to grade the quality of evidence. Seizure freedom and improved outcome (Engel 1 or ILAE class 1-2) ≥1 year after surgery were defined as the primary and secondary seizure outcome. A random-effects meta-analysis by DerSimonian and Laird method was performed to obtain pooled risk ratio (RRs) with 95% confidence interval (CIs). The memory impairment was narratively reviewed because of various evaluation tools. Fifteen cohort studies with 2485 patients were eligible for the meta-analysis of seizure outcome. Six cohorts with detailed information on postoperative memory outcome were included. The pooled RRs of seizure freedom, with moderate to substantial heterogeneity, were .98 (95% CI = .84-1.15) between HS type 2 and type 1, 1.11 (95% CI = .82-1.52) between type 3 and type 1, and .80 (95% CI = .62-1.03) between the no-HS and HS groups. No significant difference of improved outcome was found between different subtypes (p > .05). The quality of evidence was deemed to be low to very low according to GRADE. The long-term seizure outcome (≥5 years after surgery) and memory impairment remained controversial.

[Functional morphology of the bladder and its vascular system in relation to age changes and evolution of BPH].

INTRODUCTION: The increase in the proportion of people with various urinary disorders, which are based on anatomical and functional bladder remodeling due to aging, is currently un-deniable. This problem becomes more relevant due to the elevation in life expectancy. At the same time, the features of bladder remodeling, in particular, the structural changes of its vascular bed, are still practically not described in the literature. In men, the lower urinary tract undergoes additional transformation associated with age due to bladder outlet obstruction caused by benign prostatic hyperplasia (BPH). Despite the long history of studying BPH, the morphological basics of its evolution have not yet been fully elucidated, including the development of lower urinary tract decompensation and, in particular, the role of vascular changes. In addition, structural re-modeling of the bladder muscles in BPH is formed in those with pre-existing age-related changes in both the detrusor and its vascular system, which cannot but influence the dynamics of disease progression. AIM: To study the structural changes of detrusor and its vascular bed associated with age, and to establish the role of their patterns in patients with BPH. MATERIALS AND METHODS: The material was a specimen of the bladder wall obtained dur-ing: a) autopsies of 35 men aged 60-80 years who died from diseases not related to urological or cardiovascular pathology; b) autopsies of 35 men aged 60-80 years who had BPH without blad-der decompensation; c) intraoperative biopsies of 25 men of the same age who undergone surgi-cal treatment for chronic urinary retention (postvoid residual volume of more than 300 ml), bilat-eral hydronephrosis, as complications of BPH. As a control, we used the specimens obtained from 20 males aged 20-30 years who died as a result of violence. Histological sections of the bladder wall were stained with hematoxylin-eosin, according to Mason and Hart. Standard microscopy and stereometry of detrusor structural components and morphometry of the urinary bladder vessels were performed using a special ocular insert with 100 equidistant points. During morphometric examination of the vascular bed the thickness of the middle layer of arteries wall (tunica media) was measured, as well as a thickness of the entire wall of the veins in microns. In addition, a Schiff test and Immunohistochemistry (IHC) of these histological sections were performed. The IHC was evaluated using a semi-quantitative method, taking into account the degree of staining in 10 fields of vision (200). The digital material was processed with the STATISTICA program using the Student's t-test. The distribution of the obtained data corresponded to normal. The data were considered re-liable if the probability of making error did not exceed 5% (p<0.05). RESULTS AND DISCUSSION: In the course of natural aging, a structural remodeling of bladder vascular bed was observed, from the development of atherosclerosis of extra-organ arteries to restructuring of intra-organ arteries due to arterial hypertension. The progression of angiopathy leads to the development of chronic detrusor ischemia, which initiates the formation of focal at-rophy of the smooth muscles, destructive changes in the elastic fibers, neurodegeneration and stroma sclerosis. A long-term BPH leads to compensatory detrusor remodeling with hypertrophy of previously unchanged areas. At the same time, age-related atrophic and sclerotic changes in smooth muscles are accompanied with hypertrophy of individual areas of the bladder detrusor. To maintain adequate blood supply to hypertrophied detrusor areas in the arterial and venous bladder vessels, a complex of myogenic structures is formed that can regulate blood circulation, making it dependent on the energy consumption of specific areas. However, progressive age-related changes in the arteries and veins eventually lead to an increase in chronic hypoxia, im-paired nervous regulation and vascular dystonia, increased blood vessels sclerosis and hyalinosis, and sclerosis of intravascular myogenic structures with loss of their function of blood flow regu-lation, as well as the development of vein thrombosis. As a result, increasing vascular decom-pensation in patients with bladder outlet obstruction results in bladder ischemia and accelerates the decompensation of the lower urinary tract.

Pathophysiology of encapsulating peritoneal sclerosis: lessons from findings of the past three decades in Japan.

Encapsulating peritoneal sclerosis (EPS), a condition with a high mortality rate, is a serious complication of peritoneal dialysis (PD). In Japan, EPS became a central issue in the clinical setting during the mid-90s and the beginning of this century. However, following the introduction of biocompatible neutral PD solutions containing lower levels of glucose degradation products, the incidence and clinical severity of EPS has been greatly lessened. During the past three decades, the etiology of EPS has been elucidated by findings obtained by peritoneal biopsy, laparoscopy, and surgical intervention. Accumulating findings suggest the need for a paradigm change on the nature of EPS pathophysiology; notably, EPS appears not to reflect peritoneal sclerosis per se, but rather the formation of a neo-membrane as a biological reaction to peritoneal injury. This narrative review looks back on the history of EPS in Japan, and discusses EPS pathophysiology, the impact of neutral PD solution on peritoneal protection, and a future novel diagnostic approach, ultra-fine endoscope, for the identification of patients at high risk of EPS.

Lumbosacral endplate contour defect is frequently observed concurrent with other lumbosacral abnormalities on spinal CT of French Bulldogs.

Lumbosacral osteochondrosis has been previously described in large breed dogs with variable clinical signs. Its CT features are a contour defect at the dorsal aspect of either vertebral endplate, commonly with an adjacent fragment. Descriptions of this condition have not been previously published in an increasingly popular breed, French Bulldogs. Aims of this retrospective, descriptive, single center study were to evaluate CT lumbosacral abnormalities and the frequency of lumbosacral endplate contour defect in a large sample of French Bulldogs. The presence and location of lumbosacral endplate contour defect and the presence of a concurrent osseous fragment were recorded. Other abnormal CT findings such as L7-S1 disc herniation, cauda equina nerve roots compression or thickening, disc mineralization, endplate sclerosis, spondylosis deformans, S1 articular processes hypertrophy, transitional vertebrae, hemivertebrae, spina bifida, and block vertebrae were recorded. Lumbosacral CT abnormalities were present in 91.8% of dogs (168/183). The most common abnormality was an L7-S1 dorsal disc herniation (77.4%, 130/168). Among dogs with lumbosacral abnormalities, the frequency of a lumbosacral endplate contour defect was 47% (79/168). It mostly involved L7 (78.5%, 62/79) at its dorsolateral aspect (61.3%, 38/62). A mineralized fragment was identified in 62% of the defects (49/79). Endplate contour defects were most frequently seen with a concurrent disc herniation (93.7%, 74/79) causing nerve root compression in 63.3% (50/79) and with sclerosis (65.8%, 52/79). There was no definitive evidence of a relationship with clinical presentation in this sample of French Bulldogs; therefore, this finding should be interpreted with caution. The etiology remains unclear.

Humeral intracondylar fissures and intracondylar sclerosis are common CT findings in the limb contralateral to a humeral condylar fracture in French bulldogs and spaniel breeds.

French bulldogs (FBD) are reported to be at an increased risk of developing humeral condylar fractures (HCF). Spaniel breeds have been reported to be at increased risk of HCF due to a high prevalence of humeral intracondylar fissures (HIF), which can predispose to fracture. The aims of this retrospective, single center, observational study were twofold. First, to compare the signalment, fracture configuration, and etiology of populations of FBD and spaniel breeds presenting with HCF at a single hospital. Second, to evaluate the computed tomographic (CT) findings of the contralateral elbow joints of FBD and spaniel breeds presenting with HCF for concomitant pathology. FBDs were overrepresented for HCF, most commonly presenting when skeletally immature, with a median age of 3.7 months. Fractures of the lateral condyle were the most common configuration for both breed groups. Sclerosis at the fracture site was identified in 62% of FBD and 95% of spaniels undergoing CT scans. Humeral intracondylar fissures and intracondylar sclerosis were commonly observed in the contralateral elbows of FBD (44% and 50%, respectively) and spaniel breeds (58% and 95%, respectively). Medial coronoid pathology, elbow incongruency, and periarticular osteophytes were not identified in the contralateral elbows of FBD but were identified in 29%, 4%, and 20% of spaniels, respectively. The high prevalence of HIF in the FBD group may be a predisposing factor for HCF in this breed.

Effect of Nonimmune Factors on Renal Prognosis in Adult IgA Vasculitis With Nephritis: A Long-Term Retrospective Cohort Study.

OBJECTIVE: Adult immunoglobulin A vasculitis (IgAV) is documented to be associated with more renal involvement and poorer renal outcomes compared to children, but adult IgAV nephritis (IgAV-N) data are rather limited. The present study aimed to describe the characteristics of adult IgAV-N and investigate the long-term prognostic factors. METHODS: Clinical and morphological data from 106 adult patients with biopsy-proven IgAV-N and follow-up data from 94 patients in a single Chinese center were analyzed in this retrospective study. Median follow-up time interval was 102 months. RESULTS: The median age of patients with IgAV-N at biopsy was 38 (IQR 24-53) years, and 52.8% were male. The median blood pressure was 126/80 mmHg, and 25.5% of patients were hypertensive at baseline. The median initial proteinuria was 1.4 (IQR 0.7-2.2) g/day and estimated glomerular filtration rate (eGFR) was 103 (IQR 84-121) mL/min/1.73 m2. The median time interval of onset to biopsy was 8 (IQR 3-40) weeks. In biopsy, the median percentage of global sclerosis was 5.9% (IQR 0.0-13.8), whereas 45.3% of patients had interstitial fibrosis and tubular atrophy. Further, during follow-up, 7.4% patients died, 4.3% patients progressed to endstage kidney disease (ESKD), and 6.4% patients developed > 30% eGFR reduction from baseline. Multivariate Cox proportional analyses revealed hypertension (HTN) history and > 10% global sclerosis at presentation were independent prognostic factors for poor outcome. CONCLUSION: The present adult IgAV-N cohort revealed a relatively young onset age, and lower incidence of nephrotic syndrome and ESKD. Moreover, nonimmune factors such as history of HTN and renal chronic histological lesions in biopsy played a crucial role in prognosis of IgAV-N.

Interrater reproducibility of the Myoton and durometer devices to quantify sclerotic chronic graft-versus-host disease.

Chronic graft-versus-host disease (cGVHD) is a severe complication in long-term survivors of allogeneic hematopoietic stem cell transplantation. This disease is challenging to manage clinically due to a lack of validated tools to quantitatively measure skin sclerosis. The current gold standard for measuring skin sclerosis is the NIH Skin Score which has only moderate agreement among clinicians and experts. To more accurately assess skin sclerosis in cGVHD, the Myoton and durometer devices can be used to directly measure biomechanical parameters of the skin. However, the reproducibility of these devices is not known in patients with cGVHD. To determine this reproducibility, three observers independently measured 10 anatomic sites in each of seven patients with sclerotic cGVHD using the Myoton and durometer. Clinical reproducibility was measured by mean pairwise differences (U-statistic) and intraclass correlation coefficients (ICCs) with 95% confidence intervals (CIs). Mean pairwise differences, expressed in true physical units, were used to report typical errors for each anatomic site and device. Mean pairwise differences were less than 11% of the average overall values for all five Myoton parameters and durometer hardness. These were lower for Myoton creep (4.1%), relaxation time (4.7%), and frequency (5.1%) than decrement (9.0%), stiffness (10.4%), and durometer hardness (9.0%). Myoton parameters creep, relaxation time, and frequency showed promise for capturing skin biomechanics more accurately than Myoton stiffness, decrement, or durometer hardness. Mean pairwise differences trended highest in the shin and volar forearm and lowest in the dorsal forearm. The interobserver ICC for overall (averaged across all measured body sites of a patient) creep (0.94; 95% CI 0.87-1.00), relaxation time (0.96; 95% CI 0.90-1.00), and frequency (0.95; 95% CI 0.88-1.00), trended higher than that for decrement (0.43; 95% CI 0.00-0.88), stiffness (0.92; 95% CI 0.81-1.00), and durometer hardness (0.82; 95% CI 0.61-1.00). Similar trends were observed in healthy participants. These findings can help clinicians design better studies to assess therapeutic response to new cGVHD treatments and support the interpretation of future measurements.