Clinical Presentation, Pathological Spectrum, and Outcomes of Alcoholic Cirrhosis-Related Immunoglobulin A Nephropathy.

Introduction: Immunoglobulin A nephropathy (IgAN) associated with cirrhosis is frequent but often overlooked because it is largely considered silent. Until now, little has been known about their presentation and outcomes. Methods: We conducted a retrospective multicenter study on patients with kidney biopsy-proven cirrhosis-related IgAN (cirrhosis-IgAN), diagnosed between 2009 and 2022. We mixed them up with 83 primary IgAN (pIgAN) diagnosed during the same period, using a partitioning clustering approach, to determine common clinicopathological profiles. Results: All the 46 patients with cirrhosis-IgAN had an excessive alcoholic consumption. Clinical presentation was severe with acute kidney injury (AKI) in 79%; alternative causes of AKI was found in 62% of cases. Three clinicopathological clusters were identified as follows: the first one represented chronic involvement, the second one could be assimilated to mild disease, and the third one corresponded to a membranoproliferative glomerulonephritis (MPGN) pattern and was associated with heavy proteinuria and intrinsic AKI (without alternative causes). Whereas the first 2 clusters were equally distributed between pIgAN and cirrhosis-IgAN, the third was more frequent in patients with cirrhosis. The cumulative mortality rate in cirrhosis-IgAN was 26% and 46% at 1-year and 3-years, respectively. Steroid exposure and moderate or severe AKI were associated with higher mortality and steroid exposure was associated with the occurrence of severe infection. Conclusion: Our results suggest that high AKI incidence is related to extrinsic causes in most cases but can also be driven by IgA-dominant MPGN in a subset of patients. Steroid use was associated with infectious disease and mortality. Further studies are needed to clarify the role of immunosuppressive treatment in cirrhosis-IgAN patients.

Prophylactic treatment of FSGS recurrence in patients who relapsed in a previous kidney graft.

BACKGROUND AND HYPOTHESIS: Recurrence of focal segmental glomerulosclerosis (FSGS) is common after kidney transplantation and is classically associated with a significant decrease in graft survival. A major risk factor is a prior history of FSGS recurrence on a previous graft. This analysis reports the impact of a prophylactic treatment of FSGS recurrence in very high-risk patients who experienced a recurrence on a previous graft. METHODS: We performed a retrospective multicentre observational study in 25 French transplantation centres. The inclusion criteria were patients aged more than 18 years who had undergone kidney transplant between December 31, 2004, and December 31, 2020, and who had a history of FSGS recurrence on a previous graft. RESULTS: We identified 66 patients: 40 received prophylactic treatment (PT+), including intravenous cyclosporine and/or rituximab and/or plasmapheresis, and 26 did not receive any prophylactic treatment (PT-). The time to progression to end-stage kidney disease was similar between groups. The PT + group was younger at FSGS diagnosis and at the time of kidney retransplantation and lost their previous graft faster. The overall recurrence rate was 72.7% (76.9% in the PT- group and 70.0% in the PT + group, P = 0.54). At least partial remission was achieved in 87.5% of patients. The 5-year graft survival was 67.7% (95% CI: 53.4 to 78.4%): 65.1% (95%CI: 48.7 to 77.4%) in patients with FSGS recurrence vs. 77.3% (95% CI: 43.8 to 92.3%) in patients without recurrence (P = 0.48). CONCLUSION: Our study suggests that prophylactic treatment should not be used routinely in patients receiving a second transplantation after recurrence of FSGS on a previous graft. The recurrence rate is high regardless of the use of prophylactic treatment. However, the 5-year graft survival remains satisfactory.

KiT-GENIE, the French genetic biobank of kidney transplantation.

KiT-GENIE is a monocentric DNA biobank set up to consolidate the very rich and homogeneous DIVAT French cohort of kidney donors and recipients (D/R) in order to explore the molecular factors involved in kidney transplantation outcomes. We collected DNA samples for kidney transplantations performed in Nantes, and we leveraged GWAS genotyping data for securing high-quality genetic data with deep SNP and HLA annotations through imputations and for inferring D/R genetic ancestry. Overall, the biobank included 4217 individuals (n = 1945 D + 2,272 R, including 1969 D/R pairs), 7.4 M SNPs and over 200 clinical variables. KiT-GENIE represents an accurate snapshot of kidney transplantation clinical practice in Nantes between 2002 and 2018, with an enrichment in living kidney donors (17%) and recipients with focal segmental glomerulosclerosis (4%). Recipients were predominantly male (63%), of European ancestry (93%), with a mean age of 51yo and 86% experienced their first graft over the study period. D/R pairs were 93% from European ancestry, and 95% pairs exhibited at least one HLA allelic mismatch. The mean follow-up time was 6.7 years with a hindsight up to 25 years. Recipients experienced biopsy-proven rejection and graft loss for 16.6% and 21.3%, respectively. KiT-GENIE constitutes one of the largest kidney transplantation genetic cohorts worldwide to date. It includes homogeneous high-quality clinical and genetic data for donors and recipients, hence offering a unique opportunity to investigate immunogenetic and genetic factors, as well as donor-recipient interactions and mismatches involved in rejection, graft survival, primary disease recurrence and other comorbidities.

Surgery of abdominal wall endometriosis associated with clear-cell carcinoma: Case report and review.

Abdominal wall is a rare location for endometriosis, with a reported incidence of parietal endometriosis of approximately 0.03 to 0.4%. It most often occurs in the aftermath of a caesarean section and is associated with pelvic endometriosis in only 5 to 15% of cases. Rare cases of malignant transformation have been described, mainly in the form of clear-cell tumours. We report the case of a 52-year-old patient with a history of endometriosis who presented with a retractile parietal mass at the level of her caesarean scar. Histological analysis confirmed a clear-cell adenocarcinoma (CCC). Few cases of endometriosis - associated CCC are described in the literature. A review of the literature suggests radical surgical treatment combined with adjuvant radio-chemotherapy. However, the prognosis is poor. The aim of this case report is to suggest the diagnosis of malignant transformation in the presence of a rapidly evolving parietal mass in the context of endometriosis and a history of caesarean section.

The Joel-Cohen laparotomy: A dilapidating access source of wide and complex suprapubic incisional hernia.

PURPOSE: This retrospective study aims to describe morphological and therapeutic peculiarities of the suprapubic incisional hernia (SIH) encountered after a Joël-Cohen laparotomy. PATIENTS AND METHOD: Serie-report: 9 patients had an SIH, 2 were sub-umbilical and did not concern the suprapubic scar, 3 were central, 2 on the whole length of the suprapubic scar, and 2 were bilateral in one case associated to a sub-umbilical incisional hernia. RESULTS: SIH were wide openings, with a hernial fascia constituted from the anterior fascia, without connexion with the parietal peritoneum, in a sub-umbilical position above the suprapubic scar, or through the suprapubic scar. Rectus muscle was ruptured or sclerosed. There were 2 distinct defects, an anterior one through the anterior fascia, and a posterior one between the rectus muscles. The parietal peritoneum was retracted leaving bare the posterior side of the rectus muscles. There was an interstitial retro-fascial space, so the SIH was bisaccular. When releasing the parietal peritoneum was not feasible, the prosthesis was placed in a retro-fascial space. When the parietal peritoneum was released, the prosthesis was placed in a preperitoneal space. The anterior defect closure was not always completely feasible, fulfilled with a Vicryl prosthesis. One patient presents an abdominal wall bulging in case of efforts. CONCLUSION: SIH after a Joël-Cohen laparotomy is wide and dilapidating. The cure is difficult. This technique should be reserved to real emergency obstetrical procedure. We highlight the importance of the parietal peritoneum closure after gynecological or obstetric surgery.

[Genetic screening is essential in polycystic kidney disease: It is never too late!] / Une polykystose peut en cacher une autre. Il n'est jamais trop tard pour l'analyse génétique !

In France, numerous patients suffered from chronic kidney disease on polycystic kidney disorder. If PKD1 and PKD2 inactivating mutations are the most prevalent, several other genetic polycystic kidney diseases are responsible for similar kidney features and may be associated with severe extrarenal phenotypes. Genetic analysis in front of a polycystic disorder is not systematic, but is essential to assess the genetic diagnosis, discuss the intensity of treatment (vaptan) and precise the prognostic and the transmission of the phenotype. We detailed the case of a patient with end stage renal disease due to a polycystic kidney disease. Genetic analysis at 70 year of age revealed an oral-facial-digital syndrome type 1. The diagnosis had an important impact in the familial history and to attach the extrarenal phenotype to the syndrome. Our case illustrates that, in front of a polycystic kidney disease (even in aged patients with end stage renal disease) genetic screening is essential, for the propositus and their family and to take care of the extrarenal manifestations.

Novel calcium phosphate/PCL graded samples: Design and development in view of biomedical applications.

Scaffolds for bone tissue engineering require a combination of bioactivity and bioresorption at the sample surface and high mechanical properties in the bulk. This work presents a novel calcium phosphate (CaP)/polycaprolactone (PCL) scaffold with graded composition and porosity fraction. The scaffold is made of (i) a dense hydroxyapatite (HA)/ß-tricalcium phosphate (ß-TCP) core, (ii) a macroporous HA/ß-TCP transition layer and (iii) a macroporous PCL/(HA/ß-TCP) external layer. The ceramic layers were fabricated by gel-casting whereas the outer composite layer was obtained by a solvent casting/particle leaching process. The microstructure, phase composition and biodegradation of the scaffolds were characterized. The gradient of porosity was clearly obtained whereas the gradation of phase composition was less pronounced. An in vitro dissolution test was performed by immersing the scaffolds in a TRIS solution. The results showed a dissolution phenomenon with possible differentiated mechanisms in the different layers, in relation with the targeted multi-functionality.

A review of recent advances in data analytics for post-operative patient deterioration detection.

Most deaths occurring due to a surgical intervention happen postoperatively rather than during surgery. The current standard of care in many hospitals cannot fully cope with detecting and addressing post-surgical deterioration in time. For millions of patients, this deterioration is left unnoticed, leading to increased mortality and morbidity. Postoperative deterioration detection currently relies on general scores that are not fully able to cater for the complex post-operative physiology of surgical patients. In the last decade however, advanced risk and warning scoring techniques have started to show encouraging results in terms of using the large amount of data available peri-operatively to improve postoperative deterioration detection. Relevant literature has been carefully surveyed to provide a summary of the most promising approaches as well as how they have been deployed in the perioperative domain. This work also aims to highlight the opportunities that lie in personalizing the models developed for patient deterioration for these particular post-surgical patients and make the output more actionable. The integration of pre- and intra-operative data, e.g. comorbidities, vitals, lab data, and information about the procedure performed, in post-operative early warning algorithms would lead to more contextualized, personalized, and adaptive patient modelling. This, combined with careful integration in the clinical workflow, would result in improved clinical decision support and better post-surgical care outcomes.

Limits of the Epworth Sleepiness Scale in older adults.

PURPOSE: Excessive daytime sleepiness (EDS) in older adults is associated with obstructive sleep apnea, falls, reduced quality of life, and mortality. The Epworth Sleepiness Scale (ESS) is widely used to assess sleepiness. However, EDS assessment with the ESS may not be accurate in older adults. We aimed to (1) describe the responsiveness of nondemented older subjects to the ESS and (2) compare the self-report ESS scores to those of close relatives (CR) proxy and identify factors influencing any discrepancies between them. METHODS: This is a cross-sectional observational study including 104 independently living nondemented older subjects with daytime sleepiness complaints and 104 nondemented CRs. Cognitive tests (Mini-Mental State Examination) and the ESS were completed separately by subjects and CRs to assess the subject's daytime sleepiness. RESULTS: Almost 60 % of subjects and CRs were not able to answer at least one question on the ESS. Despite the fact that all subjects complained of EDS, only 24 % of them had an abnormal ESS score (>10). Subjects rated their sleepiness lower (7.10 ± 4.31) than their CR proxy did (9.70 ± 5.14) (p < 0.0001). In multivariate analysis, an increase in age and a decrease in cognitive status of the subjects appeared related to the difference in ESS between subject and CR. CONCLUSIONS: The majority of older adults were not able to answer all of the ESS items. The ESS may underestimate sleepiness severity in older subjects. Despite EDS complaints in all subjects, only one quarter of them had a pathological ESS score.

Kawasaki disease in adults: report of 10 cases.

Kawasaki disease (KD) is an acute multisystemic vasculitis occurring predominantly in children and rarely in adults. Diagnosis is made clinically using diagnostic guidelines; no specific test is available. "Incomplete" KD is a more recent concept, which refers to patients with fever lasting > or =5 days and 2 or 3 clinical criteria (rash, conjunctivitis, oral mucosal changes, changes of extremities, adenopathy), without reasonable explanation for the illness. To describe the clinical and laboratory features of classical (or "complete") KD, and incomplete KD in adults, we report 10 cases of adult KD, including 6 patients who fulfilled the criteria for incomplete KD, diagnosed either at presentation (n = 4) or retrospectively (n = 2). At the time of clinical presentation, complete KD was diagnosed in 4 patients, while 4 patients fulfilled the criteria for incomplete KD. For 3 of the 4 patients with incomplete KD, presence of severe inflammation, laboratory findings (hypoalbuminemia, anemia, elevation of alanine aminotransferase, thrombocytosis after 7 days, white blood cell count > or =15,000/mm, and urine > or =10 white blood cell/high power field), or echocardiogram findings were consistent with the diagnosis. In 2 patients, the diagnosis of KD was made retrospectively in the presence of myocardial infarction due to coronary aneurysms, after an undiagnosed medical history evocative of incomplete KD. Seven patients received intravenous immunoglobulins (IVIG), after a mean delay of 12.5 days, which appeared to shorten the course of the disease. This relatively large series of adult KD highlights the existence of incomplete KD in adults and suggests that the algorithm proposed by a multidisciplinary committee of experts to diagnose incomplete KD in children could be useful in adults. Further studies are needed to determinate whether prompt IVIG may avoid artery sequelae in adult patients with complete or incomplete KD.