[Guideline for the diagnosis and management of multiple myeloma-related renal impairment (2024 version)].

Renal impairment is a common complication of multiple myeloma (MM). All patients with MM should be assessed for the presence and severity of renal impairment. The clinicopathological manifestations of MM-related renal impairment are diverse and complex; accordingly, except for light-chain nephropathy, which can often be diagnosed without biopsy based solely on clinical criteria, a renal biopsy is needed for an accurate diagnosis. Supportive care, such as adequate hydration, is required for all patients with MM-related renal impairment. The guideline provide the principles for dose adjustment of the drugs used for MM with renal impairment, including proteasome inhibitors, immunomodulators, monoclonal antibodies, small molecule inhibitors, and alkylating agents, as well as those used for myeloma bone disease. Autologous stem cell transplantation (ASCT) and chimeric antigen receptor T-cell immunotherapy (CAR-T) are effective in patients with moderate renal impairment and are tolerated by the patients. The Chinese Hematology Association; the Chinese Geriatrics Association, Society of Hematology; and the Chinese Research Hospital Association, Society of Nephrology asked experts to collate information on current progress in clinical research relating to MM with renal impairment. This guideline was developed based on the gathered data combined with the latest international consensus and clinical practice guidelines.

Difference Between Cardiopulmonary Bypass Time and Aortic Cross-Clamping Time as a Predictor of Complications After Coronary Artery Bypass Grafting.

INTRODUCTION: Along with cardiopulmonary bypass time, aortic cross-clamping time is directly related to the risk of complications after heart surgery. The influence of the time difference between cardiopulmonary bypass and cross-clamping times (TDC-C) remains poorly understood. OBJECTIVE: To assess the impact of cardiopulmonary bypass time in relation to cross-clamping time on immediate results after coronary artery bypass grafting in the Registro Paulista de Cirurgia Cardiovascular (REPLICCAR) II. METHODS: Analysis of 3,090 patients included in REPLICCAR II database was performed. The Society of Thoracic Surgeons outcomes were evaluated (mortality, kidney failure, deep wound infection, reoperation, cerebrovascular accident, and prolonged ventilation time). A cutoff point was adopted, from which the increase of this difference would affect each outcome. RESULTS: After a cutoff point determination, all patients were divided into Group 1 (cardiopulmonary bypass time < 140 min., TDC-C < 30 min.), Group 2 (cardiopulmonary bypass time < 140 min., TDC-C > 30 min.), Group 3 (cardiopulmonary bypass time > 140 min., TDC-C < 30 min.), and Group 4 (cardiopulmonary bypass time > 140 min., TDC-C > 30 min.). After univariate logistic regression, Group 2 showed significant association with reoperation (odds ratio: 1.64, 95% confidence interval: 1.01-2.66), stroke (odds ratio: 3.85, 95% confidence interval: 1.99-7.63), kidney failure (odds ratio: 1.90, 95% confidence interval: 1.32-2.74), and in-hospital mortality (odds ratio: 2.17, 95% confidence interval: 1.30-3.60). CONCLUSION: TDC-C serves as a predictive factor for complications following coronary artery bypass grafting. We strongly recommend that future studies incorporate this metric to improve the prediction of complications.

Oxalate nephropathy and chronic turmeric supplementation: a case report.

We present a case of a 69-year-old man who presented for routine check-up and was incidentally found to have kidney failure with an initially unrevealing history and bland urinary sediment. He was diagnosed with oxalate nephropathy in the setting of chronic turmeric supplementation and chronic antibiotic therapy with associated diarrhea. Our case provides several key insights into oxalate nephropathy. First, the diagnosis requires a high index of clinical suspicion. It is uncommonly suspected clinically unless there is an obvious clue in the history such as Roux-en-Y gastric bypass or ethylene glycol poisoning. Diagnosis can be confirmed by histopathologic findings and corroborated by serum levels of oxalate and 24-hour urinary excretion. Second, the diagnosis can often be missed by the pathologist because of the characteristics of the crystals unless the renal pathologist has made it a rule to examine routinely all H&E sections under polarized light. This must be done on H&E, as the other stains dissolve the crystals. Third, one oxalate crystal in a routine needle biopsy is considered pathologic and potentially contributing to the AKI or to the CKD in an important way. Fourth, secondary oxalosis can be largely mitigated or prevented in many cases, especially iatrogenic cases. This can come through the surgeon or the gastroenterologist providing proper instructions to patients on an oxalate-restricted diet or other specific dietary measures. Lastly, this case highlights the success that results from cooperation and communication between the pathologist and the treating physician.

Impacto de la edad, las comorbilidades y las complicaciones en pacientes con COVID-19 con síndrome de dificultad respiratoria aguda en la ventilación mecánica invasiva. Estudio multicéntrico observacional / Impact of Age, Comorbidities and Complications in COVID-19 Patients with Acute Respiratory Distress Syndrome on Invasive Mechanical Ventilation. An Observational Multicenter Study

Objetivos: Millones de pacientes con COVID-19 fueron internados en terapia intensiva en el mundo, la mitad desarrollaron síndrome de dificultad respiratoria aguda (SDRA) y recibieron ventilación mecánica invasiva (VMI), con una mortalidad del 50%. Analiza-mos cómo edad, comorbilidades y complicaciones, en pacientes con COVID-19 y SDRA que recibieron VMI, se asociaron con el riesgo de morir durante su hospitalización.Métodos: Estudio de cohorte observacional, retrospectivo y multicéntrico realizado en 5 hospitales (tres privados y dos públicos universitarios) de Argentina y Chile, durante el segundo semestre de 2020.Se incluyeron pacientes >18 años con infección por SARS-CoV-2 confirmada RT-PCR, que desarrollaron SDRA y fueron asistidos con VMI durante >48 horas, durante el se-gundo semestre de 2020. Se analizaron los antecedentes, las comorbilidades más fre-cuentes (obesidad, diabetes e hipertensión), y las complicaciones shock, insuficiencia renal aguda (IRA) y neumonía asociada a la ventilación mecánica (NAV), por un lado, y las alteraciones de parámetros clínicos y de laboratorio registrados.Resultados: El 69% era varón. La incidencia de comorbilidades difirió para los diferentes grupos de edad. La mortalidad aumentó significativamente con la edad (p<0,00001). Las comorbilidades, hipertensión y diabetes, y las complicaciones de IRA y shock se asociaron significativamente con la mortalidad. En el análisis multivariado, sólo la edad mayor de 60 años, la IRA y el shock permanecieron asociados con la mortalidad. Conclusiones: El SDRA en COVID-19 es más común entre los mayores. Solo la edad >60 años, el shock y la IRA se asociaron a la mortalidad en el análisis multivariado.

Objectives: Millions of patients with COVID-19 were admitted to intensive care world-wide, half developed acute respiratory distress syndrome (ARDS) and received invasive mechanical ventilation (IMV), with a mortality of 50%. We analyzed how age, comor-bidities and complications in patients with COVID-19 and ARDS who received IMV were associated with the risk of dying during their hospitalization.Methods: Observational, retrospective and multicenter cohort study carried out in 5 hospitals (three private and two public university hospitals) in Argentina and Chile, during the second half of 2020.Patients >18 years of age with SARS-CoV-2 infection confirmed by RT-PCR, who devel-oped ARDS and were assisted with IMV for >48 hours, during the second half of 2020, were included. History, the most frequent comorbidities (obesity, diabetes and hyper-tension) and the complications of shock, acute renal failure (AKI) and pneumonia as-sociated with mechanical ventilation (VAP), on the one hand, and the alterations of re-corded clinical and laboratory parameters, were analyzed.Results: 69% were men. The incidence of comorbidities differed for different age groups. Mortality increased significantly with age (p<0.00001). Comorbidities, hyper-tension and diabetes, and complications of ARF and shock were significantly associat-ed with mortality. In the multivariate analysis, only age over 60 years, ARF and shock remained associated with mortality.Conclusions: ARDS in COVID-19 is more common among the elderly. Only age >60 years, shock and ARF were associated with mortality in the multivariate analysis

Intrinsic ureteric obstruction secondary to endometriosis: a rare clinical entity causing obstructive uropathy and renal failure.

Ureteral endometriosis is rare and can be a silent clinical entity, which can potentially lead to serious complications such as obstructive uropathy, sepsis and renal failure. A high clinical suspicion is required especially in childbearing age groups due to non-specific presentation such as renal colic, recurrent urinary tract infection (UTI), renal failure or asymptomatic hydronephrosis.A woman in her 40s presented with febrile UTI and flank pain. She reportedly suffered from recurrent UTIs in the past. Initial workup revealed an infected, obstructed left renal collecting system with gross hydronephrosis and hydroureter to the distal ureter on a significant gynaecological background of severe endometriosis requiring hysterectomy in the past.CT showed chronic obstructive changes and soft tissue nodules within the renal pelvis with no radio-opaque stones. She underwent emergent ureteric stent insertion. Functional imaging demonstrated only 1% contribution of the left kidney with a preserved estimated glomerular filtration rate of 65 mL/min/1.73 m2Endoscopic evaluation of ureters found extensive soft tissue lesions throughout the dilated left collecting system with biopsy-confirmed endometriosis. Subsequently, she underwent laparoscopic nephroureterectomy due to extensive ureteric involvement and chronically obstructed non-functioning kidney. Histopathology demonstrated completely obstructing ureteral endometriosis.Ureteric obstruction secondary to endometriosis can be due to extrinsic or intrinsic disease. In addition to initial assessment with CT urogram MRI may be helpful to evaluate soft tissue thickening. Endoscopic assessment with ureteroscopy and biopsy is required for tissue diagnosis. Surgery is often the treatment of choice, ranging from ureteroureterostomy, ureteroneocystostomy or nephroureterectomy in severe cases.Ureteral endometriosis is a rare clinical entity, clinicians should remain vigilant about common presentations of this rare entity, early diagnosis and prompt treatment is crucial to prevent progression to renal failure.

Health outcomes after myocardial infarction: A population study of 56 million people in England.

BACKGROUND: The occurrence of a range of health outcomes following myocardial infarction (MI) is unknown. Therefore, this study aimed to determine the long-term risk of major health outcomes following MI and generate sociodemographic stratified risk charts in order to inform care recommendations in the post-MI period and underpin shared decision making. METHODS AND FINDINGS: This nationwide cohort study includes all individuals aged ≥18 years admitted to one of 229 National Health Service (NHS) Trusts in England between 1 January 2008 and 31 January 2017 (final follow-up 27 March 2017). We analysed 11 non-fatal health outcomes (subsequent MI and first hospitalisation for heart failure, atrial fibrillation, cerebrovascular disease, peripheral arterial disease, severe bleeding, renal failure, diabetes mellitus, dementia, depression, and cancer) and all-cause mortality. Of the 55,619,430 population of England, 34,116,257 individuals contributing to 145,912,852 hospitalisations were included (mean age 41.7 years (standard deviation [SD 26.1]); n = 14,747,198 (44.2%) male). There were 433,361 individuals with MI (mean age 67.4 years [SD 14.4)]; n = 283,742 (65.5%) male). Following MI, all-cause mortality was the most frequent event (adjusted cumulative incidence at 9 years 37.8% (95% confidence interval [CI] [37.6,37.9]), followed by heart failure (29.6%; 95% CI [29.4,29.7]), renal failure (27.2%; 95% CI [27.0,27.4]), atrial fibrillation (22.3%; 95% CI [22.2,22.5]), severe bleeding (19.0%; 95% CI [18.8,19.1]), diabetes (17.0%; 95% CI [16.9,17.1]), cancer (13.5%; 95% CI [13.3,13.6]), cerebrovascular disease (12.5%; 95% CI [12.4,12.7]), depression (8.9%; 95% CI [8.7,9.0]), dementia (7.8%; 95% CI [7.7,7.9]), subsequent MI (7.1%; 95% CI [7.0,7.2]), and peripheral arterial disease (6.5%; 95% CI [6.4,6.6]). Compared with a risk-set matched population of 2,001,310 individuals, first hospitalisation of all non-fatal health outcomes were increased after MI, except for dementia (adjusted hazard ratio [aHR] 1.01; 95% CI [0.99,1.02];p = 0.468) and cancer (aHR 0.56; 95% CI [0.56,0.57];p < 0.001). The study includes data from secondary care only-as such diagnoses made outside of secondary care may have been missed leading to the potential underestimation of the total burden of disease following MI. CONCLUSIONS: In this study, up to a third of patients with MI developed heart failure or renal failure, 7% had another MI, and 38% died within 9 years (compared with 35% deaths among matched individuals). The incidence of all health outcomes, except dementia and cancer, was higher than expected during the normal life course without MI following adjustment for age, sex, year, and socioeconomic deprivation. Efforts targeted to prevent or limit the accrual of chronic, multisystem disease states following MI are needed and should be guided by the demographic-specific risk charts derived in this study.

Efficacy of fresh frozen plasma transfusion in decompensated cirrhosis patients with coagulopathy admitted to ICU: a retrospective cohort study from MIMIC-IV database.

We aimed to explore the association between FFP transfusion and outcomes of DC patients with significant coagulopathy. A total of 693 DC patients with significant coagulopathy were analyzed with 233 patients per group after propensity score matching (PSM). Patients who received FFP transfusion were matched with those receiving conventional therapy via PSM. Regression analysis showed FFP transfusion had no benefit in 30-day (HR: 1.08, 95% CI 0.83-1.4), 90-day (HR: 1.03, 95% CI 0.80-1.31) and in-hospital(HR: 1.30, 95% CI 0.90-1.89) mortality, associated with increased risk of liver failure (OR: 3.00, 95% CI 1.78-5.07), kidney failure (OR: 1.90, 95% CI 1.13-3.18), coagulation failure (OR: 2.55, 95% CI 1.52-4.27), respiratory failure (OR: 1.76, 95% CI 1.15-2.69), and circulatory failure (OR: 2.15, 95% CI 1.27-3.64), and even associated with prolonged the LOS ICU (ß: 2.61, 95% CI 1.59-3.62) and LOS hospital (ß: 6.59, 95% CI 2.62-10.57). In sensitivity analysis, multivariate analysis (HR: 1.09, 95%CI 0.86, 1.38), IPTW (HR: 1.11, 95%CI 0.95-1.29) and CAPS (HR: 1.09, 95% CI 0.86-1.38) showed FFP transfusion had no beneficial effect on the 30-day mortality. Smooth curve fitting demonstrated the risk of liver failure, kidney failure and circulatory failure increased by 3%, 2% and 2% respectively, for each 1 ml/kg increase in FFP transfusion. We found there was no significant difference of CLIF-SOFA and MELD score between the two group on day 0, 3, 7, 14. Compared with the conventional group, INR, APTT, and TBIL in the FFP transfusion group significantly increased, while PaO2/FiO2 significantly decreased within 14 days. In conclusion, FFP transfusion had no beneficial effect on the 30-day, 90-day, in-hospital mortality, was associated with prolonged the LOS ICU and LOS hospital, and the increased risk of liver failure, kidney failure, coagulation failure, respiratory failure and circulatory failure events. However, large, multi-center, randomized controlled trials, prospective cohort studies and external validation are still needed to verify the efficacy of FFP transfusion in the future.

Ultrasound-Guided Radiofrequency Ablation in Tertiary Hyperparathyroidism: A Prospective Study.

OBJECTIVE: To prospectively evaluate the outcomes of ultrasound (US)-guided radiofrequency ablation (RFA) in tertiary hyperparathyroidism (THPT). MATERIALS AND METHODS: Patients with THPT underwent RFA between September 2017 and January 2022. Laboratory parameters, including serum intact parathyroid hormone (iPTH) levels, were monitored for 48 months after RFA and compared with the levels at baseline. Complications related to RFA and changes in hyperparathyroidism-related clinical symptoms were recorded before and after RFA. RESULTS: A total of 42 patients with THPT were recruited for this study. Ultimately, 36 patients with renal failure and 2 patients who underwent successful renal transplantation (male:female, 17:21; median age, 54.5 years) were enrolled. The follow-up time was 21.5 ± 19.0 months in the 36 patients with renal failure. In these 36 patients, iPTH levels were significantly decreased to 261.1 pg/mL at 48 months compared with the baseline value of 1284.9 pg/mL (P = 0.012). Persistent hyperparathyroidism, defined as iPTH levels maintained at > 585.0 pg/mL for 6 months after treatment, occurred in 4.0% of patients (1/25). Recurrent hyperparathyroidism, defined as iPTH levels > 585.0 pg/mL after 6 months, were 4.0% (1/25) and 0.0% (0/9) at 6 months and 4 years after treatment, respectively. In two patients with THPT after successful renal transplantation, iPTH decreased from the baseline value of 242.5 and 115.9 pg/mL to 171.0 and 62.0 pg/mL at 6 months after treatment. All complications resolved within 6 months of ablation without medical intervention, except in 10.5% (4/38) patients with permanent hypocalcemia. The overall symptom recovery rate was 58.8% (10/17). The severity scores for bone pain, arthralgia, and itchy skin associated with hyperparathyroidism improved after treatment (P < 0.05). CONCLUSION: US-guided RFA is an effective and safe alternative to surgery in the treatment of patients with TPTH and improves hyperparathyroidism-related clinical symptoms.

A 6-year review of acute post-streptococcal glomerulonephritis at a public children's hospital in Cape Town, South Africa.

BACKGROUND: Acute post-streptococcal glomerulonephritis (APSGN) is the most common cause of acute nephritis in children globally and, in some cases, may be associated with progressive kidney injury and failure, cumulating in the need for long-term dialysis and/or kidney transplantation. METHODS: Our retrospective study describes the occurrence of APSGN among children (< 14 years) admitted to a tertiary children's hospital in Cape Town, South Africa, from January 2015 to December 2020. RESULTS: Of 161 children who presented with acute nephritis (haematuria, oedema, oliguria, and hypertension), 100 met the inclusion criteria. Demographic, clinical features, laboratory findings, management, and outcome data were collected. APSGN was defined by the clinical presentation of at least two clinical signs of acute nephritis, and low serum complement 3 (C3) level or evidence of a recent streptococcal infection. Most cases of APSGN were associated with streptococcal skin infections: 55/100 (55%); 10/100 (10%) children presented with hypertensive seizures; C3 levels were low in 86/92 (93.5%) children; 94/94 (100%) children had elevated anti-deoxyribonuclease-B (anti-DNase-B) levels; and 80/94 (85%) also had elevated anti-streptolysin O titre (ASOT) at presentation. Eleven (11%) children had a percutaneous kidney biopsy; 4/11 (36%) showed histological features of post-infectious nephritis, and 7/11(64%) also had crescentic glomerulonephritis with immune complex deposits. Sixty-two (62%) children confirmed recovered, and five (5%) progressed to kidney failure, but 29 presumed recovered as they did not return for follow-up to our institution. CONCLUSIONS: Childhood APSGN remains an important health problem in South Africa (SA) with favourable outcomes in most, apart from those with crescentic glomerulonephritis who progressed to kidney failure.

Adjuvant trastuzumab for triple-positive breast cancer with chronic renal failure: A case report and review of literature.

RATIONALE: Although the occurrence of combined renal insufficiency among patients with breast cancer is even rarer, it poses a significant challenge in the treatment of these patients. Treating such patients often requires both targeted and endocrine therapies. However, oncologists lack evidence-based guidelines for managing renal function in patients with renal insufficiency. PATIENTS CONCERN: A 56-year-old menopausal female with a history of renal failure was diagnosed with triple-positive breast cancer and administered endocrine therapy and targeted therapy associated with hemodialysis after surgery. OUTCOMES: Under the premise of regular dialysis, the patient successfully completed endocrine therapy and targeted therapy for 1 year. DISCUSSION: Patients with advanced triple-positive breast cancer, including those undergoing hemodialysis, require a combination of anti-human epidermal growth factor receptor-2 and endocrine therapies, The side effects of these 2 treatment methods are worth considering in patients with renal insufficiency. CONCLUSION: We report a case of triple-positive breast cancer in a patient undergoing hemodialysis. There was no difference in the treatment approach between patients with and without normal renal function.

Propensity score matched analysis for the safety and effectiveness of remdesivir in COVID-19 patients with renal impairment.

BACKGROUNDS: Remdesivir (RDV) is an antiviral agent approved for the treatment of coronavirus disease 2019 (COVID-19); however, is not recommended for patients with renal impairment. Due to limitations associated with prospective clinical trials, real-world data on the safety and efficacy of RDV in patients with renal impairment are necessary. METHODS: Propensity score-matched (PSM) retrospective analysis was conducted between March 2020 and September 2022 in COVID-19 patients with an eGFR < 30 mL/min in four Korean hospitals. The RDV treatment group was matched to the untreated control group. The safety and clinical outcomes in patients who received RDV were analyzed. RESULTS: A total of 564 patients were enrolled; 229 patients received RDV either for treatment or prophylaxis. On day 5, no difference in nephrotoxicity was observed between the two groups, and liver enzyme levels were within the normal range. In multivariate analysis for new dialysis, RDV treatment was not a risk factor for new dialysis. Among the 564 patients, 417 were indicated for a 5-day course of RDV treatment and 211 patients were treated with RDV. After PSM, no differences in the clinical outcomes were observed between the two groups. CONCLUSION: RDV use in COVID-19 patients with renal impairment did not result in significant nephrotoxicity or hepatotoxicity.

Assessment of clinical prognosis improvement in children with concomitant anterior and posterior urethral valves: A case series report.

BACKGROUND: Concomitant anterior urethral valves (AUVs) and posterior urethral valves (PUVs) is an extremely rare congenital urologic anomaly, which may be easily overlooked in the clinic. OBJECTIVE: This study assessed the prognosis of children with concomitant PUVs and AUVs. METHODS: The clinical data of inpatients with concomitant AUVs and PUVs in our hospital were collected from January 1983 to June 2022. The clinical manifestations, auxiliary inspection, and treatment were described in detail. RESULTS: In total, 6 cases of concomitant AUVs and PUVs in boys were found in our hospital, with ages ranging from 3 months to 9 years; the main clinical manifestation was abnormal urination. Four patients exhibited concomitant AUVs and PUVs preoperatively and underwent simultaneous anterior and posterior urethral valvotomy. Follow-up studies showed that 3 patients' clinical symptoms substantially improved with well-maintained renal function. One patient died of renal failure. In the other 2 patients, PUVs were initially identified and excised, but their clinical symptoms did not show substantial improvement. Following voiding cystourethrography (VCUG), the AUVs were found and obstructions were then completely relieved. However, 2 patients died of renal failure. CONCLUSIONS: If urinary symptoms cannot be substantially relieved after posterior urethral valvotomy, VCUG and cystoscopy should be repeated to shorten the interval between anterior and posterior urethral valvotomies to improve patient prognosis.

Combined Heart Kidney Transplantation Versus Heart Transplant in Patients with Renal Failure: Contemporary Insights and Future Perspectives.

PURPOSE OF REVIEW: In this review, we aim to outline the criteria regarding the evaluation of patients with chronic renal disease (CKD) awaiting heart transplantation and discuss the outcomes of combined heart/kidney transplantation. Herein, we also review pathophysiology and risk factors that predispose to chronic kidney disease (CKD) and acute kidney injury (AKI) in patients with HF and after OHT. RECENT FINDINGS: In patients with end-stage systolic heart failure (HF) and an estimated glomerular filtration rate (eGFR) < 30 mL/min/1.73 m2, orthotopic heart transplantation (OHT) alone is a relative contraindication, with a consensus that these patients are better served with heart-kidney transplant (HKT). However, there is significant variation between institutions regarding timing and indication for heart/kidney transplantation, with little data available to predict post-transplant outcomes. A Scientific Statement from American Heart Association was published detailing the indications, evaluation, and outcomes for Heart-Kidney Transplantation, and noted a steady rise in the incidence of heart/kidney dual organ transplants. Recently, the Organ Procurement and Transplantation Network (OPTN) Multi-Organ Transplantation Committee implemented a safety net policy for heart transplant recipients who do need meet criteria for simultaneous heart-kidney transplant in 2023 but with a likely need for sequential kidney transplantation. Optimization of organ distribution and patient outcomes after cardiac transplantation requires appropriate recipient selection. This review also outlines the criteria regarding the evaluation of patients with CKD awaiting heart transplantation and outcomes of combined HKT.

Monoclonal Gammopathy and Its Association with Progression to Kidney Failure and Mortality in Patients with CKD.

BACKGROUND: Little is known about the prognostic significance of monoclonal gammopathy of undetermined and renal significance (MGUS and MGRS) in patients with CKD. The objective of this study was to determine the clinical and kidney outcomes of patients with CKD with either MGUS or MGRS compared with those with CKD without MGUS or MGRS. METHODS: We conducted a retrospective cohort study from 2013 to 2018. Patients who had both CKD diagnosis and monoclonal testing were identified. Patients were divided into MGRS, MGUS, and no monoclonal gammopathy groups. Cumulative incidence functions and Cox proportional hazards regression were used to model time to event data and to evaluate the association between monoclonal gammopathy status and risk of kidney failure, with death treated as a competing risk. RESULTS: Among 1535 patients, 59 (4%) had MGRS, 648 (42%) had MGUS, and 828 (54%) had no monoclonal gammopathy. Unadjusted analysis showed that compared with no monoclonal gammopathy patients, patients with MGRS were at higher risk of kidney failure (hazard ratio [HR] [95% confidence interval]: 2.5 [1.5 to 4.2] but not patients with MGUS (HR [95% confidence interval]: 1.3 [0.97 to 1.6]), after taking death into account as a competing risk. However, in the multivariable analysis, after adjusting for age, sex, eGFR, proteinuria, and Charlson Comorbidity Index, the risk of progression to kidney failure (with death as competing risk) in the MGRS group was no longer statistically significant (HR: 0.9 [0.5 to 1.8]). The same was also true for the MGUS group compared with the group with no monoclonal gammopathy (HR: 1.3 [0.95 to 1.6]). When evaluating the association between MGUS/MGRS status and overall survival, MGRS was significantly associated with mortality in fully adjusted models compared with the group with no monoclonal gammopathy, while MGUS was not. CONCLUSIONS: After adjusting for traditional risk factors, MGUS/MGRS status was not associated with a greater risk of kidney failure, but MGRS was associated with a higher risk of mortality compared with patients with no monoclonal gammopathy.

The role of liver transplantation in COACH syndrome (Joubert syndrome with congenital hepatic fibrosis): A review of the literature.

BACKGROUND: COACH syndrome is a rare autosomal recessive genetic disease characterized by liver fibrosis, which leads to severe complications related to portal hypertension. However, only a few patients with COACH syndrome undergoing liver transplantation (LT) have been reported. MATERIALS AND METHODS: We herein report the outcomes of four children who underwent LT for COACH syndrome at our institute and review three previously reported cases to elucidate the role of LT in COACH syndrome. RESULTS: All four patients in our institute were female, and three received living donors LT. All patients were diagnosed with COACH syndrome by genetic testing. LT was performed in these patients at 3, 7, 9, and 14 years old. The indication for LT was varices related to portal hypertension in all patients. One showed an intrapulmonary shunt. Blood tests revealed renal impairment due to nephronophthisis in three patients, and one developed renal insufficiency after LT. The liver function was maintained in all patients. A literature review revealed detailed information for three more patients. The indication for LT in these three cases was portal hypertension, such as bleeding from esophageal varices. One patient had chronic renal failure on hemodialysis at LT and underwent combined liver and kidney transplantation. Of these three previous patients, one died from hepatic failure due to de novo HCV infection 3 years after LT. CONCLUSIONS: LT should be considered an effective treatment for COACH syndrome in patients with severe portal hypertension. However, a detailed follow-up of the renal function is necessary.

Kidney and urological involvement in Down syndrome: frequent, underestimated, but associated with impaired quality of life and risk of kidney failure.

Patients with Down syndrome (DS) are at risk of multiorgan dysfunction; kidney and urological impairment are common. This is due to a likely increased risk of congenital kidney and urological malformations (odds ratio of 4.5 compared to the general population in one study), more frequent associated comorbidities at risk of kidney dysfunction (such as prematurity in 9-24% of children, intrauterine growth retardation or low birth weight in 20%, and congenital heart disease in 44%), and more frequent lower urinary tract dysfunction (reported in 27-77% of children with DS). If present, malformations and comorbidities at risk of kidney dysfunction warrant regular kidney monitoring in addition to their treatment. Serum creatinine in children with DS has been shown to be higher than in the general population and asymptomatic hyperuricemia is reported in 12-33% of children or young adults with DS. Moreover cryptorchidism and testicular cancer are also more common and should be detected by clinical examination. Thus, persons with DS at risk of presenting kidney and urological impairment should be identified by prenatal ultrasonography, comorbidities at risk of kidney sequelae considered, and during regular medical follow-up, clinically examined and questioned to diagnose testicular anomalies and lower urinary tract dysfunction. This is of importance as such kidney and urological impairments are associated with impaired quality of life and mental health, and risk of kidney failure.

Risk Factors Associated With Postoperative Cardiac Events Following Total Hip and Knee Arthroplasty.

BACKGROUND: This study investigated the prevalence of adverse cardiac events following a total joint arthroplasty and subsequently analyzed risk factors that may increase the likelihood of these events. METHODS: Data for this study were extracted from a large national database. Chi-squared analyses and multivariate modelings were performed to determine the risk factors associated with 30-day perioperative troponin elevation, myocardial infarction (MI), and heart failure. We identified 80,544 total hip arthroplasty (THA) patients and 112,531 total knee arthroplasty (TKA) patients and analyzed the following cardiac risk factors: diabetes, renal insufficiency, prior MI, hypertension, and cerebrovascular disease. RESULTS: There were 34% of THA patients and 52% of TKA patients who had at least one of the studied risk factors. At-risk THA patients had 2.2, 5.9, and 5.3 times the odds of troponin elevation, MI, and postoperative heart failure, respectively, within 1 month compared to the control group (P < .0001). The TKA group had 2.9, 5.3, and 5.9 times the odds of troponin elevation, MI, and postoperative heart failure within 1 month compared to the control group (P < .0001). For both procedures, prior MI had the highest odds of resulting in perioperative troponin elevation and MI. Renal insufficiency had the highest odds of resulting in perioperative heart failure. CONCLUSIONS: Risk stratification for postoperative complications in orthopedic surgery is important to minimize adverse outcomes. This study highlights the need for consideration of risk factors prior to joint arthroplasty surgery. LEVEL OF EVIDENCE: Level III, Prognostic.

Outcomes of Head and Neck Free Tissue Transfer in Renal Failure Patients.

OBJECTIVE: To assess if there is increased risk of free flap failure in renal failure patients undergoing head and neck reconstruction. We seek to primarily assess free flap outcomes based on stages of chronic kidney disease (CKD) and secondarily determine increased risk for postoperative complications. METHODS: Retrospective chart review was performed at five tertiary care centers. Patients were identified that had undergone microvascular free flap reconstruction of the head and neck with diagnosis of renal failure, classified as Stage 3 CKD or higher. Demographic data was collected. Outcomes in the postoperative period were examined. RESULTS: Seventy-three patients met inclusion criteria. The average patient age was 69 years with a male predominance (n = 48). The majority of patients had CKD Stage 3 (n = 52). Overall flap failure rate was 12.33% (n = 9, CKD stage 3 = 7.69%, CKD stage 4 = 30%, CKD stage 5 = 18%). There was an increased risk of flap failure on multivariate analysis for CKD stage 4/5 patients when compared to CKD 3 patients (p = 0.0095). When compared to matched controls, there was an increased risk of flap failure in CKD patients (p = 0.01) as well as an increased risk of overall complications (p < 0.0001). CONCLUSIONS: Patients with CKD undergoing head and neck reconstruction are at a higher risk of flap failure and overall complications. When comparing CKD stages there may be increased risk of flap failure in later stages of CKD compared to CKD 3. Appropriate patient counseling is recommended pre-operatively in this patient population with consideration for regional flaps in the appropriate patient. LEVEL OF EVIDENCE: 3 Laryngoscope, 134:688-694, 2024.

Association of mild kidney dysfunction with diastolic dysfunction and heart failure with preserved ejection fraction.

AIMS: We aim to investigate the association between kidney dysfunction and left ventricular diastolic dysfunction parameters and heart failure with preserved ejection fraction (HFpEF) and whether this is sex-specific. METHODS AND RESULTS: We included participants from the HELPFul observational study. Outpatient clinical care data, including echocardiography, and an expert panel judgement on HFpEF was collected. Estimated glomerular filtration rate (eGFR) was calculated by creatinine and cystatin C without race. The association between eGFR with E/e', left ventricular mass index, relative wall thickness, and stage C/D heart failure was tested by multivariable adjusted regression models, stratified by sex, reporting odds ratios and 95% confidence intervals (95% confidence interval). We analysed 880 participants, mean age 62.9 (standard deviation: 9.3) years, 69% female. Four hundred six participants had mild (37.6%) kidney dysfunction (eGFR: 60-89 mL/min/1.73 m2 ) or moderate (8.5%) kidney dysfunction (eGFR: 30-59 mL/min/1.73 m2 ). HFpEF was significantly more prevalent in participants with mild and moderate kidney dysfunction (10.3% and 16.0%, respectively) than participants with normal kidney function (3.4%). A lower kidney function was associated with higher E/e' and higher relative wall thickness values. Participants with moderate kidney dysfunction had a higher likelihood of American College of Cardiology/American Heart Association stage C/D HF (odds ratio: 2.07, 95% confidence interval: 1.23, 3.49) than participants with normal kidney functions. CONCLUSIONS: Both mild and moderate kidney dysfunction are independently associated with left ventricular diastolic dysfunction parameters and HFpEF. This association is independent of sex and strongest for moderate kidney dysfunction. Considering mild-to-moderate kidney dysfunction as risk factor for HFpEF may help identify high-risk groups benefiting most from early intervention.