Pretransplant Parathyroidectomy in Patients with Severe Secondary Hyperparathyroidism and Long-Term Effectiveness After Kidney Transplantation.

Kidney transplantation (KT) is the best option for patients with end-stage renal disease, but recipients still have legacy bone mineral disease from the pretransplant period, especially patients with severe secondary hyperparathyroidism (sHPT). Patients who had severe sHPT and underwent KT were analyzed retrospectively. Two groups were identified (patients with severe sHPT who had parathyroidectomy or calcimimetic before KT). Bone mineral density (BMD) was measured in the first year and last follow-up at the femoral neck, total hip, and lumbar spine using the dual-energy X-ray absorptiometry (DXA). Persistent hyperparathyroidism (perHPT) incidence was significantly higher in the calcimimetic group (75% vs. 40%, p=0.007). In patients with parathyroidectomy, BMDs were higher at femoral neck (0.818±0.114 vs. 0.744±0.134, p=0.04) and lumbar spine (1.005±0.170 vs. 0.897±0.151, p=0.01) at the first assessment. The BMD comparison between patients treated with parathyroidectomy and calcimimetic found a significant difference only in the femoral neck at second evaluation (0.835±0.118 vs. 0.758±0.129; p=0.03). In multivariate, linear regression revealed a positive association between the last BMD of the femoral neck with body mass index (CC: 0.297, 95% CI, 0.002-0.017) and parathyroidectomy (CC: 0.319, 95% CI, 0.021-0.156). Parathyroidectomy is associated with a significantly better femoral neck BMD and a lower incidence of perHPT in patients with severe sHPT.

Comparison of homologous and heterologous inactivated and mRNA vaccination programme against SARS-CoV-2 in dialysis patients.

AIM: To evaluate the vaccine response and the effect of the booster dose on COVID-19 positivity in haemodialysis (HD) and peritoneal dialysis (PD) patients who received and did not receive BNT162b2 as a booster dose after two doses of CoronaVac. METHODS: The study included 80 PD and 163 HD patients, who had been administered two doses of the CoronaVac. Antibody levels were measured on Days 42 and 90 after the first dose. Measurements were repeated on Day 181 after the first dose in the patients that received two vaccine doses and on Day 28 after the third dose in those that also received the booster dose. Antibody levels below 50 AU/mL were considered negative. RESULTS: The seropositivity rate was similar in the HD and PD group on Days 42 and 90 (p = 0.212 and 0.720). All patients were seropositive in the booster group. The antibody level was lower in the patients that received CoronaVac as the booster compared to those administered BNT162b2 in HD and PD groups (p < 0.001 and 0.002). COVID-19 positivity was detected in 11 patients (7 = had not received the booster dose, 4 = had received third dose of CoronaVac). The multivariate analysis revealed that as age increased, COVID-19 positivity also increased (OR: 1.080, 95% CI: 1.017 - 1.146, p = 0.012), while booster dose administration decreased this positivity (OR: 0.113, 95% CI: 0.028 - 0.457, p = 0.002). CONCLUSION: Our results may indicate the need for additional vaccination doses in patients with HD and PD. Our findings indicate a higher antibody response in dialysis patients with heterologous BNT162b2 as a booster dose after two doses of CoronaVac compared to homologous CoronaVac.

Diagnostic accuracy of venous system ultrasound for subtypes of acute kidney injury.

BACKGROUND: Management of acute kidney injury (AKI) in the ED can be difficult due to uncertainty regarding the aetiology. This study investigated the diagnostic value of venous system ultrasound for determining the aetiological subtypes of AKI in the ED. METHODS: This multidisciplinary prospective cohort study was conducted in a single academic ED over the course of a year. Adult patients with AKI were evaluated using the venous excess ultrasound (VExUS) score, which is a four-step ultrasound protocol. The protocol begins with the inferior vena cava (IVC) measurement and examines organ flow patterns, including portal, hepatic and renal veins in the presence of dilated IVC. The AKI subtypes (hypovolaemia, cardiorenal, systemic vasodilatation and renal) were adjudicated by nephrologists and emergency physicians, considering data that became available during the hospitalisation. We determined the diagnostic test characteristics of VExUS for identifying each of the four AKI aetiological subtypes. RESULTS: 150 patients with AKI were included in the study. Hypovolaemia was the most frequent finally adjudicated cause of AKI (66%), followed by cardiorenal (18%), systemic vasodilatation (8.7%) and renal (7.3%). In diagnosing the cardiorenal subtype, the area under the curve (AUC) for VExUS grade >0 was 0.819, with 77.8% sensitivity and 80.5% specificity, and the AUC for IVC maximum diameter >20.4 mm was 0.865, with 74.1% sensitivity and 86.2% specificity. For the hypovolaemia subtype, the AUC for VExUS grade ≤0 was 0.711, with 83.8% sensitivity and 56.9% specificity, and the AUC for IVC maximum diameter ≤16.8 mm was 0.736, with 73.7% sensitivity and 68.6% specificity. None of the parameters achieved adequate test characteristics for renal and systemic vasodilatation subtypes. CONCLUSION: The VExUS score has good diagnostic accuracy for cardiorenal AKI and fair accuracy for hypovolaemic AKI but cannot identify renal and systemic vasodilatation subtypes. It should not therefore be used in isolation to determine the cause of AKI in the ED. TRIAL REGISTRATION NUMBER: NCT04948710.

Association of longitudinal high-sensitive C-reactive protein levels with changing membrane characteristics in peritoneal dialysis.

BACKGROUND: Increasing peritoneal permeability with ultrafiltration and solute removal inadequacy is a challenging issue in peritoneal dialysis (PD). Decreasing permeability is less frequent but also results in diminished solute clearance. We evaluated the association between longitudinal high-sensitive C-reactive protein (hs-CRP) values and the change in transport characteristics of the peritoneal membrane in PD patients. METHODS: This is a retrospective, single-center study of incident PD patients. An increase or decrease in peritoneal transport status is defined as two or more categories of a rise or decline in the peritoneal equilibration test (PET) from their baseline during follow-up. The 4-h dialysate/plasma creatinine ratio was used to classify transport characteristics. Hs-CRP values were obtained from the routine annual examinations of the patients. RESULTS: Baseline demographics, residual kidney function, frequency of high glucose-containing dialysate, and icodextrin use were similar between the groups. Total episodes of peritonitis within the first 5 years of follow-up were higher in stable transporters than in increased and decreased transporters (p = 0.009). Stable transporters' mean hs-CRP values did not change within 5 years (Wilks' λ = 0.873, F (2.317, 180.740) = 2.210, p = 0.10). Increased and decreased transporters' hs-CRP values significantly raised over the years (Wilks' λ = 0.422, F (1.979, 77.163) = 3.405, p = 0.04 and Wilks' λ = 0.558, F (3.673, 66.107) = 4.396, p = 0.001, respectively). CONCLUSIONS: Our study shows that the peritoneal membrane may change into different characteristics in many patients over time, despite very low peritonitis frequencies and similar baseline characteristics that may be significantly affected by systemic inflammation.

First Composite Woman-to-Woman Facial Transplantation in Turkey: Challenges and Lessons to Be Learned.

ABSTRACT: After the first face transplantation from woman to woman we performed in our clinic, it was aimed to eliminate the lack of knowledge about the subject in the literature by transferring our experiences and long-term results to the problems we had with the patient. A 20-year-old patient underwent partial osteomyocutaneous facial transplant (22nd facial transplant), which included 2 functional units of the face. The patient had no major problems in the early period and had a good aesthetic appearance. In the postoperative period, the patient ended her social isolation and adopted the transplanted face.In the late period, secondary surgical interventions, management of the problems caused by immunosuppression, and the patient's living in a remote location to our clinic were the difficulties encountered. Six revision surgeries were performed after the transplantation. Due to immunosuppression, opportunistic infections and metabolic problems required intermittent hospitalization. The patient died at the end of 56 months because of complications secondary to immunosuppression.A successful transplant involves the management of long-term problems rather than a successful tissue transfer in the early period. In today's conditions, long-term success can be achieved with a good patient compliance, as well as each team member should take an active role in the team at the transplantation centers. More case series are needed to adapt the standard treatment and follow-up protocols for solid organ transplantations for composite tissue allotransplantations. This will be possible by sharing the results and experiences transparently in the centers where face transplantation is performed worldwide.

Obstetric and long-term graft outcomes in pregnant kidney transplant recipients: A single-center experience.

BACKGROUND: Kidney transplantation (KT) is the best option for many women with end-stage renal disease desiring pregnancy. The aim of this study was to investigate obstetric and graft outcomes among KT recipient women in our center. METHODS: Maternal and fetal data were assessed in 29 pregnancies of 18 female KT recipients. Each patient was matched with two controls without pregnancy history for factors known to affect graft function. According to pre-pregnancy levels, serum creatinine and eGFR slope in the gestational and postpartum periods were calculated as percentages. RESULTS: The main maternal and fetal complications were preeclampsia (38%) and preterm births (38%), respectively. Pregnancy (odds ratio [OR]: 5.09; p = .02), proteinuria in the third trimester (OR: 5.52; p = .02), proteinuria in postpartum third months (OR: 7.4; p = .008) and stable creatinine levels in the first 6 months of pregnancy (OR: 11.25 p = .03) were associated with graft dysfunction. Postpartum first year eGFR decline (-16.8% vs. -6.7%; p = .04) and second-year eGFR decline (-18.5% vs. -8.3%; p = .04) were significantly higher in the pregnancy group than those matched controls. CONCLUSION: Pregnancy after KT is associated with high rates of maternal and fetal complications. The sustained decline of eGFR may suggest an increased risk of graft loss compared to recipients with similar clinical characteristics.

The reliability and success of peritoneal dialysis during the COVID-19 pandemic.

We evaluated the symptoms, changes in laboratory findings during the novel coronavirus disease (COVID-19) pandemic, and the effect of depression in patients with peritoneal dialysis (PD). This is an observational and cross-sectional study. All patients were asked to fill the clinical assessment form and Beck depression and anxiety inventory. Also, the last two laboratory evaluations during this period were examined. A total of 123 patients performing PD were included. None of the patients were diagnosed with COVID-19. In the total study population, parathyroid hormone (PTH), serum albumin, phosphorus and ferritin levels significantly elevated at the end of 97 ± 31 days. PTH and phosphorus levels remained stable in remote monitoring automated PD (RM-APD) group (p = 0.4 and p = 0.5), they tended to increase in continuous ambulatory PD group and significantly increased in automated PD group (p = 0.09 and p = 0.01 for PTH and p = 0.06 and p = 0.001 for phosphorus, respectively). Moderate to severe depression was associated with dyspnoea, weight gain more than 5 kg, fatigue, palpitation and increased anxiety. PD is a reliable and successful form of dialysis and can be safely administered even if hospital access is restricted. Also, RM-APD may be a better choice because of providing more stable bone-mineral metabolism. Moreover, evaluating depression and anxiety is essential for the accurate clinical assessment.

Effects of phospholipase A2 receptor and thrombospondin type-1 domain-containing 7A expression in glomerular basement membranes on treatment response and renal outcome in membranous nephropathy.

BACKGROUND: The aim of this study was to define the clinicopathologic features of phospholipase A2 receptor (PLA2R) and/or thrombospondin type-1 domain-containing 7A (THSD7A) associated membranous nephropathy(MN) focusing on their impact to disease relapse and response to treatment. METHODS: A total of 201 patients were enrolled for baseline clinical and histopathological features and 102 patients with a clinical follow-up for more than 1 year were evaluated for outcomes. Immunohistochemical staining was performed with PLA2R and THSD7A antibodies on kidney biopsies and glomerular staining was evaluated. RESULTS: PLA2R expression was observed in 75% of the patients' biopsies; however, THSD7A expression was present only in 7 patients' biopsies (3.5%). No significant difference was found between histopathological and clinical features of PLA2R positive and negative patients, collectively. Glomerular PLA2R expression was significantly associated with complete and complete/partial remission with first-line treatment; however, overall complete, and complete/partial remission rates did not differ from PLA2R negative patients (p = 0.2 and p = 0.8). Male gender, the presence of IgG4 staining and a necessity of immunosuppressive treatment were significantly associated with glomerular PLA2R expression. One patient, who developed end-stage renal disease, had glomerular expression for both PLA2R and THSD7A. Three patients with THSD7A-positive MN achieved complete remission. CONCLUSIONS: The probability of achieving complete remission is high in patients with PLA2R-positive MN for whom the relapse rate was also higher. The overall renal outcome did not differ from PLA2R negative cases. Low incidence of THSD7A-positive MN reduces the possibility of future randomized controlled trials.

Vitamin D receptor polymorphisms and bone health after kidney transplantation

Background/aim: Bone disease is one of the most prominent complications after kidney transplantation. Bone diseases include osteoporosis, persistent secondary hyperparathyroidism, and avascular necrosis (AVN). We investigated the relationship between the polymorphisms of the vitamin D receptor (VDR) gene and bone diseases occurring after kidney transplantation. Materials and methods: The study consists of 234 kidney allograft recipients with a minimum follow-up of five years after kidney transplantation. Patients with glomerular filtration rates less than 30 mL/min/1.73m2, a history of parathyroidectomy, bisphosphonate use pre- or post-transplantation, and cinacalcet use posttransplantation excluded. We evaluated associations between the polymorphisms of the VDR gene (BsmI, TaqI, ApaI, FokI, and Cdx2), the first-year bone mineral density (BMD) scores, persistent secondary hyperparathyroidism, and AVN. Results: Patients with low BMD scores were significantly younger (P = 0.03) and had higher intact parathormone (iPTH) levels (P = 0.03). Cdx2 TT genotype significantly increases the risk of low BMD scores (OR: 3.34, P = 0.04). Higher phosphate levels were protective against abnormal BMD scores (OR: 0.53; P = 0.03). Patients with persistent hyperparathyroidism had significantly longer dialysis vintage and higher pretransplantation iPTH levels (P = 0.02 and P < 0.001, respectively). Cdx2, CT/TT, and ApaI CA/AA genotypes significantly increase the risk of persistent hyperparathyroidism (OR: 6.81, P < 0.001, OR: 23.32, P < 0.001, OR:4.01, P = 0.02, and OR: 6.30, P = 0.01; respectively). BsmI CT/TT genotypes were found to increase AVN risk with an HR of 3.48 (P = 0.03). Higher hemoglobin levels were also found to decrease AVN risk with an HR of 0.76 (P = 0.05). Conclusion: Certain VDR gene polymorphisms are associated with a higher risk for bone diseases after kidney transplantation.

Two diseases one remedy? Systemic amyloidosis secondary to hidradenitis suppurativa: Treatment with infliximab.

Hidradenitis suppurativa, known as acne inversa, is a relapsing and chronic inflammatory skin disease affecting the skin folds. During the chronic course of the disease many local complications like fistulae to other tissues or systemic complications including anemia, secondary amyloidosis, lymphedema, nephrotic syndrome, artropathy may take place. Amyloid A amyloidosis is a rare complication of hidradenitis suppurativa, which has been described in a limited number of case reports. Herein, we present such a patient that had developed AA amyloidosis during the course of hidradenitis suppurativa. Both AA amyloidosis and hidradenitis suppurativa have responded to infliximab therapy which was shown by clinical recovery and by the improvement in renal functions.

Higher thrombin activatable fibrinolysis inhibitor levels are associated with inflammation in attack-free familial Mediterranean fever patients.

BACKGROUND: Coagulation abnormalities have been reported in familial Mediterranean fever (FMF) patients with amyloidosis and nephrotic syndrome; but there is not enough data about the continuity of the thrombogenic activity in FMF patients in clinical remission. The purpose of this study was to assess thrombin activatable fibrinolysis inhibitor (TAFI) levels and its relationship with fibrinolytic activity and also evaluate relationships between mutations and clinical signs in attack-free patients without amyloidosis. METHODS: Seventy-nine FMF patients and 40 healthy adults were included. The study group was divided into five groups as follows: first group, homozygote M694V; second group, homozygote M680I; third group, M694V in one allele, the other allele have other mutations or not; fourth group, other mutations; and fifth group, no mutation. RESULTS: Serum TAFI levels were significantly increased in patients compared with healthy individuals (116.64 ± 21.8 vs. 78.48 ± 19.7 µg/mL, p < 0.001) and a positive correlation was detected between TAFI antigen level and erythrocyte sedimentation rate and C-reactive protein levels (r = 0.247, p = 0.029 and r = 0.252, p = 0.032, respectively). Mean fibrinogen and TAFI levels were significantly higher in Group 1 than the other groups (p = 0.04 and p = 0.001, respectively) and in Group 3 it was higher than Groups 2, 4 and 5 (p = 0.04 and p = 0.001, respectively). CONCLUSIONS: High level of TAFI antigen in attack-free period of FMF disease shows ongoing subclinical inflammation and hypercoagulability. Clinicians should be careful about thrombosis even in patients at clinical remission. Also, genetic tests must be considered to predict clinical outcome and to reduce complications of FMF disease.

Fibromyalgia and nondipper circadian blood pressure variability.

BACKGROUND AND OBJECTIVES: Aberrant circadian rhythm with persistent nocturnal sympathetic hyperactivity has pointed out malfunctioning autonomic nervous system in fibromyalgia (FM) patients. This is a common pathogenesis shared also by patients with nondipping blood pressure (BP) pattern. Therefore, we aimed to investigate the frequency of nondipping BP pattern in normotensive women with newly diagnosed FM compared with healthy women. METHODS: Sixty-seven normotensive women with new diagnosis of FM and 38 age-matched healthy volunteer women were recruited into the study. All subjects underwent 24-hour ambulatory BP monitoring on a usual working day. Individuals were defined as "dippers" if their nocturnal BP values decreased by more than 10% compared with daytime values; defined as "nondippers" in case of a decline less than 10%. Serum creatinine, fasting blood glucose, cholesterol levels, albumin, and thyroid-stimulating hormone levels were assessed. RESULTS: Ambulatory measurements showed significantly higher diastolic BP values in patients with FM for both average of 24-hour recordings. Patients with FM had significantly lower systolic (9.1 ± 3.9 vs 11.5 ± 4.9, P = 0.010) and diastolic dipping ratios (12.3 ± 6.1 vs 16.1 ± 6.4, P = 0.004). The number of nondippers in the FM group was significantly higher than that of controls for both systolic (66% vs 34%, P = 0.002) and diastolic BP measurements (42% vs 21%, P=0.031). Patients with FM were 3.68 times more likely to be systolic nondipper and 2.69 times more likely to be diastolic nondipper. CONCLUSIONS: We have demonstrated a significant relationship between FM and nondipping BP pattern, and we suggest that nondipping profile, which has been closely associated with cardiovascular morbidity, may appear as an additional risk factor in patients with FM.

The significance of finerenone as a novel therapeutic option in diabetic kidney disease: a scoping review with emphasis on cardiorenal outcomes of the finerenone phase 3 trials.

This scoping review prepared by endocrinology and nephrology experts aimed to address the significance of finerenone, as a novel therapeutic option, in diabetic kidney disease (DKD), based on the biological prospect of cardiorenal benefit due to non-steroidal mineralocorticoid receptor antagonist (MRA) properties, and the recent evidence from the finerenone phase 3 program clinical trials. The importance of finerenone in slowing DKD progression was critically reviewed in relation to the role of MR overactivation in the pathogenesis of cardiorenal disease and unmet needs in the current practice patterns. The efficacy and safety outcomes of finerenone phase III study program including FIDELIO-DKD, FIGARO-DKD and FIDELITY were presented. Specifically, perspectives on inclusion of patients with preserved estimated glomerular filtration rate (eGFR) or high albuminuria, concomitant use of sodium-glucose co-transporter-2 inhibitor (SGLT2i) or glucagon-like peptide 1 receptor agonist (GLP-1 RA), baseline glycated hemoglobin (HbA1c) level and insulin treatment, clinically meaningful heart failure outcomes and treatment-induced hyperkalemia were addressed. Finerenone has emerged as a new therapeutic agent that slows DKD progression, reduces albuminuria and risk of cardiovascular complications, regardless of the baseline HbA1c levels and concomitant treatments (SGLT2i, GLP-1 RA, or insulin) and with a favorable benefit-risk profile. The evolving data on the benefit of SGLT2is and non-steroidal MRAs in slowing or reducing cardiorenal risk seem to provide the opportunity to use these pillars of therapy in the management of DKD, after a long-period of treatment scarcity in this field. Along with recognition of the albuminuria as a powerful marker to detect those patients at high risk of cardiorenal disease, these important developments would likely to impact standard-of-care options in the setting of DKD.

Effects of incremental peritoneal dialysis with low glucose-degradation product neutral pH solution on clinical outcomes.

PURPOSE: Incremental peritoneal dialysis (IPD) could decrease unfavorable glucose exposure results and preserve (RKF). However, there is no standardization of dialysis prescriptions for patients undergoing IPD. We designed a prospective observational multi-center study with a standardized IPD prescription to evaluate the effect of IPD on RKF, metabolic alterations, blood pressure control, and adverse outcomes. METHODS: All patients used low GDP product (GDP) neutral pH solutions in both the incremental continuous ambulatory peritoneal dialysis (ICAPD) group and the retrospective standard PD (sPD) group. IPD patients started treatment with three daily exchanges five days a week. Control-group patients performed four changes per day, seven days a week. RESULTS: A total of 94 patients (47 IPD and 47 sPD) were included in this study. The small-solute clearance and mean blood pressures were similar between both groups during follow-up. The weekly mean glucose exposure was significantly higher in sPD group than IPD during the follow-up (p < 0.001). The patients with sPD required more phosphate-binding medications compared to the IPD group (p = 0.05). The rates of peritonitis, tunnel infection, and hospitalization frequencies were similar between groups. Patients in the sPD group experienced more episodes of hypervolemia compared to the IPD group (p = 0.007). The slope in RKF in the 6th month was significantly higher in the sPD group compared to the IPD group (65% vs. 95%, p = 0.001). CONCLUSION: IPD could be a rational dialysis method and provide non-inferior dialysis adequacy compared to full-dose PD. This regimen may contribute to preserving RKF for a longer period.

Is colchicine therapy effective in all patients with secondary amyloidosis?

OBJECTIVE: Although colchicine is effective on prevention and regression of amyloidosis in many cases, rate of unresponsiveness to colchicine therapy is not too low. However, there is no sufficient data about which factors effect to response of colchicine therapy on regression of amyloidosis. MATERIALS AND METHODS: 24 patients with renal amyloidosis were enrolled into the study. The patients were divided in two groups according to urinary protein excretions: non-nephrotic stage (14/24) and nephrotic stage (10/24). The patients were also categorized according to the etiology of amyloidosis; familial Mediterranean fever (FMF)-associated amyloidosis (15/24) versus rheumatoid disorders (RD)-associated amyloidosis (9/24). The changes of amount of proteinuria and estimated glomerular filtration rates were investigated after colchicine treatment started in these groups. RESULTS: The mean follow-up period was 27.7 ± 19.2 months. After initiating colchicine therapy, the degree of proteinuria was decreased higher than 50% in 11/14 (78%) of non-nephrotic patients and elevated only in three (22%) patients. In nephrotic group, proteinuria was increased in 5/10 (50%) of patients. Glomerular filtration rates were stable in nephrotic and non-nephrotic groups. Presenting with nephrotic syndrome was higher in RD-associated amyloidosis (RD_A) group (5/9) than FMF-associated amyloidosis (FMF_A) group (5/15) without statistical significance (p > 0.05). After colchicine treatment, proteinuria was decreased in 12/15 patients in FMF_A group, however, the significant decreasing of proteinuria was not observed in RD_A group (p = 0.05 vs. p > 0.05). CONCLUSION: Colchicine therapy was found more effective in low proteinuric stage of amyloidosis. The beneficial effect of colchicine therapy was not observed in patients with RD- associated amyloidosis.

The prevalence of Fabry disease in a Turkish population with chronic kidney patients.

BACKGROUND AND OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal storage disease with various clinical symptoms due to a deficiency of an enzyme called alpha-galactosidase A. The likelihood of nephropathy increases with age and the severity of the mutation in Fabry patients. Fabry disease is difficult to diagnose. The exact incidence and prevalence of Fabry disease are unknown due to its atypical or oligosymptomatic forms. DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: GLA gene mutations were examined in patients over the age of 18 who were followed up on with a diagnosis of chronic kidney disease and who had or did not receive renal replacement therapy from October 2017 to December 2019. RESULTS: A total of 18 sites in 8 locations around Turkey volunteered to participate in the study, including people aged 18 and older with stages 1-5 of chronic kidney disease (CKD) or getting renal replacement therapy. 1904 patients were screened in total. In 13 cases, a D313Y pseudo mutation in the GLA gene was discovered. GLA gene mutations were found and pathologically assessed in four of the tested cases. CONCLUSIONS: The range of clinical symptoms of Fabry disease, as well as the frequent delays in diagnosis, result in treatment being too late. We believe that screening chronic renal patients at high risk for Fabry disease is warranted.

Sunitinib- and sorafenib-induced nephrotic syndrome in a patient with gastrointestinal stromal tumor.

OBJECTIVE: To report a case of nephrotic syndrome (NS) induced by both sunitinib and sorafenib therapy. CASE SUMMARY: A 61-year-old woman with metastatic gastrointestinal stromal tumor (GIST) presented with NS and hypertension following therapy with sunitinib 400 mg/day. Because of grade 3 toxicity, the drug was discontinued. After sunitinib discontinuation, NS and hypertension resolved. However, NS recurred on rechallenge. A similar picture developed following therapy with sorafenib 800 mg/day. A renal biopsy revealed a focal segmental glomerulosclerosis (FSGS). A few months after sorafenib cessation, resolution of NS and hypertension was again achieved. DISCUSSION: Several cases of NS have been reported among patients receiving sunitinib and sorafenib. However, renal histopathologic data were obtained in only a few patients. Although biopsy-proven cases of FSGS associated with sunitinib have been reported, this is, to our knowledge, the first reported case of biopsy-proven FSGS associated with sorafenib. The Naranjo probability scale indicated probable causality for NS developing with sorafenib, and definite causality with sunitinib. The clinical and histopathologic findings have led us to agree with the class effect proposal that all antiangiogenic drugs share a similar toxicity profile. Evidence supporting this hypothesis includes worsening of hypertension and proteinuria by both drugs, with full recovery occurring within a few months after cessation of the drugs, which favors the role of vascular endothelial growth factor receptor inhibition in FSGS development. CONCLUSIONS: The clinical adverse spectrum of antiangiogenic drugs may be broader than initially observed because of a lack of renal biopsy data and routine screening for proteinuria. It can be speculated that proteinuria, as well as hypertension, is a class effect of all antiangiogenic drugs.

Remission of de novo membranous nephropathy in a kidney allograft recipient: a case report.

Membranous nephropathy (MN), one of the most frequent causes of nephrotic syndrome in native kidneys, is also a common glomerular pathology in transplanted kidneys(Davison AM, Johnston PA. Allograft membranous nephropathy, Nephrol Dial Transplant, 1992;7(Suppl. 1):114-118. Specific treatment modalities have not been described for this population. However, renal transplanted patients presented with MN could have spontaneous remission as those with idiopathic MN. Here, we report a kidney allograft recipient diagnosed with de novo MN in early phases of posttransplantation period having a clinical remission over months.

The Effects of CNI and Mtori-Based Regimens on Bone Mineral Density After Renal Transplantation.

Background: Since glucocorticoids are used in low maintenance doses today, the relationship between calcineurin inhibitors (CNI) and osteoporosis has become clinically significant in osteoporosis after solid organ transplantation. However, there is evidence that the mammalian target of rapamycin inhibitors (mTORi) may be beneficial via osteoclast inhibition. Objective: The bone mineral density (BMD) changes are investigated in renal transplant patients under CNI or mTORi-based maintenance regimens during the first five-year post-transplant course. Methods: This study consists of thirty-three renal allograft recipients with less than one year of dialysis history. The exclusion criteria were: being older than 50 years old, history of bisphosphonate use, parathyroidectomy, CNI-mTORi switch after the post-transplant third month, diuretic use, and history of malignancy. First and fifth-year BMD scores and simultaneous laboratory parameters were evaluated. Results: CNI (n=21) and mTORi group (n=12) had similar demographics, dialysis vintages, first and fifth-year serum parathormone, calcium, phosphate, magnesium, alkaline phosphatase, and 25-OH-vitamin D levels. The femur neck scores of the CNI group decreased from -0.82 (±0.96) to -1.52 (±0.92) (p=0.020). We observed a significant decrease in the CNI group compared to the mTORi group [-0.70 (±0.68) and 0.30 (±0.36), respectively; p<0.01] when the BMD score changes were evaluated among years. The mean femur neck score of the mTORi group increased insignificantly from -1.13 (±0.65) to -0.82 (±0.56) at the fifth-year DXA scan (p=0.230). Similar trends were also observed in L1-4 scores. Conclusion: Our study suggests that CNI-based treatment is associated with decreased femur neck BMD scores, and mTORi-based treatment tends to be beneficial in the post-transplant five-year follow-up.