Kidney tissue elastography and interstitial fibrosis observed in kidney biopsy.

INTRODUCTION: Kidney interstitial fibrosis is an important risk factor for the progression of chronic kidney disease. Kidney elastography is a noninvasive imaging modality that might be used to assess tissue fibrosis. In this study, we aimed to investigate the relationship between tissue stiffness detected in kidney elastography and interstitial fibrosis observed in kidney biopsy. MATERIALS AND METHODS: Patients who were hospitalized in a tertiary care university hospital with a kidney biopsy indication were included in this study. In all patients, the transverse and sagittal elastography measurements were made using a sonoelastography device before the biopsy. The total histological score was calculated. RESULTS: Fifty-seven native kidney patients with proteinuria were included in the study. Patients were divided into two groups according to the presence (n = 6) and absence of fibrosis (n = 51) as detected by kidney biopsy. A significant correlation was found between the presence of fibrosis detected by biopsy and elastography outcomes (p = .046, r = .192). A significant correlation was found between the urea and creatinine levels and transverse elastography measurements (p = .036, r = .240). No correlation was observed between the transverse elastography measurements and total histological score consisting of glomerular, vascular, and tubular scores (r = .006, p = .967). CONCLUSION: The findings of our study suggest a significant relationship between the elastography measurements and interstitial fibrosis. Because of the high negative predictive value (91%), we suggest that elastography should mainly be used as an exclusion test for the presence of fibrosis. We also believe that elastography may be useful to evaluate the fibrosis status in kidney diseases.

Reflex Decay Test Can Reveal Ear Involvement in Fabry Disease.

OBJECTIVES: Fabry disease (FD) is an X-linked lysosomal storage disorder that causes multisystem involvement, including ear disease. In this study, we aimed to investigate the nature of auditory issues in FD using a wide spectrum of audiological tests. DESIGN: This cross-sectional study was conducted between June 2017 and December 2018. We collected the clinical and laboratory data of 40 eligible FD patients, 45 healthy subjects, and 26 diabetic controls. All patients and controls completed audiologic evaluations that included tympanometry, acoustic reflex threshold test, reflex decay test, pure-tone audiometry, speech audiometry, transient otoacoustic emissions (TEOAEs), high-frequency audiometry, and distortion product otoacoustic emission (DPOAE). RESULTS: In our study population, hearing was reduced at higher frequencies starting at 4 kHz in both the FD and diabetic groups. Regarding the acoustic reflex threshold test, FD and diabetic patients had similar results. In all frequencies, positive decay was significantly more frequent in FD patients when compared with the diabetic patients and healthy controls (p < 0.001 for each ear). The FD patients and healthy controls had similar results for DPOAE testing. CONCLUSIONS: We showed that FD patients had a higher rate of reflex decay, indicating retrocochlear involvement. Thus, further investigation of factors associated with retrocochlear involvement could be investigated, such as ABR and speech in noise tests.

Fabry Disease Prevalence in Renal Replacement Therapy in Turkey.

BACKGROUND: Fabry disease (FD) is an X-linked lysosomal storage disorder resulting from lack of alpha-galactosidase A (AGALA) activity in lysosomes. OBJECTIVE: In this multicenter study, we aimed to evaluate the prevalence of FD in renal transplant (Tx) recipients in Turkey. We also screened dialysis patients as a control group. METHODS: All Tx and dialysis patients were screened regardless of the presence of a primary disease. We measured the AGALA activity in all male patients as initial analysis. Mutation analysis was performed in male patients with decreased AGALA activity and in female patients as the initial diagnostic assay. RESULTS: We screened 5,657 patients. A total of 17 mutations were identified. No significant difference was observed between the groups regarding the prevalence of patients with mutation. We found FD even in patients with presumed primary kidney diseases. Seventy-one relatives were analyzed and mutation was detected in 43 of them. We detected a patient with a new, unknown mutation (p.Cys223) in the GLA gene. CONCLUSIONS: There are important implications of the screening. First, detection of the undiagnosed patients leads to starting appropriate therapies for these patients. Second, the transmission of the disease to future generations may be prevented by prenatal screening after appropriate genetic counseling. In conclusion, we suggest screening of kidney Tx candidates for FD, regardless of etiologies of chronic kidney disease.

Recurrent and de novo glomerulonephritis following renal transplantation: higher rates of rejection and lower graft survival.

PURPOSE: In this retrospective study with case-control design, we aimed to determine the clinical and pathological characteristics of post-transplant glomerulonephritis (GN), and their effects on transplant recipients. METHODS: One hundred and twenty renal transplant recipients with biopsy-proven recurrent or de novo primary GN were compared with two matched control groups including 120 transplant recipients with nonrecurrent primary GN (nonrecurrent GN group) and 120 transplant recipients with non-GN etiology (non-GN group). Primary outcome was allograft loss, and secondary outcomes were biopsy-confirmed cellular or antibody-mediated rejection. RESULTS: In recurrent/de novo GN, nonrecurrent GN and non-GN groups, 54.2% (n = 65), 16.7% (n = 20) and 8.3% (n = 10) of patients reached primary outcome after a median follow-up of 96 (IQR: 56-149) months, respectively. Allograft loss was significantly higher in recurrent/de novo GN group compared to nonrecurrent GN and non-GN groups (p < 0.001). At 10 years, allograft loss rates in recurrent/de novo GN group were 54.2% for focal segmental glomerulosclerosis, 53.2% for membranoproliferative glomerulonephritis, and 33.4% for IgA nephropathy cases. Biopsy-confirmed rejection rate was significantly higher in the recurrent/de novo GN group (n = 25, 20.8%) compared to non-GN (n = 8, 6.7%) group (p = 0.001). CONCLUSIONS: Recurrent/de novo GN is associated with higher risk of rejection and worse allograft survival.

The Frequency and Associated Factors for BK Virus Infection in a Center Performing Mainly Living Kidney Transplantations.

PURPOSE: BK virus (BKV) nephropathy has increasingly become an important cause of morbidity in renal transplant recipients. We evaluated the frequency and associated factors for BKV infection in a center performing mainly living donor transplantations over a long time period. METHODS: One hundred consecutive renal transplant patients were included. Quarterly visits were planned to examine urine for decoy cells and to measure the BKV DNA in the blood and urine. Renal biopsy was performed in case of deteriorated allograft function. Serological examinations for BKV immunoglobulin G (IgG) were performed in donors. RESULTS: Throughout the entire follow-up period, the rates of viruria, viremia, and the positivity of decoy cells were 12%, 6%, and 13%, respectively. The negative and positive predictive values of decoy cells were 93.1% and 69.2%, respectively, for viruria, and 99.2% and 45.5%, respectively, for viremia. Biopsy-proven BKV nephropathy was observed in 1 patient. The BKV IgG was positive in all living donors. Viruria and viremia were associated with deceased donor transplantation, acute rejection, and pulse steroid therapy. In addition, viremia was associated with antithymocyte globulin therapy and a short duration of the posttransplant period. CONCLUSIONS: The frequency of BKV infection was lower in our transplant unit compared to previous reports. Reduced doses of immunosuppression seem to be the main factor that may explain the reduced frequency. However, an active screening strategy is still of importance for this patient group.

A Solid Mass in the Chest Wall.

Posttransplant lymphoproliferative disorder (PTLD) is one of the most common malignancies after kidney transplantation. Different clinical and histopathological forms of PTLD related to immunosuppression can be observed after organ transplantations. We report a 42-year-old woman who had undergone deceased donor renal transplantation with an unusual presentation of PTLD. The immunosuppressive treatment was discontinued and appropriate chemotherapy was started. However, the patient died despite this treatment. Different presentations of PTLD in transplant patients should also be kept in mind.