Epidemiology of Fabry disease in patients in hemodialysis in the Madrid community.

This study screened for Fabry disease (FD) in patients in hemodialysis (HD) in the region of Madrid (CAM) with a cross-sectional design to evaluate HD-prevalent patients, followed by a three-year period prospective design to analyze HD-incident patients. INCLUSION CRITERIA: patients older than 18 years on HD in the CAM, excluding patients diagnosed with any other hereditary disease with renal involvement different from FD, that sign the Informed Consent (IC). EXCLUSION CRITERIA: underaged patients or not agreeing or not being capable of signing the IC. RESULTS: 3470 patients were included, 63% males and with an average age of 67.9±9.7 years. 2357 were HD-prevalent patients and 1113 HD-incident patients. For HD-prevalent patients, average time in HD was 45.2 months (SD 51.3), in HD-incident patients proteinuria was present in 28.4%. There were no statistical differences in plasmatic alpha-galactosidase A (α-GAL-A) activity or Lyso-GL-3 values when comparing HD-prevalent and HD-incident populations and neither between males and females. A genetic study was performed in 87 patients (2.5% of patients): 60 male patients with decreased enzymatic activity and 27 female patients either with a decreased GLA activity, increased Lyso-Gl3 levels or both. The genetic variants identified were: p.Asp313Tyr (4 patients), p.Arg220Gln (3 patients) and M290I (1 patient). None of the identified variants is pathogenic. CONCLUSIONS: 76% of HD Centers of the CAM participated in the study. This is the first publication to describe the prevalence of FD in the HD-population of a region of Spain as well as its average α-GAL-A-activity and plasmatic Lyso-Gl3 levels. It is also the first study that combines a cross-sectional design with a prospective follow-up design. This study has not identified any FD patient.

Volumen renal total y función renal en pacientes nefrectomizados por neoplasias renales / Total renal volume and renal function after radical nephrectomy in renal neoplasm

Introducción: La reducción de la masa renal tras la nefrectomía radical en pacientes con neoplasias renales puede producir la hipertrofia compensadora del riñón contralateral. La capacidad de compensación determinará la evolución de la función renal. La medición del volumen renal total (VRT) del riñón remanente antes y después de la nefrectomía puede ayudar a evaluar la evolución de la función renal. Objetivos: Determinar la correlación entre el VRT pre y posnefrectomía con la función renal al año de seguimiento. Materiales y métodos: Estudio retrospectivo de observación en 47 pacientes adultos con neoplasias renales que fueron sometidos a nefrectomía radical. El VRT pre y posnefrectomía (al año de seguimiento) fue calculado mediante la ecuación de la elipsoide (TAC y/o RNM), que fueron comparados con datos clínicos y analíticos. Los resultados fueron analizados mediante regresión lineal uni y multivariante. Resultados:La mediana de edad al momento de la nefrectomía fue de 70 años (44-88). La mayoría fueron hombres (66%). El filtrado glomerular estimado (FGe) pre y posnefrectomía fue de 78 (40-100) y 53,3ml/min/m2 (20-90) respectivamente (p=0,01). El VRT pre y posnefrectomía fue de 168,2ml (100,4-257,2) y 187,8ml (115,5-273,1) respectivamente (p=0,001). El FGe prenefrectomía (β=0,62; p=0,034) y el VRT pre (β=1,08; p<0,0001) se correlacionaron positivamente con el VRT posnefrectomía. Sin embargo, el FGe al año se correlacionó negativamente (β=–1,18; p=0,047). Conclusiones: En pacientes con neoplasias renales tratados con nefrectomía radical la medición del VRT pre y posnefrectomía pueden ayudar a predecir la evolución de la función renal al año de seguimiento. (AU)

Introduction: The reduction of renal mass after radical nephrectomy (RN) for renal neoplasm, could be associated with compensatory hypertrophy of the contralateral kidney. The capacity of compensation will determine the renal function (RF) evolution. Measuring of total renal volume (TRV) of the remaining kidney pre and post RN can help assess the RF evolution. Objectives: To determine the correlation between TRV pre and post nephrectomy (a year of follow-up) with RF. Materials and methods: A retrospective cohort study was carried out in 47 patients who had undergone RN from 2014 to 2018, due to renal cell carcinoma (confirmed by histopathology). The TRV was calculated, pre and post (a year of follow-up) RN, using ellipsoid formula equation, which were compared with clinical and analytical data. The results were analyzed by multivariate linear logistic models. Results: The median age at the time of RN was 70 years old (range, 40-88 years). Most of them were men, 66%. The estimated glomerular filtration rate (eGFR) pre and post nephrectomy was 78 (40-100) and 53.3ml/min/ m2 (30-90) respectively (P=.01). The TRV pre and post-nephrectomy was 168.2ml (100.4-257.2) and 187.8ml (115.5-273.1) respectively (P=.001). The pre-nephrectomy eGFR (β=0.62; P=.034) and the TRV (β=1.08; P<.0001) were positively correlated with the post-nephrectomy TRV, while the eGFR at year of follow-up was correlated negatively (β=–1.18; P=.047) Conclusions: The measurement of pre and post nephrectomy TRV can help to predict renal function evolution at a year of follow-up. (AU)

Total renal volume and renal function after radical nephrectomy in renal neoplasm.

INTRODUCTION: The reduction of renal mass after radical nephrectomy (RN) for renal neoplasm, could be associated with compensatory hypertrophy of the contralateral kidney. The capacity of compensation will determine the renal function (RF) evolution. Measuring of total renal volume (TRV) of the remaining kidney pre and post RN can help assess the RF evolution. OBJECTIVES: To determine the correlation between TRV pre and post nephrectomy (a year of follow-up) with RF. MATERIALS AND METHODS: A retrospective cohort study was carried out in 47 patients who had undergone RN from 2014 to 2018, due to renal cell carcinoma (confirmed by histopathology). The TRV was calculated, pre and post (a year of follow-up) RN, using ellipsoid formula equation, which were compared with clinical and analytical data. The results were analyzed by multivariate linear logistic models. RESULTS: The median age at the time of RN was 70 years old (range, 40-88 years). Most of them were men, 66%. The estimated glomerular filtration rate (eGFR) pre and post nephrectomy was 78 (40-100) and 53.3ml/min/m2 (30-90) respectively (p=0.01). The TRV pre and post-nephrectomy was 168.2ml (100.4-257.2) and 187.8ml (115.5-273.1) respectively (p=0.001). The pre-nephrectomy eGFR (ß=0.62; p=0.034) and the TRV (ß=1.08; p<0.0001) were positively correlated with the post-nephrectomy TRV, while the eGFR at year of follow-up was correlated negatively (ß=-1.18; p=0.047). CONCLUSIONS: The measurement of pre and post nephrectomy TRV can help to predict renal function evolution at a year of follow-up.

Fracaso renal agudo anúrico persistente en paciente infectado con Plasmodium malariae: la importancia de la biopsia renal / Persistent acute renal failure in a patient infected with Plasmodium malariae: The importance of renal biopsy

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Incidence of Hypersensitivity Reactions During Hemodialysis.

BACKGROUND/AIMS: A recent alert from Spanish health authorities warned of a higher incidence of reported hypersensitivity reactions to hemodialysis membranes with polysulfone, in the 2017 review of acute reactions to dialyzers found only published reports in the 21st century on polysulfone and its derivatives. The aim is to assess/evaluate the current incidence and characteristics of hypersensitivity reactions in hemodialysis patients. METHODS: A retrospective multicentre study in 9 Spanish hospitals evaluated patients in whom a hypersensitivity reaction required a change in dialyzer membrane. RESULTS: A total of 37 patients out of 1561 (2.37%) had hypersensitivity reactions and clinical, epidemiological and analytical data were available for 33 patients (2.11%). The membranes involved were polysulfone (n=23), polynephron (n=8), polyethersulfone (n=1) and polyacrylonitrile (n=1). This distribution reflected the frequency of use of membranes in the participating dialysis units. The reactions were described as type A in 18 cases and type B in 15 cases. There were no significant differences between the two types in clinical symptoms, the composition of the membrane involved, the method of sterilization, the season, or the time during the session in which they occurred. The most frequent symptom was dyspnea/breathlessness (64% of reactions). Eosinophilia was common (74%). 54% of the reactions occurred within the first 30 minutes of hemodialysis, 64% occurred during the first year of dialysis, and 54% required discontinuation of dialysis session. Cellulose triacetate was used as an alternative dialyzer in 78% of the cases. CONCLUSION: The incidence of hypersensitivity reactions was in the range found in reports from 20 years ago and is observed associated with synthetic membranes, not just polysulfones. Cellulose triacetate appears to be a good alternative for these patients.

Rituximab treatment of adult patients with steroid-resistant focal segmental glomerulosclerosis.

BACKGROUND AND OBJECTIVES: Isolated case reports have shown a beneficial effect of rituximab on pediatric patients with primary FSGS, but there is no information about rituximab treatment of FSGS in adults. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: All patients who had biopsy-proven FSGS and were treated with rituximab in Spain were identified, independent of their positive or negative response, among the nephrology departments that belong to the Spanish Group for the Study of Glomerular Diseases (GLOSEN). Their characteristics and outcome after rituximab treatment were studied. RESULTS: Eight patients were identified. Rituximab failed to improve nephrotic syndrome in five of eight patients, who continued to show massive proteinuria and exhibited a rapidly deteriorating renal function in two cases. Among the remaining three patients, two of them showed an improvement of renal function and a remarkable proteinuria reduction and one experienced a beneficial but transitory effect after rituximab. There were no differences in clinical or laboratory characteristics or in the CD20 B lymphocyte count after rituximab between these three patients and the five who had a negative response. The only difference was in the regimen of rituximab administration: Whereas the five patients with a negative response received only four weekly consecutive infusions of 375 mg/m(2), the three remaining patients received additional doses of rituximab. CONCLUSIONS: Only a minority (three of eight) of patients in our series of adult patients with FSGS showed a positive influence of rituximab. More studies are necessary to characterize further the optimal dosages and the mechanisms of action of rituximab in FSGS.

Severe everolimus-associated pneumonitis in a renal transplant recipient.

Inhibitors of mTOR (mammalian target of rapamycin) are immunosuppressants with less nephrotoxic potential than calcineurin inhibitors and antiproliferative effects, which are advantageous in the case of malignancy. However, a series of adverse events has been reported with the first-generation mTOR inhibitor sirolimus that includes hypersensitivity-like interstitial pneumonitis. To our knowledge, only one case of a pneumonitis associated with everolimus in a heart transplant patient has been reported, and it was related to elevated trough blood levels. We report herein the first case of a kidney graft recipient who developed everolimus-associated pneumonitis with normal trough blood levels that was completely reversed after drug withdrawal.

Emphysematous pyelonephritis in dialysis patient after embolization of failed allograft.

Emphysematous pyelonephritis is an uncommon acute infection characterized by the presence of gas in the renal parenchyma. Diabetics account for most cases, and the mortality rate is high. We report a case of emphysematous pyelonephritis after therapeutic embolization of a nonfunctioning renal graft in a nondiabetic dialysis patient. Given the increasing popularity of therapeutic embolization to control graft intolerance syndrome associated with rejected kidneys, physicians should be aware of this potentially severe complication. We discuss the differential diagnosis from entities requiring different management strategies, such as postembolization syndrome, persistence of graft intolerance, and the presence of sterile intrarenal.