Long-term remission with rituximab in a patient with severe hepatitis C virus-induced mixed cryoglobulinemia.

Mixed cryoglobulinemia (MC) is a small-vessel systemic vasculitis characterized by the presence of cryoglobulins, immunoglobulin complexes that precipitate at low temperatures ( < 37 ºC) inducing the inflammatory process. The most frequent etiology is hepatitis C infection (HCV) (1). Rituximab (RTX), an anti-CD20 monoclonal antibody, has recently emerged as the treatment of choice for severe MC (2). We present a case of severe hepatitis C virus-induced MC that was controlled and maintained in remission with RTX for 26 months, a remarkable prolonged period of time.

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.

More difficult still: Treating severe rapidly progressive glomerulonephritis in the context of COVID-19 pneumonia.

We present the case of a male patient with severe SARS-CoV-2 pneumonia, with simultaneous onset of p-ANCA positive rapidly progressive glomerulonephritis. We discuss the different therapeutic possibilities, emphasising the appropriateness of their administration according to the time in the course of the infection.

More difficult still: Treating severe rapidly progressive glomerulonephritis in the context of COVID-19 pneumonia. / Más difícil todavía: tratar una glomerulonefritis rápidamente progresiva grave en el seno de una neumonía por COVID-19.

We present the case of a male patient with severe SARS-CoV-2 pneumonia, with simultaneous onset of p-ANCA positive rapidly progressive glomerulonephritis. We discuss the different therapeutic possibilities, emphasising the appropriateness of their administration according to the time in the course of the infection.

Immunosuppression as a trigger for hyperinflammatory syndrome due to Strongyloides stercolaris in membranous nephropathy. / Inmunosupresión como desencadenante de un síndrome de hiperinfestación por Strongyloides stercolaris en la nefropatía membranosa.

The relationship between parasites and glomerulonephritis (GN) is well documented in certain parasitoses, but not in cases of Strongyloides stercolaris (S. stercolaris) where there are few cases described being the majority GN of minimal changes. We report a case of hyperinfestation by S. stercolaris in a patient affected by a membranous GN treated with oral corticosteroids with fatal outcome for the patient. This case provides a double teaching: first about a rare association of strongyloid and membranous GN and second about the importance of establishing a diagnosis of suspected and appropriate treatment for certain infections or diseases with little clinical expression before starting any immunosuppressive treatment.

What is the optimum dialysate flow in post-dilution online haemodiafiltration?

INTRODUCTION: In post-dilution online hemodiafiltration (OL-HDF), the only recommendation concerning the dialysate, or dialysis fluid, refers to its purity. No study has yet determined whether using a high dialysate flow (Qd) is useful for increasing Kt or ultrafiltration-infusion volume. OBJECTIVE: Study the influence of Qd on Kt and on infusion volume in OL-HDF. MATERIAL AND METHODS: This was a prospective crossover study. There were 37 patients to whom 6 sessions of OL-HDF were administered at 3 different Qds: 500, 600 and 700ml/min. A 5008(®) monitor was used for the dialysis in 21 patients, while an AK-200(®) was used in 17. The dialysers used were: 20 with FX 800(®) and 17 with Polyflux-210(®). The rest of the parameters were kept constant. Monitor data collected were effective blood flow, effective dialysis time, final Kt and infused volume. RESULTS: We found that using a Qd of 600 or 700ml/min increased Kt by 1.7% compared to using a Qd of 500ml/min. Differences in infusion volume were not significant. Increasing Qd from 500ml/min to 600 and 700ml/min increased dialysate consumption by 20% and 40%, respectively. CONCLUSIONS: With the monitors and dialysers currently used in OL-HDF, a Qd higher than 500ml/min is unhelpful for increasing the efficacy of Kt or infusion volume. Consequently, using a high Qd wastes water, a truly important resource both from the ecological and economic points of view.

Sodium set-point in haemodialysis: is it what we see clinically?

BACKGROUND: Natraemia in haemodialysis (HD) patients is considered constant contrary to daily clinical observations. Its relationship with clinical parameters, dialysis parameters and body water (BW) distribution is not clear. OBJECTIVES: The aims of this study were to know 1) the intraindividual variability of natraemia, 2) the relationship between natraemia and clinical and dialysis parameters and 3) the relationship between natraemia and BW distribution by bioimpedance. MATERIAL AND METHOD: Observational retrospective study on 98 chronic HD patients. Clinical, HD and natraemia, glucose and bioimpedance data were collected. RESULTS: We included 63 males and 35 females of 69.6 (21-91) years of age, with a follow-up of 23.2 (10) months. Variability: 1802 sodium measurements: mean natraemia 138 (3.2) mEq/l and corrected for glucose: 139.1 (3.6) mEq/l, p<.0001. Intraindividual coefficient of variation (CV) was 2% (0.8) (range 1-5.6%) and it correlated negatively with natraemia (r=-0.63, p<.0001). Clinical parameters: corrected natraemia was lower in diabetics than in non-diabetics 138 (2.4) compared with 139 (2) mEq/l, p<.003, CV 2.3 (0.9) compared with 1.9 (0.7)% (p<.01) and SD 3.2 (1.2) compared with 2.5 (0.9) mEq/l (p<.04). No differences according to gender, age, HD time, cardiac or liver disease, medication use, residual renal function or mortality were found. HD parameters: a positive relationship was found between natraemia and total dialysate conductivity and it was negative with interdialysis weight gain (IDG). - Bioimpedance: no relationship was found between natraemia and BW distribution. CONCLUSIONS: Natraemia varies in each patient and is related positively with conductivity and negatively with IDG. In diabetics natraemia is lower and CV is higher. There is no relationship between natraemia and BW distribution.

Pregnancy in women on chronic dialysis: a review.

The frequency of pregnancy in women on dialysis is extremely low, but the percentage of successful pregnancies in this context has increased over the years, with some studies placing the survival rate above 70%. These pregnancies are not exempt from both maternal and foetal complications, and so their management requires the joint efforts of nephrologists, gynaecologists, nurses, and nutritionists. Currently, we have been unable to establish consistent systematic treatment from both nephrological and gynaecological specialists in these patients. The main changes that need to be made are: increased time on dialysis, maintaining low levels of pre-dialysis urea, avoiding: maternal hypertension and hypotension, anaemia, urinary tract infections, and fluctuations in electrolytes. Adequate foetal monitoring is also necessary.