Angiotensin converting enzyme inhibitors (ACEi) are widely used for the treatment of multiple conditions such as hypertension, heart failure and chronic kidney disease. Angioedema is a rare but potentially fatal complication of ACEi use and unilateral tongue edema is a very rare presentation. We report a case of a 55-year-old man, with a history of hypertension, on enalapril for three years, who presented to the hospital with unilateral tongue swelling, without airway compromise. Other causes were excluded and the diagnosis of angioedema due to enalapril was established. The patient was discharged with discontinuation of ACEi with total resolution of symptoms and without relapse after several months. Although very rare, unilateral tongue swelling should be considered in the presentation of angioedema associated with ACEi. Tight surveillance is important to prevent fatal complications such as airway obstruction. ACEi discontinuation is crucial to avoid clinical relapse.

Background and Aims Minimal change disease (MCD) is the main cause of nephrotic syndrome in pediatric age and it is responsible for 10-25% of the cases in adults. It is characterized by minimal glomerular abnormalities in light microscopy, usually without immunoglobulins or complement deposits in immunofluorescence (IF). There is a subgroup of patients that presents with IgM deposition in IF, which has been described as a more aggressive disease, with frequent relapses [1], dependence of steroids [2] and worse outcomes. Method This is a retrospective study that analyses the follow up of 50 adult MCD patients in the nephrology consult, with diagnosis in childhood or adulthood. They had renal biopsies performed between 2009 and 2022. We did not include inappropriate biopsy samples, biopsies without IF or positive for C1q. We compared the characteristics between IgM positive (IgM+) and IgM negative (IgM-) patients at diagnosis, as well as the number of relapses per year, age of presentation and association with steroid-dependence or steroid-resistance. Results There were 23 (46%) patients in the IgM+ group and 27 (54%) in the IgM- group, with a mean follow-up of 10.2± 7.8 years. The IgM- subgroup was older at biopsy time (52 (30-74) years vs IgM+ 15 (13-23) years; p = 0.001) and this is probably related with the age of disease presentation, which was younger in the IgM+ group (12 (5-16) years vs IgM- 52 (73-13) years; p = 0.001). The groups were similar in sex distribution (IgM+ male n = 15 (55.6%) vs IgM- male n = 10 (44.0%); p = 0.395). There were no differences in diabetes and dyslipidemia frequencies between the two groups (Diabetes: IgM+ n = 1 (4.3%), IgM- n = 3 (11.3%), p = 0.614; Dyslipidemia: IgM+ n = 7 (30.4%), IgM- n = 7 (25.9%), p = 0.723), but the IgM- group had more patients with hypertension (IgM+ n = 2 (8.7%), IgM- n = 9 (33%), p = 0.046). The estimated glomerular filtration rate based on creatinine (eGFRcr) at biopsy time was higher in the IgM+ group (124 (104-130) ml/min/1.73 m2 vs IgM- with 96 (84-111) ml/min/1.73 m2; p = 0.003), but this happens probably due to the age as a confounding variable. In regard to the number of relapses per year, the IgM+ group had significantly more relapses than the IgM- group (IgM+ with 0.61 (0.27-1.00) relapses per year; IgM- with 0.17 (0.01-0.65) relapses per year; p = 0.011). The steroid-dependence (IgM+ n = 15 (65%); IgM- n = 11 (47%); p = 0.084) and the steroid-resistance (IgM+ n = 6 (26%); IgM- n = 3 (11%); p = 0.270) isolated outcomes were not significantly different in the two groups, but when we analyze the composed outcome of steroid-dependence plus steroid-resistance we have worse outcomes in the IgM+ subgroup (IgM+ n = 21 (91.3%); IgM- n = 14 (51.8%); p = 0.04; ORR 9.72; IC 95% 2.37-33.33). An IgM+ patient has 9.7 times the odd of being steroid-dependent or steroid-resistant of an IgM- patient. From our 50 patients, 3 evolved to stage 5 chronic kidney disease with dialysis dependency, being all of those patients IgM+. One of those patients had a recurrency of MCD after kidney transplant. There were 3 deaths with non-related to kidney disease causes, 1 in the IgM+ and 2 in the IgM- groups. Genetic test was performed in 5 of the IgM+ patients, with 4 positive results for mutations. Only 2 IgM- patients did genetic test, all without mutation´s identification. Conclusion This study supports the evidence that MCD patients with IgM+ biopsies have younger age MCD's presentation, more relapses per year and more steroid dependence plus resistance. Dialysis was started in 3 IgM+ patients and this group seems to have higher frequency of genetic mutations. This suggests that genetic testing could be important for future prognosis prediction and treatment options in the IgM+ patients, but further studies with a bigger study population need to be done to establish this relation.

Background and Aims Chronic kidney disease (CKD) prevalence has been increasing and estimates show it will be the fifth leading cause of lost life years worldwide by 2040. CKD patients should be evaluated by Nephrology even in early stages of disease, but patients substantially outnumber nephrologists. Telemedicine consists of delivery of health-care services and information from distant locations and its utility has been largely demonstrated in CKD patients. In our centre, teleconsultation began being used to support requests from family doctors regarding CKD patients that are at that point stable and in an early stage of their disease. Method Since January 2020 and December 2022, 456 patients were evaluated using teleconsultation. Their demographical characteristics and the savings on dislocation cost, based on the price per kilometre, were analysed. In addition, a questionnaire about the use and utility of telenephrology was shared through general practice centres. Results From 456 patients, 43% male and 57% female, with an average age of 78 years. One out of five patients were dependent for daily life activities. On average, the patients lived within 22kms of the hospital, the maximal distance was 540kms. Based on this data we estimate 6660 euros savings on dislocation costs alone. A total of 47 general medicine doctors answered the survey, out of which 21% were male, with an average age of 40 years, 23% had under 5 years of experience, 26% had between 5 and 10 years of experience, 26% between 11 and 15 and the remaining over 16 years of experience. Around 70% had never used the teleconsultation, the majority of which due to lack of knowledge of this possibility. In the group that had used it, 77% were satisfied with the patient's orientation and 85% would consider using it again. Most of the general practitioners (62%) had referenced patients between 2 and 5 times. A fast answer to the request was the most frequently pointed advantage and the time between request and patient orientation was considered appropriate. Conclusion From this data, we conclude that telenephrology is not well known among family doctors in Portugal, yet it has the potential to reduce the distance between patients and nephrologists and build bridges between different areas of specialisation. It will also allow for cost reduction, a matter more pressing each day, and to increase patients comfort by reducing unnecessary hospital visits.

Background and Aims Lupus nephritis is a well know manifestation of systemic lupus erythematosus (SLE) and it can progress to end stage kidney disease (ESKD) in 10-30% of patients [1]. Several studies have been conducted to find out whether proteinuria detected earlier in the course of lupus nephritis is associated with worse kidney related outcomes, such as progression to chronic kidney disease [2]. Method We retrospectively reviewed a cohort of 67 patients referenced to our center with the diagnosis of lupus nephritis until 2019. We evaluated proteinuria (g/g) in a random urine sample measured 12 months after diagnosis and beginning of treatment of lupus nephritis and divided patients into two groups: those with proteinuria less than or equal to 0.3 g/g– group 1(n = 11; 35%) and those with proteinuria superior to 0.3 g/g– group 2 (n = 20; 65%) that had measures of our variables of interest. These groups were compared regarding a continuous variable: serum creatinine measured in the 7th year of follow up, as evaluated in previous studies [3]. We compared the median of serum creatinine values between group 1 and group 2 using Mann-Whitney test, due to the absence of normal distribution of serum creatinine. We also compared the median of serum creatinine in the 7th year to proteinuria measured at 3 and 6 months. Results Mean follow up was 13.36 ± 7.8 years and 81.7% (n = 57) were women, with a median age of 33.0 ±12.85 years. A total of 8 patients (11.9%) progressed to ESKD defined by the need for renal replacement therapy or being submitted to renal transplantation. Only 1 patient died due to a nosocomial infection. We had a sample of 31 patients, 26 women and 5 men (p = 0.591) (missing n = 36). Baseline median serum creatinine in group 1 was 0,95 mg/dl and in group 2 was 0.78 mg/dl (p = 0.086). We obtained a median serum creatinine of 0.69 mg/dl (Interquartile range (IQR): 0.61-0.87) in group 1 versus0.92 mg/dl (IQR: 0.66-1.25) in group 2 (p = 0.04). Medians of serum creatinine in the 7th year regarding proteinuria measured at 3 and 6 months were not significantly different (p>0.05). Conclusion In our cohort, the group with a higher persistent proteinuria (> 0.3 g/g), one year after lupus nephritis diagnosis, had a significantly higher serum creatinine (7 years after diagnosis). Although previous studies have shown that reducing proteinuria to less than 0.5–0.8g/d 3 by 1 year of treatment predicts good long-term kidney outcomes, our particular data suggest that further reducing to less than 0.3 g/g may provide additional benefit in the long run [2], even though, due to our reduced sample, more investigation is required. From the above mentioned, one may consider 12 months proteinuria as a potential predictor to long-term renal outcomes with lupus nephritis. More studies are necessary to validate this hypothesis.