Thirst intensity survey in ADPKD patients.

INTRODUCTION: With increased fluid intake and tolvaptan treatment, the growth rate of cysts can be theoretically decelerated in autosomal polycystic kidney disease. In this prospective study, it was planned to evaluate thirst sensation in these patients and the parameters affecting its intensity. METHODS: Forty-one ADPKD patients on tolvaptan and 40 ADPKD patients not on tolvaptan as the control group were evaluated for thirst distress sensation and intensity. The feeling of thirst and the discomfort caused by excessive fluid intake was assessed with Thirst Distress Scale-HF 12 questions (60/12). Thirst intensity was evaluated with a 100 mm visual scale. RESULTS: Of the whole group, 35.8% (29) were males, and 64.2% (52) were females. The mean age of the tolvaptan group was 39.17 ± 9.35 years and for the control group, it was 41.95 ± 12.29 years. There was a negative correlation between the thirst distress score of the patients and an increase in creatinine level after a year of tolvaptan treatment (r = - 0.335, p = 0.035). The patients not taking thiazide had higher thirst intensity scores (p = 0.004). There was no impact of tolvaptan dosage, total kidney volume, serum sodium, urinary osmolarity or eGFR on thirst distress and thirst intensity scores. DISCUSSION/CONCLUSION: Only thiazide co-treatment had a positive impact on thirst distress and intensity when given tolvaptan. Thirst Distress Scale for ADPKD patients can be used to classify patients before and during tolvaptan treatment.

Characteristics and outcomes of hospitalised older patients with chronic kidney disease and COVID-19: A multicenter nationwide controlled study.

OBJECTIVE: Older adults with co-morbidities have been reported to be at higher risk for adverse outcomes of coronavirus disease 2019 (COVID-19). The characteristics of COVID-19 in older patients and its clinical outcomes in different kidney disease groups are not well known. METHODS: Data were retrieved from a national multicentric database supported by Turkish Society of Nephrology, which consists of retrospectively collected data between 17 April 2020 and 31 December 2020. Hospitalised patients aged 18 years or older with confirmed COVID-19 diagnosis suffering from stage 3-5 chronic kidney disease (CKD) or on maintenance haemodialysis (HD) treatment were included in the database. Non-uraemic hospitalised patients with COVID-19 were also included as the control group. RESULTS: We included 879 patients [388 (44.1%) female, median age: 63 (IQR: 50-73) years]. The percentage of older patients in the CKD group was 68.8% (n = 188/273), in the HD group was 49.0% (n = 150/306) and in the control group was 30.4% (n = 70/300). Co-morbidities were higher in the CKD and HD groups. The rate of presentation with severe-critical disease was higher in the older CKD and HD groups (43.6%, 55.3% and 16.1%, respectively). Among older patients, the intensive care unit (ICU) admission rate was significantly higher in the CKD and HD groups than in the control group (38.8%, 37.3% and 15.7%, respectively). In-hospital mortality or death and/or ICU admission rates in the older group were significantly higher in the CKD (29.3% and 39.4%) and HD groups (26.7% and 30.1%) compared with the control group (8.6% and 17.1%). In the multivariate analysis, in-hospital mortality rates in CKD and HD groups were higher than control group [hazard ratio (HR): 4.33 (95% confidence interval [CI]: 1.53-12.26) and HR: 3.09 (95% CI: 1.04-9.17), respectively]. CONCLUSION: Among older COVID-19 patients, in-hospital mortality is significantly higher in those with stage 3-5 CKD and on maintenance HD than older patients without CKD regardless of demographic characteristics, co-morbidities, clinical and laboratory data on admission.

Severe Metabolic Acidosis, Acute Renal Failure, and Delayed Paralysis Leading to Respiratory Depression due to Manganese ethylene-bis-dithiocarbamate.

Maneb is a widely used agricultural fungicide, which can lead to parkinsonism due to its neurotoxic effects on the dopaminergic system after chronic low-dose exposure. Previous acute human maneb poisoning cases occurred with low-dose maneb exposure through the dermal route causing renal failure. This report presents a case of acute renal failure and delayed paralysis due to ingestion of a large dose of maneb for a suicide attempt. A 16-year-old female patient was admitted to the emergency room because of drinking almost a whole bottle of maneb (400 mL [2 g L-1]) about 2 hours before. The patient was transferred to the intensive care unit with severe metabolic acidosis and renal failure. On the 4th day in intensive care unit, although the severe acidosis was resolved with haemodialysis, the patient was intubated because of ascending muscle weakness and dyspnoea. After staying in the intensive care unit for 9 days and in the nephrology ward for 2 weeks, the patient was discharged well from the hospital with no further need for haemodialysis but a persistent bilateral drop foot. One year after the event, renal functions were normal, and motor function in the lower extremities improved entirely.

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.