Renin-angiotensin system activation: may it increase frequency of obstructive sleep apnea in patients with autosomal dominant polycystic kidney disease?

PURPOSE: Renin-angiotensin system (RAS) hyperactivity is a common entity in both autosomal dominant polycystic kidney disease (ADPKD) and obstructive sleep apnea (OSA). We aimed to investigate the frequency of OSA in adults with ADPKD either with stages 3-4 or stages 1-2 chronic kidney disease (CKD) and evaluate the effect of RAS blockade on OSA in these patients. METHODS: This is a comparative, prospective, two-center clinical study. Eligible patients with ADPKD were enrolled in a polysomnography (PSG) study. Presence of OSA in patients with ADPKD was compared with individuals who underwent polisomnography study due to OSA symptoms. A subgroup analysis was performed in terms of the presence of OSA in ADPKD with eGFR values lower or higher than 60 ml/min/1.73 m2 (stages 3-4 and stages 1-2 CKD, respectively). RESULTS: Frequency of OSA (65%) was higher than in the general population and similar between the two groups (p = 0.367). Patients with ADPKD and eGFR ≥ 60 ml/min/1.73 m2 presented a similar frequency of OSA to the control group (p = 0.759). However, OSA was significantly more frequent in ADPKD with eGFR < 60 ml/min/1.73 m2 (p = 0.018). Subgroup analysis revealed that presence of OSA also was significantly higher in ADPKD with lower eGFR levels (eGFR < 60 ml/min/1.73 m2 and eGFR > 60 ml/min/1.73 m2) 14/17 (82%) and 12/23 (52%), respectively (p: 0.048). CONCLUSION: As kidney disease progresses, uremia and related factors of renal failure rather than RAS activation seem to play a more important role for the development of OSA in patients with ADPKD.

A prospective study of living kidney donors: 6 years follow-up from a cardiovascular disease risk perspective

SUMMARY OBJECTIVE: The purpose of this prospective study was to evaluate the clinical, laboratory, and donation-specific outcomes of living kidney donors 6 years after donation. METHODS: We included a total of 93 kidney donors and 54 age- and sex-matched individuals as control group through a type 2 cohort consecutive recruitment. We detected kidney function abnormalities and the presence of hypertension, diabetes, and cardiovascular events during the 6 years follow-up period. RESULTS: The mean serum creatinine levels were higher (p<0.001), and the estimated glomerular filtration rate levels were lower (p<0.001) in living kidney donors 6 years after donation when compared with controls. The protein/creatinine ratio of the study population was also higher (p=0.014). There was no difference in outcomes between the groups for end-stage kidney disease and cardiovascular mortality. A higher rate of new-onset hypertension (6.4 vs. 32.9%), diabetes mellitus (0.0 vs. 4.3%), chronic kidney disease (0.0 vs. 2.1%), and cardiovascular disease (0.0 vs. 2.1%) was demonstrated among donors 6 years after donation (p<0.001, respectively). CONCLUSION: Our data have demonstrated that the reduction in Glomerular filtration rate induced by kidney donation might cause an increase in adverse renal and cardiovascular events.

Trends of primary glomerular disease in Turkey: TSN-GOLD registry report.

BACKGROUND: Although several renal biopsy registry reports have been published worldwide, there are no data on primary glomerular disease trends in Turkey. METHODS: Three thousand eight-hundred fifty-eight native kidney biopsy records were assessed in the Turkish Society of Nephrology Primary Glomerulopathy Working Group (TSN-GOLD) Registry. Secondary disease and transplant biopsies were not recorded in the registry. These records were divided into four periods, before 2009, 2009 to 2013, 2013-2017, and 2017-current. RESULTS: A total of 3858 patients (43.6% female, 6.8% elderly) were examined. Nephrotic syndrome was the most common biopsy indication in all periods (58.6%, 53%, 44.1%, 51.6%, respectively). In the whole cohort, IgA nephropathy (IgAN) (25.7%) was the most common PGN with male predominance (62.7%), and IgAN frequency steadily increased through the periods (× 2 = 198, p < 0.001). MGN was the most common nephropathy in the elderly (> 65 years), and there was no trend in this age group. An increasing trend was seen in the frequency of overweight patients (× 2 = 37, p < 0.0001). Although the biopsy rate performed with interventional radiology gradually increased, the mean glomeruli count in the samples did not change over the periods. CONCLUSIONS: In Turkey, IgAN is the most common primary glomerulonephritis, and the frequency of this is increasing.

Multidisciplinary approach to Fabry disease: from the eye of a neurologist.

Fabry Disease (FD) is an X-linked lysosomal storage disease that emerges as a result of the mutations in the galactosidase A gene encoding alpha-galactosidase. The peripheral nervous system (PNS) involvement manifests itself as acroparesthetic complaints due to the small-fiber involvement. Our goal was to assess the PNS involvement of 14 patients with FD both clinically and electrophysiologically besides the other systemic features. 14 patients (11 female and 3 male) of the same family whose enzyme level and genetic mutation analysis confirmed the FD diagnosis were evaluated retrospectively in terms of systemic and neurological findings of the FD. Neurological examination and nerve conduction studies were performed to evaluate the PNS involvement. PNS involvement was more common in females. Eight of the patients had acroparesthesia. The neurological examinations of all patients were normal. Two patients presented sensory axonal polyneuropathy, one of whom had no acroparesthesia. Other patients with acroparesthesia had normal nerve conduction studies. There was no significant relationship between the presence of acroparesthesia and the results of conduction studies (p > 0.05). Acroparesthetic complaints in patients with normal results were attributed to small-fiber involvement. Since small-diameter nerve fibers cannot be evaluated by routine conduction studies, especially in the early stages of FD, these studies may be normal. Early diagnosis through the symptoms such as acroparesthesia may contribute to the survival of the patient by preventing and/or delaying the development of renal, cardiac, and cerebrovascular diseases, which are the main causes of morbidity and mortality.

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.

A randomized study to assess the efficacy of herbal product to prevent cisplatin-induced nephrotoxicity in a rat model.

OBJECTIVES: This study aimed to investigate the protective effect and antioxidant activity of an herbal product that made from multiple plants in a rat model of kidney dysfunction induced by intraperitoneal cisplatin. MATERIALS AND METHODS: Twenty-four rats were divided into four different groups namely: Group 1 - control healthy animals without any specific medication, Group 2 - Herbal product only 5 mg/kg, Group 3 - cisplatin only and Group 4 - Herbal product 5 mg/kg + cisplatin. RESULTS: Evaluation of our findings demonstrated a significant (p = 0.017) reduction in Catalase activities and a significant increase (p = 0.001) in renal tissue Malondialdehyde levels in cisplatin- treated rats when compared with the control group. Also, Glutathion and Glutathione peroxidase content revealed significant (p = 0.031) reduction in renal tissues of cisplatintreated rats compared with the control group. Pre-treatment of rats with the herbal product ameliorated these cisplatininduced changes of the antioxidant enzymes. No statistically significant changes were demonstrated in Superoxide dismutase activities in the tissue specimens of any group. CONCLUSIONS: This potent antioxidant herbal medicine was found to have potential antioxidant activity, which may in turn to be effective in the protection of kidney tissue resulting from cisplatin application. Therefore, much attention should be given to the possible role of natural dietary antioxidants for protecting the kidney.