The sodA, tuf, and 16S rRNA genes were very useful for CoNS identification. Each has its own characteristics of similarity, discriminative power, and inclusion in databases.

Patient: Male, 52Final Diagnosis: Fahr’s syndromeSymptoms: SeizureMedication: —Clinical Procedure: —Specialty: Endocrinology and MetabolicObjective:Rare diseaseBackground:Idiopathic basal ganglia calcification, also known as Fahr’s disease or Fahr’s syndrome, is a rare neurological disorder characterized by abnormal calcified deposits in the basal ganglia. Here, we report a case of Fahr’s syndrome with calcification of the basal ganglia due to hypoparathyroidism in a patient with seizures.Case Report:A 52-year-old male patient visited our clinic with seizures. Brain computed tomography (CT) showed bilateral symmetrical calcifications in cerebellar white matter, the corpus striatum, the posterior thalami, and the centrum semiovale of both cerebral hemispheres. He had symptoms of hypocalcemia and low parathyroid hormone levels. The patient was diagnosed with Fahr’s syndrome due to primary hypoparathyroidism. He underwent calcium supplementation and calcifediol treatment. His symptoms improved, and he was discharged from the hospital.Conclusions:In patients with hypocalcemia accompanied by parathyroid dysfunction, neurological examination and CT should be performed to confirm abnormal intracranial calcification.

Backgound: This study evaluated whether the hydration status affected health-related quality of life (HRQOL) during 12 months in peritoneal dialysis (PD) patients.Methods: The hydration status and the HRQOL were examined at baseline and after 12 months using a bioimpedance spectroscopy and Kidney Disease Quality of Life-Short Form, respectively in PD patients. Four hundred eighty-one patients were included and divided according to the baseline overhydration (OH) value; normohydration group (NH group, -2L≤ OH ≤+2L, n=266) and overhydration group (OH group, OH >+2L, n=215). Baseline HRQOL scores were compared between the two groups. The subjects were re-stratified into quartiles according to the OH difference (OH value at baseline - OH value at 12 months; <-1, -1 - -0.1, -0.1 - +1, and ≥+1L). The relations of OH difference with HRQOL scores at 12 months and the association of OH difference with the HRQOL score difference (HRQOL score at baseline - HRQOL score at 12 months) were assessed.Results: The OH group showed significantly lower baseline physical and mental health scores (PCS and MCS), and kidney disease component scores (KDCS) compared with the NH group (all, P<0.01). At 12 months, the adjusted PCS, MCS, and KDCS significantly increased as the OH difference quartiles increased (P<0.001, P=0.002, P<0.001, respectively). In multivariate analysis, the OH difference was independently associated with higher PCS (β = 2.04, P< .001), MCS (β=1.02, P=0.002), and KDCS (β=1.06, P<0.001) at 12 months. The OH difference was independently associated with the PCS difference (β = -1.81, P<0.001), MCS difference (β=-0.92, P=0.01), and KDCS difference (β=-0.90, P=0.001).Conclusion: The hydration status was associated with HRQOL and increased hydration status negatively affected HRQOL after 12 months in PD patients.

Background and Purpose The aim of this study was to evaluate the structural and functional connectivities of brain network using graph theoretical analysis in neurologically asymptomatic patients with end-stage renal disease (ESRD). We further investigated the prevalence of cognitive impairment (CI) in ESRD patients and analyzed the association between network measures of brain connectivity and cognitive function. Methods We prospectively enrolled 40 neurologically asymptomatic ESRD patients, 40 healthy controls, and 20 disease controls. All of the subjects underwent diffusion-tensor imaging (DTI) and resting-state functional magnetic resonance imaging (rs-fMRI). We calculated measures of structural and functional connectivities based on DTI and rs-fMRI, respectively, and investigated differences therein between the ESRD patients and the healthy controls. We assessed cognitive function in the ESRD patients using the Korean version of the Consortium to Establish a Registry for Alzheimer's Disease neuropsychological battery. Results The ESRD patients exhibited decreased global structural and functional brain connectivities, as well as alterations of network hubs compared to the healthy controls and disease controls. About 70% of the ESRD patients had CI. Moreover, ESRD patients without CI exhibited decreased global connectivity and alterations of network hubs. Furthermore, there was a significant positive association between measures of brain connectivity and cognitive function. Conclusions We found that ESRD patients exhibited decreased structural and functional brain connectivities, and that there was a significant association between brain connectivity and cognitive function. These alterations in the brain network may contribute to the pathophysiological mechanism of CI in ESRD patients.

BackgroundAppropriate immunosuppressive therapy for patients with idiopathic membranous nephropathy (MN) remains controversial. The effect of mycophenolate mofetil (MMF) versus cyclosporine (CsA) combined with low-dose corticosteroids was evaluated in patients with idiopathic MN in a multi-center randomized trial (NCT01282073).MethodsA total of 39 biopsy-proven idiopathic MN patients with severe proteinuria were randomly assigned to receive MMF combined with low-dose corticosteroids (MMF group) versus CsA combined with low-dose corticosteroids (CsA group), respectively, and followed up for 48 weeks. Complete or partial remission rate of proteinuria and estimated glomerular filtration rate (eGFR) at 48 weeks were compared.ResultsThe level of proteinuria at baseline and at 48 weeks was 8.9 ± 5.9 and 2.1 ± 3.1 g/day, respectively, in the MMF group compared to 8.4 ± 3.5 and 3.2 ± 5.7 g/day, respectively, in the CsA group. In total, 76.1% of the MMF group and 66.7% of the CsA group achieved remission at 48 weeks (95% confidence interval, −0.18 to 0.38). There was no difference in eGFR between the two groups. Anti-phospholipase A2 receptor Ab levels at baseline decreased at 48 weeks in the complete or partial remission group (P = 0.001), but were unchanged in the no-response group. There were no significant differences between the two groups in changes in the Gastrointestinal Symptom Rating Scale and Gastrointestinal Quality of Life Index scores from baseline to 48 weeks.ConclusionIn combination with low-dose corticosteroids, the effect of MMF may not be inferior to that of CsA in patients with idiopathic MN, with similar adverse effects including gastrointestinal symptoms.Trial RegistrationClinicalTrials.gov Identifier: NCT01282073

Objectives The aim of this study was to investigate alterations in structural and functional brain connectivity between patients with end‐stage renal disease (ESRD) who were undergoing peritoneal dialysis (PD) and hemodialysis (HD). Methods We enrolled 40 patients with ESRD who were undergoing PD (20 patients) and HD (20 patients). We also enrolled healthy participants as a control group. All of the subjects underwent diffusion tensor imaging (DTI) and resting‐state functional magnetic resonance imaging (rs‐fMRI). Using data from the structural and functional connectivity matrix based on DTI and rs‐fMRI, we calculated several network measures using graph theoretical analysis. Results The measures of global structural connectivity were significantly different between the patients with ESRD who were undergoing PD and healthy subjects. The global efficiency and local efficiency in the patients with PD were significantly decreased compared with those in healthy participants. However, all of the measures of global structural connectivity in the patients with HD were not different from those in healthy participants. Conversely, in the global functional connectivity, the characteristic path length was significantly increased and the small‐worldness index was decreased in patients with HD. However, the measures of the global functional connectivity in the patients with PD were not different from those in healthy subjects. Conclusion This study revealed that alterations in structural and functional connectivity in patients who were undergoing PD and HD were different than those in healthy controls. These findings suggest that brain networks may be affected by different types of renal replacement therapy.

Health-related quality of life (HRQOL) is an important clinical outcome for dialysis patients. However, relative superiority in HRQOL between automated peritoneal dialysis (APD) and continuous ambulatory peritoneal dialysis (CAPD) are not clearly known. We compared HRQOL over time between APD and CAPD patients and evaluated factors associated with HRQOL.All 260 incident patients initiating APD or CAPD at multiple centers throughout Korea were prospectively enrolled in this study between October 2010 and February 2013. HRQOL, depressive symptoms, and renal treatment satisfaction were assessed 1 and 12 months after the start of dialysis by the Kidney Disease Quality of Life Short Form 36 (KDQOL-36), the Beck Depression Inventory (BDI), and the Renal Treatment Satisfaction Questionnaire (RTSQ), respectively.Of 196 patients who completed all questionnaires and did not change the peritoneal dialysis (PD) modality during the 1-year follow-up period, 160 were matched. APD patients showed better baseline HRQOL than CAPD patients for the symptoms, patient satisfaction, pain, and social function domains. There were no differences in HRQOL between the 2 groups at 12 months, and CAPD patients had significantly greater improvements in symptoms (P = 0.02), the mental composite summary (P = 0.03), and health status domains (P = 0.03) than APD patients. There were similar improvements in depressive symptoms (P = 0.01) and patient satisfaction with treatment (P = 0.01) in CAPD and APD patients. Interestingly, depressive symptoms, not PD modality, was the most influential and consistent factor for HRQOL. Despite the spontaneous improvement of depressive symptoms, considerable PD patients still had depressive symptoms at the 1-year appointment.APD has no advantage over CAPD for HRQOL. Considering the substantial negative effect of depressive symptoms on HRQOL, it is important to evaluate PD patients for depression and to treat those with depression to improve their HRQOL.

BackgroundThe aim of this multicenter study was to evaluate the safety and efficacy of tolvaptan (TLV) in Korean patients with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH).MethodsOf 51 enrolled patients with SIADH, 39 patients (16 female patients, aged 70.8 ± 11.3 years) were included in an intention to treat analysis. All patients received 15 mg/day as the initial dose, and the dose was then increased up to 60 mg/day (as needed) until day 4.ResultsSerum sodium increased significantly from baseline during the first 24 hours (126.8 ± 4.3 vs. 133.7 ± 3.8 mmol/L, P < 0.001), rose gradually between days 1 and 4 (133.7 ± 3.8 vs. 135.6 ± 3.6 mmol/L, P < 0.05), and then plateaued until day 11 (136.7 ± 4.5 mmol/L). The correlation between the change in serum sodium for the first 24 hours and initial serum sodium concentration was significant (r = −0.602, P < 0.001). In severe hyponatremia (< 125 mmol/L), the change was significantly higher (11.1 ± 4.8 mmol/L) than in moderate (6.4 ± 2.5 mmol/L, P < 0.05) or mild hyponatremia (4.3 ± 3.3 mmol/L, P < 0.01). In addition, logistic regression analysis showed that body weight (odds ratio [OR], 0.858; 95% confidence interval [CI], 0.775–0.976; P = 0.020) and body mass index (BMI) (OR, 0.692; 95% CI, 0.500–0.956; P = 0.026) were associated with rapid correction. No serious adverse events were reported, but in 13% of patients hyponatremia was overcorrected.ConclusionTLV is effective in correcting hyponatremia and well-tolerated in Korean patients with SIADH. However, those with low body weight, low BMI or severe hyponatremia, could be vulnerable to overcorrection with the initial dose of 15 mg TLV.