Primary aldosteronism is a disease entity characterized by hypertension, hypokalemia, metabolic alkalosis and muscle weakness. Aldosteronoma is the most common cause of primary aldosteronism. The prevalence of primary aldosteronism in patients with hypertension appears to be low, less than 1%. However, primary aldosteronism is the one of common cause of secondary hypertension that is one of a few potentially curable forms of hypertension by surgical treament. The malignant hypertension in primary aldosteronism is very rare and the renal vascular damage due to hypertension seldom occurs. There has been no known reports about primary aldosteronism complicated with chronic renal failure in Korea. We report the rare case of primary aldosteronism in patient with hypokalemia, metabolic alkalosis complicated with chronic renal failure due to malignant hypertension with evident nephrosclerosis.
Alkalosis ; Humans ; Hyperaldosteronism* ; Hypertension ; Hypertension, Malignant ; Hypokalemia ; Kidney Failure, Chronic* ; Korea ; Muscle Weakness ; Nephrosclerosis ; Prevalence

Hypertensive nephrosclerosis is usually associated with chronic hypertension, which increases the risk of progressive renal disease. Among the causes of malignant hypertension, thrombotic microangiopathy is complicated and is associated with renal dysfunction at the time of diagnosis. In this case, a young man with hypertension presented with renal failure and thrombocytopenia in the emergency department. This case emphasizes the importance of early recognition of renal failure and thrombocytopenia among patients with uncontrolled hypertension.
Blood Pressure* ; Diagnosis ; Emergency Service, Hospital ; Humans ; Hypertension ; Hypertension, Malignant ; Nephrosclerosis ; Renal Insufficiency ; Thrombocytopenia ; Thrombotic Microangiopathies*

OBJECTIVETo investigate the clinical and pathological characteristics of patients with clinically presumed hypertensive nephrosclerosis (HN).

METHODSClinical data and renal biopsy results were obtained in 63 patients diagnosed clinically as HN (primary hypertension plus renal injury).

RESULTSHN was confirmed by biopsy in 47 out of 63 patients (74.6%, 12 malignant nephrosclerosis and 35 benign nephrosclerosis). Primary nephritis (PN) was diagnosed by biopsy in 10 patients (7 IgA nephropathy, 2 mesangial proliferative nephritis, 1 chronic interstitial nephritis) and focal and segmental glomerulosclerosis (FSGS) in 6 patients. Blood pressure, body mass index, GFR and blood lipids were similar among groups. HN patients were related to higher age, more frequent family history of hypertension, longer hypertension duration, higher left ventricular mass index, lower serum creatinine and lower incidence of microscopic hematuria. Most patients with malignant nephrosclerosis and FSGS patients showed grades III and IV retinopathy.

CONCLUSIONOur results show that HN was misdiagnosed in nearly 25% patients in this cohort. Since the clinical features are similar between HN, PN and FSGS, renal biopsy is needed to establish the diagnosis of HN.

Adult ; Aged ; Female ; Humans ; Hypertension, Renal ; complications ; diagnosis ; pathology ; Kidney ; pathology ; Male ; Middle Aged ; Nephrosclerosis ; diagnosis ; etiology ; pathology

An increased plasma aldosterone concentration, with suppressed plasma renin activity (PRA), is an abnormal finding in primary hyperaldosteronism. A suppressed PRA is caused by aldosterone- dependent sodium retention and extracellular volume expansion. A case of primary hyperaldosteronism, due to adenoma, with increased PRA, was observed. An adrenalectomy and intraoperative renal biopsy was performed. In our patient, histologically proven renal arteriosclerosis was the probable cause of the escape of the PRA from the suppression by an aldosterone-producing adenoma. Normal blood pressure was not attained after the adrenalectomy. However, the blood pressure was then controlled by small doses of antihypertensive drug before resection of the tumor. In this case, the patient was treated with spironolactone, but the blood pressure was not correctly controlled. After the adrenalectomy, the blood pressure was well controlled with smaller dose of calcium channel blockers. So, an early adrenalectomy may be beneficial as soon as the diagnosis of an aldosterone-producing adenoma is confirmed, even in patients with hypertensive nephrosclerosis.
Adenoma ; Adrenalectomy ; Aldosterone ; Arteriosclerosis ; Biopsy ; Blood Pressure ; Calcium Channel Blockers ; Diagnosis ; Humans ; Hyperaldosteronism* ; Nephrosclerosis ; Plasma* ; Renin* ; Sodium ; Spironolactone ; United Nations

The Korean Society of Nephrology (KSN) launched the official end-stage renal disease (ESRD) patient registry in 1985, and an Internet online registry program was opened in 2001 and revised in 2013. The ESRD Registry Committee of KSN has collected data on dialysis therapy in Korea through the online registry program in the KSN Internet website. The status of renal replacement therapy in Korea at the end of 2012 is described in the following. The total number of ESRD patients was 70,211 at the end of 2012, which included 48,531 hemodialysis (HD) patients, 7,552 peritoneal dialysis (PD) patients, and 14,128 functioning kidney transplant (KT) patients. The prevalence of ESRD was 1,353.3 patients per million population (PMP), and the distribution of renal replacement therapy among ESRD patients was as follows: HD, 69.1%; PD, 10.8%; and KT, 20.2%. The number of new ESRD patients in 2012 was 11,742 (HD, 8,811; PD, 923; and KT, 1,738; the incidence rate was 221.1 PMP). The primary causes of ESRD were diabetic nephropathy (50.6%), hypertensive nephrosclerosis (18.5%), and chronic glomerulonephritis (18.1%). The mean urea reduction ratio was 67.9% in male and 74.1% in female HD patients. The mean Kt/V was 1.382 in male and 1.652 in female HD patients. The 5-year survival rates of male and female dialysis patients were 70.6% and 73.5%, respectively.
Diabetic Nephropathies ; Dialysis ; Female ; Glomerulonephritis ; Humans ; Incidence ; Internet ; Kidney ; Kidney Failure, Chronic ; Korea ; Male ; Nephrology ; Nephrosclerosis ; Peritoneal Dialysis ; Prevalence ; Renal Dialysis ; Renal Replacement Therapy* ; Survival Rate ; Urea

Here we report two cases of isolated diffuse mesangial sclerosis (IDMS) with early onset end-stage renal failure. These female patients did not show abnormalities of the gonads or external genitalia. Direct sequencing of WT1 PCR products from genomic DNA identified WT1 mutations in exons 8 (366 Arg>His) and 9 (396 Asp>Tyr). These mutations have been reported previously in association with Denys-Drash syndrome (DDS) with early onset renal failure. Therefore we suggest that, at least in part, IDMS is a variant of DDS and that investigations for the WT1 mutations should be performed in IDMS patients. In cases with identified WT1 mutations, the same attention to tumor development should be required as in DDS patients, and karyotyping and serial abdominal ultrasonograms to evaluate the gonads and kidney are warranted.
Base Sequence ; DNA/chemistry/genetics ; DNA Mutational Analysis ; Fatal Outcome ; Female ; Glomerular Mesangium/*pathology ; Humans ; Infant ; Infant, Newborn ; *Mutation ; Nephrosclerosis/*genetics ; WT1 Proteins/*genetics

BACKGROUND/AIMS: Although renal manifestations are often involved in patients with rheumatoid arthritis (RA), the causal relationship between RA and renal manifestations has not been clearly defined. The prevalence and causes of renal manifestations in patients with RA were investigated in this study. METHODS: The clinical data from 457 patients with RA and who were admitted to Hanyang University Hospital between 2001 and 2005 were retrospectively analyzed. Renal manifestations were defined as proteinuria (> or =300 mg/day) or azotemia (serum creatinine > or =1.7 mg/dL), with or without hematuria. RESULTS: Renal manifestation was present in 82 (17.9%) out of 457 RA patients. Among them, proteinuria was observed in 81 (17.7%), azotemia in 37 (8.1%) and hematuria with either proteinuria or azotemia in 35 (7.7%). For the cases with proteinuria, the amount of preteinuria was 1353+/-207 (mean+/-SD) mg/day. There was no significant correlation between the degree of proteinuria and the duration of RA. For the cases with azotemia, the serum creatinine was 3.98+/-0.35 mg/dL. The presence of azotemia had no significant association with the duration of RA (14.4+/-1.5 vs. 11.6+/-1.2 years, respectively). When the etiology of the renal manifestation was classified into primary and secondary renal disease, the latter included diabetic nephropathy in 13 (15.9%), hypertensive nephrosclerosis in 8 (9.8%), drug induced chronic tubulointerstitial disease in 11 (13.4%) and AA amyloidosis in 2. Renal biopsy revealed 10 cases of primary glomerulopathy, including IgA nephropathy in 3, membranous nephropathy in 2, mesangial proliferative glomerulonephritis in 1, focal segmental glomerulosclerosis in 1 and chronic sclerosing glomerulonephritis in 3. CONCLUSIONS: The prevalence of chronic kidney disease in patients with RA is high, although direct renal invasion by RA is very rarely encountered. Renal biopsy would be of great help to identify the various causes of renal manifestations in patients with RA.
Amyloidosis ; Arthritis, Rheumatoid ; Azotemia ; Biopsy ; Creatinine ; Diabetic Nephropathies ; Glomerulonephritis ; Glomerulonephritis, IGA ; Glomerulonephritis, Membranous ; Glomerulosclerosis, Focal Segmental ; Hematuria ; Humans ; Nephrosclerosis ; Prevalence ; Proteinuria ; Renal Insufficiency, Chronic ; Retrospective Studies

BACKGROUND: The terminal features of hypertensive target organ damage include decrease in the kidney size and increase in the heart size and wall thickness. Increased apoptosis has been known in the hypertensive nephrosclerosis and in the hypertensive heart failure. We hypothesized that apoptosis may progress by different degrees in the kidneys and heart with hypertension being sustained. To test this hypothesis we examined apoptosis in the kidneys and hearts of spontaneously hypertensive rats(SHR) of various ages. In addition, we examined histopathology of the kidneys. METHODS: The kidneys and hearts of 19 SHR were excised at the age of 16 weeks(n=4), 20 weeks (n=6), and 32 weeks(n=9). Sprague-Dawley rats(SDR, n=6) were also sacrificed at the age of 16-24 weeks. Degree of apoptosis was evaluated semi-quantitati-vely by counting the number of apoptotic nuclei, stained by TUNEL method, per high power field(x400). Light microscopic and electron microscopic examination of the kidneys were performed. RESULTS: 1) In SHR kidneys, the number of apoptotic nuclei at the age of 16 weeks was similar to that in SDR kidneys(9.3+/- 0.5 vs. 10.2+/- 2.2, p=NS). However, the number was significantly, p<0.05, increased at the ages of 20 weeks and 32 weeks(31.5+/- 4.4 and 34.1+/- 4.0, respectively) as compared with that in SHR kidneys at the age of 16 weeks and that in SDH kidneys. 2) In SHR hearts, the number of apoptotic nuclei at the ages of 16, 20, and 32 weeks(4.0+/- 1,2, 2.0+/- 0.7, 1.9+/- 0.4, respectively) was neither changed nor different significantly from that in SDR hearts(0.70.5, p=N5), although the heart of SHR was hypertrophied at the age of 32 weeks. 3) Apoptosis was detected most frequently in the outer medulla of the kidneys in SHB. Histopathologic findings were the segmented sclerosis of glomeruli (1/25-50 glomeruli), edema, vacuolization and decreased villi of tubular epithelial cells. The older the age of SHR was, the more severe histopathologic changes were found. COMCLUSION: The sustained hypertansion caused increased apoptosis in the kidneys but no increased apoptosis in the heart of SHR during the specified ages of our study. The longer the duration of hyper- tension was, the more apoptotic cells and the more severe histopathologic changes, mainly in the tubulo-interstitial area, were found in the kidneys. The most frequent site of apoptosis was the outer medulla. It is suggested that apoptosis in the kidneys begin earlier than that in the heart in hypertensive target organ damage.
Apoptosis* ; Edema ; Epithelial Cells ; Heart Failure ; Heart* ; Hypertension ; In Situ Nick-End Labeling ; Kidney* ; Nephrosclerosis ; Rats, Inbred SHR* ; Rats, Sprague-Dawley ; Sclerosis

Because of increases in the elderly population and diabetic patients, the proportion of elderly among dialysis patients has rapidly increased during the last decades. The mortality and morbidity of these elderly dialysis patients are obviously much higher than those of young patients, but large analytic studies about elderly dialysis patients' characteristics have rarely been published. The registry committee of the Korean Society of Nephrology has collected data about dialysis therapy in Korea through an Internet online registry program and analyzed the characteristics. A survey on elderly dialysis patients showed that more than 50% of elderly (65 years and older) patients had diabetic nephropathy as the cause of end-stage renal disease, and approximately 21% of elderly dialysis patients had hypertensive nephrosclerosis. The proportion of elderly hemodialysis (HD) patients with native vessel arteriovenous fistula as vascular access for HD was lower than that of young (under 65 years) HD patients (69% vs. 80%). Although the vascular access was poor and small surface area dialyzers were used for the elderly HD patients, the dialysis adequacy data of elderly patients were better than those of young patients. The laboratory data of elderly dialysis patients were not very different from those of young patients, but poor nutrition factors were observed in the elderly dialysis patients. Although small surface area dialyzers were used for elderly HD patients, the urea reduction ratio and Kt/V were higher in elderly HD patients than in young patients.
Aged* ; Arteriovenous Fistula ; Diabetic Nephropathies ; Dialysis* ; Humans ; Internet ; Kidney Failure, Chronic ; Korea* ; Mortality ; Nephrology ; Nephrosclerosis ; Renal Dialysis ; Renal Replacement Therapy ; Urea

Many factors can be recognized for the acute rejection such as: degree of HLA mismatching, cytokine gene expression, ischemic time, etc. Some authors have suggested the importance of early routine biopsy of renal allograft to predict acute rejection. This prospective study on renal implantation biopsies was performed to evaluate the relationship between the implantation biopsies and the acute rejection during the immediate post-transplantation period. From December 1996 to February 1998 implantation biopsies were performed on 46 renal allografts within 40~60 minutes after vascular anastomosis using tru-cut needle (18G). Two samples were obtained from transplanted kidney in each patient. Serial sections were stained for the light microscopic examination. The slides were evaluated for histologic features such as interstitial cellular infiltration, nephrosclerosis, tubular damage, glomerular neutrophil count (GL-PMN), and peritubular neutrophil count (PTC-PMN). Forty six biopsies were grouped into acute rejection group (R group, n=10) and non-rejection group (N group, n=36) during immediate posttransplantation period (1 month). Acute rejections were confirmed by ultrasonography guided biopsy. Histologic findings were classified according to Banff schema. The statistical analysis was performed by using Chi-Square Test and Spearman Rank Sum Test. During the immediate post-transplantation period, acute rejection developed in 10 cases (21.7%) of which 9 cases were the biopsy-proven rejection. The male to female ratio was 21:25. Recipients were ranged from 22 to 54 years old with a mean age of 38.2+/- 9.1. Original disease of recipient were chronic glomerulonephritis in 15 cases (32.6%), hypertension in 8 cases, diabetes mellitus in 3 cases, RPGN in 2 cases. Fifteen cases (32.6%) were of unknown etiology. The mean number of HLA mismatches was 4.6+/- 0.9 in R group, 4.7+/- 1.2 in N group, and the mean number of HLA-B & DR mismatches was 2.2+/- 0.4 in R group, 2.3+/- 0.7 in N group. The ratio of the living vs. cadaveric donors was 34:12. No statistical difference was observed between two groups in interstitial cellular infiltration, nephrosclerosis and tubular damage. The GL-PMN was 0.6 0.9 in R group, while 0.1 +/- 0.4 in N group. The PTC-PMN was 5.3+/- 3.3 in R group and 0.3+/- 1.1 in N group (p<0.05). The presence of more than five PTC-PMN count was related with the occurrence of acute rejection (p<0.01). In conclusion, the PTC-PMN of renal implantation biopsies is a possible predicting factor for acute rejection in this preliminary report.
Allografts ; Biopsy* ; Cadaver ; Diabetes Mellitus ; Female ; Gene Expression ; Glomerulonephritis ; HLA-B Antigens ; Humans ; Hypertension ; Kidney ; Male ; Middle Aged ; Needles ; Nephrosclerosis ; Neutrophils ; Prospective Studies ; Tissue Donors ; Ultrasonography