A Case of Liddle Syndrome Associated with Torsades de pointes / 대한신장학회잡지

Liddle syndrome is a rare cause of hypokalemic hypertension and caused by renal tubular sodiurn channel defect resulting in excessive sodium absorption, potassium wasting and metabolic alkalosis. Clinically this syndrome resembles the primary aldosteronism, however, aldosterone and renin secretion are markedly suppressed due to chronic state of volume expansion. This syndrome is transmitted in an autosomal dominant pattern. We have experienced a case of Liddle syndrome, a 74 years old female accompanying severe hypokalemia, long-standing hypertension, metabolic alkalosis and suppressed aldosterone and renin level in serum and urine. She had a history of arrhythmia, torsades de pointes, of unknown cause. We believe that the arrhythmia resulted from severe hypokalemia secondary to this syndrome. Two of her siblings died suddenly, probably from cardio-, cerebrovascular accidents. Five her offspring needed to be evaluated for this syndrome due to its autosomal dominant inheritance. Endocrinologically there was no clue for us to seek other diseases of enzyme deficiency needed in aldosterone synthesis. Once the diagnosis of Liddle syndrome was suspecti, we treated her with amiloride 5mg/day for several days. Thereafter metabolic abnormalities including persistent hypertension, not responded to conventional parenteral potassium replacement and antihypertensive drugs, were reversed and normalized until now. We believe that in some of patients of secondary hypertension of unknown cause, Liddle syndrome should be ruled out, and that the incidence of this syndrome has been underes- timated due to lack of suspicion.

A Case of Drug-Induced Chylous Ascites in a Patient Undergoing Continuous Ambulatory Peritoneal Dialysis / 대한신장학회잡지

A chylous ascites, especially drug-induced, is very rare complication in CAPD. The diagnostic criteria for the drug-induced chylous peritoneal dialysate include 1) turbid dialysate developed within Chrs after the administration of causative drug, 2) no clinical symptoms being suggestive of peritoneal inflammation, 3) the fluid containing normal leukocyte counts and being negative for bacterial and fungal culture, and 4) it disappeared spontaneously after the withdrawal of the assumed causative agent and never recurred thereafter. We report a case of chylous ascites emerging after use of manidipine, dihydropyridine calcium channel blocker, in a patient undergoing CAPD. The chylous ascites in that patient was improved after discontinuation of manidipine.

Effect of High Glucose on Nitric Oxide Production in Culteured Rat Mesangial Cells / 대한신장학회잡지

Diabetic nephopathy is one of the leading causes of end-stage renal disease and characterized pathologically by the glomerular mesangial expansion and increased extracellular matrix(ECM) formation. Glomerular hyper-filtration and increased vascular permeability observed in the early stage of diabetic nephropathy have been proposed to play a significant pathophysiologic role in the eventual development of glomerulosclerosis of dia-betic nephropathy. Some studies have suggested that this glomerular hyperfiltration is mediated by increased nitric oxide(NO) production via the constitutive nitric oxide synthase(cNOS) pathway present in endothelial cells under the high glucose environment. However, the exact role of the inducible NOS(iNOS) pathway present in mesangial cells in the pathogenesis of diabetic neph-ropathy is not clearly established. The present study was carried out to examine whether NO production via the iNOS pathway is mo-dulated in cultured rat mesangial cells exposed to the high glucose environment and underlying mechanism of this modulation. For this purpose, the production of the stable metabolite of NO(nitrite), intracellular cyclic gu-anosine monophosphate(cGMP), iNOS mRNA expression and iNOS protein synthesis were examined under different glucose concentrations. Rat mesangial cells cultured in high glucose concen- tration(30mM D-glucose) increased significantly nitrit#e/ nitrate production and intracellular cGMP levels upon stimulation with lipopolysaccharide(LPS) plus interfer-on-r (IFN-r ) compared with control glucose concen- tration(5.6mM D-glucose). Mesangial iNOS mRNA expression and protein synthesis also increased signifi- cantly in response to high glucose. This enhanced iNOS mRNA expression induced by high glucose concentration was significantly suppressed by protein kinase C(PKC) inhibitor, calphostin C, and the aldose reductase inhibitor, 6-bromo-l, 3-dioxo-1H- benz[d, elisoquinoline-2(3H)-acetic acid. These results indicate that high glucose in combination with stimulation by LPS plus IFN- r enhances NO production from mesangial cells by the iNOS pathway, and that the activation of PKC and the polyol pathway may play a role in this enhancement.

Patients' Referral Pattern and Dialysis Initiation Practice: Single Center Experience / 대한신장학회잡지

Despite improvements in dialysis care, the mortality of patients with end-stage renal disease(ESRD) remains high. One factor that has so far received little attention, but which might contribute to morbidity and mortality, is the timing of referral to the nephrologist. We performed a retrospective analysis in 358 patients(male 275, female 151) who were initiated renal replacement therapy first at this hospital from Jan 1995 to Dec 1996. Patients were defined by the time of first nephrology as early referral(E, n=163) encountered after more than 8 weeks; late early referral(LE, n=19) encountered between 8 weeks and 4 weeks; late referral(L, n=55) encountered from 1 week to 4 weeks; urgent referral(U, n= 121) encountered less than 1 week. There were no differences in age, gender, primary renal disease, cause of dialysis, and renal replacement therapy modalities. However, there were significant differences in rnean arterial pressure and serum phosphate levels between these 4 groups. The mean arterial pressures (mmHg) were 109.15 +/- 17.16, 105.37+/-18.76, 117.24 +/- 27.24 and 116.98+/-24.26 for E, LE, L and U, respectively(p0.05). In the E group, there was more controlled blood pressure and serum phosphate levels compared to the U group at initiation of renal replacement therapy, but other parameters were not significantly different among the 4 groups. Delays in initiation of renal replacement therapy may result in patients entering dialysis in a compromised state, therefore adequate long-term predialysis care by a nephrologist is important. Socioeconomic - and medical factors respon-sible for late referral and late initiation of dialysis need to be evaluated and corrected to further improve the outcome of these patients.