A Novel UMOD Mutation (c.187T>C) in a Korean Family with Juvenile Hyperuricemic Nephropathy

Familial juvenile hyperuricemic nephropathy (FJHN; OMIM 162000) is an autosomal dominant disorder characterized by hyperuricemia and gouty arthritis due to reduced kidney excretion of uric acid and progressive renal failure. Gradual progressive interstitial renal disease, with basement membrane thickening and glomerulosclerosis resulting from fibrosis, starts in early life. In most cases of FJHN, uromodulin gene (UMOD) is responsible for the disease; however, there has been only one report of a genetically confirmed FJHN family in Korea. Here we report another Korean family with FJHN, in which three male members. a father and 2 sons.developed gout and progressive renal insufficiency. The clinical, laboratory, and radiological findings were consistent with FJHN, and renal biopsy showed chronic parenchymal damage, which can be found in FJHN but is not specific to this disease. In order to confirm the diagnosis, sequence analysis of the UMOD was performed, and a novel heterozygous missense variant (c.187T>C; p.Cys63Arg) in exon 3 was identified. We assume that this variant is likely to be the causative mutation in this family, as the variant segregated with the disease. In addition, approximately two-thirds of the known mutations lead to a cysteine amino acid change in uromodulin, and all such variants have been shown to cause UMOD-associated kidney disease. In summary, we report a Korean FJHN family with three affected members by genetic analysis of the UMOD, and provide the first report of a novel heterozygous missense mutation.

Characteristics of Pneumonia and Clinical Significance of Atelectasis in Patients with Chronic Kidney Disease Stage 5 / 대한신장학회지

PURPOSE: Pneumonia is a common condition in patients with chronic renal insufficiency, and the condition is closely associated with high mortality and hospitalization rate in such patients. However, limited information is available about the clinical course of pneumonia in these patients, particularly in those with coexistent pulmonary atelectasis. We studied the characteristics of pneumonia as well as the clinical significance of pulmonary atelectasis in patients with chronic renal insufficiency. METHODS: We retrospectively reviewed the medical records of 25 patients with chronic renal insufficiency that were diagnosed as having pneumonia with atelectasis. The clinical, laboratory and radiological findings in these patients were examined. We also assessed the severity of atelectasis in these patients and compared the clinical courses of patients with different grades of atelectasis. RESULTS: The mean age of the patients was 71 years, and 15 of the 25 patients (60%) had diabetes. On chest computed tomography, the incidence of lobar infiltration, atelectasis, and pleural effusion was 75%, 64%, and 56%, respectively. The incidences of severe pneumonia and death tended to increase with the severity of atelectasis; however the increase was not statistically significant. The incidence of recurrence of pneumonia was significantly higher in patients with severe atelectasis than that in those without atelectasis. CONCLUSION: The incidence of severe pneumonia and the mortality rate tended to be higher in patients with severe atelectasis than in those without atelectasis. In addition, severe atelectasis was associated with the recurrence of pneumonia in patients with chronic renal insufficiency.

A Case of End-Stage Renal Disease (ESRD) from Reflux Nephropathy in a patient with Situs Inversus and Duplicated Ureter / 대한신장학회지

Situs inversus is a rare congenital anomaly that occurs in adults at the rate of 1:5,000-1:10,000. In the total form, the thoracic organs, as well as the abdominal organs, are completely reversed in a "mirror image" of their normal arrangements. Ureteral duplication is the most common ureteral anomaly founded incidentally. However, there is a higher incidence of duplication in cases of urinary tract infection, and it may be associated with upper urinary tract stasis, obstruction, or reflux. But ureteral duplication has no relation to situs inversus. Vesicoureteral reflux (VUR) is the most common disorder usually detected during radiologic evaluation of children with urinary tract infection. Complications such as renal scarring, reflux nephropathy, chronic renal failure and hypertension are well known in patients with VUR. Reflux nephropathy is responsible for about 10% of all cases of treated ESRD and is the most common case of ESRD in children. Thus, if VUR exists, early diagnosis and intensive treatment is necessary. Herein, we present a case of reflux nephropathy related ESRD in a 41-year-old woman with total situs inversus and duplicated ureter.

A Case of Primary Amyloidosis with Nephrotic Syndrome and Factor X Deficiency / 대한신장학회지

We report a case of nephrotic syndrome and factor X deficiency secondary to primary amyloidosis. A 58-year-old man was referred to our hospital for evaluation of nephrotic syndrome and bleeding tendency. He was confirmed to have primary amyloidosis by renal biopsy, immunofixation electrophoresis and bone marrow findings. His bleeding tendency was due to prothrombin time prolongation caused by isolated factor X deficiency. If any patient with nephrotic syndrome has bleeding tendency due to coagulation abnormalities, that patient should be considered to have factor X deficiency secondary to primary amyloidosis.

An Adult Case of Tubulointerstitial Nephritis and Uveitis Syndrome in Korea / 대한신장학회지

We report a case of adult-onset tubulointerstitial nephritis and uveitis syndrome with Fanconi syndrome. A 31-year-woman presented with fever, anorexia, nausea, general weakness and weight loss for two months. Her initial laboratory findings showed anemia, high serum creatinine, hypouricemia, hypophosphatemia, hypokalemia, glucosuria, and proteinuria. She was diagnosed as having acute tubulointerstitial nephritis by renal biopsy. The etiology of tubulointerstitial nephritis was unclear. She was treated with systemic corticosteroid. Six months later and while the patient was still on systemic corticosteroid (Deflazacort 36 mg), bilateral uveitis developed. Renal function was recovered by systemic corticosteroid and mycophenolic acid. But ocular symptoms relapsed twice despite systemic corticosteroid treatment. The ocular symptoms improved after topical ophthalmic steroid drops and injection. Tubulointerstitial nephritis and uveitis syndrome should be considered in the differential diagnosis of the unexplained tubulointerstitial nephritis. And the need of the steroid treatment also should be considered in the case of adult-onset.

Efficacy and safety of high dose epoetin alfa therapy in CAPD patients by cross-over study / 대한내과학회지

BACKGROUND: Recombinant human erythropoietin (rHuEPO) is an essential and well-established treatment for renal anemia. Rcently, clinicians have moved toward administration of high dose rHuEPO to reduce the inconvenience and time efficient.We aimed to determine whether high dose subcutaneous (SC) epoetin alfa is as efficient and safe as the usual dose for treating anemia in continuous ambulatory peritoneal dialysis (CAPD) patients. METHODS: Twenty-four patients on CAPD were randomly assigned to a high-usual dose group (n=12) and an usual-high dose group (n=12) with a variable interval for 48 weeks. Patients received 10 times treatments by scheduled visiting during Period I lasting 24 weeks and received 4 times treatments by scheduled visiting in Period II lasting 24 weeks by cross-over. The high dose was 10,000 IU and the usual dose was 4,000 IU epoetin alfa regimen. If hematocrit was out of the targeted range, 30~39%, the interval of epoetin alfa was changed within 50% of the previous interval. RESULTS: Fifteen patients, out of 24, completed the study (8 patients in the high-usual dose group; 7 patients in the usual-high dose group). Mean hemoglobin levels at randomization and after 12, 24, 36 and 48 weeks were 10.8+/-1.1, 11.5+/-0.9, 11.5+/-1.5, 11.4+/-1.5, 11.5+/-0.8 g/dL, respectively, in high-usual dose group compared with 11.2+/-0.8, 11.4+/-1.2, 11.2+/-0.9, 11.2+/-1.4, 11.4+/-0.9 g/dL, respectively, in usual-high dose group. The mean weekly epoetin alfa dosages at randomization and after 12, 24, 36 and 48 weeks were 83.6+/-38.1, 87.1+/-35.8, 89.4+/-34.2, 60.1+/-25.1, 62.8+/-30.7 IU/kg/week, respectively, in high-usual dose group compared with 69.8+/-31.6, 64.9+/-12.2, 69.9+/-46.1, 78.8+/-29.3, 75.9+/-16.4 IU/kg/week, respectively, in usual-high dose group. No statistically significant differences between the two groups were apparent for hemoglobin levels or mean weekly epoetin alfa dosages. Treatment interval at Period I and Period II were 13.3+/-5.3, 8.2+/-4.3 days in high-usual dose group compared with 7.0+/-2.5, 13.4+/-4.0 days in usual-high dose group with statistically significant differences. Treatment interval in high dose was about two times as longer as usual dose. Adverse events were generally mild and transient, and pain on injection site following subcutaneous administration was rarely reported. CONCLUSIONS: This study demonstrates that epoetin alfa 10,000 IU is as efficient and safe as 4,000 IU with a similar weekly dose in CAPD patients. Epoetin alfa 10,000 IU administration can reduce frequency of injections by about one half.

Clinical Significance of Human Parvovirus B19 Infection in Kidney Transplant Recipients / 대한임상미생물학회지

BACKGROUND: Human parvovirus B19 infection has been known to cause chronic anemia, pure red cell aplasia, glomerulopathy and allograft dysfunction in kidney transplant (KT) recipients. The aim of this study was to evaluate the prevalence and clinical significance of B19 infection in KT recipients. METHODS: Five hundred and thirty seven serum samples from 167 KT recipients were included in the present study. The prevalence of B19 infection was based on either qualitative or quantatitive polymerase chain reaciton (PCR) with LightCycler Parvovirus B19 Quantification kit (Roche Diganostics, Mannheim, Germany). Clinical significance of B19 infection was investigated by retrospective review of hemoglobin levels and the results of kidney and bone marrow biopsies. RESULTS: Overall PCR positive rate was 18.3% (98/537) and 52 out of 167 (31.1%) KT recipients showed at least one positive PCR result. In addition, 20 out of 167 subjects (12.0%) showed PCRpositivity more than two consecutive times and they had significantly lower hemoglobin level than those with negative PCR result or only one-positive result (P < 0.0001 by ANOVA and multiple comparison). In addition, two patients (1.2%) suffered from pure red cell aplasia which was confirmed by bone marrow biopsy. Nevertheless, B19 infection did not seem to affect the graft outcome. CONCLUSIONS: The parvovirus B19 infection in KT recipeints was not uncommon and was associated with low hemoglobin level and pure red cell aplasia after KT. Therefore, routine examination for the B19 infection should be provided for the KT recipients. To the best of our knowledge, this is the first report of the incidence and clinical significance of B19 infection in Korean KT recipients.

Percutaneous Intervention for Permanent Hemodialysis Access

PURPOSE: To evaluate the effectiveness of percutaneous transluminal angioplasty (PTA) and pulse-spray pharmacomechanical thrombolysis (PSPMT) using urokinase for the management of insufficient hemodyalitic access, and to identify contributory patency-related factors following interventional procedures. MATERIALS AND METHODS: Between August 1995 and July 2001, 105 cases of insufficient hemodyalitic access involving 38 artificial arteriovenous fistulae (AVF) and 67 graft arteriovenous fistulae (AVG) were treated interventionally. The patients underwent PTA alone in 53 cases and PSPMT combined with PTA in 47, and procedural success and long-term patency were evaluated in terms of a patient's age and sex, the presence of diabetes, the location of access, the type of AVG, the draining vein of AVG, the presence of central vein stenosis, the degree of residual stenosis, and the method of interventional procedure, and contributory factors were thus identified. RESULTS: The overall technical success rate of interventional management was 83.8% (88/105), while the overall primary patency rate was 58.7+/-5.2% at 6 months, 43.0+/-6.0% at 1 year, and 18.1+/-6.0% at 2 years. In AVF/AVG groups, primary patency rates were 55.9+/-9.2%/57.8+/-6.5% at 6 months, 45.8+/-10.0%/42.7+/-8.4% at 1 year, and 21.8%+/-9.8%/18.9+/-6.5% at 2 years. The overall secondary patency rate was 40.0+/-8.1% at 2 years. No contributory factors were found (95% confidence level), though patency of access decreased when residual stenosis was more than 30% (p=0.054). CONCLUSION: Interventional management of insufficient hemodyalitic access has high success and patency rates, and is an effective primary method. There appear to be no contributory factors, though residual stenosis of more than 30% tends to decrease the patency of hemodialytic access.

Enoxaparin-associated Retroperitoneal Hematoma in Two Chronic Dialysis Patients / 대한신장학회잡지

Low molecular weight heparin has greater advantages over unfractionated heparin. It is more bioavailable, and laboratory monitoring is not necessary. Compared with unfractionated heparin, low molecular weight heparin does not result in increased risk of major bleeding. However, the bleeding tendency is not predictable in patients with renal failure, because elimination of low molecular weight heparin is delayed and it does not alter prothrombin times or partial thromboplastin times. Recently, we experienced two cases of enoxaparin-associated retroperitoneal hematoma in chronic dialysis patients. A 57- year-old woman developed retroperitoneal bleeding, during treatment with enoxaparin(1 mg/kg q 12 hours) and oral aspirin. The other patient, a 49- year-old man developed retroperitoneal hematoma after discontinuation of enoxaparin and aspirin. Both patients had inguinal pain, femoral neuropathy, anemia and hypotension. They recovered gradually and their hematoma size were decreased by conservative treatment. These results suggest that anti-Xa acivity monitoring may be warranted in renal insufficiency patients who are receiving low molecular weight heparin If anti-Xa activity test is not available, unfractionated heparin could be used with monitoring of activated partial thromboplastin time. And the possibility of retroperitoneal hematoma should be considered, whenever the acute symptoms including inguinal pain, leg pain, anemia, or hypotension occured during the anticoagulation therapy.

Analysis of Depression in Continuous Ambulatory Peritoneal Dialysis Patients

It is well known that depression and sense of hopelessness worsen the quality of life in end-stage renal disease (ESRD) patients receiving dialysis. However, the characteristics of depression in continuous ambulatory peritoneal dialysis (CAPD) patients have not been analyzed in detail. We performed this study to investigate the severity of depression and the factors affecting depression in CAPD patients. With 96 CAPD patients, we evaluated each patient's depressive mood and hopelessness with CES-D (Center for Epidemiologic Studies Depression) scale and Beck Hopelessness Scale. We also evaluated the degree of stress of each patient with internal individual stress scale. Most CAPD patients experienced severe depression compared with the general population. Their depression was better explained by psychological factors, such as stress and sense of hopelessness, than by demographic or physical factors. On the basis of these findings, we suggest that the treatment of depression in CAPD patients might be possible by modulation of psychological factors.

Expression of Nephrin in Puromycin Aminonucleoside Nephrosis in Rat / 대한신장학회잡지

BACKGROUND: Nephrin, a recently identified protein, could be a slit diaphragm component and it has been suggested to play a crucial role in maintaining the glomerular filtration barrier. It has been reported that mutations in the nephrin gene lead to congenital nephrosis. However, the expression of nephrin in acquired glomerular disease has not yet been fully clarified. We induced nephrotic-range proteinuria in experimental animal and performed morphologic analysis with immunoelectron microscopy. This study was designed to examine the expression and distribution of nephrin in acquired glomerular disease and to suggest a role of nephrin in pathogenesis of proteinuria. METHODS: Twenty-three rats were divided into 3 experimental groups and control(n=6). 17 rats of experimental groups had intravenous injection of puromycin aminonucleoside(PAN) singly, and were sacrificed at 1 week(n=5), 2 weeks(n=6) and 3 weeks(n= 6) later. The expression of nephrin was observed by immunoelectron microscopy employing the polyclonal antibody against nephrin and gold particle. For quantifications, the gold particles were counted from photographs. RESULTS: The average length of foot process in 1 week group(2,307+/-524 nm) was far greater than that of control(317+/-45 nm). The average number of total gold particles per unit length(10,000 nm) of the GBM was reduced at 1 week(4.4+/-1.3), compared with control(12.1+/-3.9). Also, the average number of junctional gold particles at 1 week(1.7+/-0.5) was decreased compared with control(6.7+/-2.2). No difference was observed in the number of junctional gold particles per slit diaphragm among groups. But, there were significant differences in the distribution of gold particles among groups. Gold particles were seen more frequently at apical plasma membrane and cytoplasm in 1 week group, whereas those were observed prominently at junctions in control. CONCLUSION: These data show that the expression of nephrin was decreased with effacement of foot process in PAN induced nephrosis rat. However, nephrin was preserved at not-damaged slit diaphragm. And the distribution of nephrin was changed in PAN nephrosis. Further studies for nephrin production and redistribution should be needed to understand pathogenesis of nephrotic syndrome.

Patient with Renal Hypouricemia and Exercise Induced Acute Renal Failure / 대한신장학회잡지

We have described a male patient with a episode of acute renal failure after strenuous exercise. He was found to have low serum uric acid(0.6 mg/dL, after recovery) and normal 24 hour urinary excretion in the steady state. The possibility of other diseases that cause hypouricemia could be excluded, acute renal failure associated with idiopathic renal hypouricemia was diagnosed in this case. A renal computed tomography showed the delayed wedge shaped contrast enhancement, these findings suggested that the cause of acute renal failure could be renal vasoconstriction rather than obstruction by uric acid crystals. Hypouricemia appear to play a crucial role in this reperfusion oxygen free radical induced acute renal failure. We have suggested that the renal hypouricemia should be suspected in the case of acute renal failure associated with exercise when the patient's uric acid level was within or slight alone normal range at the time of acute renal failure.

Dissociation between Clearances of Small and Middle Molecules in Incremental Peritoneal Dialysis / 대한신장학회잡지

OBJEVTIVE: To evaluate the peritoneal clearance of the middle molecule compared with that of the small molecule in incremental peritoneal dialysis(PD). METHODS: Peritoneal clearances of the creatinine and beta2-microgloblulin were compared in 57 continuous ambulatory PD patients with full dose 4 times exchange and in 54 incremental PD patients with 2 or 3 times exchange over 24 hours. The clearances were also compared when there were changes in the peritoneal dialysis regimen such as in the number of exchanges and dwelling time. RESULTS: Peritoneal creatinine clearance increased almost linearly along with the increase in the number of exchanges. In contrast, peritoneal clearance of beta2-microglobulin was 9.1+/-3.6 L/week, 8.8+/-4.4 L/ week, and 7.9+/-2.5 L/week respectively with 2, 3 and 4 exchanges per day, not different from each other. Peritoneal clearance of beta2-microglobulin did not change when there was an increase in the number of exchange from 2 to 3 times and 3 to 4 times over a period of 24 hours, whereas the peritoneal clearance of creatinine increased. Peritoneal clearance of beta2-microglobulin almost doubled from 5.4+/-2.7 L/ week with 2 times exchange over 12 hours per day, to 9.5+/-4.4 L/week with 2 times exchange over 24 hours, whereas the creatinine clearance did not change. CONCLUSION: In contrast to peritoneal clearance of small molecule which depends on the number of dialysate exchange, peritoneal clearance of middle molecule depends mainly on the total dwelling hours rather than the number of exchange per day in incremental PD. This can be another advantage of incremental PD since peritoneal clearance of middle molecules in incremental PD over 24 hours is comparable to that in full dose PD.

Cadaveric Renal Transplantation between Donor and Recipient Both are Hepatitis B Virus Positive / 대한신장학회잡지

There has been studies constantly reporting on high rate mortality of renal transplantation of hepatitis B virus(HBV)-positive subject because the direct and indirect effect of immunosuppressive agent which was administrated after a transplantation worsens hepatitis or causes hepatic failure. But recent studies have reported that there is no difference in graft rejection, infection and survival rate of the graft or the host between hepatitis positive and negative groups. And, after lamivudine which suppresses HBV replication is introduced into renal transplantation, transplantation of HBV-positive subjects has taken on a new aspect. But it has been hard to find the reports about renal transplantation between donor and recipient both are hepatitis B virus positive in documents, because, in most cases of those reports, the recipient was hepatitis B virus positive but the donor who offers kidney was hepatitis virus negative. This study selected all 9 cases of cadaveric renal transplantation between HBV-positive cadaveric donor and HBV-positive chronic renal failure(CRF) patient who were operated at Samsung medical center from March of 1997 to August of 2000, then analyzed the medical records of five donors and nine recipients retrospectively. Six cadaveric donors(5 male, 1 female, age 15-52) and nine recipients(4 male, 5 female, age 23-52, median dialysis period 23 months) were included. During following up periods of 42 to 12 months (median 24 months) after renal transplantation with HBV DNA, serum ALT and serum creatinine change of hepatic function and renal function were observed and a development of infection and other complication were also investigated. Any case didn't come out fulminant hepatitis or liver cirrhosis. Four cases came out hepatic dysfunction. Among these, one case was diagnosed to CsA hepatotoxicity. One case came out a transient increase of ALT more than six months, since then was normilized. One case came out acute hepatitis and one case recurrent hepatitis. The rest constantly came out normal hepaitc function. In all the cases lamivudine treatment was practiced and the major indication were positive HBV DNA and a increase of ALT. In the recent test the eight cases came out a normal ALT and the only one case came out a little increase of ALT, 60 IU/L. Renal function was relatively well maintained. Three cases came out acute rejection, but it was successfully recovered. Chronic rejection didn't occur. In the recent test the eight cases came out a normal serum creatinine except one case(28 month after transplantation) which 1.5 mg/dL of serum creatinine appeared. When we consider our situation possessing much more recipients than donors of renal transplantation, this trial to expand the scope of donor to HBV- positive patients as well as the activation of cadaveric renal transplantation is a clinically meaningful effort especially in Korea and Asian countries which have a plenty of hepatitis carriers and chronic hepatitis patients. And we consider this new trial needs to continue comparative analyzation through long term observation.

Cognitive Function and Quality of Life in Patients with Chronic Renal Failure on Hemodialysis / 대한신장학회잡지

BACKGROUND: Nowadays it is considered that increment of hematocrit in chronic renal failure patients improves quality of life and cognitive function. But previous studies cannot exclude the practice effect and suggestion because they were accessed in the same ways before and after the erythropoietin treatment. This study was designed into two groups by hematocrit levels to know if patients with higher hematocrit levels have better quality of life and neurocognitive function. METHODS: Fifty-two chronic renal failure patients during hemodialysis received neurocognitive function test by physician before checking several self rating scales about quality of life. RESULTS: Patients with higher hematocrit levels had better scores in neurocognitive function tests and there was a relationship between the age and cognitive function. But patients with higher hematocrit levels didn't have better quality of life than those with lower ones. Instead there was strong correlation between the Beck Depression Inventory score and quality of life. CONCLUSION: These findings suggest that higher hematocrit levels improve cognitive function in chronic renal failure patients on hemodialysis, but increased hematocrit level does not always mean the better quality of life. Other psychological managements for depressive moods are recommended for better quality of life.

Cognitive Function and Quality of Life in Patients with Chronic Renal Failure on Hemodialysis / 대한신장학회잡지

BACKGROUND: Nowadays it is considered that increment of hematocrit in chronic renal failure patients improves quality of life and cognitive function. But previous studies cannot exclude the practice effect and suggestion because they were accessed in the same ways before and after the erythropoietin treatment. This study was designed into two groups by hematocrit levels to know if patients with higher hematocrit levels have better quality of life and neurocognitive function. METHODS: Fifty-two chronic renal failure patients during hemodialysis received neurocognitive function test by physician before checking several self rating scales about quality of life. RESULTS: Patients with higher hematocrit levels had better scores in neurocognitive function tests and there was a relationship between the age and cognitive function. But patients with higher hematocrit levels didn't have better quality of life than those with lower ones. Instead there was strong correlation between the Beck Depression Inventory score and quality of life. CONCLUSION: These findings suggest that higher hematocrit levels improve cognitive function in chronic renal failure patients on hemodialysis, but increased hematocrit level does not always mean the better quality of life. Other psychological managements for depressive moods are recommended for better quality of life.

A Prospective Study on Changes of Serum Leptin and Its Expression in CAPD Patients / 대한신장학회잡지

The ob gene product leptin is thought to be an adipostatic hormone through the regulation of food intake and energy expenditure. There are many reports that serum leptin concentration was increased in CRF patients, especially CAPD patients. The causes of elevated serum leptin concentration in CRF are believed to be multifactorial. Increased body fat mass, decreased residual renal function, active inflammation and hyperinsulinemia all are suggested to influence serum leptin concentration in CAPD patients. In this study, in order to investigate the pathogenic mechanism of increased serum leptin level in CAPD patients, we observed the changes of serum leptin concentration, leptin expression in the abdominal subcutaneous fat tissue, body fat composition, residual renal function, serum insulin concentration and CRP. Thirteen patients(7 men and 6 women, mean age 53+/-14 years) were enrolled in this study. Serum leptin concentration was measured by RIA before start of CAPD(baseline data), 5 days and 1, 3 months after start of CAPD. Simultaneously, fat tissues were aspirated from the abdominal subcutaneous fat tissues for analysis of ob gene expression. Ob mRNA expression was measured by semiquantitative RT-PCR method. The changes of serum insulin concentration, C-reactive protein, residual renal function were measured. All studies were done immediately before starting CAPD, 5 days, 1 month and 3 months after starting CAPD. Total body fat was estimated by dual energy X-ray absorptiometry and abdominal visceral and parietal fat area measured by computed tomography were done at 1-3 days(baseline data), 1 month, 3 months after start of CAPD. Serum leptin concentration increased significantly as early as 5 days after start of CAPD and maintained high up to 3 months(4.3+/-2.6->8.2+/-7.6->7.4+/-6.5->10.8+/-13.8ng/mL), while leptin expression in the abdominal subcutaneous fat tissue did not change during the study period(0.24+/-0.06->0.25+/-0.08->0.20+/-0.07->0.34+/-0.21ng/mL). No significant changes of body fat composition, residual renal function and serum insulin concentration were observed during the study period. These results suggest that increase of serum leptin concentration after CAPD may be due to increase of local leptin production, especially from the peritoneum, as has also been suggested by several reports of relatively higher dialysate leptin.

Effect of Mineralocorticoid on Serum Potassium Regulation and Urine Ammonium Excretion in Chronic Renal Patients / 대한신장학회잡지

Mineralocorticoids influences on acid-base homeostasis by the regulation of urine acidification. But its mechanism of acion is not well known in human. This study compared the acid-base status and the indices of urine acidification before and after mineralocorticoid administration in human, and analyzed the effect of mineralocorticoids on human acid-base homeostasis. We administered 9a-fludrocortisone in 6 chronic renal failure patients and 6 normal controls 0.5mg daily for 7 days. The results were as following: 1) After administration of 9a-fludrocortisone in patients group, serum aldosterone level changed from 120.2+/-71.0pg/mL to 44.8+/-32.2pg/mL(mean+/-SD, p< 0.05). Serum HCO- level was not changed. Urine ammonium excretion was incresed from 24.6+/-12.3 mmol/day to 43.7+/-19.0 (p<0.05), but there were no change in urine pH and urine anion gap, Serum potassium level decreased from 5.5+/-0.7mBq/L to 4.1+/-0.5mEq/L (p<0.05), and TTKG increased from 3.9 to 8.9(p<0.05). 2) After administration of 9a-fludrocortisone in control group, serum aldosterone level changed from 99.7+/-44.5pg/mL to 25.1+/-3 mL(p<0.05). Serum HCO- level was not changed. Urine ammonium excretion was incresed from 44.3+/-21.6mmoVday to 76.3+/-19.6(p<0.05), but there were no change in urine pH and urine anion gap. Serum potassium level decreased from 4.8+/-0.5mEq/L to 3.9+/-0.2mHq/L(p< 0.05), but there was no change in TTKG. 3) No patient or control showed any discomfort after 9-fludrocortisone administration, and there was no elevation in diastolic blood pressure, increase in body weight, electrolyte abnormality. In summary, after 9alpha-fludrocortisane administration, urinary ammonium excretion increased in both patients and control group, and this phenomenon occured with correction of hyperkalemia without urine pH change. This result implies urinary ammonium excretion increase by mineralocorticoid. In human increase in renal distal acidification by mineralocorticoid is due to increase in renal ammoniagenesis rather than stimulation on proton excretion.

Increases of cAMP Production and Urinary cAMP Excretion by Oxytocin in the Inner Medullary Collecting Duct(IMCD) / 대한신장학회잡지

Oxytocin, like vasopressin, has been known to act in the IMCD by the activation of adenylyl cyclase through V2 receptor, but the exact mechanism of its action remains to be elucidated. To prove whether oxytocin is involved in the activation of adenylyl cyclase in the renal collecting duct, we measured the cAMP production and urinary cAMP excretion rate. After single IMCD segments of Sprague-Dawley rats were microdissected and treated with different con- centrations of vasopressin(10pM, 10nM) and oxytocin (10pM, 10nM), cAMP production was measured. Urinary cAMP excretion rate was measured after dehydration and intraperitoneal injection of vasopressin and oxytocin. The results are as follows. 1) cAMP production in single IMCD was significantly increased in vasopressin group(10pM: 48,9+/-4.7(mean+/-SE), 10nM:94.6+/-5.3fmol/mm) and oxy-tocin group(10pM: 11.3+/-2.9, 10nM: 65.7+/-6.1fmol/mm) compared with that in the control(3.2+/-0.2fmol/ mm). 2) Urine volume was significantly decreased in dehydration group(40+/-7Ml/hour) and vasopressin group(420+/-120Ml/hour), but urine volume of oxytocin group(1,480+/-230Ml/hour) was not different from that of control(1,550+/-120Ml/hour). Urine osmolality was significantly increased in all experimental groups(control: 737.0+/-132.6, dehydration group : 2,463.9+/- 412.5, vasopressin group : 1,702+/-412.5, oxytocin group 1,293.4+/-117.9mOsm/kg). Urinary cAMP excretion rate was significantly increased in dehydration group(4,149.5+/-1,072.3pmol/hour) and oxytocin group(4,843.3+/-2,341.8pmol/hour), but not in vasopressin group(1,358.1+/-690.2pmol/hour), compared with that in control(49+/-10.7pmoVhour). These results suggest that oxytacin has anti-diuretic effect by the activation of adenylyl cyclase through V2 receptor.

Tuberculous Abscess of the Graft in a Renal Transplant Recipient after Chronic Rejection / 대한이식학회지

Tuberculosis is a significant opportunistic infection in transplant recipients under the immunosuppressed condition, though not common and also known to have higher incidence among transplant recipients than in general population. The most common form of tuberculosis among transplant recipients is pulmonary, gastrointestinal, bone and genital tract in decreasing order. However tuberculous infection of the transplanted graft is rare and usually associated with disseminated tuberculosis with high mortality. We experienced M.. tuberculosis infection of the renal allograft after chronic rejection. A 28-year-old female received living-related renal transplantation, required high-dose steroid therapy for two episodes of acute rejection (8 and 20 months later). However, she eventually became renal failure due to chronic rejection and immunosuppression therapy was discontinued. Patient was refered back to our institute for the hemodialysis (post-transplant 40 months) when the patient was found to have pulomary tuberculosis of miliary type. Antituberculosis medication (INH, RFP, EMB & PZA) was immediately started on the basis of positive cultures from lung and bone marrow specimen for M. tuberculosis. In spite of full medication, high fever was sustained and subsequently pyonephritis of grafted kidney was detected and infected graft was removed to confirm the tuberculosis is very serious disease among immunosuppressed transplant patient and therefore more aggressive approach is needed including the search for the hidden infection even at the failed graft with cessated function