A Case of Fabry Disease, Following Renal Biopsy for the Investigation of Proteinuria / 대한신장학회잡지

Fabry disease, angiokeratoma corporis diffusum, is a rare X-linked inborn error of glycosphingolipid metabolism due to the lack of the lysosomal enzyme, alpha-galactosidase A, resulting in a progressive deposition of specific neutral glycosphingolipids within the lysosomes of endothelial and smooth muscle cells of the cardiovascular and renal systems predominantly. We reported a case of Fabry disease, following renal biopsy for the investigation of proteinuria(Creatinine clearance 87.28 mL/min/1.73, serum creatinine 1.1 mg/dL, 24-hour urine protein 1,125 mg, 24-hour urine creatinine 1,382 mg). The patient was 46 year old male. He had experienced anterior chest pain regarded as angina pectoris for a few years. A 12- lead electrocardiogram was abnormal(T-wave inversion in II, III, AVF, and V3-V6), but echocardiography and coronary angiography revealed no abnormal. Kidney biopsy findings showed lamella inclusion bodies on electron microscopy, which are typical finding of Fabry disease. The patient is followed at O.P.D without any significant complaints for 18 months after diagnosis of Fabry disease.

Determinants of erythropoietin resistance in hemodialysis patients: EPO Resistance in HD patients / 대한내과학회지

BACKGROUND: More than 90% of dialysis patients respond in a dose-dependent manner to erythropoietin(EPO) administration and the others are resistant. The causes of EPO resistance are iron deficiency, vitamin deficiency, severe hyperparathyroidism, aluminum toxicity, and inflammation. Much literature has been published concerning iron deficiency and its role in EPO resistance. However little attention has been given to the contribution of inflammation to the EPO-resistant anemia observed in hemodialysis patients. METHODS: In the present study, we examined the contribution of parathyroid hormone levels, iron idices, normalized protein catabolic rate(nPCR), Kt/Vurea, albumin, and C-reactive protein(CRP) to erythropoietin resistance index(weekly rhEPO dose/body weight/hematocrit; ERI) in clinically stable 48 hemodialysis patients. RESULTS: The factors correlated with ERI were CRP(R=0.608, p or =0.4 mg/dL) with normal CRP group(<0.4 mg/dL), there were significant differences in serum albumin, creatinine, ferritin, and ERI. CONCLUSION: Acute-phase response, assessed by the level of CRP, was the most important predictor or EPO resistance in stable hemodialysis patients.

A Case of Necrotizing FasciitisZ in Chronic Renal Failure Patient / 대한신장학회잡지

Necrotizing fasciitis is an uncommon severe infection involving the subcutaneous soft tissue, particularly the superficial and often deep fascia. It is usually associated with systemic toxicity, rapid progression and a mortality rate which varies between 20 and 60%. It can affect any part of the body but is most common on the extremities, especially the leg. Predisposing factors include diabetes mellitus, alcoholism, intravenous drug abusers, abdominal surgery, perineal infection. The prognosis for necrotizing fasciitis depends so heavily on early recognition and determination of the extent of necrosis. Whilist there are reports of acute renal failure occuring in the presence of necrotizing fasciitis, descriptions of the condition in patients with chronic renal failure are rare in the literature. Hence we report a case of necrotizing fasciitis, diagnosed by MRI(Magnetic Resonance Imaging) in chronic renal failure patient.

Liddle's syndrome: a report in a middle-aged woman

A 54-year-old woman with diabetes mellitus was hospitalized with generalized edema and weakness. She was also found to have hypertension, hypokalemia and metabolic alkalosis. Detailed examination showed subnormal plasma renin activity and plasma aldosterone concentration. Adrenal CT scanning revealed no adrenal tumor. A successful treatment with amiloride established the diagnosis of Liddle's syndrome for the patient. Liddle's syndrome, a rare hereditary disease usually found in young patients, should be considered in the differential diagnosis of hypertension even in elderly individuals.

Comparison of Nutritional Status According to Serum C-reactive Protein Concentration in Hemodialysis Patients / 대한신장학회잡지

Serum C-reactive protein(sCRP) is an acute-phase reactant that exhibiting negative correlation with serum albumin concentration. It was reported that sCRP is an independent predictor of survival in both hemodialysis and peritoneal dialysis patients, and an acute phase inflammation could be preceded by protein catabolism, hypoalbuminemia, anorexia and even atherosclerotic cardiovascular disease. We have evaluated serum biochemical parameters including albumin and prealbumin, Kt/Vurea, nPCR, SGA score, anthropometric parameters and diet history in 30 ESRD patients maintained on chronic hemodialysis subdivided by sCRP concentration. Upon comparing the two subgroups[high CRP group(sCRP >or= 0.4mg/dL), n=15 vs. normal CRP group (sCRP<0.4mg/dL), n=15], high CRP group showed significantly lower levels of hemoglobin(9.3+/- 0.7 vs. 9.8+/-0.6g/L, p<0.05), hematocrit(28.3+/-2.3 vs 29.8+/-1.696, p<0.05), creatinine(9.6+/-3.1 vs. 12.2+/-2.5mg/dL, p<0.05), prealbumin(20.9+/-5.0 vs. 25.8+/-6.4mg/dL, p< 0.05), SGA score(5.0+/-1.2 vs. 5.9+/-0.7, p<0.05), and percent of patients who have higher nPCR than protein intake(85.7 vs. 28.6%, p<0.05). Ferritin was significantly higher in high CRP group(503.1+/-205.7 vs. 323.3+/-186.6, p<0.05). There were no differences in age, sex, duration of hemodialysis, prevalence of diabetic nephropathy and cardiovascular disease, Kt/Vurea, nPCR, residual renal function, amount of protein intake and other nutritional parameters. In conclusion, there was higher probability of malnutrition, anemia and protein catabolism in hemo-dialysis patients with elevated sCRP concentration.

A Case of Primary Sjorgen's Syndrome Presenting as Hypokalemic Periodic Paralysis / 대한류마티스학회지

We describe a 46-year-old woman with hypokalemic paralysis as the initial manifestation of Sjorgen's syndrome. Sjorgen's syndrome is an autoimmune exocrinopathy, characterized by keratoconjuntivitis sicca and xerostomia. Among the extraglandular manifestations of Sjorgen's syndrome, renal tubular involvement, especially renal tubular acidosis, is the most often latent or minimally symptomatic. Renal tubular acidosis is estimated to be present in 25~30 percent of the cases. Hypokalemic paralysis may serve as a clinical marker for more severe renal disease in patient who has primary Sjorgen's syndrome with renal tubular acidosis, even though it is a rare manifestation of Sjorgen's syndrome.

Lower Esophageal Sphincter Pressure(LESP) and 24-hour(h) Esophageal pH Monitoring in Continuous Ambulatory Peritoneal Dialysis(CAPD) Patients / 대한신장학회잡지

Upper gastrointestinal symptoms such as epigastric fullness, nausea, and vomiting are often observed in CAPD patients. Intraabdominal pressure depends on the amount of dialysate and the body positions. It is not clear whether upper gastrointestinal symptoms are manifestations of raised intraperitoneal pressure produced by dialysate. To investigate the changes of LESP according to the amount of dialysate and the body position and the relationship between LESP and parameters of 24-h esophageal pH monitoring, esophageal manometry and 24-h esophageal pH monitoring were performed in 12 CAPD patients. The sex ratio was 1:2. The duration of CAPD of patients except one was less than 1 month. The mean age was 47.5 15.5(SD) years old. There were no changes in supine LESPs according to the infused volume of dialysate. Sitting LESPs at 500, 1500, and 2000ml were elevated significantly compared to basal sitting LESP(27.1 5.5, 27.0 6.0, and 28.5 7.0 vs. 23.9 5.7mmHg, p<0.05). Supine LESPs at basal and 500ml of dialysate significantly higher than sitting LESPs(30.2 9.1 and 31.2 8.5 vs. 23. 9 5.7 and 27.1 5.5mmHg, p<0.05). There were no differences in LESP by age, sex, and diabetic status. Supine LESP at 2000ml strongly correlated with total reflux episodes(r=-0.92, p<0.01), fraction time of pH<4.0(r=-0.85, p<0.01), and total reflux score(DeMeester)(r=-0.88, p<0.01), but other LESPs did not. When the patients were divided into group I(<30mmHg) and group II(330mmHg) by supine LESP at 2000ml, group I had more total reflux episodes(73.7 27.0 vs. 14.0 10.3, p<0.05), fraction time of pH<4.0(4.0 3.7 vs. 0.5 0.4%, p<0.05), and total reflux score(15.2 10.5 vs. 2.8 1.5, p<0.05). In conclusion, CAPD patients seem to have a risk of gastroesophageal reflux due to elevated intraperitoneal pressure by dialysate, especially if supine LESP at 2000ml was lower than 30mmHg.

A Case of Chronic Renal Failure, Caused by IgA Nephropathy Combined with Polycythemia Vera / 대한신장학회잡지

Patients with chronic renal failure, generally, sufferred from normocytic normochromic anemia caused by decreased level of erythropoietin. But, secondary erythrocytosis has been reported in patients with several renal diseases; renal artery stenosis or throm- bosis, polycystic kidney disease, bilateral hydronephrosis, etc. We report one case of chronic renal failure combined with polycythemia vera. The case was 32 year-old man whose chief complaints were dyspnea, back pain, itching sensation, headache. 6 month ago, the laboratory examination showed only proteinuria and hematuria without deterioration of renal function. The renal function was aggravated with an accelerated course, and bone marrow examination revealed hypercellularity (erythroid predominance), and renal biopsy showed the finding of the end stage of renal disease which may be originated from IgA nephropathy.

One Case of Microscopic Polyangiitis / 대한내과학회지

Microscopic polyangiitis is a primary systemic vasculitis which involve small arterioles and capillaries. Clinical manifestations show generalized weakness, fever, myalgia, weight loss. oliguria, proteinuria, skin rash, neuromuscular symtoms, and pulmonary, gastrointestinal symtoms. Prednisolone and cyclophosphamide therapy is effectve, more than 80% of patients with microscopic polyangiitis survive for longer than 5years. However, about 50% of patients will have at least one relapse in the 4-5years after initial treatment. There is a very strong correlation between the presence of ANCA(especially p-type) and microscopic polyangiitis. We herein report a case of microscopic polyandiitis with the review of literature. A 65 years-old woman admitted to our hospital due to general weakness, fever, generalized edema, and dyspnea. Laboratory result showed high serum creatinine and CRP, moderate proteinuria, and microscopic hematuria. P-ANCA was 1:1280 positive in indirect immunofluorescence method, and kidney biopsy showed focal segmental necrotizing glomerulonephritis, crescent formation, fibrin clots, PMN and lymphocytes infiltrations in small arterioles and capillries. Immunofluorescence study was negative. Her clinical feature, laboratory findings, and the kidney biopsy result were compatible with microscopic polyangiitis. The patient had been treated with prednisolone and cyclophosphamide. P-ANCA titration was decreased 1:320 at the 19th day of treatment and was negative about 2months later.

Antibodies to human T-cell lymphotropic virus type I (HTLV-I) by particle agglutination (PA) test in Korean blood donors

HTLV-I infection is a recently recognized disease entity that is common in some tropical and subtropical areas, including the southwestern district of Japan. Despite the geographical proximity and frequent cultural exchanges between Korea and Japan, it is understood that Korea is not an endemic area and HTLV-I-associated illnesses are very rare in Korea. This study was designed to evaluate the positive rate of anti-HTLV-I antibodies in Korean blood donors and its regional distribution. Sera were obtained from blood donors from various districts around Korea. Anti-HTLV-I antibodies were detected by using the microtiter particle agglutination test employing an indirect agglutination technique. A total of 9,281 donors were tested and 12 donors (0.13%) were positive for anti-HTLV-I antibodies, 10 (0.11%) out of 8,845 males and 2 (0.46%) out of 436 females, with relative female predominance. A relatively high incidence of anti-HTLV-I positive donors was observed in Cheju Island (0.80%), Kyungnam (0.31%), and Chonnam (0.15%). In conclusion, the positive rate of anti-HTLV-I antibodies seemed to be very low in Korea, but the highest positive rate of anti-HTLV-I antibodies was noticed on Cheju Island, warranting further research for confirmation.

A case of "scleroderma renal crisis" developed from diffuse scleroderma / 대한내과학회지

Polymyositis is an inflammatory, autoimmune disease of the skeletal muscle characterized by symmetrical, proximal muscle weakness, elevated muscle enzymes, and characteristic features on electromyogram and muscle biopsy. The kidneys are generally spared and myoglobinuric renal failure is very rare in polymyositis. There have been infrequent reports of polymyositis developing myoglobinuric renal failure secondary to rhabdomyolysis. The flare-up may occur in polymyositis, usually manifest within several weeks to months of achieving a remission. But, rhabdomyolysis and myoglobinuric renal failure was a very rare feature of the relapse of polymyositis. We present a case report of patient with polymyositis who initially presented and relapsed as rhabodomyolysis that lead to myoglobinuric, oliguric renal failure and required transient dialytic support.

Relationship between Plasma Taurine Concentrations and Left Ventricular Dysfunction in Hemodialysis Patients / 대한신장학회잡지

In maintenance dialysis patients, death is most often due to cardiac disease. The common causes of death in nondiabetic dialysis patients are congestive heart failure. Relation between taurine and cardiac contractility had been reported in animal studies. Uremic patients have taurine deficiency and this may induce impairment in cardiac contractility. We performed this study to evaluate the relationship between plasma taurine concentration and left ventricular(LV) function by echocardiography in clinically stable 23 hemodialysis(HD) patients. HD patients had significantly lower plasma taurine concentrations(29.1+/-9.4 micromol/L) compared to control(57.6+/-17.5 micromol/L). There were significant correlations between plasma taurine concentrations and fractional shortening(FS)(r=0.422, P<0.05), and taurine and LV end-diastolic diameter(LVEDD)(r=-0.531, P<0.01). By partial correlation analysis of controlling hematocrit, this correlations were maintained between taurine and LVEDD(r=-0.46, P<0.05) and there was a tendency of correlation between taurine and FS(r=0.37, P=0.09). There was a significant correlation between plasma taurine concentrations and normalized protein catabolic rate(NPCR)(r=0.42, P<0.05), insulin-like growth factor 1(IGF-I)(r=0.43, P<0.05) and prealbumin(r=0.54, P<0.05). Group I in whom plasma taurine concentrations were below median value had significantly lower FS(29.7+/-8.2 vs. 38.5+/-8.0%, P<0.05) and higher LVEDD(57.7+/-4.7 vs. 53.1+/-3.0mm, P<0.05) and LV mass index(299.2+/-73.1 vs. 192.4+/-49.7g/m2, P<0.05) than group II in whom plasma taurine concentrations were above median value. On linear multiple regression analysis, plasma taurine concentrations and diastolic blood pressure were independently correlated with FS. In conclusion, taurine deficiency may be one of the factors affecting LV dysfunction in maintenance HD patients.

A Case of Nutcracker Syndrome in a Patient with Gross Hermaturia / 대한신장학회잡지

Nutcracker syndrome(renal vein entrapment syndrome) is probably more common than previously suspected. The nutcracker phenomenon refers to compression of left renal vein between aorta and superior mesenteric artery that results in elevation of pressure in left renal vein and develoment of collateral veins. This syndrome occurs in relatively young and previously healthy patients and is characterized by intermittent gross hematuria due to left renal vein hypertension, at times associated with flank pain, abdominal pain or varicocele. We report a 17 years-old male patient with this syndrome presented with flank pain, abdominal pain, and intermittent gross hematuria for 3 months. Urinalysis revealed protein(-), blood(+++), many RBC with only 1% of dysmorphic RBC. IVP and cystoscopy showed no remarkable finding but doppler ultrasonography and abdominal spiral CT revealed compression of left renal vein between aorta and superior mesenteric artery. Renal venography showed compression of left renal vein and collateral circulation to left gonadal vein and the pressure gradient between left renal vein and inferior vena cava was 11mmHg. The nutcracker syndrome should be considered as one of the causes of nonglomerular hematuria. All patients with unexplained severe left flank or abdominal pain, or unilateral hematuria from the left on cystoscopy, should be studied by selective renal venography and pressure measurement in inferior vena cava and renal veins. The patient with this typical syndrome could be treated surgically, by transposition of left renal vein and resection of collateral veins as the procedure of choice to correct the underlying pathologic process and eliminate these troublesome symptoms.

Effects of Postdialysis Urea Rebound on Dialysis Adequacy in Hemodialysis Patients / 대한신장학회잡지

Urea reduction ratio (URR) and Kt/Vurea are objective parameters of dialysis delivery in hemodialysis patients and correlate with nutritional status and patient outcome. URR and Kt/Vurea depend on postdialysis blood urea nitrogen (BUN). In patients with severe postdialysis urea rebound (PDUR), these parameters do not accurately reflect dialysis adequacy. We measured PDUR 30 minutes after dialysis in 26 chronic stable hemodialysis patients. The impact of PDUR on dialysis delivery assessed by URR and Kt/Vurea and the independent factors affecting on PDUR were evaluated. All patients had been dialyzed for 4 hours thrice a week using hemophan membrane. 1) The mean age of patients was 48.6+/-14.8 years and sex ratio was 1:2.3. The mean duration of hemodialysis was 42.7+/-45.0 months. Primary renal diseases were chronic glomerulonephritis 11 (42.3%), diabetic nephropathy 7 (26.9%), and hypertension 4 (15.4%). 2) The mean blood flow was 209.2+/-17.4ml/min. URR, Kt/Vurea, and nPCR using immediate postdialysis BUN were 60+/-7%, 1.13+/-0.21, 1.09+/-0.28g/kg/ day, respectively. The mean recirculation rate was 4.4+/-2.3%. 3)The mean PDUR was 12.2+/-4.6% (range:6-22 %). URR, Kt/Vurea, and nPCR using BUN 30 minutes after dialysis were 55+/-7%, 0.99+/-0.18, and 1.02+/-0.25 g/kg/day, respectively and were significantly lower than those using immediate postdialysis BUN (P or = 12%), high PDUR group was significantly higher than low PDUR group in hematocrit (27.0+/-2.6 vs. 23.5+/-3.6%, P=0.008), URR (64.3+/-5.4 vs. 55.8+/-6.8%, P=0.002), Kt/Vurea (1.26+/-0.17 vs. 1.03+/-0.18, P=0.002), and total recirculation rate (5.6+/-2.7 vs. 3.6+/-1.7%, P=0.05). There were no differences in age, sex, postdialysis body weight, ultrafiltration rate, blood flow, serum albumin, predialysis BUN, creatinine, and nPCR. 5) In multiple regression analysis, the independent factors affecting on PDUR were Kt/Vurea (beta=0.546, P<0.001), recirculation rate (beta=0.422, P<0.001), and hematocrit (beta=0.366, P=0.0017). In conclusion, we think that PDUR should be considered in hemodialysis patients when estimating dialysis delivery, especially if they had high Kt/ Vurea, recirculation rate, and hematocrit.

Plasma Leptin Concentration in Patients with Chronic Renal Failure / 대한신장학회잡지

Leptin, which is a plasma protein produced by the obese gene, is expressed and secreted by adipocytes. The clearance of lepdn from the circulation is unknown. But, markedly elevated serum leptin concentrations have recently been reported in patients with chronic renal failure. The purpose of the present study was to investigate plasma leptin concentration of patients with chronic renal failure and evaluate the factors affecting plasma leptin levels. Plasma leptin, insulin, and body mass index were determined in 34 patients with chronic renal failure and 55 control subjects. The plasrna leptin concentrations were not significantly different between patients with chronic renal failure and control subjects (9.4+/-11.8 vs 4.9+/-4.2ng/ml, P>0.05). The serum leptin concentrations were not significantly higher in both male and female CRF patients compared with control subjects (3.96+/-5.72 vs 2.48+/-1.65, P=0.1947, 17.07+/-14.02 vs 7.49+/-4.63ng/ml, P=0.07, respectively). And, there was no significant correlation between serum creatinine and plasma leptin. However, there was significant correlation between plasma leptin concentration and insulin level (P<0.05). We fit a multiple linear regre- ssion analysis with plasma leptin level as the dependent variable in CRF. Sex (male vs female) (P< 0.001) and insulin (P=0.004) were independently associated with plasma leptin level in CRF. These results suggested that plasma leptin level was regulated or affected by multiple factors inclu- ding sex and insulin resistance. Additional study is required to evaluate relationship between plasma leptin and insulin resistance in chronic renal failure.

Anabolic Steroid(AS) on Nutritional Parameters in Malnourished Continuous Ambulatory Peritoneal Dialysis(CAPD) Patients / 대한신장학회잡지

Protein-energy malnutrition is frequently observed in CAPD patients and is closely correlated with morbidity and mortality. We investigated the effects of AS on nutritional parameters in malnourished CAPD patients. Eleven CAPD patients who were proved to be malnourished by biochemical and anthropometric measurements, not admitted for 2 months recently, received intramuscularly 200mg of Nandrolone Decanoate, monthly. After AS was administered for more than 12 weeks, the nutritional parameters were checked. The mean age of patients was 53.5+/-9.8(SD) years, the sex ratio was 4:7. The mean duration of CAPD was 34.5+/-23.2 months. Three patients were diabetics. The mean duration of AS administered was 22.0+/-3.6 weeks. Eight of eleven patients improved their subjective symptoms such as appetite and muscle strength. There were significant increases in hemoglobin(7.0+/-0.9 vs. 7.8+/-1.1g/dl), hematocrit(20.3+/-2.8 vs. 23.0+/-2.8%), insulin-like growth factor I(165.9+/-71.6 vs. 207.1+/-69.5ng/ml), prealbumin(30.1+/-11.0 vs. 39.3+/-9.1mg/dl), transferrin(205.4+/-48.8 vs. 239.6+/-47.7mg/dl) and nPCR(0.75+/-0.15 vs. 0.84+/-0.15g/kg/day)(P<0.05). There were no changes in albumin, total lymphocyte, cholesterol, weeklky Kt/Vurea, weekly creatinine clearance and urea nitrogen appearance. On anthropometric measurements, midarm muscle circumference(21.8+/-3.2 vs. 22.7+/-3.5cm, P<0.05) and % lean body mass(57.6+/-12.7 vs. 60.5+/-11.4%, P<0.05) were increased, but triceps skinfold thickness, and body mass index were not changed. Edema was observed in 8 patients and acne was noted in a patient. Severe edema and ultrafiltration failure were observed in 2 patients. The mean time that edema developed was 17.6 4.6 weeks. In conclusion, AS seems to be useful in correcting malnutrition with their protein anabolic effect in malnourished CAPD patients.

The Effect of Anabolic Steroid on Body Composition and Protein Metabolism in Malnourished CAPD Patients / 대한신장학회잡지

To evaluate the effect of anabolic steroid(AS) on body composition and protein metabolism in malnourished CAPD patients, 7 CAPD patients who were proved to be malnourished by subjective global assessment received 200mg of AS, nandrolone decanoate, by intramuscular route monthly for 5 months. We analyzed the body composition, biochemical nutritional markers, plasma and dialysate amino acids(AA) before and after administration of AS. The mean age was 53.6+/-11.5 years, the sex ratio was 2:5, and the duration of CAPD was 28.5+/-18.7 months. Two patients were diabetics. Five months after AS administration, %lean body mass seemed to increase from 52.6+/-11.7 to 57.3+/-11.0%(P>0.05), but there were no significant changes in body weight and body mass index. Serum insulin-like growth factor-I (172.4+/-88.5 vs. 204.1+/-85.8ng/ml, P<0.05) and transferrin(213.6+/-55.4 vs. 239.0+/-55.3mg/dl, P<0.05) were significantly increased, and serum total CO2(31.1+/-4.5 vs. 25.9+/-2.5mM/L, P<0.05) were significantly decreased. Urea nitrogen appearance(4.0+/-2.0 vs. 3.6+/-1.6g/ day) seemed to be decreased. Both plasma essential AA(EAA) level(738.3+/-206.5 vs. 492.5+/-187.2micromol/L, P<0.05) and peritoneal EAA loss(565.0+/-186.1 vs. 377.4+/-113.9micromol/day, P<0.05) decreased significantly after administration. Plasma total and non- essential amino acids(NEAA) level tended to be increased and peritoneal total and NEAA loss seemed to be decreased without statistical significance. In conclusion, AS seems to have anabolic effect and be useful in the correction of malnutrition in CAPD patients.

A Case of Bilateral Renal Angiomyolipoma Associated with Tuberous Sclerosis / 대한신장학회잡지

Renal angiomyolipoma is a rare entity, usually associated with tuberous sclerosis. Until a few years ago it was almost impossible to arrive at a correct diagnosis preoperatively, and most of the case underwent nephrectomy with the wrong diagnosis of renal carcinoma. Recently, abdominal computerized tomography and renal sonography have made it possible to reach the correct preoperative diagnosis in many cases, and thus to avoid unnecessary nephrectomies. We herein present a case of bilateral renal angiomyolipoma with tuberous sclerosis which was preoperatively diagnosed by abdominal ultrasound and CT. A 54 years old woman was admitted with pain on the left flank since 4 months ago. She has had multiple erythematous papules on the face, a small nodule like fibroma on the right third toe. Laboratory findings were normal range. Abdominal ultrasound revealed multiple, variable sized homogenous hyperechogenic mass in the both kidney. Abdominal CT scan showed multiple, variable sized low dense mass in both kidney. Biopsy specimens of the mass of the left kidney revealed fibronecrotic material and blood clots, in which was no evidence of malignancy. Biopsy specimen from the face revealed adenoma sebaceum.

Clinical Outcome of the HBsAg Positive Malignant Lymphoma Patients Following Anticancer Chemotherapy / 대한내과학회지

OBJECTIVES: Infection and replication of the hepatitis B virus are closely related to the host immunity. Anticancer chemotherapy decreases the immune response of the host, Especially, glucocorticoid can activate the replication of hepatitis B virus directly. It is well known that hepatitis B virus infection and hepatic complications are more common in patients with hematologic malignancies like malignant lymphoma. We studied the incidence of hepatitis B virus infection and hepatic complications following anti-cancer chemotherapy in patients with malignant lymphoma. METHODS: Among 224 cases diagnosed as malignant lymphoma from January 1989 to December 1993 at Yonsei University Medical Center, 77 cases tested for hepatitis B virus serology was studied. RESULTS: 1) Eighteen cases (23%) was HBsAg positive. 2) The results of hepatitis C virus serology in six cases were all negative. 3) Eight (57%) of 14 follow-up cases had hepatic complications, Two patients had fulminant hepatitis, two nonicteric hepatitis and four icteric hepatitis. 4) Interferon-alpha was administered in three cases among the patients with hepatic complications. Loss of HBeAg was observed in one case and loss of HBsAg in another case. CONCLUSION: Serious hepatic complications can be occurred following anticancer chemotherapy in HBsAg-positive patients with malignant lymphoma. Therefore, we recommend that patients being considered as candidates for anticancer chemotherapy should routinely undergo serologic test for Hepatitis B virus. In addition HBsAg-positive patients with anticancer chemotherapy should be regularly monitored for hepatic injury. And with the careful use of steroid and interferon, prolongation of survival might be searched for these patients.

Serum Albumin as a Predictor of Morbidity and Mortality in Continuous Ambulatory Peritoneal Dialysis(CAPD) Patients / 대한신장학회잡지

Serum albumin(SA) is a powerful predictor of morbidity and mortality in hemodialysis(HD) population. In CAPD patients, SA may be more severely compromised because of large dialysate protein losses and lower protein intake relative to HD patients. However, the usefulness of SA as a marker for increased morbidity and mortality in CAPD patients remains undefined. To investigate the role of SA and other risk factors as predictors of peritonitis, hospitalization and death in CAPD patients, we conducted this retrospective study on 782 patients who were followed-up for more than 3 months after initiation of CAPD at Severance Hospital from January 1985 through August 1995. For each patient the following data were collected: age, sex, duration on dialysis, presence of diabetes mellitus or cardiovascular disease, and monthly biochemical data including SA. 1) The mean age of the patients was 45.5+/-13.6 years(range, 14-80) with sex ratio 1.2:1 and mean duration on dialysis was 30.8+/-25.6 months(range, 3- 122). 2) Independent factors affecting serum albumin level were creatinine(beta=0.301, por=4.0g/dL), and incidence of peritonitis, hospitalization and death were compared among them. Compared to group 2 and group 3, group 1 had significantly higher peritonitis rate(1.18 vs. 0.87, 0.66 times/year/patient, p<0.05) and hospitalization(7.93 vs. 4.70, 3.36 days/year/patient, p<0.05). The 5- year survival rates in group 1, group 2 and group 3 were 43.4%, 60.9% and 84.1%, respectively (p<0.05). In conclusion, low SA is an important and strong predictor for morbidity and mortality in CAPD patients. Prospective studies are needed to determine if aggressive intervention with nutritional supplements could improve the SA level and to determine if such an improvement would impact on morbidity and mortality.