The Evolution of Lupus Activity among Patients with End-Stage Renal Disease Secondary to Lupus Nephritis

The aim of this study was to evaluate the evolution of lupus activity in end-stage renal disease (ESRD) patients due to lupus nephritis and to determine the long-term prognosis. We reviewed the clinical courses of 45 patients with ESRD due to systemic lupus erythematosus (SLE). We analyzed the course of SLE following the onset of ESRD, with special attention to the clinical and serological manifestations, survival time on dialysis, and renal transplantation outcome. Disease activity was measured using the SLE Disease Activity Index (SLEDAI). Of the 45 patients, 21 patients were being treated with hemodialysis (HD), 11 were undergoing peritoneal dialysis (PD), and 13 underwent transplantation. Duration of follow- up was 53 +/- 29 months. The SLEDAI score on commencement of renal replacement therapy was not significantly different among the 3 groups (HD: 4.2 +/- 4.2, PD: 4.3 +/- 2.3, Transplant: 3.2 +/- 1.9). However, disease activity scored by follow-up maximal SLEDAI during dialysis or transplantation showed a significant increase after peritoneal dialysis (HD: 5.0 +/- 3.6, PD: 7.4 +/- 3.7, Transplant: 2.2 +/- 1.7, p < 0.05). When the individual changes in the maximal SLEDAI score were considered, a significant increase was apparent after peritoneal dialysis (p < 0.05), but not after either hemodialysis or renal transplantation. There was no significant difference in cumulative survival rate, and also in technique or graft survival rates of the 3 groups. Among the variables tested, follow-up maximal SLEDAI score was the only significant factor associated with patient survival (odds ratio: 1.15, p < 0.05). The incidence (36% versus 19%) of high disease activity was greater, but not significantly, in the peritoneal dialysis group, as compared to the hemodialysis group. Clinical activity of SLE was apparent in 65% of patients in the first year of dialysis, but none showed any activity after the third year of dialysis. We found that although lupus disease activity declined after patients progressed to ESRD, lupus disease activity still affected patients' survival. An incremental increase in postdialysis lupus activity was not uncommon, especially during the first one year of dialysis. During the follow-up period, maximal SLEDAI score increased significantly after peritoneal dialysis. However, the long-term prognosis was not significantly different according to the treatment modality.

A Case of Penicillium merneffei CAPD Peritonitis / 대한신장학회잡지

P. marneffei is a fungus that causes life-threatening disseminated infection in a geographically distinct areas of the world. Following the first case of human infection in 1959, the incidence of this infection has risen markedly during the past 5 years. However, even in the midst of such rapid increase, the infection has always occurred only in a limited geographic distributions or in persons who have visited this limited geographic areas. These areas include Myanmar, Hong Kong, Indonesia, Laos, Malaysia, Singapore, Taiwan, Thailand, Vietnam, and the Guangxi province of southern China. P. marneffei infection occurs mostly in immunocompromised patients, particularly AIDS patients. P. marneffei infection commonly presents with skin and subcutanous tissue infection, fungemia, diarrhea, bone marrow infection, and generalized lymphadenopathy with hepatosplenomegaly. We report the case of continuous ambulatory peritoneal dialysis-associated peritonitis caused by P. marneffei. The case occurred in Korea, a non-endemic area of P. marneffei, in a non-AIDS patient who has not been exposed to any of the endemic areas. This warrants further consideration in determining the yet unknown transmission route of this fungal organism. P. marneffei was diagnosed without delay by 18sRNA PCR and sequencing, and was later confirmed by culture. PCR and sequencing may contribute to the early diagnosis of the P. marneffei infection, which is important given this infection's ability to progress to a systemic infection with high mortality rate when diagnosis and management are delayed.

Factors Affecting Malnutrition in Continuous Ambulatory Peritoneal Dialysis Patients: A Cross-sectional Study / 대한신장학회잡지

PURPOSE: Protein-calorie malnutrition is common in continuous ambulatory peritoneal dialysis (CAPD) patients and is associated with an increase in morbidity and mortality. This cross-sectional study was carried out to investigate prevalence of protein-calorie malnutrition in Korean CAPD patients. Other purposes were to ascertain relationship between various nutritional parameters and to evaluate factors independently associated with malnutrition in CAPD patients. METHODS: Subjects were clinically stable 127 patients who have been on CAPD for more than 3 months. Nutritional status was assessed by subjective global assessment (SGA), biochemical, anthropometric, and urea kinetic parameters. RESULTS: The mean age of the patients was 50.7+/-11.4 years with sex ratio (M : F) 1 : 1, and mean duration of dialysis was 67.3+/-39.7 months. Patients were divided into 3 groups according to SGA : group I (normal nutrition, n=75, 59.0%), group II (mild malnutrition, n=30, 23.6%) and group III (moderate to severe malnutrition, n=22, 17.4%). There were significant differences in age, CAPD duration, peritonitis rate and hospital-days per year between group I and group III. Among biochemical variables, serum creatinine, triglyceride, protein, albumin, prealbumin, IGF- 1, transferrin and leptin were significantly lower in group III compared to group I (p<0.05), and CRP, ferritin, and IL-6 were significantly higher in group III than group I. There were significant differences in serum albumin among all three groups. There were no differences in Kt/Vurea, nPNA and SCCr among three groups. However, there were significant differences in residual renal function (p<0.05) and PNA (p<0.05) between group I and group III. Among anthropometric variables, body weight, %IBW, BMI, %body fat mass, MAC, TSF, BSF, CAMA, and TBM were significantly lower in group III than the other two groups (p<0.05). But, there was no differences in exercise capacity and food intake among the three groups. To evaluate relationship between various parameters used for assessing malnutrition, we conducted Pearson's rank correlation test. Serum albumin (gamma=0.45), age (gamma=-0.29), %IBW (gamma=0.52), leptin (gamma=0.32), CRP (gamma=-0.24), TSF (gamma=0.45) and CAMA (gamma=0.41) significantly correlated with SGA. As a result of multiple regression analysis, albumin, leptin, and CRP were independent predictors of malnutrition (p<0.05). CONCLUSION: About 40% of CAPD patients were malnourished according to SGA, and multiple regression analysis revealed that serum albumin, leptin and CRP were independent predictors of malnutrition. These results suggest that acute and chronic inflammatory response plays an important role in the development of protein-calorie malnutrition in CAPD patients with comparable food intake and dialysis dose.

Risk Factors of Left Ventricular Hypertrophy in CAPD Patients / 대한신장학회잡지

BACKGROUND: Left ventricular hypertrophy (LVH) is a critical and an independent factor for mortality of patients with end-stage renal disease, and numerous risk factors for LVH have been discussed in previous studies. In present study, we intended to clarify the factors that affect the progression of LVH in patients with their first continuous ambulatory peritoneal dialysis (CAPD) and to analyse the influences of these risk factors on severity of LVH. METHODS: This retrospective study enrolled the patients who performed echocardiography both before and in period between 24 to 30 months after CAPD. We estimated the change of LVH by the calculated difference of left ventricular mass index (LVMI) on echocardiography. We analyzed the factors that influence the change of LVMI such as age, sex, baseline renal disease, body mass index, blood pressure, hematocrit, calcium, phosphate, intact parathyroid hormone (i-PTH), serum albumin and peritoneal transport status on peritoneal equilibration test (PET). RESULTS: The causes of renal disease of the patients (male : female=10 : 16, mean age 55.74+/-12.0 years) were as follows : 13 cases (50.0%) of diabetic nephropathy, 11 cases (47.4%) of chronic glomerulonephritis, 1 case (3.8%) of hypertensive nephrosclerosis, and 1 case (3.8%) of unknown cause. Mean duration of follow-up was 25.5+/-2.1 months. As a result, the difference of LVMI positively correlated with mean systolic blood pressure (p=0.001, r=0.598) and mean diastolic blood pressure (p<0.001, r=0.718), difference of pulse pressure (p<0.001, r=0.893), and maximal i-PTH level (p=0.041, r=0.404). On the other hand, the difference of LVMI showed negative correlation with mean hematocrit (p=0.031, r=-0.421). In multiple linear regression analysis, the mean diastolic blood pressure and the difference of pulse pressure appeared to be the independent risk factors for the difference of LVMI (R2=0.923). CONCLUSION: The factors necessary to restrict the progression of LVH after initiation of CAPD are strict blood pressure control, correction of anemia, optimal treatment of secondary hyperparathyroidism. These corrections could secure the amelioration of LVH.

A Case of Fabry Disease Diagnosed by Molecular Analysis of alpha-galactosidase A Gene / 대한신장학회잡지

Fabry disease is a X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase A. This abnormality in enzyme results intracellular accumulation of globotriaosylceramide and leads to severe painful neuropathy with progressive renal, cardiovascular, and cerebrovascular dysfunction and early death. We report a 35 year-old man who had been suffered from acroparesthesia aggravated by body temperature elevation and with asymptomatic renal function impairment, which were proven to be due to Fabry disease. We performed gene analysis by PCR direct sequencing and confirmed missense mutation of GLA gene. Recently enzyme replacement of alpha-galactosidase was introduced and we think that the importance of early diagnosis and treatment should be emphasized.

Kidney Transplantation in Diabetic Patients: Clinical Study in a Single Center / 대한이식학회지

PURPOSE: End stage renal disease caused by diabetic nephropathy is increasing throughout the world. In earlier years, the results of kidney transplantation in diabetics were not as good as those in non-diabetics and the presence of diabetes has been considered as contraindication at many centers. But the survival rate of diabetic patients treated with transplantation has improved in recent years. In this study we compared the results of kidney transplantation in diabetic patients group with those of non-diabetic patients group. METHODS: We reviewed our experience in a single center with 1,386 kidney transplantation patients in non-diabetic patients, compared with 31 kidney transplantation patients in diabetic patients. The clinical characteristics such as age, sex, duration of diabetes mellitus, serum albumin, blood urea nitrogen, hemoglobin, glycated hemoglobin, creatinine clearance, and morbidity were retrieved from medical charts. RESULTS: For diabetic transplantation patients one- and five year patient survival were 92.3% and 84%; for non-diabetic transplantation patients one- and five year patient survival were 98.7% and 93.4%. It showed statistically significant differences in patient survival between two groups. We analyzed graft survival in two ways. When all deaths were not censored, the graft survival rate of diabetic transplantation patients was significantly lower than that of non-diabetic transplantation patients: 80.6% vs 85.8% at 5 years and 27.3% vs 68.6% at 10 years (P=0.04). But the graft survival rate did not differ significantly between the diabetic and non-diabetic patients when deaths were censored: 95% vs 91.7% at 5years and 63.3% vs 79.5% at 10 years (P=0.96) In the analysis of risk factors affecting patient mortality, presence of DM and graft loss were associated with mortality and its odds ratios were 8.94 and 6.33 respectively. CONCLUSION: The overall patient survival and graft survival were significantly worse in the diabetic transplantation patient group than the non-diabetic transplantation patient group. But graft survival was not different between two groups when death was censored. This means that graft survival in diabetic transplantation group is not different actually with non- diabetic transplantation group when comorbidities are fully evaluated and treated before transplantation.

Selective Proteinuria Index as a Prognostic Index in IgA Nephropathy / 대한신장학회잡지

PURPOSE: Proteinuria is the hallmark of glomerular injury and results from alterations in glomerular permeability. The permeability of diseased glomerulus has been estimated by selectivity of proteinuria. Recently, some authors showed a significant relationship between selectivity of proteinuria and tubulointerstial damage. The present study examines the role of protein selectivity as a prognostic marker in patients with IgA nephropathy and its correlation with other prognostic indices. METHODS: The selective proteiuria index of 81 cases with IgA nephropathy diagnosed between 1990 and 2000 were reviewed, and each case was subclassified using the following : highly selective(SPI0.2). The mean age of the patients was 27+/-15 years with a follow-up period of 38+/-24 months. Six patients had highly selective proteinuria, thirty three patients had moderately selective proteinuria, and forty two patients had nonselective proteinuria. RESULTS: 1) A significant relationship was found between the SPI and Haas subclasess(p=0.01). With respect to clinical presentaion, hypertension(0, 4, 11 cases, p<0.05), proteinuria(0.52+/-0.35, 1.85+/-1.55, 2.79+/-2.51 g/day, p<0.05) were significant correlation.2) Chronic renal failure was significantly higher in patients with nonselective proteinuria in comparison with patients with selective proteinuria(p<0.05).3) Markers of renal failure by the Cox proportional hazards model were Cr(Exp(B)=4.2, p<0.001), Ccr (Exp(B)=2.1, p<0.05), SPI(Exp(B)=1.7, p<0.05), hypertension(Exp(B)=1.6, p<0.05). 4) In 28 patients of IgA nephropathy with nephrotic syndrome, 9 patients were moderately selective, 19 patients were nonselective. The response to therapy, evaluated retrospectively, was 67% and 16% in moderate and nonselective proteinuria(p=0.01). CONCLUSION: There is a significant relationship between selectivity of proteinuria and clinical para meters. Moreover, the selectivity of proteinuria has a predictive value on functional outcome.

Subglottic Stenosis in Wegener's granulomatosis: A Case Report / 대한류마티스학회지

Wegener's granulomatosis (WG)is a multisystemic inflammatory disease characterized by necrotizing granulomatous inflammation and vasculitis of unknown etiology which classically affects the upper airway,lung,and kidney.Subglottic stenosis (SGS)is rare and occurs independently of other features of active WG. SGS can be a life-threatening manifestation of disease,and prompt diagnosis is essential.Since the presenting symptoms of SGS can be nonspecific,SGS should be considered in the differential diagnosis of any patient with WG who has increasing dyspnea,voice change,or cough.Although both surgical and medical treatments have been utilized,the optimal therapeutic approach to SGS in patients with WG has not been determined.So far,a case of WG with SGS has not been reported in Korea.Recently,we experienced a case of 59-year-old woman with SGS in WG.She responded to prednisolone and methotrexate therapy and did not require surgical intervention.So we report this case with a review of literatures.

A Case of Cronkhite-Canada Syndrome with a Remission to Steroid Therapy / 대한소화기내시경학회지

Cronkhite-Canada syndrome is characterized by generalized gastrointestinal polyposis, ectodermal changes, and the eventual development of diarrhea and weight loss. The pathogenesis of the disease is unknown, and there is no established therapy. The disease has a poor prognosis because of malnutrition resulting from altered absorption in the gastrointestinal tract. We experienced a case of a 56-year-old female with Cronkhite-Canada syndrome. After enteral nutrition and administration of prednisolone for 3 months, clinical improvement was noted with cessation of diarrhea, increased serum protein, disappearance of pigmentation, and regrowth of the scalp hair, finger-and toenails. Endoscopy showed resolution of the gastrointestinal polyposis. So we report here a case of diffuse gastrointestinal polyposis which has been in remission with steroid therapy.

Squamous Cell Carcinoma of the Cornea

In this paper, a case of corneal squamous cell carcinoma is reported. Invasive squamous cell carcinoma of the cornea is a rare disorder and has not been previously described in the Korean literature. In this case, the invasive squamous cell carcinoma of the cornea was treated by complete excision and cryotherapy. No evidence of metastasis or recurrence has been found since the procedure. Complete excision and adjunctive cryotherapy has become the treatment of choice because of the higher recurrence rate following a simple excision.

Efficacy of Pre- and Postoperative Chemotherapy in Patients with Osteosarcoma of the Extremities / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: We evaluated the treatment efficacy including survival and recurrence, and factors associated with recurrence in osteosarcoma patients treated with preoperative chemotherapy, surgery, and adjuvant chemotherapy. MATERIALS AND METHODS: Forty nine patients with osteosarcoma were treated with preoperative chemotherapy with intra-arterial cisplatin and adriamycin infusion for 3 cycles, followed by surgery. According to the pathologic response, if tumor was necrotized more than 90%, the same adjuvant chemotherapy was reintroduced for 3 cycles, and if the response was not enough, then the salvage regimen was introduced. Plain chest film and chest CT scan were taken monthly and every 3 months, respectively. When tumor recurred, the metastasectomy was performed whenever possible. RESULTS: Forty three patients were evaluable with amedian follow up of 53 months. Five-year disease-free and overallsurvival rate was 47.0% and 66.9%, respectively. The recurrence was observed in 22 patients (51.2%) with median time of 12.5 months. Baseline alkaline phosphatase (ALP) was the only significant factor for recurrence (p=0.03) and the patients with the possibility of metastasectomy recurrence showed higher post-relapse survival compared to other treatment modalities (26 momths vs 5~12 months). CONCLUSION: These results indicates that pre- and postoperative chemotherapy with intra-arterial cisplatin and adriamycin infusion showed comparable treatment efficacy and acceptable toxicities.

Two Cases of Malignant Lymphoma Involving Bilateral Adrenal Glands as Huge Masses / 대한내분비학회지

Adrenal gland is a common site of metastatic tumors such as breast cancer, lung cancer, and colon cancer. When adrenal mass is found incidentally, adenoma is the most common among single adrenal masses. But in the case of bilateral adrenal masses, infection, bilateral metastases and hemorrhage are common. Secondary involvement of the adrenal gland is found in 25% of autopsy cases of non-Hodgkin's lymphoma. However, adrenal insufficiency is rare because it becomes apparent only when approximately 90% of adrenal cortex is destructed. We exprienced two cases of malignant lymphoma which involved the adrenal glands bilaterally. One case in which adrenal insufficiency was suspicious, was accompanied by hypovolemic shock and sepsis at the initial presentation. He died of sepsis combined with DIC even though hydrocortisone, intravenous saline infusion, and antibiotics therapy were started immediately. The other one was found incidentally, in which adrenal infiltraion was confirmed by CT scan. Hormonal level was normal and adrenal masses disappeared after chemotherapy.