Middle East respiratory syndrome clinical practice guideline for hemodialysis facilities

The Korean Society of Nephrology participated in the task force team consisting of government authorities and civilian experts to prevent and control the spread of Middle East respiratory syndrome (MERS) in 2015. The Korean Society of Nephrology MERS Task Force Team took an immediate action and drafted ‘the clinical recommendation for hemodialysis facilities’ to follow when the first and the only confirmed case was reported in the hemodialysis unit. Owing to the dedicated support from medical doctors, dialysis nurses, and related medical companies, we could prevent further transmission of MERS infection successfully in hemodialysis units. This special report describes the experience of infection control during MERS outbreak in 2015 and summarizes the contents of ‘the clinical practice guideline for hemodialysis facilities dealing with MERS patients’ built upon our previous experience.

Tumoral calcinosis and calciphylaxis treated with subtotal parathyroidectomy and sodium thiosulphate / 영남의대학술지

Tumoral calcinosis (TC) is a condition resulting from extensive calcium phosphate precipitation, primarily in the periarticular tissues around major joints. Calciphylaxis is a fatal ischemic vasculopathy mainly affecting dermal blood vessels and subcutaneous fat. This syndrome is rare and predominantly occurs in patients with end-stage renal disease. Here, we report on a rare case involving a patient with TC complicated with calciphylaxis. Our patient was a 31-year-old man undergoing hemodialysis who presented with masses on both shoulders and necrotic cutaneous ulcers, which were associated with secondary hyperparathyroidism, on his lower legs. He underwent subtotal parathyroidectomy, and sodium thiosulfate (STS) was administered for 27 weeks. Twenty months after beginning the STS treatment course, he experienced dramatic relief of his TC and calciphylaxis.

Methylprednisolone Pulse Therapy in Adult-Onset Minimal Change Nephrotic Syndrome / 대한신장학회지

PURPOSE: The incidence of complete remission is lower and the relapse is more frequent in adult-onset minimal change nephrotic syndrome (MCNS) are observed especially when compared with those in children. This study was designed to examine the effect of methylprednisolone pulse therapy in adultonset MCNS comparing to oral steroid as an initial therapeutic modality. METHODS: We have retrospectively reviewed the clinical data of 25 adult-onset MCNS patients. Twelve patients were treated with three intravenous pulses of methylprednisolone (1 g daily) followed by oral prednisolone 1 mg/kg daily for 4-8 weeks and also by low doses of oral prednisolone for 4-6 months (MP group) Thirteen patients were initially treated with oral prednisolone 1 mg/kg daily for 4-8 weeks and then with low doses of oral prednisolone (PD group). RESULTS: The response to therapy was similar between MP and PD group, with a complete remission obtained in 83.3% and 84.6%, respectively. No statistically significant difference between the two groups was observed in the rate of response at 8 weeks (58.3% versus 69.2%). The mean time to response was not different between MP group (37.9+/-28.0 days) and PD group (45.5+/-40.2 days). No difference was recognized between the two groups with respect to relapse rate. CONCLUSION: These data suggest that a short course of methylprednisolone pulse therapy followed by oral prednisolone is not superior to oral prednisolone therapy as an initial therapeutic modality in adult-onset MCNS.

A case of membranous glomerulonephritis induced by lithium / 대한내과학회지

The lithium is widely employed as a psychotropic agent, specially in manic illness. Lithium nephrotoxicity can be divided into three main categories: nephrogenic diabetes insipidus, acute intoxication, chronic nephropathy including interstitial nephropathy and glomerulopathy manifested as nephrotic syndrome. But, nephrotic syndrome induced by lithium is very rare. We reported a nephrotic syndrome induced by lithium in a 27-year-old woman who was taking lithium carbonate for 11 years due to bipolar disorder. Renal biopsy showed membranous nephropathy. After withdrawal of lithium, clinical symptoms and proteinuria significantly improved.

A case of early gastric cancer with IgA nephropathy and minimal change nephrotic syndrome / 대한내과학회지

The nephrotic syndrome has been reported in association with a wide variety of neoplastic disease. The relationship between early gastric cancer and glomerulonephritis has not been well elucidated. Only 6 cases (5 cases in Korea and 1 case in Japan) have been reported associated with glomerulonephritis by this time. 5 cases in Korea included three cases of membranous glomerulonephritis, one case of IgA nephropathy and membranoproliferative glomerulonephritis. Four out of six cases including presented case were mucin secreting adenocarcinomas of stomach. Minimal change disease associated with solid tumors has been reported very rarely. Three cases of colon adenocarcinoma have been reported in association with minimal change disease by this time. Complete remission of nephrotic syndrome with tumor resection without corticosteroid therapy was reported in one of three cases, but two of them had to be on corticosteroid therapy in addition to tumor resection to get complete remissions. We report a 44-year-old man case with nephrotic syndrome associated with early gastric cancer. Renal biopsy findings were indicative of minimal change disease but with scanty IgA deposition. Low grade IgA nephropathy was suggested. Endoscopic biopsy on stomach demonstrated the adenocarcinoma with signet ring cells. Serum IgA concentration was 543 mg/dL, and decreased to 336 mg/dL after tumor resection. But, owing to persistent proteinuria and acute renal failure complicated, corticosteroid therapy was applied on the 30th day after operation. So we could not prove definite relationship between early gastric cancer and the development of IgA nephropathy mimicking minimal change disease. But, it is regarded that normalized serum IgAlevel and the complete remission of nephrotic syndrome with tumor resection and corticosteroid therapy can be indirect evidences for the relationship between early gastric cancer and the development of IgA nephropathy mimicking minimal change disease.

Two cases of successful management of paraquat poisoning with pulmonary fibrosis / 대한내과학회지

Paraquat, a very potent herbicide, has produced many fatalities through indiscriminate usage and suicides. It is known that with ingestion of more than one mouthful of 20% paraquat, death usually occurs by pulmonary fibrosis. We experienced two cases of successful outcome in patients with paraquat poisoning complicated with pulmonary fibrosis through active, intensive treatment from the early phase after ingestion. They swallowed one to two mouthfuls of the 20% commercial paraquat, and received hemoperfusion within 2-3 hours after ingestion. Then, combination therapy with steroid, vitamin C, D-penicillamine, allopurinol, colchicine was administered from the early phase. They showed moderate to severe hypoxemia and pulmonary fibrosis on chest HRCT during hospital course, but recovered from hypoxemia and remained very well on follow up over 3 months and 12 months after ingestion, respectively. We report these cases with literature review on treatment and prognosis of paraquat poisoning.

Two cases of successful management of paraquat poisoning with pulmonary fibrosis / 대한내과학회지

Paraquat, a very potent herbicide, has produced many fatalities through indiscriminate usage and suicides. It is known that with ingestion of more than one mouthful of 20% paraquat, death usually occurs by pulmonary fibrosis. We experienced two cases of successful outcome in patients with paraquat poisoning complicated with pulmonary fibrosis through active, intensive treatment from the early phase after ingestion. They swallowed one to two mouthfuls of the 20% commercial paraquat, and received hemoperfusion within 2-3 hours after ingestion. Then, combination therapy with steroid, vitamin C, D-penicillamine, allopurinol, colchicine was administered from the early phase. They showed moderate to severe hypoxemia and pulmonary fibrosis on chest HRCT during hospital course, but recovered from hypoxemia and remained very well on follow up over 3 months and 12 months after ingestion, respectively. We report these cases with literature review on treatment and prognosis of paraquat poisoning.

A study on the characteristics and the pathogenesis of hemodialy sis ascites . - Hemodialy sis adequacy ( Kt / V urea ) and serum ascites albumin gradient ( SAAG ) - / 대한내과학회지

BACKGROUND: The aim of this study was to characterize the nature and elucidate the pathogenesis of hemodialysis ascites(HA), especially with regard to the levels of Serum Ascites Albumin radient(SAAG) and the degrees of hemodialysis adequacy(Kt/Vurea). METHODS: In the study group, seven cases of HA which had developed in 6 patients from Feb. 1997 through July 1998 were included. In the control group, 24 cases which had not developed HA were included. The study design was a retrospective. Analysis of ascites on WBC, total protein and albumin, cytology, ADA(Adenosine deaminase), osmolality, SAAG and routine work-up were performed in HA group. Serum total protein and albumin, C-reactive protein(CRP), osmolality, and routine liver function test were also checked. Kt/Vurea and weekly Kt/Vurea were calculated in both group. In statistical analyses, t-test and chi-square test were used. RESULTS: Mean SAAG of HA was >1.1(1.49+/-0.40) gm/dL, and mean concentration of total protein of HA was >2.5(4.26+/-0.58) gm/dL. The mean of weekly Kt/Vurea of patients with HA(2.61+/-0.85) was significantly lower than that of patients without HA(3.48+/-0.90)(p<0.05). Positive ratio of CRP in patients with HA was higher than that of patients without HA(p<0.05). Mean concentration of serum total protein was significantly higher in patients with HA than that of patients without HA but with comparable weekly Kt/Vurea levels(p<0.05). CONCLUSION: It is regarded that the nature of HA is an exudate having high SAAG over 1.1 gm/dL. Low weekly Kt/Vurea is suggested as a cause of HA. Chronic inflammation was also regarded as an important factor causing HA.

Hemoperfusion and continuous veno-venous hemofiltration for treatment of paraquat poisoning / 대한내과학회지

BACKGROUND: In Korea, paraquat accounts for most of fatal poisoning with 500 or more deaths per year. It has been known that patients who ingested more than 1/2 mouthful of 20% concentrate paraquat usually die of multiorgan failure and pulmonary fibrosis. But the effect of charcoal hemoperfusion which can enhance elimination of paraquat remains controversial. Because acute paraquat poisoning is also characterized by multiorgan failure including kidney and marked rebound in plasma paraquat level after hemoperfusion, Continuous veon-venous hemofiltration(CVVH) may have theoretical benefits in the treatment of paraquat poisoning. So we evaluated the effect of early charcoal hemoperfusion and prophylactic CVVH after hemoperfusion in patients with paraquat poisoning. METHODS: There were 80 patients with paraquat poisoning admitted within 24 hours after ingestion (August 1996 - March 1998). All of them were treated with hemoperfusion (duration of hemoperfusion, 6.4+/-3.0 hours) within 24 hours of ingestion. The amount of ingestion was 2.1+/-1.0 mouthful (as 20% concentrate) and 78 (98%) were urine sodium dithionite test positive which is a poor prognosis factor. Forty-four patients received hemoperfusion only and 36 were followed by CVVH (duration, 50.4+/-20.9 hours; ultrafiltration volume, 33.8+/-3.9 L/day) after hemoperfusion. RESULTS: There was no difference between the hemoperfusion group and hemoperfusion+CVVH group in age, sex, initial serum creatinine, arterial oxygen saturation, severity of poisoning (as assessed by serum paraquat level determined by HPLC and amount of ingestion), or in the time elapsed from ingestion to the beginning of hemoperfusion. The total mortality was 65% (52/80) with no difference between the hemoperfusion group (64%, 28/44) and hemoperfusion+CVVH group (67%, 24/36). The mortality according to amount of ingestion was as follows: or = 3 mouthful, 82% (27/33); unknown, 60% (6/10). CONCLUSION: Early hemoperfusion can be effective in reducing mortality in patients who ingest less than 2 mouthful. Even though prophylactic CVVH after hemoperfusion has no additional benefit in reducing mortality in paraquat poisoning, it prolonged the time to death after ingestion.

A case of acute myeloid leukemia in chronic renal failure patient on erythropoietin treatment / 대한내과학회지

Recombinant human erythropoietin is a synthetic glycoprotein hormone and has the effect on bone marrow erythroid progenitor cells. Therefore, recombinant human erythropoietin has been frequently used as an agent treating anemia in chronic renal failure patients. However, Stockenhuber reported that recombinant human erythropoietin also affected other bone marrow progenitor cells (colony- forming unit-granulocyte-erythrocyte-macrophage, colony-forming-gra-nulocyte-macrophage). This reports may suggest a possible casual relationship between acute myeloid leukemia and erythropoietin. Acute myeloid leukemia is a rare disease in patients with chronic renal failure especially on erythropoietin treatment. By this time only a case of acute leukemia in chronic renal failure was reported by Campistrus in 1995. Campistrus's case was on the remission state of acute myeloid leukemia, and erythropoietin was administered to treat anemia of chronic renal failure. Also no previous case of acute myeloid leukemia developed in chronic renal failure patients on erythropoietin treatment was yet reported in Korea. So, we report a case of acute myeloid leukemia developed in a pre-dialysis chronic renal failure patient but on erythropoietin treatment.

TnI, cTnT, and CK-MB in patients with chronic renal failure / 대한내과학회지

BACKGROUND: Nonspecific elevations of CK-MB, cTnT have been well known in patients with chronic renal failure(CRF) on maintenance hemodialysis. It has been suggested that recently developed cTnI seldom shows nonspecific elevations in these patients. Status of CRF patients can be divided into three groups: predialysis group, hemodialysis group and peritoneal dialysis group. Until now, most researchers have studied CK-MB, cTnT and cTnI only in CRF patients receiving maintenance hemodialysis. No previous studies have ever compared the differences of the nonspecific positivity of CK-MB, cTnT and cTnI according to the different status of CRF patients. METHODS: Nonspecific positive ratios of cTnI, cTnT, & CK-MB in were evaluated 20 predialysis patients, 13 CAPD patients and 20 hemodialysis patients. No one had had any evidence of myocardial ischemia during the previous 3 months before the study entry. The predialysis group was again divided into two groups according to the cut off level of serum creatinine of 3.0 mg/dl. Authors also compared the nonspecific positive ratios of cTnI, cTnT, CK-MB between diabetic CRF group and non diabetic CRF group. The sensitivity, specificity and false positive ratios of each enzymes were examined on and 6 hours after arrival in 21 CRF patients who visited the emergency room with the complaint of chest pain. RESULTS: 1) There were no nonspecific significant elevations of cTnI in CRF patients regardless of the status of CRF. But there were significant nonspecific elevations of CK-MB, cTnT in them. It was more marked in cTnT especially with the cut-off value of 0.1 ng/ml. 2) Nonspecific positive ratios of cTnT was significantly increased in diabetic CRF patients. 3) The sensitivity and specificity of cTnI were 100% and 93.3% each, which were significantly higher than those of CK-MB(83.3%, 66.7%) & cTnT(66.7%, 53.3%). CONCLUSION: In CRF patients, the nonspecific positive ratios of CK-MB, cTnT were higher than that of cTnI, and only cTnI did show significant specific elevations in all the CRF patients with acute myocardial infarction. It is likely that the status of CRF patients, dialysis mode, the sampling time point would not give significant changes in the nonspecific positive ratios of CK-MB, cTnT and cTnI.

The status of blood pressure control and the effect of dialysis adequacy on blood pressure in chronic hemodialysis patients / 대한내과학회지

OBJECTIVE: Volume expansion has been known to be the major factor in the development of hypertenision in chronic hemodialysis(HD) patients. But some HD patients remain hypertensive even with adequate volume control, which suggests the role of undefined uremic toxin in the pathogenesis of hypertension. So we aimed to evaluate the status of blood pressure (BP) control and the effect of Kt/V (as a marker for removal of uremic toxin) on BP in chronic HD patients. METHODS: The status of BP control was obtained from records of 8 HD session in 132 patients in November 1996 and 127 patients in November 1997. Of 132 patients studied in 1996, 70 patients underwent a follow-up evaluation in 1997. All patients were dialyzed 3 times a week, 4 hours a session. Postdialytic cyclic 3',5' guanosine monophosphate (cGMP) level was measured in 48 patients as a marker of volume status. RESULTS: The prevalence of postdialytic hypertension (>140/90mmHg) was 73.5 in 1996 and 65.3% in 1997. Normotensive patients (postdialytic mean BP<114 mmHg) had higher Kt/V value than hypertensive patients in both 1996 and 1997. But there was no difference in the degree of ultrafiltration (UF) and cGMP level between two groups. Postdialytic mean BP was inversely correlated with Kt/V level but had no relationship with degree of UF and cGMP level in both 1996 and 1997. The group in which postdialytic mean BP had been decreased during 1 year study period had higher degree of elevation in Kt/V than the group in which postdialytic mean BP had been increased. The changes of postdialytic weight and degree of UF during study period were similar between two groups. The number of antihypertensives used were also inversely correlated with Kt/V but not correlated with degree of UF and cGMP level in both 1996 and 1997. CONCLUSION: Our study indicate that increasing HD adequacy is associated with improved control of postdialytic mean BP and less use of antihypertensive drugs. UF and antihypertensive drugs may not be adequate form of hypertension treatment as once thought and increasing HD adequacy can be an alternative method.

Sleep Disturbance in Hemodialysis Patients / 대한신장학회잡지

Sleep disturbance and restless legs syndrome (RLS) among uremic patients is known to be high, but data on the prevalence, clinical significance, and causative factors are limited. A semiquantitative sleep and RLS questionnaire was distributed to 62 chronic hemodialysis(HD) patients. 74.5N had sleep complaints and frequent awakening was the most frequent specific sleep complaints followed by daytime sleepiness. Presence of diabetes mellitus(DM), RLS, depres- sion, high CRP level, low intact parathyroid hormone level, low subjective global assessment score, and short HD duration were associated with more sleep complaints. 58.3N had RLS and presence of DM, low serum albumin level were also associated with more severe RLS. But Kt/V, protein catabolic rate, hematocrit, plasma bicarbonate level, use of erythropoietin and sleep medication were not associated with sleep complaints and RLS. Sleep problems and RLS are comrnon in HD patients and seem to be associated with malnutrition. Presence of DM, de- pression, and RLS are likely to contribute the high prevalence of sleep disturbance.

The relationship between plasma leptin and nutritional status in chronic hemodialysis patients

Leptin serves an important role in suppressing appetite in mice and is known to be elevated in chronic renal failure (CRF) patients. But clinical significance of leptin as an appetite-reducing uremic toxin, remains to be determined. So we studied the relationship between plasma leptin and nutritional status in 46 chronic hemodialysis (HD) patients. Pre HD leptin was measured and divided by body mass index (BMI) to give adjusted leptin levels. KT/Vurea (K, dialyzer urea clearance; T, duration of HD; V, volume of distribution of urea), C-reactive protein (CRP), plasma insulin and nutritional parameters such as serum albumin, normalized protein catabolic rate (nPCR), subjective global assessment (SGA), BMI and mid-arm muscle circumference (MAMC) were also measured. Mean plasma leptin levels were 8.13+/-2.91 ng/mL (male 3.15+/-0.70; female 14.07+/-6.14, p<0.05). Adjusted leptin levels were positively correlated with nPCR (male r=0.47, p<0.05; female r=0.46, p<0.05), SGA (male r=0.43, p<0.05; female r=0.51, p<0.05) and MAMC (male r=0.60, p<0.005; female r=0.61, p<0.05). They did not correlate with KT/Vurea, serum albumin, hematocrit, bicarbonate, insulin and CRP. Presence of DM and erythropoietin therapy had no effect on leptin levels. These results suggest that leptin is a marker of good nutritional status rather than a cause of protein energy malnutrition in chronic HD patients.

A case of diabetic hyperosmolar coma treated successfully with conversion to hemodialysis in a chronic renal failure patient on CAPD treatment / 대한내과학회지

Continuous ambulatory peritoneal dialysis(CAPD) is now the most important and effective therapeutic modality as well as hemodialysis(HD) and renal transplantation in patients with chronic renal failure. It is frequently recommended to diabetic renal failure patients because of poor, athersclerotic vascularity of them. Hyperglycemia and obesity are not uncommon complications of CAPD therapy. But there were only few reports of very severe hyperglycemia or hyperosmolar coma in CRF patients on CAPD therapy, especially with 7% dextrose CAPD dialysate in foreign countries in the past. Moreover, no specific management for hyperosmolar coma is established in those situations yet. In Korea, only three cases of hyperosmolar coma in non diabetic renal failure patient on CAPD therapy have been reported in one case report. And only one case among them whose CAPD therapy was changed to HD survived. Authors also experienced a case of diabetic hyperosmolar coma treated successfully with conversion to HD in chronic renal failure patient on CAPD therapy. So we report this case with a review of literature. Taken together with this case and review of literature, changing CAPD therapy to HD is regarded to be an important part of treatment when hyperosmolar coma develops in chronic renal failure patients.

A case of diabetic hyperosmolar coma treated successfully with conversion to hemodialysis in a chronic renal failure patient on CAPD treatment / 대한내과학회지

Continuous ambulatory peritoneal dialysis(CAPD) is now the most important and effective therapeutic modality as well as hemodialysis(HD) and renal transplantation in patients with chronic renal failure. It is frequently recommended to diabetic renal failure patients because of poor, athersclerotic vascularity of them. Hyperglycemia and obesity are not uncommon complications of CAPD therapy. But there were only few reports of very severe hyperglycemia or hyperosmolar coma in CRF patients on CAPD therapy, especially with 7% dextrose CAPD dialysate in foreign countries in the past. Moreover, no specific management for hyperosmolar coma is established in those situations yet. In Korea, only three cases of hyperosmolar coma in non diabetic renal failure patient on CAPD therapy have been reported in one case report. And only one case among them whose CAPD therapy was changed to HD survived. Authors also experienced a case of diabetic hyperosmolar coma treated successfully with conversion to HD in chronic renal failure patient on CAPD therapy. So we report this case with a review of literature. Taken together with this case and review of literature, changing CAPD therapy to HD is regarded to be an important part of treatment when hyperosmolar coma develops in chronic renal failure patients.

The influence of maturation period of arteriovenous fistula on its survival in patients undergoing maintenance hemodialysis / 대한내과학회지

BACKGROUND: Arteriovenous fistula(AVF) has been the most important, primary mode of achieving vascular access for chronic hemodialysis by this time. In general, maturation period over 4 to 8 weeks after operation for the formation of AVF has been recommended for the long-term survival of AVF, and so insertion of central venous catheter without using AVF being matured has been primarily recommended whenever hemodialysis is needed. But not infrequently, serious complications have been reported in association with the insertion and the use of central venous catheter. So earlier use of AVF is regarded as a good method of avoiding serious complications with regard to the insertion and the use of central venous catheter. But early use of AVF has not been generally recommended, for early use of AVF has been regarded to be associated with early failure of AVF. But few studies have reported the correlation between maturation period and AVF survival. And in practice, early use of AVF has already been performed frequently by not a few nephrologists or nurses of dialysis units. So authors tried to examine the correlation between maturation period and AVF survival rate, and to find the validity of early use of AVF if it is regarded usable for the hemodialysis by experienced hemodialysis nurses and nephrologists. METHODS: A retrospective analysis using 88 AVF cases which had been created in 85 patients from Oct. 1986 through June 1996, and from which authors could get enough information for this study was done. Authors compared one year survival rates of AVF according to the maturation period, the presence of DM, and condition of AVF assessed clinically by doctors and experienced nurses in hemodialysis units. Also from the cases with AVF obstruction, authors examined the 1st, 2nd, and 3rd year survival rate of AVF according to the maturation period. RESULTS: One year survival rate of AVF with maturation period less than 4 weeks was higher than that with maturation period more than 4 weeks, but there was no statistical significance. One year survival rate, irrespective of the length of maturation period for AVFs, of AVF regarded to be usable and good for hemodialysis was higher than that of AVF regarded to be usable but not good for hemodialysis. In the study with the AVF obstruction group only, one year survival rate of AVF with maturation period less than 4 weeks was higher than that of AVF with maturation period more than 4 weeks but there was no statistical significance. And one year AVF survival rate was higher in non DM group(94.1%) than DM group(60%) regardless of maturation period of AVF(p<0.05). CONCLUSION: On the contrary to the views that longer maturation period of more than 4 weeks will be necessary for the long-term survival of AVF, our results suggest that shorter maturation period for AVF less than 4 weeks does not necessarily mean early failure of AVF once AVF is regarded to be usable for hemodialysis. So it is suggested that early use of AVF instead of inserting central venous catheter is a reasonable approach for getting an adequate vascular access for hemodialysis in chronic renal failure patients who were subjected to receive hemodialysis on waiting peroid of AVF maturation.

A Case of RPGN associated with toxic epidermal necrolysis / 대한내과학회지

RPGN is a catastrophic form of acute glomerulonephritis characterized by an abrupt onset and rapid deterioration of renal function resulting in oliguria within weeks or months. RPGN is seen in a variety of systemic disorders, including systemic lupus erythematosus, poly arteritis nodosa, Wegener's granulomatosis and subacute bacterial endocarditis. In addition, RPGN is seen in association with a variety of primary renal diseases such as poststreptococcal glomerulonephritis, membranoproliferative glomerulonephritis, and IgA nephropathy, Goodpasture's syndrome. Toxic epidermal necrolysis(TEN) is a drug induced life threatening disease characterized by extensive epidermal detachment, necrosis, and mucosal erosion. TEN may involve liver, lung, intestine, and kidney. But renal involvement has seldom been reported. We report on a 63-year-old patient who developed a RPGN with a TEN. Renal biopsy showed pauci-immune crescentric glomerulonephritis and skin biopsy showed edematous change with extravasated erythrocytes in upper dermis and several individually necrotic keratinocytes. ANCA and FANA test was negative. Our patient recovered renal function with steroid pulse therapy. The pathophysiology of TEN is unresolved but abnormal cytokine release(e.g., tumor necrosis factor) has been implicated in pathogenesis of TEN. Because various cytokines have direct toxic effect on kidney structure, the tubular and glomerular damage may be related to the cytokines involved in TEN. To our knowledge, this is the first case documenting the presence of RPGN in patients with TEN. And there maybe some relations between PRGN and TEN which require further study.

Sequential Changes of Extracellular Matrix mRNA in Anti-GBM Antibody Induced Crescentic Glomerulonephritis in the Rabbit

Progressive renal fibrosis is considered to be the final common pathway leading to chronic renal insufficiency, however, the mechanism regarding renal fibrosis in renal injury is not well understood. Recently, several kinds of cytokines have been known to be related to fibrosis after renal injury. The interaction between elements regulating fibrogenesis would be better understood by looking at the effect of TGF-beta1 on the synthesis and accumulation of extracellular matrix, especially collagenous proteins. Crescentic glomerulonephritis (CGN) was induced in New Zealand White rabbits by administration of guinea pig anti-GBM IgG after sensitization with guinea pig IgG; and their kidneys were analyzed for the development of crescents and fibrosis through sequential renal biopsies. Serum creatinine levels in a time course progressively increased until day 15. We semi-quantitatively assayed the levels of the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA factored for GAPDH mRNA using RT-PCR. We observed a progressive interstitial fibrosis and the expression of collagen I both in the cortex and medulla. The effect of repeated renal biopsy itself on pathology and on the expression of alpha1(I) collagen mRNA and TGF-beta1 mRNA in a time course were not significant, but a very mild increase of the expression of alpha1(I) collagen mRNA was noted at day 15. Histology showed a progressive crescent formation and interstitial fibrosis in a time course that roughly paralleled the expression of alpha1(I) collagen mRNA in both cortex and medulla. TGF-beta1 mRNA was hardly expressed at day 0 in cortex as well as in medulla. It was elevated from day 1, peaked at day 7, and then decreased. In medulla, TGF-beta1 mRNA was noticeably expressed at day 1, peaked at day 4, and then decreased. The expression of alpha1(I) collagen mRNA was seen even before inducing CGN. It was gradually and continuously increased until day 15 both in cortex and medulla. These results suggest that the expression of TGF-beta1 mRNA precedes that of alpha1(I) collagen mRNA in the early stage of CGN and has a central role for provoking the accumulation the collagen I, the most representative interstitial extracellular matrix, in the rabbit model CGN induced by anti-GBM antibody. We conclude that the measurement of the expression of TGF-beta1 mRNA and/or alpha1(I) collagen mRNA in a biopsy sample can be a useful predictor for renal outcome.

A Case of Simultaneous Bilateral Emphysematous Pyelonephritis and Cystitis / 대한내과학회지

Since the first clinical observation by Kelly and MacCallum, gas-forming infection of the urinary tract has been extensively studied. It is characterized by gas production within the urinary tract. The gas may often pass outside the urinary tract. Patients with this disorder invariably have uncontrolled diabetes mellitus and pos sibly associated obstructive uropathy. It is usually caused by aerobic bacteria, most commonly Escherichia coli. In the literature, the majority of cases were emphy sematous pyelonephritis alone or emphysematous cystitis separately. Bilateral renal involvement associated with emphysematous cystitis is very rarely seen and no case was reported yet in Korea. We report a case of bilateral emphysematous pye lonephritis and emphysematous cystitis which occured in 48-year-old diabetic patient simultaneously. It was caused by E. coli. The patient was cured by medical management alone.