De Novo ANCA-Negative Pauci-Immune Crescentic Glomerulonephritis After COVID-19 mRNA Vaccination: A Case Report

To prevent the spread of the coronavirus disease 2019 (COVID-19) pandemic, vaccines have been authorized for emergency use and implemented worldwide. We present a case of de novo glomerulonephritis (GN) after use of the COVID-19 mRNA vaccine BNT162b2.A 48-year-old man with no relevant medical history was referred for sudden and persistent worsening of renal insufficiency 1.5 months after the second vaccine dose. He had arthralgia and skin rash a week after vaccination. Abdominal pain and diarrhea started 2 weeks later, and he was admitted to the hospital for enteritis treatment. Colonoscopy showed multiple ulcerations and petechiae suggestive of vasculitis in the terminal ileum. After prednisolone therapy, his gastrointestinal symptoms improved, but his renal function continued to deteriorate. Based on kidney biopsy findings and nephrotic-range proteinuria (5,306 mg/24 hours), he was diagnosed with anti-neutrophil cytoplasmic autoantibody (ANCA)-negative pauci-immune crescentic GN (CrGN). He received high-dose steroid pulse therapy and oral cyclophosphamide, and then, gradually underwent steroid tapering, with improvement in proteinuria and renal function over several weeks. Several cases of GN suspected to be related to COVID-19 vaccines have been reported. To our knowledge, this is the first case report of ANCA-negative pauci-immune crescentic CrGN with extrarenal involvement after COVID-19 mRNA vaccination. Our finding expands the spectrum of COVID-19 vaccine-associated GN.

Development of End Stage Renal Disease after Long-Term Ingestion of Chaga Mushroom: Case Report and Review of Literature

Chaga mushrooms are widely used in folk remedies and in alternative medicine. Contrary to many beneficial effects, its adverse effect is rarely reported. We here report a case of end-stage renal disease after long-term taking Chaga mushroom. A 49-year-old Korean man with end stage renal disease (ESRD) was transferred to our hospital. Review of kidney biopsy finding was consistent with chronic tubulointerstitial nephritis with oxalate crystal deposits and drug history revealed long-term exposure to Chaga mushroom powder due to intractable atopic dermatitis. We suspected the association between Chaga mushroom and oxalate nephropathy, and measured the oxalate content of remained Chaga mushroom. The Chaga mushroom had extremely high oxalate content (14.2/100 g). Estimated daily oxalate intake of our case was 2 times for four years and 5 times for one year higher than that of usual diet. Chaga mushroom is a potential risk factor of chronic kidney disease considering high oxalate content. Nephrologist should consider oxalate nephropathy in ESRD patients exposed to Chaga mushrooms.

Lupus-like Glomerulonephritis Associated with Extranodal Marginal Zone B-cell Lymphoma of MALT / 대한내과학회지

Lupus-like glomerulonephritis is an immune complex disease with features of lupus nephritis in the absence of systemic lupus erythematosus (SLE). We report a 49-year-old man diagnosed with lupus-like glomerulonephritis associated with extranodal marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT). He was admitted to the hospital for edema. At admission, the serum creatinine was 2.2 mg/dL and the urine protein level was 3.9 mg/day. A renal biopsy showed features of lupus nephritis with no clinical or serological evidence of SLE. Extranodal marginal zone B-cell lymphoma of MALT was discovered concurrently. After successful chemotherapy, the lupus-like glomerulonephritis and lymphoma entered complete remission.

Complete Remission from C1q Nephropathy with Disappearance of C1q Deposition after Steroid Therapy / 대한내과학회지

C1q nephropathy is a rare glomerular disease, defined by characteristic mesangial C1q immune deposition seen in immunofluorescence microscopy with no serological evidence of systemic lupus erythematosus. C1q nephropathy can be diagnosed with a subsequent biopsy, as with IgA nephropathy. There are some cases with an initial diagnosis of hematuria and proteinuria with minimal disease changes, focal segmental glomerulonephritis, and mesangial proliferative glomerulonephritis, but lacking C1q nephropathy, in which C1q deposition on immunofluorescence subsequently develops. We report a case that was diagnosed as diffuse mesangial proliferative glomerulonephritis, but a subsequent biopsy showed C1q nephropathy, with C1q deposition in both immunohistochemistry and electron microscopy (EM). We treated the C1q nephropathy with methylprednisolone and confirmed the disappearance of C1q depositions by both immunohistochemistry and EM in a follow-up biopsy.

A Case of Secondary FSGS due to Chronic Chloride Diarrhea

Congenital chloride diarrhea (CLD) is a rare autosomal recessive disease that is difficult to diagnose. CLD requires early treatment to correct electrolyte imbalance and alkalosis and to prevent severe dehydration. Renal injury is clearly associated with defective electrolyte balance induced by CLD, particularly during the first months or years of life. A 7-year-old boy was diagnosed with CLD following detection of a homozygous mutation (c.2063-1G>T) in SLC26A3 at 6 months of age. During treatment with electrolyte supplements, mild proteinuria was detected at 8 months of age, and is still present. Renal biopsy showed the presence of focal renal dysplasia, with metaplastic cartilage and mononuclear cell infiltration, calcification, and fibrosis in the interstitium. Up to two-thirds of the glomeruli exhibited global obsolescence, mostly aggregated in the dysplastic area. In nondysplastic areas, the glomeruli were markedly increased in size and severely hypercellular, with increased mesangial matrix, and displayed segmental sclerosis. The marked glomerular hypertrophy with focal segmental glomerulosclerosis suggested a compensatory reaction to the severe nephron loss or glomerular obsolescence associated with renal dysplasia, with superimposed by CLD aggravating the tubulointerstitial damage.

Bacteriocin from Purple Nonsulfur Phototrophic Bacteria, Rhodobacter capsulatus

To find whether productivity of bacteriocin is controlled between different species under unusual cultural conditions, we used Rhodobacter capsulatus ATCC 17016 as a producer and Rhodopseudomonas palustris ATCC 17003 as an indicator. Rhodobacter capsulatus was cultured under aerobic conditions in the dark in Lascelles medium containing 0.3% Triton X-100. As a result, bacteriocin productivity increased enormously. The optimal pH range of bacteriocin production was 6~7.8. Through partial purification of bacteriocin, the molecular weight was roughly estimated at 14 kDa. Plasmid had no influence on bacteriocin production by Rhodobacter capsulatus. Our findings indicate that culture conditions affect bacteriocin productivity between more distantly related species, and bacteriocin of Rhodobacter capsulatus is not encoded by a plasmid.

Atypical Hemolytic Uremic Syndrome Associated with Streptococcus pneumoniae Infection / 소아과

Hemolytic uremic syndrome is the most common cause of acute renal failure in childhood. Atypical hemolytic uremic syndrome which is not usually associated with prodromal symptoms has a higher mortality rate and more tendency to progress to chronic renal failure. Children younger than two years of age are a more susceptible to pneumococcal infection and can develop more serious disease. We report a 23 month-old male who suffered from pneumococcal pneumonia and progressed to hemolytic uremic syndrome requiring peritoneal dialysis. He recovered completely from acute renal failure after peritoneal dialysis and intravenous use of vancomycin.

Expression Patterns of PTEN (Phosphatase and Tensin Homologue Deleted on Chromosome 10) in Osteosarcoma / 대한정형외과학회잡지

PURPOSE: To characterize PTEN gene alterations and their expressions during the development of osteosarcoma. MATERIALS AND METHODS: We studied the pattern of deletion, mutation and expression of PTEN in normal bone tissues, tumor samples of 22 patients of osteosarcoma, and 4 osteosarcoma cell lines (HOS, U2-OS, MG-63 and Saos-2). The tissue was analyzed for deletion and mutational inactivation of PTEN by polymerase chain reaction-single strand conformation polymorphism (PCR-SSCP) and direct sequence analysis, and examined for abnormalities in expression by immunohistochemistry. RESULTS: In this study, neither mutation nor deletion of PTEN was found. Expression of PTEN protein was increased, without deletion or mutation of the PTEN gene, in 22 patients of osteosarcoma and in 4 osteosarcoma cell lines. Nuclear staining was more intense than the cytoplasmic staining in normal bone tissues and osteosarcoma cell lines, but in osteosarcoma tissues PTEN was expressed mainly in the cyto-plasm. CONCLUSION: These results suggest that abnormal expressions of PTEN by differential compartmentalization may play a role in the development and progression of osteosarcoma, instead of genetic alterations of PTEN.

A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children

PURPOSE: Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. METHODS: Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed after 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. RESULTS: The median onset age was 13.4 years. Clinical manifestations were nephrotic syndrome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. CONCLUSION: With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done

A Clinicopathological Study of Idiopathic Membranous Nephropathy in Children

PURPOSE: Idiopathic Membranous Nephropathy(IMN) is a rare renal disease in children. To help better understanding of its clinical course and treatment strategies, we reviewed the clinical manifestations and pathological findings of children with IMN. METHODS: Among 58 cases with MN, from 1977 to 2003, 42(72.4%) were hepatitis B virus (HBV) associated and 16(27.6%), 6 males and 10 females, were idiopathic. All cases diagnosed after 2000 were IMN. Several clinicopathological findings(sex, onset age, proteinuria, serum albumin, cholesterol, creatinine clearance, tubulointerstitial changes, glomerular sclerosis, hypertension, renal vein thrombosis, the use of ACE inhibitor, and immunosuppressive therapy) were compared between the remission and the non-remission group of the patients with IMN. RESULTS: The median onset age was 13.4 years. Clinical manifestations were nephrotic syndrome(7 cases, 43.8%), gross hematuria(5 cases, 31.3%) and microscopic hematuria with proteinuria(3 cases, 18.8%). Hypertension, hypocalcemic tetany and renal vein thrombosis were accompanied in 2, 1 and 2 cases, respectively. In addition to the typical findings of MN, the kidney biopsies showed segmental sclerosis(5 cases, 31.3%) or global sclerosis(6 cases, 37.5 %), diffuse crescents(1 case), and mild(11 cases, 68.7%) or moderate tubulointerstitial changes(3 cases, 18.8%). Thirteen cases(86.7%) received oral steroid. Among them 2 cases received cyclophophamide and 1 received cyclosporin as well. Ten cases(62.5%) received ACE inhibitors. In the patients followed up, 7 cases(46.7%) became free from proteinuria (remission group) while 8(53.3%) presented continous proteinuria (non-remission group), two (13.3%) of which progressed to renal failure. Clinicopathological findings showed no significant differences between the two groups. CONCLUSION: With HBV vaccination, HBV associated MN decreased markedly and IMN has taken up most of MN in children. For better understanding of this rare disease, a prospective multicenter study of the clinical course and treatment strategies should be done

Quantification of the Glomerular Structural Changes by Morphometry / 대한신장학회잡지

No abstract available.

Quantification of the Glomerular Structural Changes by Morphometry / 대한신장학회잡지

No abstract available.

Glomerular Disease in Young Korean Men / 대한신장학회잡지

BACKGROUND: We performed this study to elucidate the incidence and the types of glomerular diseases present in young Korean men. METHODS: Between January 1998 and December 2000, 222 military conscripts were submitted to 13 military hospitals for renal biopsy after poststreptococcal glomerulonephritis (PSGN) had been excluded. Clinical data and renal biopsies were reviewed retrospectively. On the basis of this data, we obtained the annual detection rate of glomerular disease in military conscripts. We also ascertain the prevalence of glomerular disease in pre-induction medical examination during the same period. RESULTS: Total two hundred and ten cases of glomerulonephritis including 30 cases of PSGN were diagnosed on renal biopsy. After additional six patients with nephrotic syndrome were included, total 216 patients were diagnosed as glomerular disease. Among one hundred and eighty six patients excluding the patients with PSGN, 90 cases presented with asymptomatic urinary abnormality, 63 nephrotic syndrome, 25 acute nephritis, 8 chronic GN. In exclusion of PSGN, the most common glomerular diseases observed were those due to IgA nephropathy (42.8%), minimal change disease (15.6%) and focal segmental glomerulosclerosis (14.4%). IgA nephropathy was most common (57%) in 96 patients presented with asymptomatic urinary abnormality. On the basis of the figures in this study the average number of annually detected cases of GN was 13.8 per 100,000 Korean Amy. During the same period an annual average of 70.0 per 100,000 young men were exempted from military service because of Glomerulonephritis. CONCLUSION: Average number of annually detected cases of glomerulonephritis is 13.8 per 100,000 and IgA nephropathy is most common in young Korean conscripts.

Glomerular Disease in Young Korean Men / 대한신장학회잡지

BACKGROUND: We performed this study to elucidate the incidence and the types of glomerular diseases present in young Korean men. METHODS: Between January 1998 and December 2000, 222 military conscripts were submitted to 13 military hospitals for renal biopsy after poststreptococcal glomerulonephritis (PSGN) had been excluded. Clinical data and renal biopsies were reviewed retrospectively. On the basis of this data, we obtained the annual detection rate of glomerular disease in military conscripts. We also ascertain the prevalence of glomerular disease in pre-induction medical examination during the same period. RESULTS: Total two hundred and ten cases of glomerulonephritis including 30 cases of PSGN were diagnosed on renal biopsy. After additional six patients with nephrotic syndrome were included, total 216 patients were diagnosed as glomerular disease. Among one hundred and eighty six patients excluding the patients with PSGN, 90 cases presented with asymptomatic urinary abnormality, 63 nephrotic syndrome, 25 acute nephritis, 8 chronic GN. In exclusion of PSGN, the most common glomerular diseases observed were those due to IgA nephropathy (42.8%), minimal change disease (15.6%) and focal segmental glomerulosclerosis (14.4%). IgA nephropathy was most common (57%) in 96 patients presented with asymptomatic urinary abnormality. On the basis of the figures in this study the average number of annually detected cases of GN was 13.8 per 100,000 Korean Amy. During the same period an annual average of 70.0 per 100,000 young men were exempted from military service because of Glomerulonephritis. CONCLUSION: Average number of annually detected cases of glomerulonephritis is 13.8 per 100,000 and IgA nephropathy is most common in young Korean conscripts.

Altered Expression of Nephrin, Glomerular Epithelial Cell Protein-1 (GLEPP1) and WT-1 in Glomerular Disease

BACKGROUND: Glomerular epithelial cell protein-1 (GLEPP1) and WT-1 expressed in mature visceral glomerular epithelial cell (VGEC) is required for maintenance of the mature status of VGEC. Nephrin protein is located at the filtration slit and regarded as a molecular component of the slit diaphragm. Alterations of these proteins in proteinuric diseases are not clearly defined. METHODS: We investigated the expression of GLEPP1, WT-1 and nephrin in 28 renal biopsies diagnosed with minimal change nephropathy (n=10), focal glomerulosclerosis (n=10) and membranous nephritis (n=8) by immunohistochemical staining. Normal control biopsies were obtained from six nephrectomy specimens. RESULTS: The patients consisted of 15 males and 13 females. The mean age was 40.7 years. Nephrotic range proteinuria (> or =3.5 g/day) was noted in 15 (54%) patients. GLEPP1 and nephrin expression were significantly decreased in patients as compared with those of the controls (p<0.05). The mean number of WT-1 expressing cells per glomerulus was also significantly decreased in patients as compared with those of the controls (p<0.05). However, there was no significant difference in the number of WT-1 expressing cells among the disease groups. CONCLUSIONS: These results suggest that the loss of biological markers of mature VGEC may play an important role in the pathogenesis of proteinuria.

A Case of Glomerulonephritis Associated with POEMS Syndrome / 대한신장학회잡지

A POEMS syndrome(polyneuropathy, organomegaly, endocrinopathy, monoclonal M protein, and skin lesion) is a rare multisystem disease of unknown cause with varying clinical manifestations. POEMS- associated renal disease is also a rare condition. We experienced a 42-year-old male who had been suffered from generalized edema, low extremity weakness and pain. He also had a significant hematuria, proteinuria, azotemia, hepatospenomegaly, intraperitoneal lymph node enlargement, hypothyroidism, IgA lamda type monoclonal gammopathy, specific skin change and ascites. In renal biopsy, membranoproliferative features with enlarged glomeruli, mesangial proliferation, capillary wall thickening and paucity of immune deposit was noted. We suppose that renal insufficiency could come from POEMS syndrome. His symptoms such as low extremity pain, azotemia and generalized edema were improved by hemodialysis and use of prednisolone. At present, he is under the treatment of prednisolone and melphalan.

Unilateral Renal Lymphangiectasia with Renal Vein Thrombosis / 대한신장학회잡지

Renal lymphangiectasia is a rare cystic kidney disorder. This disorder has been termed renal lymphangioma, renal lymphangiomatosis, pararenal lymphatic cyst. We report on a 32-year-old man admitted with complaints of abdominal discomfort, in whom unilateral renal lymphangiectasia with renal vein thrombosis was diagnosed. Abdominal computed tomography revealed left perirenal fluid collection with multiloculation. Left renal vein was completely obliterated due to thrombosis and inferior vena cava (IVC) was partially obliterated. Perirenal fluid collection and renal vein thrombosis were treated initially with percutaneous drainage and anticoagulation therapy. The biochemical features of the drained fluid were compatible with lymphatic fluid. Since there was no decrease in the amount of daily drainage, left nephrectomy and IVC thrombectomy was performed on the 34th day of hospitalization. Left kidney was enlarged (15x10x18 cm, 750 g) and composed of numerous smooth, thin-walled cysts. Pathologic diagnosis was renal lymphangiectasia.

A Case of Membranous Glomerulonephropathy Associated with Idiopathic Autoimmune Thrombocytopenic Purpura / 대한신장학회잡지

Membranous glomerulonephropathy is the most common cause of nephrotic syndrome in adults and idiopathic autoimmune thrombocytopenic purpura is autoimmune disease caused by autoantibody to platelet membrane glycoprotein. Although there are some pathologic similarity between two diseases that 'membrane attack complex' play a role in pathologic process, but only 3 cases worldwide are reported about membranous glomerulonephropathy associated with idiopathic autoimmune thrombocytopenic purpura. So we report a case of sixty eight years of woman who had symptoms of generalized edema, foamy urine, anorexia and thrombocytopenia on admission and developed more severe symptomatic thrombocytopenia there after. She was diagnosed membranous glomeulonephropathy on renal biopsy and also diagnosed idiopathic autoimmune thrombocytopenic purpra on bone marrow biopsy and on the basis of exclusion.

Correlations between the Intrarenal Expression of Various Cytokines and Chemokines and the Clinicopathologic Characteristics in IgA Nephropathy / 대한신장학회잡지

IgA nephropathy is one of the most common forms of primary glomerulonephritis in adults, and the pathogenetic mechanisms seem to be diverse. Proinflammatory cytokines, Th1/Th2 cytokines, and chemokines would be involved in the pathogenetic pathways and would affect the functional and histologic consequences. To evaluate this hypothesis, we tried to quantify the magnitude of intrarenal gene expression of various cytokines(TNF-alpha, IL-1beta, IL- 6, IL-15, IFN-gamma, IL-2, IL-10) and chemokines(IL-8, RANTES) in 61 renal core biopsy specimens confirmed as IgA nephropathy by immunofluorescent microscopy. Semiquantitative reverse-transcriptase polymerase chain reactions(RT-PCR) using the internal competitors were done for the quantification of gene transcripts. And using the immunohistochemistry (IHC), we tried to determine the degree of expression and the location of various cytokines and chemokines in renal tissues in 29 patients among the above patients. The IFN-gamma/IL-10 ratio was higher in patients with renal dysfunction than that in patients with normal renal function(p=0.0483). Gene transcript levels of proinflammatory cytokines(TNF-alpha, IL-1beta) were high in patients with significant proteinuria. In patients with severe glomerular sclerosis, the ratio of IFN-gamma/IL-10 gene transcripts was high(p=0.0363). IL-10 gene transcript level was related to the severity of tubulointerstitial damage. The levels of gene expression of TNF-alpha(p=0.0026), IL-10(p=0.0092) and IFN-gamma(p=0.0188) were related to the degree of mesangial matrix expansion, and the extent of intrarenal arteriolar lesions correlated with the expression of the IL-8 genetranscript(r=0.3828, p=0.0033). The cellular infiltration in glomeruli was related with chemokine(IL-8) gene expression, but the relation was not significant statistically. The degree of IgA deposition in glomeruli was related with the expression of IL-6 and IL-15. The expression of intrarenal gene transcripts of various cytokines and chemokines were closely interrelated. Th1 or Th2 cytokine polarization was not present in IgA nephropathy. In IHC, TNF-alpha, IFN-gamma and IL-2 were immunostained dominantly in mesangial region, but not in tubulointerstitial region. In contrast, positive reactions for IL-10 were observed mainly in tubules. The significant reactions for IL-8 were noted in the periarteriolar and arteriolar areas. The results of RT- PCR and IHC showed positive relationships, but those were not significant statistically. This study suggests that proinflammatory, Th1/ Th2 cytokines and chemokines are involved in the specific processes of inflammation and immunologic injury, and their predominance and the level of expression could determine the pathogenetic processes and the severity of the clinical manifestations in IgA nephropathy.

Clinical and Histopathological Analysis of 494 Cases of Percutaneous Renal Biopsies / 대한신장학회잡지

Percutaneous renal biopsy is an essential component for diagnosis and management of glomerular diseases. In order to elucidate clinical and pathological features, 494 renal patients who had undergone renal biopsies at Gil Medical Center from January 1989 to June 1999 were studied retrospectively. The male to female ratio was 1.3 : 1 and average age was 33.2 years. There were 370(83.5%) cases of primary glomerular disease and 58(13.1%) cases of secondary glomerular disease. Among primary glomerular disease, IgA nephropathy was the most common(175 cases), followed by minimal change disease(84 cases), membranous glomerulonephritis(34 cases), and focal segmental glomerulosclerosis(33 cases). In secondary glomerular disease, lupus nephritis was the most common(21 cases), followed by 11 cases of hepatitis B associated glomerulonephritis, 9 cases of Henoch-Sch nlein purpura, and 4 cases of diabetic nephropathy. Among 99 cases of asymptomatic urinary abnormalities, IgA nephropathy was most common(69 cases) followed by 12 cases of thin basement membrane disease, 4 cases of minimal change disease, and 3 cases of focal segmental glomerulosclerosis, membranous glomerulonephritis, nonspecific glomerulonephritis. Among 159 cases of nephrotic syndrome, minimal change disease was most common(60 cases) followed by 25 cases of IgA nephropathy, 23 cases of focal segmental glomerulosclerosis, 21 cases of membranous glomerulonephritis, and 13 cases of lupus nephritis. Documented complication of renal biopsies included 23 cases of gross hematuria, 6 cases of perirenal hematoma, and 4 cases of infection. Death, AV fistula, aneurysm or serious compications that required surgical intervention were not reported. In conclusion, the percutaneous renal biopsy is relatively safe, and useful for diagnosis and management of glomerular diseases. The most common type of primary glomerular disease was IgA nephropathy.