An analysis of 4,514 cases of renal biopsy in Korea

To evaluate the distribution and changing patterns of renal diseases in Korea, a total of 4,514 cases of renal biopsy collected over a 23-year period between 1973 and 1995 were reviewed. Of 4,200 cases excluding 314 unsatisfactory biopsies, adult cases comprised 59.5% and pediatric cases, 40.5%. The male to female ratio was 1.5:1 in adults and 2.2:1 in children. Glomerulonephritis (GN) comprised 80.0% of the total. The most common primary GN in adults was minimal change disease (MCD) (26.6%), followed by IgA nephropathy (IgAN) (22.1%), membranous GN (MGN) (11.8%), and membranoproliferative GN (MPGN) (5.9%). In children, the primary GN incidence rates were MCD (24.8%), IgAN (10.3%), poststreptococcal (including postinfectious) GN (PSGN) (8.6%), and focal segmental glomerulosclerosis (FSGS) (4.0%). The most common secondary GN in adults was lupus nephritis and in children Henoch-Schonlein purpura nephritis. The most common cause of nephrotic syndrome was MCD in both adults and children, followed by MGN and FSGS. The elderly, aged sixty years and older, comprised 2.7% of cases and recorded equal numbers of MCD and MGN. The proportion of the biopsies found to be seropositive for HBs antigen was 27.9%, and these showed either MGN or MPGN pattern. Repeat biopsy was performed in 168 patients, due to previous biopsy failure in 15.5%. When the primary GN cases were analyzed at 5-year intervals, the prevalence of PSGN, which was greater than 25% during the 1973-1982 period, decreased abruptly in children thereafter, whereas the prevalence of FSGS increased slowly since the 1988-1992 period in both adults and children. The decrease of PSGN and the increase of FSGS suggest a role for socioeconomic and environmental factors in Korea.

A Case of Membranoproliferative Glomerulonephritis Type II: Dense Deposit Disease, DDD

Type II membranoproliferative glomerulonephritis (Dense deposit disease ) is an acquired primary glomerular disease characterized by electron microscopic evidence of a continuous dense membrane deposition replacing the lamina densa. It is a subtype of idiopathic membra- noproliferative glomrulonephritis, and was described as a separate entity by Berger and Galle in 1963. It frequently occurs in older chilren and young adults and the clinical course is variable, but is generally progressive. The presenting feature is nephrotic syndrome in many patients, and proteinuria and hematuria are also seen frequently. The purpose of this paper is to present a case of DDD (Dense deposit disease) from a 10 year old boy who was diagnosed as a acute poststreptococcal glomurulonephritis with protenuria, hematuria, and facial edema by renal biopsy 4 years ago.

Renal Pathology: Retrospect and Prospect / 대한신장학회잡지

No abstract available.

Incidence of Chronic Pathologic Nephrotoxicity of Cyclosporine A in Pediatric Nephrotic Syndrome

Pigmented villonodular synovitis is a destructive, fibrohistiocytic proliferation producing innumerable villous and nodular synovial protrusions. Its common locations are knee, ankle, foot, and hip. Although histologic feature of this tumor is well known, there have been few reports on the fine needle aspiration cytology findings. We report the cytologic features of a biopsy-proven case of pigmented villonodular synovitis. The patient was a 21-year-old male with a mass of the right knee for 2 years. On fine needle aspiration cytology, the aspirates was composed of abundant mononuclear histiocytic cells, singly and in clusters, multinucleated giant cells, and hemosiderin pigments.

Treatment of Membranous Glomerulonephritis with Cyclosporine A / 대한신장학회잡지

We investigated the relationship between hepatitis B and membranous glomerulonephritis and progress of disease after treatment with cyclosporine A. Twenty seven patients were reviewed retrospectively about clinical manifestations, laboratory findings, pathologic findings and progress of disease after treatment with steroid alone or steroid plus cyclosporine A. The male to female ratio was 3.5:1 and mean age was 8.9 years ranged from 2.8 to 16.9 years. The common clinical manifestations were edema(81%), asymptomatic proteinuria(19%) and micro- or macroscopic hematuria(78%). In viral marker studies, seventeen patients(63%) showed positive HBsAg and among them, 16 patients(59%) showed positive HBeAg. Eleven patients treated with steroid and cyclosporine A showed complete remissions and 12 cases among 16 patients treated with steroid alone showed complete remissions. In conclusion, sixty three percents of children with membranous glomerulonephritis have strong association with HBsAg. The most common clinical manifestation was edema. Patients treated with cyclosporine A and steroid showed better results than patients treated with steroid alone(P=0.03).

Prognostic Factors in Childhood IgA Nephropathy

The authors report series of 360 cases of transthoracic fine-needle aspiration cytology (TFNA) from Oct. 1982, through Aug. 1986 at the Seoul National University Hospital. A diagnosis of neoplastic lesion was established in 50.3% of the cases. A non-neop- lastic diagnosis was made in 38.5%, nondiagnostic one in 6.5% and inadequate one in 4.7% of the total. Statistical findings on cytological diagnoses were as follows. Specificity was 100%: sensitivity, 92%; predictive value for positive, 1.0: predictive value for negative, 0.9; concordance rate, 84.2%: diagnostic accuracy in non-neoplastic lesion, 65.4%, and typing accuracy in malignant tumor, 0.77.

Giant Cell Tumor of the Larynx: Report of a case

Giant cell tumor arising in the osteocartilaginous tissue of the larynx is extremely rare. The few reported cases have all occurred in men older than 20 years and the site of origin has been exclusively localized to the thyroid and cricoid cartilages that are known to undergo enchondral ossification. The formation of giant cell tumor in larynx is thought to be related to this enchondral ossification process. We report a case of giant cell tumor arising in the thyroid cartilage of a 39 year old man. CT scan of the neck revealed a well defined mass of soft tissue density replacing the right thyroid cartilage. Grossly the tumor was well demarcated with a distinctive pushing margin except for the area bordering the submucosa of the larynx where it showed focally an infiltrative pattern of growth. Microscopically multinucleated giant cells were found dispersed regularly among the spindle cells. Although mitoses were frequently noted in the spindle cells there was no atypicality or pleomorphism. Total laryngectomy was performed without further treatment. Postoperative follow up for 6 months has proved the patient to be alive and well without recurrence.

Long-term results of cyclosporine-induced remission of relapsing nephrotic syndrome in children

Twenty-nine children with nephrotic syndrome were treated with cyclosporine (CsA), 100 mg/m2/day for 6 months and prednisone, 2 mg/kg every other day for 1 month and then subsequently 1 mg/kg every other day for 5 months. A renal biopsy had shown minimal change disease (MCD) in 18 children, focal segmental glomerulosclerosis (FSGS) in 3 children, membranous glomerulonephritis (MGN) in 4 children, membranoproliferative glomerulonephritis (MPGN) in 2 children, and IgA nephropathy in 2 children. All MCD patients went into complete remission during therapy. Five out of 11 steroid-sensitive patients (45.5%) remained in complete remission, while the remaining 6 (54.5%) had 2 to 3 relapses, 19 to 47 months after CsA discontinuation. Two out of 7 steroid-resistant patients (28.6%) were still in complete remission and 5 (71.4%) had 1 to 6 relapses 25 to 49 months after CsA withdrawal. The mean number of relapses in the steroid-sensitive group before and after CsA treatment decreased more (8.5 vs 1.4) than in the steroid-resistant group (8.1 vs 2.4) (p < 0.05). At the most recent examination, 1 of 3 FSGS patients achieved complete remission and 2 had a partial response. Three of 4 MGN patients were in complete remission and 1 was in partial remission. One of 2 MPGN patients achieved complete remission and 1 showed partial remission. Two patients with IgA nephropathy were in partial remission. We compared MCD patients in sustained remission and relapse; the mean CD4/CD8 ratio decreased from 1.5 to 0.9 in the remission group, in comparison with no change in the relapsed group (p < 0.05). The posttreatment renal biopsy showed lesions of nephrotoxicity in 3 of 18 children with MCD whose renal function did not alter after CsA treatment. We concluded: 1) A 6-month treatment of CsA, in combination with a low-dose alternate-day steroid, proved to be effective in maintaining the remission of steroid-sensitive and steroid-resistant MCD patients. 2) The CD4/CD8 ratio can be used as a index to predict remission or relapse after CsA therapy.

Successful Transplantation of 3 Cases of Oligomeganephronia

"We retrospectively reviewed the results of 1,850 fine needle aspiration cytology (FNAC) of thyroid nodules performed from 1990 to 1991 in the Department of Pathology, Seoul National University Hospital. Among 1,528 cases and 322 cases aspirated by clinicians and a pathologist, 465 cases (30.4%) and 13 cases (4.0%) of the aspirates were inadequate, respectively. In 227 cases, correlation of the FNAC diagnosis and histologic diagnosis was done. Excluding the inadequate cases, the sensitivity for the detection of neoplasm (malignancy together with follicular adenoma) was 86.4% and the specificity was 70.7%. The overall diagnostic accuracy was 79.0%. There were 16 false-positive cases (7.0%), and 19 false-negative cases (8.4%). The predictive value of each cytologic diagnosis was 92% in papillary carcinoma, and 100% in Hashimoto's thyroiditis. The expectancy of malignancy was 52.8% in ""suspicious malignancy"" and 26.7% in ""atypical lesion""."

Pancreatic serous cystadenoma mimicking pseudocyst

The serous cystadenoma of the pancreas is a rare lesion that is usually found incidentally. It is mostly observed as a spongy microcystic mass but rare variants such as macrocystic, unicystic, or multicentric are also seen. We recently experienced a unique case of unicystic serous cystadenoma mimicking a pseudocyst. It was grossly a unilocular cyst with surrounding dense fibrosis resembling a pseudocyst. Microscopically, the cyst was partly lined by low columnar-to-cuboidal cells with clear cytoplasm containing glycogen.

A Morphometric Study of Glomerular Dimensions in Relation to Glomerular Location, Age and Sex in Koreans

As measurement of glomerular size in the assessment of several renal diseases becomes increasingly important, it has become necessary to devise rapid simple methods for the assessment of glomerular size and to have on hand reference ranges. A few reports on glomerular size have been published in Western literature, but their body builds are different from Koreans. In this study, 100 glomeruli(50 glomeruli each from the outer cortical and the juxtamedullary area) were measured in sections taken from 74 kidneys(ages 3 days~73 years) obtained from autopsy utilizing the semi-automatic image analyser. The percentage of glomerular sclerosis was measured based on its location. The sphere diameter, maximum diameter, area and sphere volume of non-sclerotic glomeruli were measured and evaluated with respect to age, sex and the location of the glomeruli. The results were as follows; 1) Mean glomerular dimensions including sphere and maximum diameter, area and sphere volume increased until 40 years of age, then reached a plateau. The percentage of sclerotic glomeruli then increased slowly with age but without statistical significance. 2) The glomerular dimensions and sclerosis showed no significant differences according to sex. 3) Juxtamedullary glomeruli were larger than the outer cortical ones which was statistically significant in age groups of 0~10, 11~20 and 41~50 years. The percentage of sclerotic glomeruli was generally greater in the outer cortex. 4) Differences in the values of glomerular dimensions between outer cortical and juxtamedullary area were similar in all age groups. 5) All parameters of measurement showed consistent and similar trends between the different groups. 6) The measurements of the largest 12 glomeruli out of randomly-selected 50 glomeruli gave similar results when compared with those of 50 glomeruli. It was evident from our results that glomerular size is influenced by age and glomerular location, but not by sex. The method of assessing glomerular size used in this study will not necessarily give the true, absolute value of size but it may be a simple, practical and useful method of comparing glomerular size in different groups of patients.

Interstitial mononuclear cell infiltrates in chronic rejection of the kidney and correlation with peripheral blood

To investigate the characteristics of interstitial inflammatory cells and possible involvement of nudelta T cells, 16 renal allograft biopsies showing chronic rejection were stained by immunohistochemical method and correlated with the data of peripheral blood evaluated by flow cytometry. For immunophenotyping, fresh frozen sections were stained with monoclonal antibodies against CD3, CD4, CD8, CD68, CD56, TCRdelta1 and HLA DR. Paraffin embedded tissue was stained with CD45RO, CD20-Cy and CD68. Nine cases of nonspecific tubulointerstitial change and 4 cases of nonallograft tubulointerstitial nephritis were used as a control. Inflammatory infiltration was present in all cases studied. T cells predominated in the interstitium of chronic rejection and were followed by macrophages and B cells. The degree of interstitial infiltration of frozen section was not accordant with that of paraffin sections. Allografts with nonspecific tubulointerstitial changes or tubulointerstitial nephritis of native kidneys showed similar distribution pattern in terms of type and degree. However, the degree of infiltrate did not give any statistical significance among groups. The CD4/CD8 ratios in interstitial infiltrates were less than 1.0 in 6 cases and was not accordant with those of peripheral blood. Proportion of nudelta T cells increased over 10% in 2 cases in tissue and in 3 cases in peripheral blood. In 3 cases of chronic rejection in which both tissue and blood results were available, there was no concordance of CD4/CD8 or nudeltaT/CD3 between them. Tubular expression of HLA DR was, however, present only in 4 cases of chronic rejection. In conclusion, T lymphocytes were predominant regardless of diagnosis or disease activity. T lymphocyte subset did not give any suggestion as to the diagnosis or disease activity in chronic rejection. Furthermore nudelta T cells had only limited value. Lymphocytic subsets in peripheral blood would not be predictors of tissue destruction in chronic rejection.

Interstitial mononuclear cell infiltrates in chronic rejection of the kidney and correlation with peripheral blood

To investigate the characteristics of interstitial inflammatory cells and possible involvement of nudelta T cells, 16 renal allograft biopsies showing chronic rejection were stained by immunohistochemical method and correlated with the data of peripheral blood evaluated by flow cytometry. For immunophenotyping, fresh frozen sections were stained with monoclonal antibodies against CD3, CD4, CD8, CD68, CD56, TCRdelta1 and HLA DR. Paraffin embedded tissue was stained with CD45RO, CD20-Cy and CD68. Nine cases of nonspecific tubulointerstitial change and 4 cases of nonallograft tubulointerstitial nephritis were used as a control. Inflammatory infiltration was present in all cases studied. T cells predominated in the interstitium of chronic rejection and were followed by macrophages and B cells. The degree of interstitial infiltration of frozen section was not accordant with that of paraffin sections. Allografts with nonspecific tubulointerstitial changes or tubulointerstitial nephritis of native kidneys showed similar distribution pattern in terms of type and degree. However, the degree of infiltrate did not give any statistical significance among groups. The CD4/CD8 ratios in interstitial infiltrates were less than 1.0 in 6 cases and was not accordant with those of peripheral blood. Proportion of nudelta T cells increased over 10% in 2 cases in tissue and in 3 cases in peripheral blood. In 3 cases of chronic rejection in which both tissue and blood results were available, there was no concordance of CD4/CD8 or nudeltaT/CD3 between them. Tubular expression of HLA DR was, however, present only in 4 cases of chronic rejection. In conclusion, T lymphocytes were predominant regardless of diagnosis or disease activity. T lymphocyte subset did not give any suggestion as to the diagnosis or disease activity in chronic rejection. Furthermore nudelta T cells had only limited value. Lymphocytic subsets in peripheral blood would not be predictors of tissue destruction in chronic rejection.

Prognostic factors and renal survival rates in IgA nephropathy

A retrospective study of 223 patients with IgA nephropathy (IgAN) was performed to clarify the prognostic factors and the renal survival rates of the disease. One hundred twenty-two patients were followed-up for more than 6 months after their renal biopsy (mean follow-up duration: 43.0 months), and 20 of them (16.4%) had progressed to end-stage renal disease (ESRD). Using univariate analysis, 8 risk factors (2 clinical and 6 histopathological findings) for developing ESRD were identified: renal insufficiency at initial presentation (serum creatinine > or = 1.5 mg/dl); heavy proteinuria(> or = 3.5 gm/day); moderate to severe histopathologic findings such as class IV/V lesions by W.H.O. classification, mesangial hypercellularity, glomerular sclerosis, interstitial infiltration, interstitial fibrosis, and tubular atrophy. In multivariate regression analysis, class IV/V lesions and renal insufficiency at initial presentation were the independent prognostic factors of IgAN. The renal survival rates were 100% at 1 year, 97.0% at 3 years, and 78.9% at 5 years. In conclusion, it seems that about 20% of IgAN patients have a risk to progress to ESRD within 5 years, and a careful follow-up is recommended especially in patients who have either renal insufficiency at the time of presentation or severe renal pathology (class IV/V lesions).

The Effect of Vitamin E on Bleomycin-InducedPulmonary Injury and Fibrosis in Rat: Comparison of Penicillamine- or Deferoxamine-Treated Group / 결핵

BACKGROUND: Pulmonary toxicity by bleomycin has multiple mechanisms including direct tissue toxicity due to oxygen-derived free radicals and indirect toxicity through amplification of pulmonary inflammation. To evaluate the effect of chelators or free radical scavenger to lung damage induced by bleomycin, penicillamine as a copper chelator, deferoxamine as an iron chelator and vitamin E as a free radical scavenger were administered. METHODS: Two hundred Wistar rats were divided into five groups: Control, bleomycin treated, bleomycin-penicillamine treated, bleomycin-deferoxamine treated, and bleomycin-vitamin E treated groups. Rats sacrificed on day 1, day 3, day 4, day 7, day 14, and day 28 after treatment. Bronchoalveolar lavage, light microscopic and immunohistologic studies for type I, III, IV collagens, fibronectin, laminin and NBD phallicidin were evaluated. RESULTS: There was a significant increase in the total cell counts of bronchoalveolar lavage on day 1 from all treated animals and vitamin treated group showed an abrupt decrease in total cell counts with decrease of neutrophils on day 3. Bleomycin-vitamin E treated group had the least histologic changes such as pulmonary fibrosis. The alveolar basement membranes were positive for type IV collegen and laminin. Basement membranes of bleomycin, bleomycin-penicillamine, or bleomycin-deferoxamine treated groups were disrupted and fragmented on day 4 or 7. The bleomycin-vitamin E treated group had intact basement membranes until day 28. CONCLUSION: Bleomycin-induced pulmonary fibrosis was related to the severity of acute injury to oxygen radicals or activation of neutrophils and disruption of basement membrane. Vitamin E seemed to be the most effective antioxidant in the inhibition of bleomycin-induced pulmonary injury and fibrosis.

Interstitial Mononuclear Cell Infiltration and its Phenotypes in IgA Nephropathy

To know the correlation between glomerular and tubulointerstitial lesion and to define the characteristics of interstitial inflammatory cell in IgA nephropathy and classified according to WHO classification and graded tubulointerstitial lesion as mild, moderate and severe. Paraffin-embedded 5u sections were stained with UCHL-l, L26 and CD68 antibodies. More than 20 fields were examined in each case under the high power microscopy and the number of positive cells were counted. There was positive correlation between the severity of glomerular and that of tubulointerstitial lesion. The mostcommoninflammatory cells in the interstitiuin were UCHL-l positive cells followed by CD68 and L26 positive cells. As the WHO grade or tubulointerstitial lesion increased, the numbers of positive cells were increased in all three groups. The proportion of UCHL-1 Positive cells were increased in cases with high WHO grade whereas that of L26 positive cells incases with severe tubulointerstitial lesion Proteinuria was correlated with the degree of inflammatory cell infiltration, especially with that of L26 positive cells.

Immunohistochemical Study on Expression of Extracellular Matrix Components in Glomerular Diseases

Most glomerular lesions are associated with qualitative and quantitative alterations of the extracellular matrix components, having relation to progressive glomerular sclerosis. We aimed to investigate the characteristic alteraltions in distribution of extracellular matrix components, such as fibronectin, laminin, collagen type III and IV in human glomerular diseases by immunohistochemical method. The materials included are 3 nephrectomy as normal control, 51 renal biopsies and I autopsy; 3 normal, 5 minimal change disease, 5 minimal change disease with minimal mesangial lgA deposit, 5 benign recurrent hematuria, 10 focal segmental glomerulosclerosis, 15 lgA nephropathy, 10 membranoproliferative glomerulonephritis, 2 diffuse mesangial sclerosis of infancy. Type IV collagen and laminin were present normally in the mesangium, GBM, TBM and interstitial vessels, and were increased at the portion of increased mesangial matrix, of sclerosis and thickened GBM in cases of lgA nephropathy, membranoproliferative glomerulonephritis, focal segmental glomrulosclerosis and diffuse mesangial sclerosis in the proportion to the glomerular damage. Type III collagen was absent in the normal glomeruli, but was detectable focally and segmentally in cases of membranoproliferative glomerulonephritis, IgA nephropathy and focal segmental glomerulosclerosis at the sclerotic portion. Fibronectin was normally detectable mainly in the mesangium, and partly and incompletely in GBM, and was increased at the portion of increased mesangial matrix, sclerosis and thickened GBM in cases of focal segmental glomerulosclerosis, membranoproliferative glomerulonephritis, IgA nephropathy and diffuse mesangial sclerosis, but was diminshed at the old slcerotic portion or global sclerosis. The expression of these antibodies in cases of minimal change disease, minimal change disease with minimal mesangial IgA deposit, benign recurrent hematuria was not different, quantitatively and qualitatively, from that of normal glomeruli. These findings suggest that progressive glomerular sclerosis was due to the increase of extraceuular matrix components such as type IV collagen, laminin, fibronectin and new appearance of type III collagen, and the expression was in proportion to the degree of sclerosis, but had no relation to the disease entity.

Muscular Hamartoma of the Breast: A case report

Hamartoma of the breast is relatively rare benign tumor, which is a well-circumscribed mass mainly composed of fibrous stroma, a(tipose tissue, ducts and acini. Muscular hamartoma of the breast is mainly composed of smooth muscle, and is extremely rare because proper smooth muscle is normaly absent in the breast except in the nipple. We describe a rare case of muscular hamartoma of the breast in a 38-year-old woman. This tumor was located in the upper outer quadrant and a 3 x 2.5 x 2 cm sized, well-demarcated but not encapsulated mass, The mass consisted mainly of irregularly arranged smooth muscle bundles in the fibrous stroma with lobular units and admixed fat cells. The origin of smooth muscle in hamartoma is not well known.

Aggressive AngiOmYxoma Occuring in Ischiorectal Fossa: A case report

We report a case of aggressive angiomyxoma in ischiorectal fossa of a 39-year-old women. The tumor is characterized by relatively large size(13 x 11 cm), grossly gelatinous appearance and locally infiltrative nature. Microscopically, it consists of many variable sized blood vessels and spindle or stellate cells widely separated in myxoid or collagenous stroma. Immunohistochemical stains reveal that the tumor cells are strongly positive for actin and desmin. Electron microscopic findings are that of a few cells dispersed in abundant intercellular substance and collagen bundles. These cells form irregular cytoplasmic process without basal lamina and contain endoplasmic reticulum having cistern.

Homogenous fetal dopaminergic cell transplantation in rat striatum by cell suspension methods

The transplantation of dopaminergic neurons in the brain has been attempted in experimental animals and humans as the new treatment modality of Parkinson's disease. Before the trial of dopaminergic neuronal transplantation in human, the authors proceeded with the animal experiment of fetal dopaminergic cell transplantation in a rat Parkinson's disease model. The aims of this experiment were to confirm the availability of fetal mesencephalic cells as the donor, to compare the viability of cells according to different cell manipulation methods, and to follow up the functional recovery in the transplanted Parkinson's disease model. As a result, the authors concluded that the simple enzyme digestion method had a better cell survival rate than the multiple enzyme digestion method. Also, the transplanted mesencephalic cells could not only survive in the host animal but also promote functional recovery.