Ciliary metaplasia in a patient with antineutrophil cytoplasmic autoantibody-associated pauci-immune glomerulonephritis

No abstract available.

Effects of aristolochic acid I and/or hypokalemia on tubular damage in C57BL/6 rat with aristolochic acid nephropathy

BACKGROUND/AIMS: This study was designed to investigate the roles of aristolochic acid I (AA-I) and hypokalemia in acute aristolochic acid nephropathy (AAN). METHODS: After an adaptation period (1 week), a total of 40 C57BL/6 mice (male, 8 weeks old) were divided into four groups: I (control group), II (low potassium [K] diet), III (normal K diet with administration of AA-I [10 mg/kg weight]), and IV (low K diet with AA-I). After collecting 24 hours of urine at 2 weeks, the mice were sacrificed, and their blood and kidneys were obtained to perform immunochemical staining and/or Western blot analysis. RESULTS: Proteinuria, glycosuria, and increased fractional excretion of sodium and K were prominent in groups III and IV (p < 0.05). Diffuse swelling and poor staining of collecting duct epithelial cells were evident in the medullas of group II. Typical lesions of toxic acute tubular injury were prominent in the cortices of groups III and IV. Α-Smooth muscle actin (α-SMA) was higher in the cortices of the mice in groups III and IV versus group II (p < 0.05), and higher in the medullas of group IV than groups I and III (p < 0.05). E-cadherin was higher in the cortices of groups III and IV compared to group I (p < 0.05). The F4/80 value was higher in the cortices and medullas of groups II, III, and IV compared to group I (p < 0.05), particularly in the case of group II. CONCLUSIONS: AA-I can induce acquired Fanconi syndrome in the acute stage of AAN. Macrophages appear to play a key role in the pathogenesis of AAN and hypokalemic nephropathy. It remains uncertain whether hypokalemia plays any role in AAN and hypokalemia.

Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature

Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.

Rare Location of Castleman's Disease in the Temporal Region: A Case Report Involving a Young Korean Woman and Review of the Literature

Castleman's disease (CD) is an uncommon benign lymphoproliferative disorder of unknown etiology. Histopathologically, it is divided into three types: hyaline-vascular, plasma cellular, and multicentric CD. The mass usually presents asymptomatically; however, it can cause non-specific symptoms such as fever and fatigue. Although CD can be found wherever lymph nodes are present, 75% of cases are reported in the mediastinum, and occurrence in the head and neck is rare. Herein, we report a rare case of CD presenting as a superficial mass in the temporal region. To the best of our knowledge, this is the first report of temporal CD in Korea involving a young patient.

An Adult Case of Bartter Syndrome Type III Presenting with Proteinuria

Bartter syndrome (BS) I-IV is a rare autosomal recessive disorder affecting salt reabsorption in the thick ascending limb of the loop of Henle. This report highlights clinicopathological findings and genetic studies of classic BS in a 22-year-old female patient who presented with persistent mild proteinuria for 2 years. A renal biopsy demonstrated a mild to moderate increase in the mesangial cells and matrix of most glomeruli, along with marked juxtaglomerular cell hyperplasia. These findings suggested BS associated with mild IgA nephropathy. Focal tubular atrophy, interstitial fibrosis, and lymphocytic infiltration were also observed. A genetic study of the patient and her parents revealed a mutation of the CLCNKB genes. The patient was diagnosed with BS, type III. This case represents an atypical presentation of classic BS in an adult patient. Pathologic findings of renal biopsy combined with genetic analysis and clinicolaboratory findings are important in making an accurate diagnosis.

Crystals in a patient with asymptomatic proteinuria

No abstract available.

Dedifferentiated Endometrioid Adenocarcinoma of the Uterus: Highly Aggressive and Poor Prognostic Tumor

No abstract available.

A Case of Elderly-Onset Crescentic Henoch-Schonlein Purpura Nephritis with Hypocomplementemia and Positive MPO-ANCA

Henoch-Schonlein purpura (HSP) is common in childhood and often self-limiting. There have been limited studies on elderly-onset HSP nephritis (HSPN). A 76-yr-old man was transferred to our hospital with a 1-month history of oliguria, abdominal pain, edema and palpable purpura in the legs. Three months ago, he was admitted to another hospital with jaundice, and consequently diagnosed with early common bile duct cancer. The patient underwent a Whipple's operation. Antibiotics were administrated because of leakage in the suture from the surgery. However, he showed progressive renal failure with edema and purpura in the legs. Laboratory investigations showed serum creatinine 6.4 mg/dL, 24-hr urine protein 8,141 mg/day, myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) 1:40 and C3 below 64.89 mg/dL. Renal biopsy showed crescentic glomerulonephritis, as well as mesangial and extracapillary Ig A deposition. We started steroid therapy and hemodialysis, but he progressed to end-stage renal failure and he has been under maintenance hemodialysis. We describe elderly onset HSPN with MPO-ANCA can be crescentic glomerulonephritis rapidly progressed to end stage renal failure.

Clinicopathologic Features of IgA-Dominant Postinfectious Glomerulonephritis

BACKGROUND: IgA-dominant acute postinfectious glomerulonephritis (APIGN) is a recently recognized morphologic variant of APIGN, but its clinicopathologic features were not clearly characterized. We will present demographic, clinical and renal biopsy findings from seven patients with IgA-dominant APIGN with a literature review. METHODS: All renal biopsy specimens (n=1,119) processed by the Department of Pathology in Hanyang University Hospital from 2005 to 2009 were reviewed. Seven patients with IgA-dominant APIGN were identified, and their clinical data analyzed. RESULTS: All patients had renal failure, hematuria and proteinuria. One was diabetic, and none of the patients had previous renal diseases. Three had clinical infections at the time of presentation: 2 with methicillin-resistant Staphylococcus aureus and one with rickettsial infection. Light microscopically diffuse endocapillary proliferative and exudative glomerulonephritis was found in all cases. Immunofluorescence microscopy showed granular IgA deposits along peripheral capillary walls and in mesangium. Ultrastructurally, subepithelial 'humps' with mesangial deposits were noted. End-stage renal disease developed in two patients, chronic renal failure was stationary in two, and azotemia improved in three. CONCLUSIONS: Various infections including rickettsiosis preceded IgA-dominant APIGN in both diabetics and nondiabetics. Because the prognosis of IgA-dominant APIGN is poor, early diagnosis based on renal biopsy is required.

Cytologic Features and BRAF Mutation of Hyalinizing Trabecular Adenoma of the Thyroid: A Case Report with Review of the Literature

A hyalinizing trabecular adenoma (HTA) is a rare benign thyroid tumor of follicular epithelial cell origin with a trabecular-alveolar growth pattern and marked intratrabecular hyalinization. The cytological and histological features of HTA are very similar to those of papillary and medullary carcinomas of the thyroid. Therefore, an accurate diagnosis of HTA is important to avoid unnecessary and potentially harmful management of patients. However, the results of BRAF gene mutation analysis shown by many studies are distinctly different between HTAs and papillary thyroid carcinomas. Herein, we describe a rare case of HTA of the thyroid in a 49-year-old female and consider its characteristic cytological features and BRAF gene mutation analysis results with a brief review of the literature.

Subcutaneous Myxoid and Round Cell Liposarcoma

Myxoid and round cell liposarcoma is a variant of liposarcoma characterized by a morphologic continuum in which tumor progression from low-grade myxoid to high-grade hypercellular or round cell areas may be observed. A 28-year old man presented with a painful skin colored subcutaneous mass on his left flank. The skin lesion was discovered about two months ago. Computed tomography revealed a well-defined tumor in the subcutaneous area without significant lymphadenopathy. A histopathologic study showed a nonencapsulated nodular growing tumor mass under the deep dermis composed of a mixture of atypical proliferating lipoblasts in a prominent myxoid stroma, occasional uniform round to oval shaped primitive nonlipogenic mesenchymal cells and plexiform, chicken wire shaped capillaries. Based on these findings, we diagnosed this case as myxoid and round cell liposarcoma. Because liposarcomas are large tumors of the deep subcutis or deeper soft tissue, they rarely come to the attention of the dermatologist. However, liposarcoma should be of concern, especially when rapid growing subcutaneous tumors are shown.

MPO-ANCA Associated Rapidly Progressive Glomerulonephritis in A Patient with Mixed Connective Tissue Disease / 대한신장학회지

Mixed connective tissue disease (MCTD) has the clinical feature of other collagen vascular diseases. According to several reports recently published, MCTD sometimes involved kidney as benign course. ANCA associated glomerulonephritis occurred in systemic autoimmune disease such as systemic sclerosis or rheumatoid arthritis, not in MCTD. In this case, as we experienced that MPO-ANCA associated glomerulonephritis arose and proceeded to rapid progressive glomerulonephritis (RPGN) in women with MCTD, we report it with review of reference. A 60-year-old woman was admitted with uremia and joint pain. In physical finding and serum test, she was diagnosed with MCTD according to Khan`s criteria. Also MPO antibody and ANCA were noted in specific immunological test and the histologic findings showed crescentic glomerulonephritis. So we diagnosed RPGN induced by MPO-ANCA associated glomerulonephritis. She was treated with immunosuppressive treatment and uremia was improved. RPGN induced by MPO-ANCA associated glomerulonephritis should be ruled out by prompt renal biopsy and require proper treatment.

The expression of clusterin, bax, p53, Ki-67, and apoptotic index in epithelial ovarian tumors / 대한산부인과학회지

OBJECTIVE: The purpose of this study was (1) to evaluate the expressions of clusterin, bax, Ki-67, p53, and apoptotic index in epithelial ovarian tumors, borderline and malignant ovarian tumors, (2) to find out the correlation between their expressions and clinicopathological parameters, and (3) to evaluate the effect on the patient's survival according to their expressions. METHODS: The histological and clinical findings of 22 cases of ovarian cystadenomas, 44 cases of borderline tumors and 96 cases of carcinomas were evaluated. Expressions of clusterin, bax, Ki-67, p53, and apoptotic index were studied on paraffin-embedded tissue sections by immunohistochemical methods. RESULTS: The expressions of clusterin, p53, and Ki-67 were higher in ovarian carcinomas than borderline tumors. The overexpression of p53, and Ki-67 were frequent in high stage, poorly differentiated and bilateral ovarian carcinomas. The overexpressions of clusterin, bax, p53, and Ki-67 showed a statistically significant correlation with histologic type. Apoptotic index was higher in bax overexpression group, but there was no correlation with overexpression of clusterin or p53. Ki-67 was higher in p53 overexpression group, but there was no correlation with overexpression of clusterin or p53. There was no statistically significant correlation with each other between the overexpressions of clusterin, bax, p53, and Ki-67. The overexpressions of clusterin, Ki-67, p53 was associated with overall patient's survival in borderline significance. CONCLUSION: The overexpression of p53, and Ki-67 were frequent in poorly differentiated ovarian carcinomas. So the overexpression of p53, and Ki-67 can be used as prognostic factor. The overexpression of clusterin was more in epithelial ovarian carcinomas than in borderline tumors but showed no significant correlation with the overall patient's survival. Further studies are required to clarify the possibility of using clusterin for target therapy in epithelial ovarian carcinomas.

Practical Standardization in Renal Biopsy Reporting

BACKGROUND: To standardize renal biopsy reporting and diagnosis, The Renal Pathology Study Group of the Korean Society of Pathologists (RPSKSP) has developed a renal pathology reporting format for the native and allograft kidney. METHODS: A consensus checklist of a provisional renal biopsy format was sent to all members of the RPSKSP. Feed back opinions regarding the practical application of the checklist to the diagnostic work were received. RESULTS: Kidney biopsies require three essential examinations: by light microscopy, immunofluorescence (IF), and electron microscopy (EM). A final report of a renal biopsy should include information on specimen adequacy and a description of the morphologic change using a systematic semiquantitative method for each of the compartments, with optional separate IF and EM reports. CONCLUSIONS: A standard renal biopsy report format is important in establishing clinicopathologic correlations, making reliable prognostic considerations, comparing the findings in sequential biopsies and evaluating the effects of therapy.

A Case of IgA Nephropathy Associated with Systemic Lupus Erythematosus / 대한류마티스학회지

Renal involvement is frequently seen in patients with systemic lupus erythematosus (SLE). The occurrence of non-lupus nephritis, and especially IgA nephropathy, in SLE patients has rarely been reported. We describe here the case of a 30-year-old woman who had systemic lupus erythematosus and nontuberculous mycobacterial lung disease, and her biopsy of a renal lesion was unexpectedly diagnostic of IgA nephropathy. Although both IgA nephropathy and lupus nephritis are immune complex mediated diseases, their laboratory and histopathologic findings and the extra-renal clinical manifestations are different and these all support a different pathogenesis for the 2 diseases. Renal biopsy plays a crucial role in identifying and diagnosing renal lesions, which may have prognostic and therapeutic implications that are distinct from those of lupus nephritis. In conclusion, performing a renal biopsy in SLE patients who have urinary abnormalities is important since a correct diagnosis would permit the most appropriate treatment to be started and so avoid unnecessary immunosuppressive treatments.

Secondary Amyloidosis Associated with Multiple Sclerosis

BACKGROUND: Multiple sclerosis (MS) is a demyelinating disease of the central nervous system. Secondary amyloidosis can occur as a complication of chronic systemic inflammatory and infectious diseases. Until now there has been no report of secondary amyloidosis associated with MS. We report herein a case of renal biopsy-proven secondary amyloidosis in a patient with MS. CASE REPORT: A 41-year-old woman with MS was hospitalized due to aggravated quadriparesis and edema in both lower extremities. Laboratory findings showed nephrotic-range proteinuria and hypoalbuminemia. A percutaneous renal biopsy procedure was performed, the results of which revealed secondary amyloid-A-type amyloidosis associated with MS. CONCLUSIONS: This is the first report of secondary amyloidosis associated with MS.

The Morphologic Patterns of Diabetic Nephropathy in Koreans

BACKGROUND: Diabetic nephropathy is the most common cause of end-stage renal disease and it has various pathologic features. We investigated the clinicopathologic differences between the histologic classes of diabetic nephropathy. METHODS: A total of 46 patients with diabetic nephropathy were evaluated. Morphologically, the renal lesions were divided into three categories: class 1, diffuse or nodular glomerulosclerosis: class 2, vascular change without evidence of glomerulosclerosis: and class 3, non-diabetic renal disease superimposed on diabetic glomerulosclerosis. We evaluated the laboratory findings and the histologic findings, including mesangial expansion, interstitial fibrosis and inflammation, arteriolar hyalinosis and tubular atrophy. RESULTS: The proportion of each class was 32 cases (70%), 4 cases (9%) and 10 cases (21%), respectively. The clinical and laboratory data showed no significant difference among the classes. For the groups of class 1, the group with nodular sclerosis showed a higher serum creatinine level than did the diffuse group (p=0.003). IgA nephropathy was the most common non-diabetic renal disease superimposed on diabetic glomerulosclerosis in our study. CONCLUSIONS: The patients with nodular glomerulosclerosis presented with a more progressed clinicopathological features than did the patients with class 1 diffuse glomerulosclerosis. We also found 21% of all the patients with diabetic nephropathy had superimposed non-diabetic renal disease in a Korean population.

Acute Renal Failure in Coexisting IgA Nephropathy and Acute Interstitial Nephritis: Complete Recovery after Hemodialysis and Steroid Therapy / 대한신장학회지

Acute renal failure in Immunoglobulin A nephropathy (IgAN), a rare event, is associated with acute tubular necrosis mainly induced by intratubular erythrocytic cast and crescentic glomerulonephropathy (rapidly progressive glomerulonephritis) and the severity paralleled to the degree of glomerular damage. The changes are regarded as those of secondary atrophic response to the glomerular lesions. In that case, renal progression correlates more closely with the severity of tubulointerstitial lesions than with the degree of glomerular lesions in IgAN. Rarely, acute tubulointerstitial nephritis (TIN) could develop independently in primary glomerulonephritis. In this case, the severity of tubulointerstitial lesion was out of proportion with damage of glomerular lesion. To the best of our knowledge, we report the first case of a patient with independently developed severe acute TIN complicating IgAN in Korea. A 38-year-old man was admitted with recurrent hematuria. Proteinuria (<1 g) and severe renal failure were noted and hemodialysis was started. In renal biopsy, IgAN associated with acute TIN was diagnosed. He showed good response to steroid therapy and maintained normal renal function after discontinuation of medication.

Clinicopathological Correlation of Lupus Nephritis / 대한신장학회지

PURPOSE: Clinical treatment for lupus nephritis largely depends upon histological renal biopsy classification. But it has been reported that serologic biochemical markers are not strongly associated with pathologic classification. The aim of this study is to see whether serologic markers could predict pathologic class of lupus nephritis for appropriate treatment. METHODS: We investigated 67 patients, who underwent renal biopsy with lupus nephritis at Hanyang University Hospital between January, 2005 and August, 2007. Biological markers for this study are hematuria, proteinuria, serologic data of lupus activity and azotemia. They were retrospectively analyzed from patients grouped by ISN/RPS 2003 lupus nephritis classification. RESULTS: Total 67 patients (men 5, women 62) were enrolled and the mean age of the patients was 30.6+/-9 years. The number of patient group by pathologic classification was 4 cases for class II, 15 cases for class III, 30 cases for class IV and 15 cases for class V. Spot urine protein to creatinine ratio more than 3 increased in class IV group statistically (p=.007). C3 level decreased more in class IV group than class III, V groups. Ten patients showed azotemia, and 9 of them were class IV group (p=.048). CONCLUSION: The patients with more increased proteinuria, decreased C3 level and azotemia showed more frequently in class IV group. Hence those three biological markers may be a clinical clue to pathologic diagnosis.

Cytohistologic Correlation of Phyllodes Tumors of the Breast: A Study on 17 Cases

Phyllodes tumor (PT) is a rare fibroepithelial tumor of the breast, and these tumors are subdivided into benign, borderline, and malignant tumors. The criteria for their histologic diagnosis have been relatively well-described. However, the cytologic diagnosis presents more difficulties and only a few cytologic studies concerned with their subclassification have been published. The objective of the current study is to describe the cytologic features of benign, borderline and malignant PTs in an attempt to distinguish one from the others. Cytohistologic correlation for 11 benign, 3 borderline and 3 malignant PTs was performed. For all these cases, the preoperative fine needle aspiration (FNA) findings were available for review. The features we examined were a necrotic background, cellularity, stromal tissue fragments, stromal pleomorphism and atypism, dissociated stromal cells and mitosis. The overall diagnostic accuracy of FNA for the PT grading was 88.2% (15/17). Two benign PTs were cytologically misinterpreted as "atypical epithelial and stromal cells" and "highly suspicious for ductal carcinoma". Nevertheless, the cytologic diagnosis and the grading of PTs on FNA were relatively reliable. Semiquantitative analysis for the cellular stromal tissue fragments, stromal pleomorphism and atypism, dissociated stromal cells and mitosis might be helpful for subclassifing PTs on FNA. In the case of encountering a markedly necrotic background, special concern about degenerative change such as infarction is needed.