Expression of Phospholipase A2 Receptor in PediatricHepatitis B Virus-Related Membranous Nephropathy

Purpose@#Hepatitis B virus (HBV) infection is among etiologies of secondarymembranousnephropathy (MN) in pediatric patients. We evaluated expressionof phospholipase A2 receptor (PLA2R), a specific target antigen of primary MN, inpediatric HBV-related MN. @*Methods@#We retrospectively reviewed patients with biopsy-proven HBV-relatedMN from the renal biopsy registry and electronic medical records of SeveranceHospital, Seoul, Korea, from 1993 to 2004. Paraffin-embedded human kidneytissues were retrieved and immunohistochemically stained for PLA2R. @*Results@#Ten pediatric patients with 13 biopsied specimens were reviewed. Thepredominant pathological stage was stage II–III, and second was stage II. Theintensity of staining for IgG was greatest, with less intense staining for IgM, IgA,C3, C4, and C1q. All the patients had angiotensin-converting enzyme inhibitorcombined with glucocorticoid, and four patients converted to cyclosporine treatmentfrom glucocorticoid monotherapy. Urinalysis of all the patients normalizedafter variable period. PLA2R staining was demonstrated in the outer glomerulus in3 out of 13 biopsies, 2 of which were obtained from the same patient over a 5-yearinterval. @*Conclusions@#PLA2R was expressed in a small number of cases diagnosed aspediatricHBV-related MN, indicating that some HBV-related MN cases may beprimary MN concurrent with HBV infection.

IgG4-Related Hepatic Inflammatory Pseudotumor Complicated by Actinomycosis during Steroid Therapy

For a 67-year-old man with diabetes mellitus, a 9-cm liver mass was found on CT during the diagnostic work-up for weight loss and fever. Dynamic CT and MRI showed a layered pattern of contrast enhancement suggesting the imaging features of the solid inflammatory mass. After tissue diagnosis of immunoglobulin G4 (IgG4)-related disease by gun needle biopsy, steroid therapy induced partial shrinkage of the mass on the follow-up CT at 4 weeks. On the 5-month follow-up CT with the maintenance of low-dose oral steroid medication, disease progression with invasion to diaphragm brought surgical intervention of right hemihepatectomy considering the possibility of combined malignancy. In the area of diaphragmatic destruction, focal actinomycosis was complicated in the main mass of IgG4-related disease. We are the first to describe a rare case of IgG4-related inflammatory pseudotumor, complicated by actinomycosis, showing an invasive nature that mimicked malignancy during steroid therapy in a diabetic patient.

Usefulness of Immunohistochemistry for Microsatellite Instability Screening in Gastric Cancer

BACKGROUND/AIMS: The usefulness of immunohistochemistry to screen for the microsatellite instability (MSI) phenotype in gastric cancer remains unclear. Moreover, the prognostic value of MSI phenotypes in gastric cancer has been debated. METHODS: The clinicopathologic parameters and survival outcomes of 203 MSI-high (MSI-H) and 261 microsatellite-stable (MSS) advanced gastric cancers (AGCs) were compared. Next, we compared the immunohistochemistry results for hMLH1 and hMSH2 with those of a polymerase chain reaction (PCR)-based method. Kaplan-Meier curves and a Cox proportional hazard regression model were used to conduct survival analyses. RESULTS: The MSI-H AGCs were correlated with older age (p<0.001), female gender (p=0.018), distal location (p<0.001), larger size (p=0.016), and intestinal type (p<0.001). Multivariate analysis revealed that the MSI-H phenotype was an independent favorable factor that was related to overall survival in patients with AGC (p<0.001). Compared with the PCR-based analysis, immunohistochemistry exhibited high sensitivity (91.1%) and specificity (98.5%) in the detection of MSI phenotypes. CONCLUSIONS: MSI-H gastric cancers have distinct clinicopathologic features and better prognoses, which suggests the necessity of MSI analysis in gastric cancer. Immunohistochemistry can be a useful and reliable screening method in the assessment of MSI status in gastric cancer.

Delayed Hemolytic Uremic Syndrome Presenting as Diffuse Alveolar Hemorrhage / 대한구급학회지

Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical intravascular hemolytic anemia with schistocytosis, thrombocytopenia and acute renal failure. Pulmonary involvement in HUS is known to be rare. We present the case of a 25-year-old male with diffuse alveolar hemorrhage and myocarditis followed by atypical hemolytic uremic syndrome. In this case, successful treatments included steroid pulse therapy for the fatal alveolar hemorrhage and plasma exchange for the hemolytic uremic syndrome.

Perivascular Epithelioid Cell Tumor Arising in the Sacrum: A Case Report

No abstract available.

Usefulness of Nuclear Protein in Testis (NUT) Immunohistochemistry in the Cytodiagnosis of NUT Midline Carcinoma: A Brief Case Report

No abstract available.

Diagnostic Accuracy of Cerebrospinal Fluid (CSF) Cytology in Metastatic Tumors: An Analysis of Consecutive CSF Samples

BACKGROUND: Cerebrospinal fluid (CSF) examination can be used to verify the presence of primary malignancies as well as cases of central nervous system (CNS) metastasis. Because of its importance, there have been several studies concerning the sensitivity of CSF cytology. To determine the practical use and reproducibility of diagnoses based on CSF cytology, we evaluated this test by analyzing cytology results from consecutive CSF samples. METHODS: Between July 2010 and June 2013, 385 CSF cytology samples from 42 patients were collected. The samples were gathered using a ventricular catheter and reservoir. CSF cytology of all patients was examined more than two times with immunocytochemistry for cytokeratin. RESULTS: Primary neoplastic sites and histologic types of patients' metastatic cancer were diverse. The overall sensitivity for detecting malignancy was 41.3%. Even within short-term intervals, diagnoses frequently changed. CONCLUSIONS: Our results were inconsistent, with low sensitivity, when compared to the results of previous studies. However, CSF evaluation can still provide valuable diagnostic and prognostic information because adjuvant treatments are now routinely performed in patients with CNS metastasis. Negative CSF cytology results should not be ignored, and continuous CSF follow-up is essential for following the clinical course of patients with metastatic cancer involving the CNS.

A Case of Immunoglobulin A Nephropathy in a Patient with Kimura's Disease / 영남의대학술지

Kimura's disease is an angiolymphoid-proliferative disorder that manifests with benign subcutaneous swelling predominantly in the head and the neck. Kidney involvement, including proteinuria, occurs in 12-16% of patients with the disease, and 60-78% of such cases is nephrotic syndrome. Reported etiologies of nephrotic syndrome in Kimura's disease include membranous glomerulonephritis, mesangial proliferative glomerulonephritis, minimal-change disease, focal segmental glomerulosclerosis, diffuse proliferative glomerulonephritis and immunoglobulin A (IgA) nephropathy. There have been only two case reports of IgA nephropathy in Kimura's disease, in 1998. In this report, we present a third case of IgA nephropathy associated with Kimura's disease.

Recurrent Esophageal Stricture Caused by Candidiasis in an HIV-Infected Patient / 대한내과학회지

Esophageal candidiasis is a common opportunistic infection that develops in human immunodeficiency virus (HIV)-infected patients. It is usually effectively treated with fluconazole, and the occurrence of an esophageal stricture in association with esophageal candidiasis has rarely been reported in HIV-infected patients. In the case presented here, a 49-year-old man was diagnosed with severe esophageal candidiasis with HIV infection. At the time of the HIV diagnosis, he had immunodeficient status with a CD4+ T lymphocyte count of 150 cells/mm3. He received antifungal treatment with fluconazole and combined antiretroviral therapy. Although the esophageal candidiasis improved with rapid recovery of the CD4+ T lymphocyte count, a recurrent esophageal stricture developed. To treat the recurrent esophageal stricture, the patient received repeated balloon dilatation and stent insertion. We report this rare case of severe esophageal stricture complication of esophageal candidiasis in an HIV-infected patient.

Recurrent Esophageal Stricture Caused by Candidiasis in an HIV-Infected Patient / 대한내과학회지

Esophageal candidiasis is a common opportunistic infection that develops in human immunodeficiency virus (HIV)-infected patients. It is usually effectively treated with fluconazole, and the occurrence of an esophageal stricture in association with esophageal candidiasis has rarely been reported in HIV-infected patients. In the case presented here, a 49-year-old man was diagnosed with severe esophageal candidiasis with HIV infection. At the time of the HIV diagnosis, he had immunodeficient status with a CD4+ T lymphocyte count of 150 cells/mm3. He received antifungal treatment with fluconazole and combined antiretroviral therapy. Although the esophageal candidiasis improved with rapid recovery of the CD4+ T lymphocyte count, a recurrent esophageal stricture developed. To treat the recurrent esophageal stricture, the patient received repeated balloon dilatation and stent insertion. We report this rare case of severe esophageal stricture complication of esophageal candidiasis in an HIV-infected patient.

Cellular Angiofibroma of the Perianal Space: MR Imaging and Pathologic Correlation

A variety of benign and malignant tumor can occur in the perianal space. Among them, cellular angiofibroma is a rare tumor that belongs to fibroblastic/myofibroblastic tumors of the WHO classification, sometimes called as "angiomyofibroblastoma-like tumor". Cellular angiofibroma can be found at various anatomic sites such as the vulva, perineum, genital tract, and inguinal regions with approximately equal gender ratio. This tumor is usually a well-circumscribed mass showing isosignal intensity to muscle on T1-weighted images. On T2-weighted images, cellular angiofibroma may show as a mass with inhomogeneous signal intensity depending on amount of composed spindle cell, collagenous stroma, myxoid matrix, and fat tissue; however it is characterized by low signal intensity due to fibrous tissues. This highly vascular mass shows strong enhancement on post contrast images. Recommended treatment is simple local excision and no recurrence or metastasis have been reported up to date.