A Case of Focal Segmental Glomerulosclerosis in Turner Syndrome / 고신대학교의과대학학술지

Turner syndrome is usually accompanied with various anomalies. Congenital urological and renal abnormalities are often associated with this syndrome. The occurrence of glomerulonephritis is uncommon. An 18-year-old woman showed fatigue and profound proteinuria. She had been diagnosed with Turner syndrome in her age of 15. The kidney biopsy specimen examined by light microscopy, immunofluorescence and electron microscopic examination revealed focal segmental glomerulosclerosis. This is the first case report of focal segmental glomerulosclerosis in turner syndrome in South Korea.

A Case of Hypersensitivity Pneumonitis with Giant Cells in a Female Dental Technician

OBJECTIVES: Dental technicians are exposed to methyl methacrylate(MMA) and hard metal dusts while working, and several cases of hypersensitivity pneumonitis caused by the exposure have been reported. The authors experienced a case of hypersensitivity pneumonitis in a female dental technician who had 10 years' work experience and report the case with clinical evidence. METHOD: The patient's work, personal, social, and past and present medical histories were investigated based on patient questioning and medical records. Furthermore, the workplace conditions and tools and materials the patient worked with were also evaluated. Next, the pathophysiology and risk factors of pneumonitis were studied, and studies on the relationship between hypersensitivity pneumonitis and a dental technician's exposure to dust were reviewed. Any changes in the clinical course of her disease were noted for evaluation of the work-relatedness of the disease. RESULTS: The patient complained of cough and sputum for 1 year. In addition, while walking up the stairs, the patient was not able to ascend without resting due to dyspnea. She visited our emergency department due to epistaxis, and secondary hypertension was incidentally suspected. Laboratory tests including serologic, electrolyte, and endocrinologic tests and a simple chest radiograph showed no specific findings, but chest computed tomography revealed a centrilobular ground-glass pattern in both lung fields. A transbronchial biopsy was performed, and bronchoalveolar washing fluid was obtained. Among the findings of the laboratory tests, microcalcification, noncaseating granuloma containing foreign body-type giant cells, and metal particles within macrophages were identified histologically. Based on these results, hypersensitivity pneumonitis was diagnosed. The patient stopped working due to admission, and she completely quit her job within 2 months of restarting work due to reappearance of the symptoms. CONCLUSION: In this study, the patient did not have typical radiologic findings, but pathological evaluation of the lung biopsy from the bronchoscope led to the suspicion of pneumonitis. Under the microscope, the sample contained fibrotic changes in the lung, multinucleated giant cells, and particles in macrophages and was diagnosed as dental technician pneumoconiosis by the pathology. Working as a dental technician had directly exposed her to light metal dust and MMA, and her clinical symptoms and radiologic findings subsided after withdrawal from exposure to the workplace. These outcomes led to the diagnosis of hypersensitity pneumonitis due to MMA exposure and strong work-relatedness.

Expression of E-cadherin, Heat Shock Protein 47, Transforming Growth Factor beta1 and C4d in Chronic Allograft Nephropathy / 대한이식학회지

BACKGROUND: Chronic allograft nephropathy (CAN), which causes graft failure, is related to tubular atrophy and interstitial fibrosis. E-cadherin is a well-known epithelial marker and heat shock protein (HSP)-47 is a collagen-specific molecular chaperone that regulates collagen synthesis. Transforming growth factor (TGF)-beta1, a profibrotic cytokine, downregulates E-cadherin and induces expression of mesenchymal markers in an in vitro model. C4d expression is considered a poor prognostic marker for graft survival. This study evaluated the relationship between the expression of E-cadherin, HSP47, TGF-beta1, and C4d with the prognosis for CAN. METHODS: Between March 1991 and August 2007, we performed renal allograft biopsies on 42 recipients with deteriorating renal function. CAN was diagnosed according to the chronic allograft damage index (Banff classification). Renal allograft biopsies were examined for the expression of E-cadherin, HSP47, TGF-beta1, or C4d by immunohistochemistry. The HSP47, TGF-beta1, and E-cadherin staining was scored semiquantitatively by analyzing ten different fields of cortical interstitium and tubules. Biopsies with endothelial C4d staining in peri-tubular capillaries (> or =25%) were designated as C4d-positive. RESULTS: Of 42 recipients, 17 (40.5%) were in the graft survival group (GS) and 25 (59.5%) were in the graft failure group (GF). E-cadherin expression in tubular cells of the GS was much higher than that of the GF (94.1% vs 52%, P=0.04). HSP47 expression in tubular cells and interstitium in the GF was much higher than that in the GS (84% vs 35.3%, P=0.001). TGF-beta1 expression in tubular cells and interstitium in the GF was much higher than that in the GS (72% vs 23.5%, P=0.02). CONCLUSIONS: E-cadherin, HSP47, and TGF-beta1 expression was strongly correlated with the CAN prognosis.

A Case of IgA Nephropathy Associated with Thin Basement Membrane Disease / 대한신장학회지

IgA nephropathy and thin basement membrane disease are common glomerular diseases in persistent microscopic hematuria with or without proteinuria. However, these two conditions cannot be easily distinguished on the biochemical or urinary findings alone. Therefore, renal biopsy is required for correct identification of the two conditions in most cases. Recently, it has been reported that thinning of glomerular basement membrane is accompanied with precipitation of electron dense deposits in some patients with IgA nephropathy. We report a case of IgA nephropathy associated with thin basement membrane disease in a 19-year-old male with microscopic hematuria and mild proteinuria. After 2 years' treatment with angiotensin II receptor blocker, the patient exhibited persistent microscopic hematuria but decreased proteinuria. Our finding concurs with the previous reports indicating that patients with both IgA nephropathy and thin basement membrane disease do not have different clinical features compared to those with IgA nephropathy alone. In addition, clinical outcome does not appear to be affected by thin basement membrane disease when these two conditions are combined.

Kaposi's Sarcoma on Mediastinum after Renal Retransplantation

Kaposi's sarcoma occurs in higher rates in the setting of immunosuppression, especially in patients with acquired immunodeficiency syndrome (AIDS), immunosuppressive therapy or posttransplantation, commonly involving the skin, visceral, oral cavity or respiratory tract. Of the de novo malignancies in transplantation patients, the incidence of Kaposi's sarcoma is increasing steadily. We report a case of a 37-year-old male patient who was diagnosed with Kaposi's sarcoma 16 years after his first renal transplantation and 5 months after his second transplantation. He presented with lymphoproliferative lesions in the mediastinum and supraclavicular area without showing any typical cutaneous lesions. Diagnosis was confirmed by gun biopsy of the enlarged axillary lymph nodes. Tacrolimus, the initial immunosuppressive drug, was tapered while sirolimus therapy and chemotherapy with vincristine was initiated. The enlarged lymph nodes decreased in size and the patient has been treated with vincristin and conversion of tacrolimus to sirolimus.

Recovery from crescentic glomerulonephritis with bacterial endocarditis with antibiotics alone / 대한내과학회지

A 59-year-old woman presented to our hospital with fever, petechiae, pyuria, gross hematuria, and rapidly progressive glomerulonephritis (GN). She had a history of urinary tract infection. A renal biopsy specimen revealed crescentic GN and an echocardiogram showed a vegetation 3.15x1.73 cm in size on the mitral valve and severe mitral valve regurgitation. Blood cultures grew Enterococcus faecalis. Treatment with antibiotics alone resulted in clinical improvement of the renal function and resolution of the fever. Three months after hospitalization, the echocardiogram showed mild mitral valve regurgitation. This case suggests that crescentic glomerulonephritis associated with even a huge vegetation of infectious endocarditis can be treated with antibiotics alone and result in stable renal function.

CT, MR, and Angiography Findings of a Solitary Fibrous Tumor of the Larynx: a Case Report

This report details the CT, MR, and angiography findings of a solitary fibrous tumor involving the larynx of a 34-year-old man. A precontrast CT scan revealed a well-defined isodense mass in the submucosal region of the supraglottic larynx. The tumor appeared as a mixed intensity lesion on the T1- and T2-weighted MR images. A T2-weighted MR image showed a central, round, and low signal intensity area within the mass. For both the CT and MR images, the mass demonstrated heterogeneous enhancement following the administration of contrast material. The angiography showed a hypervascular tumor with heterogeneous persistent staining.

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.

Living Donor Liver Transplantation in a Child with Unresectable Hepatoblastoma / 대한이식학회지

Hepatoblastoma is the most common primary malignant liver tumor encountered during childhood. Complete surgical resection is the most important factor in predicting which children would achieve cure. Some chemotherapy regimens are effective in reducing tumor size, often rendering initially unresectable lesions amenable to complete surgical resection, and dramatically improving disease-free survival. Liver transplantation provides an additional treatment option for patients whose tumor remains unresectable even after preoperative chemotherapy. We report a case of unresectable hepatoblastoma in a child who underwent living related liver transplantation with pre and post-operative chemotherapy.

Epidermoid Cyst of the Ovary: Two case reports

Two cases of epidertnoid cyst of the ovary are added to the ten reported in the literature. This rare lesion is usually an incidental finding. Its main interest is in its histogenesis, which includes metaplasia of the coelomic surface epithelium, monophyletic development of teratoma, and metaplasia of the rete ovarii. We investigated two cases of epidertnoid cyst. Histologic examination of the cysts showed a thin lining of mature keratinizing squamous epithelium rectum: unaccompanied by skin appendages or other teratomatous elements.