A case of Fanconi syndrome accompanied by crystal depositions in tubular cells in a patient with multiple myeloma

Fanconi syndrome (FS) is a rare condition that is characterized by defects in the proximal tubular function. A 48-year-old woman was admitted for evaluation of proteinuria. The patient showed normal anion gap acidosis, normoglycemic glycosuria, hypophosphatemia, and hypouricemia. Thus, her condition was compatible with FS. The M peak was found behind the beta globulin region in urine protein electrophoresis. Upon bone marrow examination, we found that 24% of cells were CD138+ plasma cells with kappa restriction. From a kidney biopsy, we found crystalline inclusions within proximal tubular epithelial cells. Thereafter, she was diagnosed with FS accompanied by multiple myeloma. The patient received chemotherapy and autologous stem cell transplantation, and obtained very good partial hematologic response. However, proximal tubular dysfunction was persistent until 1 year after autologous stem cell transplantation. In short, we report a case of FS accompanied by multiple myeloma, demonstrating crystalline inclusion in proximal tubular cells on kidney biopsy.

Fibrous hamartoma of infancy: an experience of a single institute / 대한외과학회지

PURPOSE: Fibrous hamartoma (FH) of infancy is a distinctive fibrous growth that most frequently occurs at birth and during the postnatal period. It is important for clinicians and pathologists to recognize this entity to avoid an aggressive approach. METHODS: We herein describe the clinicopathologic features of 9 FHs diagnosed at a single institution between 1997 and 2010. RESULTS: There were 7 boys and 2 girls, and the mean age of presentation was 14.7 months. The common locations were the lower back and gluteal region (n = 3) and scrotum (n = 2). They were solitary lesions, and measured 1.0 to 7.0 cm in maximum diameter (mean, 4.9 cm). The excised masses tended to be poorly circumscribed, and consisted of an intimate mixture of firm, gray-white tissue with fat. Histologically, these lesions were composed of 3 components forming a vague, irregular, organoid pattern: well-defined intersecting trabeculae of fibrocollagenous tissue; loosely textured areas of small, rounded, primitive mesenchymal cells; and mature fat. Over a median follow-up of 72 months, no patient showed recurrence. CONCLUSION: FH should be distinguished from other forms of fibromatosis and malignant tumors because it is benign and usually cured by local excision.

Three Cases of Minimal Change Nephrotic Syndrome in Patients with Type 2 Diabetes / 대한신장학회지

Diabetic nephropathy is a common and serious complication of diabetes characterized by persistent proteinuria, hypertension and a progressive decline of renal function. However, non-diabetic renal disease can be present in diabetic patients and differential diagnosis of treatable disease is important. Minimal change nephrotic syndrome is characterized by normal light microscopic finding and effacement of foot process in electron microscope, but foot process effacement is not specific and it can be present in the glomeruli of the most glomerulopathy including diabetic nephropathy. Therefore, pathologic diagnosis of minimal change nephrotic syndrome combined with diabetic nephropathy is very difficult. However, we could exclude other glomerulopathy and diagnose minimal change nephrotic syndrome by clinical features in three type 2 diabetic patients with diabetic nephropathy and have successfully treated with corticosteroid.

A Case of Crescentic Glomerulonephritis Superimposed on Membranous Glomerulopathy / 대한신장학회지

Membranous glomerulopathy (MGN) is a common cause of nephrotic syndrome in adults. Renal failure gradually develops in patients with MGN and crescentic glomerulonephritis (CGN) superimposed on MGN is a rare cause of acute renal failure. In most cases patients showed nephrotic syndrome with acute renal failure. We report a 33-year-old woman with azotemia but with no other symptoms such as nephrotic syndrome she had been diagnosed to have MGN 15 months before. There seemed to be no other cause of azotemia. Renal biopsy was performed and revealed CGN on existing MGN. She was treated with immunosuppression treatment and azotemia was improved. When unexplained azotemia develops in patients with MGN, we should promptly investigate superimposed conditions including CGN. In CGN superimposed on MN, a potentially reversible condition with appropriate immunosuppression therapy should be considered.

Application of the Revised Case Matrix Format to Tutorial in Pathology Teaching: An Interim Approach toward Problem-Based Learning under Traditional Curricular Structure

This paper describes a student-centered case study program concerning the tumor pathology course for first year students in medical school under the traditional curricular structure. A traditional, discipline-oriented, lecture-laboratory approach was partly modified by introducing a tutuorial session using a modified case matrix format during the laboratory hours without altering the general scheme of the existing system. Small group tutorial sessions were set with the development of learning objectives emphasizing clinicopathologic reasoning and early exposure to future practical presentation which was followed by the large class session; each tutorial was supplied with a short clinical history, gross kodachrome slides, and microslides. The session for problem identification was replaced by proving a series of instructor-designed questions for both pathology and interdisciplinary correlation during which pedagogical implication was stressed the most. Student's active participation, development of self learning skill and vigorous teaching-learning process among students, and motivation/relevance for forthcoming pathology study were among the benefits conferred by this modification. We conclude that this approach is an interim step to meet the advantages of problem-based learning even in a traditional curricular structure.

Application of the Revised Case Matrix Format to Tutorial in Pathology Teaching: An Interim Approach toward Problem-Based Learning under Traditional Curricular Structure

This paper describes a student-centered case study program concerning the tumor pathology course for first year students in medical school under the traditional curricular structure. A traditional, discipline-oriented, lecture-laboratory approach was partly modified by introducing a tutuorial session using a modified case matrix format during the laboratory hours without altering the general scheme of the existing system. Small group tutorial sessions were set with the development of learning objectives emphasizing clinicopathologic reasoning and early exposure to future practical presentation which was followed by the large class session; each tutorial was supplied with a short clinical history, gross kodachrome slides, and microslides. The session for problem identification was replaced by proving a series of instructor-designed questions for both pathology and interdisciplinary correlation during which pedagogical implication was stressed the most. Student's active participation, development of self learning skill and vigorous teaching-learning process among students, and motivation/relevance for forthcoming pathology study were among the benefits conferred by this modification. We conclude that this approach is an interim step to meet the advantages of problem-based learning even in a traditional curricular structure.