Sarcomatoid carcinomas are malignant epithelial neoplasms in which the tumor cells assume a partial or complete spindle cell pattern of growth, leading to the erroneous classification of some true carcinomas as sarcomas. These spindle cells are malignant and manifest various amount of both vimentin and cytokeratin. Positive reaction of some of the spindle cells for cytokeratin antibodies is confirmatory. Clinical features do not differ significantly from those of patients with high-grade transitional cell carcinoma. So far, 13 cases of sarcomatoid transitional cell carcinoma of the renal pelvis have been reported in English and Korean literature. In this report, we describe clinicopathologic features of recently observed two cases of sarcomatoid transitional cell carcinoma of the renal pelvis and summarize the pathologic findings of previously reported cases with review of the literature.
Antibodies ; Carcinoma ; Carcinoma, Transitional Cell* ; Carcinosarcoma ; Classification ; Humans ; Keratins ; Kidney Pelvis* ; Sarcoma ; Vimentin

PURPOSE: Membranoproliferative glomeulonephritis(MPGN) has been diagnosed in an increasing number of asymptomatic cases. These cases have been detected by school urinary screening test even though the total cases of MPGN show a decreasing trend. We have analyzed the clinical and pathological characteristics of children with MPGN according to the clinical manifestations at the time of disease presentation. METHODS: A total of 18 patients who had been diagnosed with idiopathic MPGN by percutaneous renal biopsy from January 1990 to February 2004 were involved in our study. The patients were divided into 2 groups as the school urinary screening(A) group and the symptomatic(S) group according to the clinical manifestations at the time of disease presentation. RESULTS: Out of the total 18 patients, 8(44.4%) were in the S group and 10(55.6%) were in the A group. The mean serum total protein, albumin and C3 levels in the S group were significantly lower than those levels of the A group, respectively(4.9+/-1.2 g/dL vs 7.0+/-0.5 g/dL P=0.002, 2.8+/-0.9 g/dL vs. 4.1+/-0.3 g/dL P=0.002, 63.9+/-36.4 mg/dl vs. 100.8+/-39.5 g/dl P=0.041). The mean total protein amount of 24 hour collected urine in the S group were significantly higher than that of the A group(3684.0+/-2601.3 mg/m2 vs. 559.4+/-406.9 mg/m2, respectively, P=0.001). Hypocomplementemia was observed in 11(61.1%) out of 18 patients at the time of disease onset, 7(87.5%) in the S group and 4(40%) in the A group. However the hypocomplementemia was decreased in 6(33.3%) out of 18 patients at the time of final follow-up, 3(37.5%) in the S group and 3(30%) in the A group. According to the pathologic type, hypocomplementemia was observed 8 patients(61.5%) with type I disease, 1 patients (100%) with type II disease, 2 patients(50%) in type III disease at the disease onset, but 4 patients(30.8%) in type I disease, 1 patient(100%) in type II disease, 1 patient(33.3%) with type III disease at the time of last follow-up. The incidence of cellular crescent formation and tubular atropy, as observed on light microscopy, were higher in the S group compared to the A group. Mean grade of capillary wall thickening and, mesangial proliferation were significantly higher in the S group. CONCLUSION: MPGN, as diagnosed in patients with only asymptomatic urinary abnormalities, has been increasing, it is more frequent in asymptomatic patients than in patients with presenting symptoms. Our result suggests that MPGN should be considered in the renal biopsy diagnosis regardless of serum C3 level when urinary abnormalities are found by school urinary screening test.
Biopsy ; Capillaries ; Child* ; Diagnosis ; Follow-Up Studies ; Glomerulonephritis, Membranoproliferative* ; Humans ; Incidence ; Mass Screening ; Microscopy

BACKGROUND: Childhood obesity leads to a high possibility of adult obesity. Previous studies show cases of research on obesity control programs led by doctors specializing in obesity, and dietitians. However, no such research have yet targeted schools, where such obesity control program are led by the school's staff (dietitian, school nurse and physical education teacher) instead. Thus, this research set out to determine whether obesity control programs could be effectively utilized within elementary schools as a part of the school health problems. As a start, a book on Elementary School Obese Children Control Program was used as the main text to provide the basics of the program. METHODS: The research was targeted at an elementary school located in NoWon district, where 16 moderate obese children from grades 3 to 6, were chosen to participate in training once every week for 6 weeks, together with exercises once every week for 5 weeks. RESULTS: The evaluation of the obesity control program showed significant results in obesity index, body fat and HDL-cholesterol. The school nurse contacted each student's teacher every day and an exercise or training was scheduled, so that students could participate in the program. This contributed to a high participation rate, with an overall participation rate reaching 85% and an exercise participation rate reaching 79%. During the evaluation of the program, it was found that the regular exercises carried out once a week in the school led the students to get in the habit of exercising regularly after school. CONCLUSION: It is considered that there would be a considerable change in childhood obesity control if the school nurse, dietitian and physical education teachers each played a part in carrying out an obesity control program as part of the school health care.
Adipose Tissue ; Adult ; Child ; Exercise ; Humans ; Nutritionists ; Obesity* ; Pediatric Obesity ; Physical Education and Training ; School Health Services

Fabry disease is an X-linked lysosomal storage disorder caused by a deficiency of the enzyme α-galactosidase A, resulting in the accumulation of glycosphingolipids within the lysosomes of various cell types. It has a wide spectrum of clinical phenotypes, and renal failure is a serious complication. Fabry disease is confirmed either by measurement of α-galactosidase A activity or by genetic testing for GLA mutations. Renal biopsy findings on light microscopy, specifically enlarged podocytes with foamy cytoplasm, and osmiophilic inclusion bodies in the cytoplasm in all types of renal cells on electron microscopy, are characteristic of this disease. The predominant differential diagnosis is iatrogenic phospholipidosis in association with certain drugs that can cause cellular injuries indistinguishable from Fabry disease. Here, we report the case of a 10-year-old boy with microscopic hematuria who underwent a renal biopsy that showed morphological findings consistent with Fabry disease, although the patient had neither a GLA mutation nor a history of drug consumption. Six years later, spontaneous regression of this renal pathology was observed in a second renal biopsy examination.
Biopsy* ; Child* ; Cytoplasm ; Diagnosis, Differential ; Fabry Disease* ; Genetic Testing ; Glycosphingolipids ; Hematuria* ; Humans ; Inclusion Bodies ; Lysosomes ; Male* ; Microscopy ; Microscopy, Electron ; Pathology ; Phenotype ; Podocytes ; Renal Insufficiency

It is uncommon that Anesthesiologist experience thyroid storm during the elective surgery because of marked reduction of operation for the treatment of hyperthyroidism owing to the development of radio-active iodide, antithyroid drugs and the use of beta-receptor blockers such as propranolol. In the treatment of Graves' disease, the conditions such as persistant symptoms of hyperthyroidism with resistance to the antithyroid drugs, frequent relapses and increasing size of thyroid gland must be considered for the need of surgery. It is essential to maintain the euthyroid state before surgery in these cases, if not, the possibility of thyroid storm is increased from increased release of thyroid hormones owing to manipulation of surgery. In our case, the patient had been medicated for 6 years, however, the euthyroid state had not been achieved before the day of surgery. Under the judgement of difficulty for more control of hyperthyroidism the surgeon requested for operation without delay. Immediately after starting operation, the symptoms suggesting thyroid storm such as the abrupt onset of high fever, the increasing blood pressure and heart rate were occured. The sugery was stopped and then the management for thyroid storm was begun. At arterial blood gas analysis, respiratory acidosis and metabolic acidosis were developed and increased hilar haziness suggesting severe pulmonary edema were present in both lung fields at chest x-ray. The patient was mechanically ventillated until improvement of metabolic and respiratory acidosis and pulmonary edema. Two days after operation, vital signs were stabilized and the patient was transferred to general wards.
Acidosis ; Acidosis, Respiratory ; Antithyroid Agents ; Blood Gas Analysis ; Blood Pressure ; Fever ; Graves Disease ; Heart Rate ; Humans ; Hyperthyroidism ; Lung ; Patients' Rooms ; Propranolol ; Pulmonary Edema ; Recurrence ; Thorax ; Thyroid Crisis* ; Thyroid Gland* ; Thyroid Hormones ; Thyroidectomy* ; Thyrotoxicosis* ; Vital Signs

PURPOSE: It was well known that transforming growth factor (TGF)-beta1 plays a pivotal role in interstitial fibrosis and loss of podocyte. We explored the effects of exogenous administration of TGF-beta1 latency-associated peptide (LAP) in a model of renal fibrosis induced by unilateral ureteral obstruction (UUO) and examined whether TGF-beta1 LAP can inhibit apoptosis of podocyte. METHODS: Twenty four male BALB/c mice were unilaterally obstructed of proximal ureters by ligation. Half of the mice with operation and half of 8 control were administered recombinant human LAP intraperitoneally. One to three mice per group were euthanized on days 3, 7, 14, and 21 after operation for observation of renal fibrosis and apoptosis of podocyte. RESULTS: Interstitial fibrosis was less severe in LAP-treated group. Obstructed kidneys from LAP- untreated mice had more glomerular apoptotic podocytes (TUNEL assay) compared to LAP-treated mice at day 7, 14, and 21 after operation. CONCLUSION: Intraperitoneal administration of TGF-beta1 LAP prevents the loss of podocyte & renal damage partially up to day 14 after operation.
Animals ; Apoptosis ; Fibrosis ; Humans ; Kidney ; Ligation ; Male ; Mice ; Podocytes ; Transforming Growth Factor beta1 ; Transforming Growth Factors ; Ureter ; Ureteral Obstruction

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.
Child ; Female ; Humans ; Hypertension/*etiology ; Ovarian Neoplasms/complications/*diagnosis/pathology ; Puberty, Precocious/enzymology/*etiology ; Receptors, Cell Surface/metabolism ; Receptors, Progesterone/metabolism ; Renin/blood ; Sex Cord-Gonadal Stromal Tumors/complications/*diagnosis/pathology ; Steroids/biosynthesis ; Tomography, X-Ray Computed ; Vacuolar Proton-Translocating ATPases/metabolism

BACKGROUND: Friedewald equation is the most widely used method for estimating low-density lipoprotein cholesterol (LDL-C) level. However, due to potential over- or underestimation, many studies have used a modified equation. This study aimed to compare estimates by 4 different equations to directly measured LDL-C concentrations in order to propose the most appropriate method for LDL-C estimation in the Korean population. METHODS: We studied data of 4,350 subjects that included total cholesterol, high-density lipoprotein cholesterol (HDL-C), triglyceride (TG), and LDL-C concentrations that had been measured at one university hospital in Seoul. We investigated 4 equations: LDL-C by Friedewald's original equation (LDL-C(F)) and its 3 modifications. Pearson correlation analysis was performed to compare these estimates to the direct measurement. RESULTS: Pearson correlation analysis revealed a good correlation among all 4 estimated LDL-C values and the directly measured LDL-C value. The Pearson coefficients were 0.951 for LDL-C(F), 0.917 for LDL-C by Hatta equation (LDL-C(H)), 0.968 for LDL-C by Puavilai equation (LDL-C(P)), and 0.983 for LDL-C by Martin equation (LDL-C(M)). Martin equation (LDL-C(M)) resulted in the best approximation (mean difference from the direct measurement, 5.5 mg/dL; mean percentage difference from the direct measurement, 5.1%) and the best agreement with the direct measurement (86.1%). LDL-C(P) resulted in the second-best approximation (mean difference, 7.0 mg/dL; mean percentage difference, 6.2%; concordance, 82.5%). LDL-C(M) was found to be less influenced by TG and HDL-C levels than by LDL-C(F). CONCLUSION: Estimates by Martin equation had the best agreement with direct LDL-C concentrations and both Martin and Puavilai equations were superior to Friedewald equation for estimating LDL-C concentrations in Korean adults.
Adult* ; Cholesterol* ; Dyslipidemias ; Humans ; Lipoproteins* ; Methods* ; Seoul ; Triglycerides

Amyloidosis comprises a diverse group of systemic and local diseases characterized by organ involvement by the extracellular deposition of fibrils composed of subunits of a variety of normal serum proteins. Secondary amyloidosis is caused by the deposition of amyloid A(AA) protein in chronic inflammatory disease. Juvenile rheumatoid arthritis(JRA) has been known to be the most common cause of secondary amyloidosis. We experienced one case of secondary renal amyloidosis in a 12-year-old girl who had suffered from JRA for several years who had visited our renal clinic to evaluate the proteinuria with microscopic hematuria which was detected by chance at school urine screening examination. Apple green birefringence was observed under polarized light with Congo red stain and characteristic electron microscopic findings was also noted in renal tissues which was obtained by percutaneous renal biopsy. In our knowledge, this is the first case report of secondary renal amyloidosis developed in pediatric age in Korea.
Amyloid ; Amyloidosis* ; Arthritis, Juvenile* ; Biopsy ; Birefringence ; Blood Proteins ; Child* ; Congo Red ; Female ; Hematuria ; Humans ; Korea ; Mass Screening ; Proteinuria

PURPOSE: In order to evaluate the value of the renal expression of ICAM-1 as a marker of renal injury, we analyzed the relationship between abnormal tubular expression of ICAM-1 and histopathological features and clinical manifestations in children with IgA nephropathy (IgAN). METHODS: The clinical data from 43 patients with IgAN were analyzed retrospectively and compared to the histopathologic subclassification proposed by Haas. ICAM-1 in tubular epithelium was assessed using the LSAB(Labeled streptavidine biotin) kit on the renal biopsy specimens. RESULTS: In 43 patients with primary IgAN, 28 males and 15 females aged 12.2+/-2.2 years were studied. There were no differences of renal tubular expression of ICAM-1 between patients with gross hematuria and without gross hematuria. But renal tubular expression of ICAM-1 in patients with proteinuria was significantly higher than that of in patients without proteinuria(78.72+/-14.19% vs. 55.80+/-32.20%, P<0.05). Renal tubular expression of ICAM-1 was also associated with the severity of histopathological degree using Haas classification method. In subclass I, renal tubular expression of ICAM-1 was significantly lower than those of other subclasses. A significant correlation was found between the tubular expression of ICAM-1 and the total amount of protein in 24 hour collected urine(rs=0.47236, P<0.05). But there were no significant correlations between the renal tubular expression of ICAM-1 and interstitial cellular infiltration, tubular atrophy, and interstitial fibrosis respectively(F=0.89, P>0.05; F=0.31, P>0.05; F=0.21, P>0.05). CONCLUSION: Renal tubular expression of ICAM-1 can be a useful marker of renal injury in children with IgAN.
Atrophy ; Biopsy ; Child* ; Classification ; Epithelium ; Female ; Fibrosis ; Glomerulonephritis, IGA* ; Hematuria ; Humans ; Immunoglobulin A* ; Intercellular Adhesion Molecule-1* ; Male ; Proteinuria ; Retrospective Studies ; Streptavidin