Thirty cases of rhabdomyosarcoma in patients under the age of 15 years were reviewed retrospectively to find out characteristics of histologic subtypes related to prognosis. Histologic diagnosis were embryonal in 21(70%), alveolar in 7(23%) and mixed type in 2(7%). Cellular anaplasia was noted in 6 cases. Round to polygonal cells with abundant eosinophilic cytoplasmic rim or globules were most characteristic cytologic feature or rhabdomyosarcomas, being followed by myofibrils. Myxoid and edematous stroma in embryonal type and alveolar pattern in alveolar type wee nost characteristic growth pattern. Immunohistochemically, tumors were positive for desmin, actin and myoglobin in 97%, 27%, and 40%, respectively. Consequently, characteristic cytologic features are important to differentiate rhabdomyosarcomas form other kinds of small round cell tumor and growth pattern is more critical in categorizing histologic subtypes. Desmin is far more useful than actin or myoglobin in the diagnosis of rhabdomyosarcoma.
Child ; Male ; Female ; Humans

Drash syndrome, which was first reported by Denys et al. in 1967 is a complex disorder which associates a nephropathy, Wilms' tumor, and male pseudohermaphroditism. The common denominator is a nephropathy. The nephropathy may be associated with either genital abnormalities or Wilms' tumor, and these associations are called incomplete form of Drash syndrome. This syndrome appears early in life and the first sign usually is genital ambiguity. The nephropathy presents with proteinuria, hematuria and hypertension, and eventually progresses to end stage renal failure. Renal biopsy may reveal a variety of glomerular and interstitial changes. Wilms' tumor may appear as s mass on ultrasound or it may not be recognized until nephrectomy or even autopsy. We report on a boy with nephropathy and genital abnormalities. A nephrotic syndrome with hypertension was present when first seen at 15 days of age. The karyotype was 46, XY and external genitalia was ambiguous. The nephrotic syndrome and signs of renal insufficiency persisted and he died at the age of 40 days. Histopathologic findings of kidney at autopsy revealed those of diffuse mesangial sclerosis. The case was presented with brief review of literatures.
46, XY Disorders of Sex Development ; Autopsy ; Biopsy ; Denys-Drash Syndrome* ; Disorders of Sex Development ; Genitalia ; Hematuria ; Humans ; Hypertension ; Karyotype ; Kidney ; Male ; Nephrectomy ; Nephrotic Syndrome ; Proteinuria ; Renal Insufficiency ; Sclerosis ; Ultrasonography ; Wilms Tumor

Pigmented(melanotic) schwannoma is a very rare variant of schwannoma that characteristically has massive cytoplasmic melanin. Since it was described in 1946 by Bjorneboe, about 44 cases have been reported in the English literature. It has a relatively benign clinical course, but the cases arising in the cranial nerve and sympathetic chain show aggressive behavior with malignant potential. We herein report a typical case of pigmented schwannoma with light microscopic findings. The results of immunohistochemical and electronmicroscopic study are also presented. The patient was a 30 year-old Korean male who had a mass in his posterior neck for 10 years that recently.began to induce neurologic manifestations. The tumor, which was mainly in the extramedullary intradural space of the cervical canal extending to the extradural space and soft tissues of the neck, was relatively well defined and composed of black solid tissue. Microscopic, densely pigmented spindle cells forming fascicles, nuclear palisading, whorling and polygonal cells with vacuolated or clear cytoplasm were characteristic. Immunohistochemically, the tumor cells were positive for vimentin, S-100 protein, and HMB-45. Electron microscopic study revealed that the tumor cells have interdigitating cytoplasmic processes containing varying stages of melanosomes with a very focal basal lamina and Luse body like collagen bundles.
Male ; Humans

Renal calcifications are a recognized complication of furosemide therapy in premature infants. Particularly in infants with chronic lung disease, the use of this potent diuretic is associated with hypercalciuria, which may predispose the infant to medullary nephrocalcinosis and renal calculi, We experienced two cases of nephrocalcinosis in very low birth weight infants. One had bronchopulmonary dysplasia, pneumonia, patent ductus arteriosus, ventricular septal defect and congestive heart failure and the other had systemic cytomegalovirus infection with cytomegalovirus pneumonitis and ricket of prematurity. Both patients received a large amount of furosemide. We presented these cases with brief review of literatures.
Bronchopulmonary Dysplasia ; Cytomegalovirus ; Cytomegalovirus Infections ; Ductus Arteriosus, Patent ; Furosemide ; Heart Failure ; Heart Septal Defects, Ventricular ; Humans ; Hypercalciuria ; Infant* ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight* ; Kidney Calculi ; Lung Diseases ; Nephrocalcinosis* ; Pneumonia

Objectives:This study was conducted to evaluate the ventilatory function in shipyard welders using the lifetime cumulative exposure to welding fumes. METHODS: Ventilatory function tests were performed on 241 male welders at one large shipyard,exposed to welding fumes for over five years,and was compared to 80 male control subjects. Ventilatory function indices, such as forced vital capacity(FVC), one-second forced expiratory volume(FEV 1), ratio of FEV 1 to FVC(FEV 1%), maximal mid-expiratory flow(MMF), peak expiratory flow rate(PEFR)and maximal expiratory flow at 50 and 75% of expired FVC(FEF50 and FEF75), were obtained by analyzing forced expiratory spirograms and maximal expiratory flow-volume curves that were simultaneously measured by an Impulse ocillometry(IOS)program in the standing position. RESULTS: FEF50 and FEF75 expressed as a percentage of the predicted values were significantly lower in the exposure group than in the controls, and the MMF tended to decrease.The means of all the ventilatory indices,expressed as percentage of the predicted values, were not significantly increased at the current exposure levels. The MMF, FEF50 and FEF75, expressed as a percentage of the predicted values, were significantly decreased with an increase in exposure duration.Only the FEF75 , expressed as a percentage of the predicted value, was significantly decreased with an increase in the cumulativeexposure to welding fumes(C1). The FEF75, expressed as a percentage of the predicted value, was significantly decreased with an increase in the cumulative exposure to welding fumes(C2). The above parameters were calculated to consider the current exposure level, exposure duration, application of the ventilatory system, production rate, personal protection evice, electrode and welding wire. The MMF and FEF50, expressed as a percentage of the predicted value, tended to decrease. The proportion of workers with abnormal MMF, FEF50 and FEF75 were significantly increased with an increase in the cumulative exposure to welding fumes(C2). CONCLUSIONS: These findings suggested that the MMF, FEF50 and FEF75 are sensitive and practical indices for the evaluation of the ventilatory function of workers exposed to welding fumes, when the lifetime cumulative exposure(C2) is considered as a factor affecting exposure.
Electrodes ; Humans ; Male ; Maximal Expiratory Flow-Volume Curves ; Welding*

Embryonal sarcomas of the liver, often called undifferentiated sarcomas or malignant mesenchymomas, are extremely rare tumors that occur chiefly in children with poor prognosis and uncertain histogenesis. Histologically, tumor cells are stellate or spindle shaped, loosely arranged in edematous or myxoid pattern alternating with highly cellular zones and do not show evidence of differentiation. Ultrastructural and immunohistochemical studies by previous investigators indicate that the neoplastic cells are mostly primitive mesenchymal cell which occasionally differentiate to fibroblasts, lipoblasts, histiocytes and smooth muscle cells via intermediate cell types. The authors experienced a case of 6-year old boy who presented with an enlarging abdominal mass of the liver which revealed typical histological and ultrastructural features of embryonal sarcoma with focal areas of unusual chondrosarcomatous differentiation.
Child ; Male ; Female ; Humans

No abstract available.
Animals ; Coal* ; Dust* ; Lung* ; Rats* ; Silicon Dioxide*

No abstract available.
Animals ; Rats*

OBJECTIVES: This study was planned to compare the epidemiological features such as radiological and clinical features between coal worker's pneumoconiosis and manufacturing pneumoconiosis in connection with their age and dust exposure duration. METHODS: For the study, detailed examination records of those workers who had been confirmed to have pneumoconiosis in coal mining and manufacturing industries by the Ministry of Labour during two year period of 1991 and 1992. Total number of study subjects was 895 pneumoconiosis cases; 504 from coal mining and 391 from manufacturing industries. Information variables for the data analysis were sex, age, dust exposure duration, work position as the independent variables and radiological pneumoconiosis category, pulmonary function test results and pulmonary tuberculosis complication status as the dependent study variables. RESULTS: There was a significant difference in distribution of radiological categories of both pneumoconiosis groups. The proportion of suspicious and category 1 pneumoconiosis was higher in manufacturing pneumoconiosis group than in coal mine pneumoconiosis group whereas category 2 and large opacity pneumoconiosis was higher in coal mine pneumoconiosis group than in manufacturing pneumoconiosis group. Major ventilatory indices such as FVC and FEV1 were significantly lower in coal mine pneumoconiosis group than in manufacturing pneumoconiosis group even after other variables such as age and smoking history were statistically adjusted for. CONCLUSIONS: It is suggested that some selected outcome variables such as radiological category of pneumoconiosis, ventilatory impairment, and pulmonary tuberculosis complication rate were significantly different between coal mine pneumoconiosis and manufacturing pneumoconiosis.
Anthracosis ; Coal ; Coal Mining ; Dust ; Korea* ; Mining* ; Pneumoconiosis* ; Respiratory Function Tests ; Smoke ; Smoking ; Statistics as Topic ; Tuberculosis, Pulmonary

PURPOSE: Febrile seizure (FS) is the most common type of seizure in children between 6 months to 5 years of age. A family history of febrile seizures can increase the risk a child will have a FS. Yet, prevalence of FS regarding external environment has not been clearly proved. This study attempts to determine the association between prevalence of FS and weather. METHODS: This study included medical records from the Korea National Health Insurance Review and Assessment Service. Data were collected from 29,240 children, born after 2004, diagnosed with FS who were admitted to one of the hospitals in Seoul, Korea, between January 2009 and December 2013. During the corresponding time period, data from the Korea Meteorological Administration on daily monitoring of four meteorological factors (sea-level pressure, amount of precipitation, humidity and temperature) were collected. The relationships of FS prevalence and each meteorological factor will be designed using Poisson generalized additive model (GAM). Also, the contributory effect of viral infections on FS prevalence and weather will be discussed. RESULTS: The amount of precipitation was divided into two groups for comparison: one with less than 5 mm and the other with equal to or more than 5 mm. As a result of Poisson GAM, higher prevalence of FS showed a correlation with smaller amount of precipitation. Smoothing function was used to classify the relationships between three variables (sea-level pressure, humidity, and temperature) and prevalence of FS. FS prevalence was correlated with lower sea-level pressure and lower humidity. FS prevalence was high in two temperature ranges (-7 to -1℃ and 18–21℃). CONCLUSION: Low sea-level pressure, small amount of precipitation, and low relative air humidity may increase FS prevalence risk.
Child* ; Fever ; Humans ; Humidity ; Korea ; Medical Records ; Meteorological Concepts ; National Health Programs ; Prevalence* ; Seizures ; Seizures, Febrile* ; Seoul ; Weather*