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

BACKGROUND: Benzoic acid contained in some food can hinder the biological monitoring of hippuric acid in urine, which is frequently used as an exposure index of toluene and xylene. METHOD: 20 male examinees who are not exposed to organic solvents in their jobs were given a bottle of drink containing benzoic acid(70 mg/dl) and took it. Urine samples were taken from every person just before, in 1.5 hrs after, and in 3 hrs after taking the drink. Hippuric acid in urine was analyzed with improved Ogata and Taguchi method and creatinine with Jaffe method. RESULT: Mean hippuric acid concentrations in urine just before, in 1.5 hrs after, and in 3 hrs after taking the drink were 0.59+/-0.21 g/g creatinine, 2.75+/-0.98 g/g creatinine, 1.04+/-0.58 g/g creatinine, respectively. And, each group had statistically significant differences (p<0.01). There were no statistically significant differences between two groups categorized by age, smoking, and drinking. CONCLUSION: From the results, we suggest that when hippuric acid concentration in urine is used as a exposure index of toluene, it should be surveyed whether the food containing benzoic acid was taken or not.
Benzoic Acid* ; Creatinine ; Drinking ; Environmental Monitoring ; Humans ; Male ; Smoke ; Smoking ; Solvents ; Toluene ; Xylenes

The sputum eosinophilia has been observed in various allergic disease of respiratory tract, even non allergic disease, and as well as malignant disease of respiratory tract by several authors in adult patient. This studies were undertaken to evaluate sputum eosinphils in normal and in acute respi ratory tract infection in children. Sputum eosinophils were studied in 10 cases of normal child and in 30 cases of acute respiratory infection. Sputum was liquified by trypsin for 2 hours at 37degrees C in incubator and stained sputum eosinophils with Hinkelman's solution specifically. The stained eosinophils in 1mm3 of sputum were counted by hemocytometer. The results were as follows : 1. Average sputum eosinophil counts in normal child were 120+316 cell/mm3. 2. Average sputum eosinophil counts in acute respiratory tract infection were 10597+/-13207 cell/mm3, which was significantly increased than normal child(p<0.005). 3. Average sptum eosinophil counts in acute bronchitis and bronchiolitis were 10417+/-9295 cell/mm3, 7724+/-6136 cell/mm3 in acute pnemonia, 800 cell/mm3 in bronchial asthma, and 1945+/-2468 cell/mm3 in pulmonary tuberculosis. There were no significant differences of sputum eosinophils between acute respiratory infections. 4. Average sputum eosinophils in acute respiratory infection by different age group were 11178+9632 cell/mm3 below 1 year of age, 8816+/-8933 cell/mm3 in 1~6 years of age, 18798+/-29847 cell/mm3 in 6~10 years of age, and 1400+/-1697 cell/mm3 above 10 years of age. Average sputum eosinophil counts in male patient were 8428+/-9067 cell/mm3, 10597+/-13207 cells/mm3 in female patient. There were no significant differences of sputum eosinophils by age and sex in acute respiratory infection.
Adult ; Asthma ; Bronchiolitis ; Bronchitis ; Child ; Eosinophilia ; Eosinophils* ; Female ; Humans ; Incubators ; Male ; Respiratory System ; Respiratory Tract Infections ; Sputum* ; Trypsin ; Tuberculosis, Pulmonary

A case of intracerebral ependymal(neuroepithelial) cyst of developmental origin is presented. The cyst had no communication with ventricular system or subarachnoid space. The protein content of cyst fluid was higher than that of the CSF. The preoperative, postoperative CT scan and operative findings are presented. The authors reviewed and discussed the previously reported cases. .
Cyst Fluid ; Subarachnoid Space ; Tomography, X-Ray Computed

The authors analysed 74 cases of ruptured aneurysms confirmed by CT, angiography and surgery during the period from September 1982 to August 1984 at Keimyung University Dongsan Hospital for the study of the relationship between the amount of subarachnoid hemorrhage(SAH) detected by CT and the preoperative clinical grade (Hunt & Hess), the later development of cerebral vasospasm, ischemic neurologic deficit(IND) and the postoperative prognosis. We also attempted to investigate the relationship between the low density in the hypothalamus and/or midbrain on CT and the preoperative clinical grade and the angiographic vasospasm. There was no definite difference in age and sex distribution according to the severity of SAH on CT except in predominantly female cases of severe SAH(CT grade III). 75% of the cases with the absence of SAH(CT grade I) were preoperatively clinical grade I or II. However, 76% of the severe SAH cases(CT grade III) showed preopertively clinical grade III, IV or V. In case with no angiographic vasospasm(Ag0), 70% were shown to be clinical grade I or II. But in cases of severe vasospasm seen by angiography(Ag3), 80% were clinical grade III or IV. 17% of the cases that showed an absence of angiographic vasospasm(Ar0) showed a low density in the hypothalamus and/or midbrain on CT as did 48% of the cases with mild angiographic vasospasm(Ag1), 75% with moderate angiographic vasospasm(Ag2) and 100% of the cases with severe angiographic vasospasm(Ag3). It was noticed on the CT in cases of clinical grade I and II, there was a 14% and 24% low density in the hypothalamus and/or midbrain respectively. But in the cases of clinical grade III and IV, it was noticed that there was a 65% and 100% low density in hypothalamus and/or midbrain respectively. Ischemic neurological deficit(IND) developed in 9% of the cases having an absence of SAH(CT grade I) on CT and in 48% of the cases of severe SAH(CT grade III) on CT during the care if the patients. 100% of the cases showed good postoperative results in patients who had an absence of or thin SAH(CT grade I or II) on CT and only 50% of the cases who had severe SAH(CT grade III) obtained good postoperative results. The authors found a correlation between the amount of SAH and the preoperative clinical grade, the development of angiographic cerebral vasospasm, IND and the postoperative prognosis. The low density in the hypothalamus and/or midbrain on CT also has a relationship with the preoperative clinical grade and the development of angiographic vasospsm. We suggest that the severity of SAH on CT and the low density in the hypothalamus and/or midbrain may be of great value in the prediction of cerebral vasospasm and treatment of aneurysmal patients.
Aneurysm* ; Aneurysm, Ruptured ; Angiography ; Female ; Humans ; Hypothalamus ; Intracranial Aneurysm ; Mesencephalon ; Prognosis ; Sex Distribution ; Subarachnoid Hemorrhage* ; Vasospasm, Intracranial

Sixteen cases of intracranial arachnoid cyst were diagnosed with computerized tomography(CT). Four patients also had subdural hematoma, two of them following head trauma. Arachnoid cysts in Sylvian fissure are divided into three types, according to their shape and extension on CT. Male was predominant in sex distribution and Sylvian fissure was prevalent in location. Seizure and focal thinning of bone were the most frequently encountered clinical and radiological findings. Surgical procedure was performed in nine cases with partial excision of the cyst membrane or cystoperitoneal shunt. The authors discussed the pathogenesis, clinical findings and treatment of the intracranial arachnoid cyst. We support the congenital origin as pathogenesis, with the experience of one case, which was diagnosed before delivery with ultrasonogram. Considering the potential morbidity of the cyst, as subdural hematoma formation, we propose the surgical treatment as soon as the diagnosis is made.
Arachnoid Cysts ; Arachnoid* ; Craniocerebral Trauma ; Diagnosis ; Hematoma, Subdural ; Humans ; Male ; Membranes ; Seizures ; Sex Distribution ; Ultrasonography

No abstract available.
Pubic Bone* ; Technetium Tc 99m Medronate*

BACKGROUND: Apoptosis has been implicated in pathogenesis of various disease. Apo-1/Fas (CD95) is one of the main pathway of apoptosis. To examine the possible relationship between Apo-1/Fas (CD95) and primary knee osteoarthritis, MvaI restriction length polymorphism (RFLP) in human Apo-1/Fas (CD95) gene was assessed. METHODS: Genotype and allele frequencies in promoter region in the Apo-1/Fas (CD95) gene were studied by PCR-RFLP in 226 Korean controls and 148 Korean patients with primary knee osteoarthritis. RESULTS: No statistically significant difference in the genotypic distribution and allelic frequencies was found between the control and the knee oateoarthritis patients. But in the severe grade (grade 3, 4) Kellgren-Lawrence score patients, the frequency of MvaI*1 (G) allele was significantly decreased (P=0.0392) and the of MvaI*2 (A) allele frequency was significantly increased (P=0.0473) compared to the normal controls. CONCLUSION: Apo-1/Fas (CD95) gene polymorphism is a part a determinant factor of severity in knee osteoarthritis, the patients with MvaI*2 (A) allele is more severe radiologic progression. Further substantiation studies are needed in larger patient samples and various other apoptosis related genes to elucidate the mechanism of osteoarthritis, including the Fas ligand gene analysis.
Alleles ; Apoptosis ; Fas Ligand Protein ; Gene Frequency ; Genotype ; Humans ; Knee* ; Osteoarthritis ; Osteoarthritis, Knee* ; Promoter Regions, Genetic

No abstract available.
Pneumocystis carinii* ; Pneumocystis* ; Pneumonia ; Pneumonia, Pneumocystis*

PURPOSE: To evaluate the mammographic and ultrasonographic findings of mucocele-like tumors. MATERIALS AND METHODS: Twelve breast lesions from 1994 through 2004, coded as mucocele or mucocele-like tumors, were retrieved from the surgical pathology database files at our institution. Eleven of the patients had undergone mammography, and sonography had been performed in all 12 patients. We retrospectively reviewed the mammographic, sonographic and pathologic findings. RESULTS: The mammographies showed calcifications alone (n=6), calcification with mass or asymmetric density (n=3), and normal mammogram (n=2). The shapes of the calcifications were pleomorphic (n=4, 44.4%), amorphous (n=3, 33.3%) and round (n=2, 22.2%). Sonography was performed in all patients (n=12) and showed cysts (n=8), cystic mass (n=2), tubular hypoechoic structure (n=1) and hypoechoic mass (n=1). Pathologic examination revealed 5 cases of benign mucocele-like tumor that included epithelial hyperplasia without atypia (n=2) and atypical ductal hyperplasia (n=4), and 3 cases of associated intraductal carcinoma. Calcification was more frequently detected in the mucocele-like tumors with atypical ductal hyperplasia or intraductal carcinoma than in the benign tumors. Pleomorphic calcification was only visualized in those cases involving atypical hyperplasia or intraductal carcinoma. Of the 9 cases of calcification seen in the mammograms, 7 cases (77.8%) were detected in the associated sonograms and all were located within the lesion. CONCLUSION: The most common mammographic finding of mucocele-like tumors was segmentally distributed pleomorphic or amorphous calcifications, and the most common sonographic finding was cyst or cystic mass.
Adenocarcinoma, Mucinous ; Breast Neoplasms ; Breast* ; Carcinoma, Intraductal, Noninfiltrating ; Humans ; Hyperplasia ; Mammography ; Mucocele ; Pathology, Surgical ; Retrospective Studies ; Ultrasonography