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

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

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

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

PURPOSE: To evaluate prognosis-related CT findings in hypoxic ischemic encephalopathy. MATERIALS AND METHODS: For the purpose of prognosis, 28 children with a clinical history and CT findings suggestive of hypoxic ischemic encephalopathy (HIE) were restrospectively reviewed. The diagnostic criteria for HIE, as seen on CT scanning, were as follows : 1, ventricular collapse ; 2, effacement of cortical sulci ; 3, prominent enhancement of cortical vessels ; 4, poor differentiation of gray and white matter ; 5, reversal sign ; 6, obliteration of perimesencephalic cistern ; 7, high density on tentorial edge, as seen on precontrast scans ; and 8, low density in thalamus, brain stem and basal ganglia. On the basis of clinical outcome, we divided the patients into three groups, as follows : group I(good prognosis) ; group II(neurologic sequelae), and group III(vegetative state or expire), and among these, compared CT findings. RESULTS: There were thirteen patients in group I, six in group II, and nine in group III. Ventricular collapse, effacement of cortical sulci, and prominent enhancement of cortical vessels were noted in all groups, whereas poor differentiation of gray and white matter, reversal sign, obliteration of perimesencephalic cistern, high density on tentorial edge, on precontrast scan, and low density in brain stem and basal ganglia were observed only in groups II and III. CONCLUSION: CT findings showed distinct differences between groups in whom prognosis was good, and in whom it was poor. An awareness of poor prognostic CT findings may be clinically helpful in the evaluation of patients with hypoxic ischemic encephalopathy.
Basal Ganglia ; Brain Stem ; Child* ; Humans ; Hypoxia-Ischemia, Brain* ; Prognosis* ; Thalamus ; Tomography, X-Ray Computed

Kerion celsi is an inflammatory type of tinea capitis. It is usually seen with zoophilic pathogens, with common examples being Microsporum(M.) canis, Trichophyton(T.) mentagrophytes and T. verrucosum. Hamster is the mammal which belongs to the rodent family, usually small in size and frequently nocturnal in their habits and small pet kept in many countries today. We report a case of kerion celsi caused by M. canis probably transmitted from hamster in a 8-year-old boy. The patient had various sized, erythematous boggy nodules and pustules with irregular hair loss on the scalp for 2 months. Culture from a scalp lesion of patient on Sabouraud's dextrose agar showed M. canis. He was treated with 125 mg of terbinafine daily for 12 weeks and short term therapy of low dose of prednisolone. Skin lesions were cured without recurrence.
Agar ; Animals ; Child ; Cricetinae ; Glucose ; Hair ; Humans ; Mammals ; Microsporum ; Naphthalenes ; Prednisolone ; Recurrence ; Rodentia ; Scalp ; Skin ; Tinea Capitis

PURPOSE: The efficacy of the artificial urinary sphincter(AUS) in treating sphincteric incontinence has been clearly demonstrated. We report on 20 years of experience using artificial sphincter implantation at a single institute. MATERIALS AND METHODS: The follow-up data for 37 patients who received AUS(AMS 800(TM)) implantation between 1987 and 2006 at Yonsei University were available for this study. We investigated various components of the medical records, such as the number of pads used per day, results of pre-operative urodynamic studies, operative and post-operative complications, and revision rate. RESULTS: Mean patient age was 35.6 years(range 15-64 years), and mean follow-up duration was 12.4 years(range 1.4-19.8 years). Of the 37 patients, 21 had neurogenic bladder, and 9 had traumatic injury. Other causes of incontinence included post-operative complications(4 patients) and congenital anomalies(3 patients). The cuffs were placed were in the bladder necks of 21 patients and in the bulbous urethrae of 16 patients. The average number of pads used daily decreased significantly from 6.2 to 1.2 after the operation, and 27 patients(72.9%) were able to maintain 'dry-up status' (number of pads used< or=1). A total of 32 artificial sphincters remained in place(86.4% survival rate), with 8 revisions(21.6%) required secondary to infection, mechanical failure, or urethral stone. CONCLUSIONS: AUS implantation is a safe and durable treatment for urinary incontinence in patients with intrinsic sphincter deficiency from various underlying diseases.
Follow-Up Studies ; Humans ; Medical Records ; Neck ; Urethra ; Urinary Bladder ; Urinary Bladder, Neurogenic ; Urinary Incontinence ; Urinary Sphincter, Artificial ; Urodynamics

No abstract available.
Korea ; Latent Tuberculosis