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

No abstract available.
Pulmonary Artery* ; Takayasu Arteritis*

The purpose of this study is to evaluate the efficacy of the balloon bronchoplasty in the treatment of the tuberculous bronchostenosis. Balloon bronchoplasty was performed in thirteen patients with stenosis of the left main bronchus (two with combined left upper and lower lobar bronchostenosis) using a inflatable balloon catheter under a fluoroscopic guide. We analysed the changes in the changes in the severity of dyspnea and wheezing, serial FEV1/FVC as a parameter of the airflow obstruction, and bronchial diameter and lung volume on chest radiographs. The extent of pulmonary tuberculosis was correlated with the improvement of FEV1/FVC. There was an improvement of dyspnea in 69%(9/13), decrease of wheezing in 69%(9/13), significant increase of FEV1/FVC in 18% (2/11). The increase of the bronchial diameter and lung volume were seen in 84%(11/13) and 53% (7/13), respectively. The significant increase of FEVI/FVC was seen in 28% (2/7) of the patients with lung involvement of tuberculosis less than one third of left upper lobe, whereas there was no increase in those of more than one third. There was no complication except transient leukocytosis, fever and blood-tinged sputum. In conclusion, balloon bronchoplasty is effective in the treatment of medically intractable tuberculous bronchostenosis, and can be considered as an initial method of treatment.
Bronchi ; Catheters ; Constriction, Pathologic ; Dyspnea ; Fever ; Humans ; Leukocytosis ; Lung ; Methods ; Radiography, Thoracic ; Respiratory Sounds ; Sputum ; Tuberculosis ; Tuberculosis, Pulmonary

The purpose of this study is to evaluate the efficacy of the balloon bronchoplasty in the treatment of the tuberculous bronchostenosis. Balloon bronchoplasty was performed in thirteen patients with stenosis of the left main bronchus (two with combined left upper and lower lobar bronchostenosis) using a inflatable balloon catheter under a fluoroscopic guide. We analysed the changes in the changes in the severity of dyspnea and wheezing, serial FEV1/FVC as a parameter of the airflow obstruction, and bronchial diameter and lung volume on chest radiographs. The extent of pulmonary tuberculosis was correlated with the improvement of FEV1/FVC. There was an improvement of dyspnea in 69%(9/13), decrease of wheezing in 69%(9/13), significant increase of FEV1/FVC in 18% (2/11). The increase of the bronchial diameter and lung volume were seen in 84%(11/13) and 53% (7/13), respectively. The significant increase of FEVI/FVC was seen in 28% (2/7) of the patients with lung involvement of tuberculosis less than one third of left upper lobe, whereas there was no increase in those of more than one third. There was no complication except transient leukocytosis, fever and blood-tinged sputum. In conclusion, balloon bronchoplasty is effective in the treatment of medically intractable tuberculous bronchostenosis, and can be considered as an initial method of treatment.
Bronchi ; Catheters ; Constriction, Pathologic ; Dyspnea ; Fever ; Humans ; Leukocytosis ; Lung ; Methods ; Radiography, Thoracic ; Respiratory Sounds ; Sputum ; Tuberculosis ; Tuberculosis, Pulmonary

Increased death rate resulted from cardiovascular disease called for the study on the autonomic nervous system and cardiovascular system. It is known that an HRV (heart rate variability) spectrum analysis based on ECG (electrocardiograph) signals could be used to define activity of sympathetic and parasympathetic nervous system noninvasively. However, it is important to prove whether suggested HRV analysis method could provide the useful information for observing autonomic nervous system quantitatively and objectively before clinical application. In this study, 14 rats were used and divided into two groups, sympathectomy group and vagotomy group, respectively. During the experiment, ECGs of rats were collected three times at each experimental condition. After the application of Berger's series algorithm to the ECG raw data, HRV spectrum was obtained via FFT (fast Fourier transform). Power contents for each frequency bands were calculated from HRV waveforms. Two peak values, HF (high frequency) and LF(low frequency), representing autonomic nervous system status were used to extract the parameter, HF/LF ratio. Results showed that HF/LF values were increased for the sympathectomy group and decreased for the vagotomy group. It implies that the variations in HF/LF components exhibits the information for the classification of ANS(autonomic nervous system) function quantitatively. HRV analysis algorithm developed from this study could be expanded for the observation of autonomic nervous system variations in human.
Animals ; Autonomic Nervous System ; Cardiovascular Diseases ; Cardiovascular System ; Classification ; Electrocardiography ; Humans ; Mortality ; Parasympathetic Nervous System ; Rats ; Spectrum Analysis ; Sympathectomy* ; Vagotomy*

"Capsaicin, the pungent principle of hot peppers, is a neurotoxin that depletes primary sensory neurons of neuropeptides like tachykinin. The objectives of these experiment was to examine the effects of capsaicin on Salmonel/a typhimurium-induced production of cytokines such as TNF-a, IL-1B, IL-6, IL-10 and IL-12 and on production of nitric oxide in peritoneal macrophages. In addition, the effects of capsaicin on survival rates of S. typhimurium-infected mice and on nuclear transcription factor (NF-kB) activation were also investigated. Mice were pretreated with a single s.c. injection of 100 ug of capsaicin and were infected i.v. with S. typhimurium (5xO5/mouse) in 0.2 ml volume after capsaicin pretreatment. The serum cytokine levels were measured 30, 60, 120, 180 and 240 min after Salmonella infection, using ELISA kits. The activation of NF-B was also examined by gel shift assay in spleens, thymuses and brains of mice that had been pretreated with a single s.c. injection of 100 ug of capsaicin. It was found that Sa/mone/la infection induced the production of TNF-a, IL-1B, IL-6, IL-10 and IL-12, but capsaicin pretreatment inhibited the production of TNF-a, IL-1B, IL-10 and IL-12, but enhanced IL-6 production 120 min after Salmonella infection. Interestingly, the capsaicin pretreatment inhibited the activation of NF-kB in spleens and thymuses. There were no differences in the numbers of bacteria in livers, brains, spleens, kidneys and lungs between capsaicin- pretreated mice and the control animals in applied experimental conditions. Suprisingly, however, capsaicin pretreatment increased both the survival rates of Sa/mone//a-infected mice and production of nitric oxide by peritoneal macrophages compared with capsaicin-untreated control mice. Taken together, these results indicate that the capsaicin-sensitive primary sensory neurons may play an important modulatory role in the production of cytokine, nitric oxide and NF-B activation and the pathogenesis of salmonellosis."
Animals ; Bacteria ; Brain ; Capsaicin* ; Cytokines* ; Enzyme-Linked Immunosorbent Assay ; Interleukin-10 ; Interleukin-12 ; Interleukin-6 ; Kidney ; Liver ; Lung ; Macrophages, Peritoneal ; Mice ; Neuropeptides ; NF-kappa B* ; Nitric Oxide* ; Salmonella Infections* ; Salmonella typhimurium ; Salmonella* ; Sensory Receptor Cells ; Spleen ; Survival Rate ; Tachykinins ; Thymus Gland ; Transcription Factors

PURPOSE: To analyze CT and follow-up chest radiographic findings in patients with necrotizing pneumonia and to evaluate clinical significance of the extent of necrosis. MATERIALS AND METHODS: We reviewed medical records and retrospectively analysed CT scans and follow-up chest radiographs of 22 patients with necrotizing pneumonia, confirmed by biopsy(n=7) and culture (n=15). Inclusion criteria for necrotizing pneumonia was necrotic low attenuation, with or without cavitation on postcontrast enhanced CT scan. The study group included 15 men and seven women, aged 11-66 years (average: 47years). RESULTS: The pathogens of necrotizing pneumonia were Klebsiella spp(n=7), Enterobacter spp(n=5), Actinomyces spp(n=4), Pseudomonas spp(n=4), Nocardia spp(n=4), and others(n=5). Average duration of pneumonia was 4.1 months. On CT scan, pneumonic consolidations were well-marginated in 14 patients and there were cavities on initial CT scan in 16 cases. Margins of the necrotic portion on CT scan were well-demarcated in majority of the patients(16/22). Low attenuation areas on initial CT scan resulted in cavitation, fibrosis and volume loss as shown on follow-up chest radiographs. The larger the necrotic areas on CT, the more the volume loss was. CONCLUSION: CT findings of necrotizing pneumonia were well-marginated air-space consolidation with low attenuation area, with or without cavity. The extent of necrotic area was closely related with the degree of fibrotic change later on. CT is important tool for diagnosis and prediction of parenchymal damage in necrotizing pneumonia.
Actinomyces ; Diagnosis ; Enterobacter ; Female ; Fibrosis ; Follow-Up Studies ; Humans ; Klebsiella ; Male ; Medical Records ; Necrosis ; Nocardia ; Pneumonia* ; Pseudomonas ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed

PURPOSE: To analyze CT and follow-up chest radiographic findings in patients with necrotizing pneumonia and to evaluate clinical significance of the extent of necrosis. MATERIALS AND METHODS: We reviewed medical records and retrospectively analysed CT scans and follow-up chest radiographs of 22 patients with necrotizing pneumonia, confirmed by biopsy(n=7) and culture (n=15). Inclusion criteria for necrotizing pneumonia was necrotic low attenuation, with or without cavitation on postcontrast enhanced CT scan. The study group included 15 men and seven women, aged 11-66 years (average: 47years). RESULTS: The pathogens of necrotizing pneumonia were Klebsiella spp(n=7), Enterobacter spp(n=5), Actinomyces spp(n=4), Pseudomonas spp(n=4), Nocardia spp(n=4), and others(n=5). Average duration of pneumonia was 4.1 months. On CT scan, pneumonic consolidations were well-marginated in 14 patients and there were cavities on initial CT scan in 16 cases. Margins of the necrotic portion on CT scan were well-demarcated in majority of the patients(16/22). Low attenuation areas on initial CT scan resulted in cavitation, fibrosis and volume loss as shown on follow-up chest radiographs. The larger the necrotic areas on CT, the more the volume loss was. CONCLUSION: CT findings of necrotizing pneumonia were well-marginated air-space consolidation with low attenuation area, with or without cavity. The extent of necrotic area was closely related with the degree of fibrotic change later on. CT is important tool for diagnosis and prediction of parenchymal damage in necrotizing pneumonia.
Actinomyces ; Diagnosis ; Enterobacter ; Female ; Fibrosis ; Follow-Up Studies ; Humans ; Klebsiella ; Male ; Medical Records ; Necrosis ; Nocardia ; Pneumonia* ; Pseudomonas ; Radiography, Thoracic ; Retrospective Studies ; Tomography, X-Ray Computed

Epilepsy can be divided into idiopathic epilepsy and symptomatic epilepsy according to the existence ofunderlying organic disease. It has been said that the incidence of the symptomatic epilepsy caused by underlyingorgainic brain disease is higher in late-onset epilepsy after the age of 20 than in childhood-onset epoilepsy. CTis very sensitive and non-invasive method for detection of organic brain disease. 168 cases of late-onset epilepsyafter the age of 20 were studied by CT in recent 2 years were analyzed. The results were as follows; 1. The 3rddecade was the most frequent age group, and the ratio of male to female was 2.5;1. 2. Structural abnormality onbrain CT was demonstrated in 51.8% of hte patient. 3. The older the onset of age was, the higher the rate of theabnormal CT finding, except 5th decade which showed less CT abnormality than 4th decade. 4. The most frequenthistory related to epilepsy was trauma. 63.1% of the patients had no relevant history; and they showed CT findingsof brain tumor, atrophy and infarction in decreasing order of frequency. 5. Abnormal CT finding was demonstratedin 49.2% of normal neurologic examination and in 46.4% of normal EEG study. 6. The most frequent lesion ofabnormal CT scan in late-onset epilepsy was 30 cases(18.4% )of brain atrophy. The next frequent lesion was 18cases (10.7%) of brain tumor. Infarction, parasites and calcification were other frequnet lesions.
Atrophy ; Brain ; Brain Diseases ; Brain Neoplasms ; Electroencephalography ; Epilepsy ; Female ; Humans ; Incidence ; Infarction ; Male ; Methods ; Neurologic Examination ; Parasites ; Tomography, X-Ray Computed

Over a period of recent two years, 100 patients who had localized pulmonary lesions and underwent percutaneousneedle aspiration and biopsy, were analized. There were 56 malignancies and 44 benign and 79% respectively.Differentiation of malignancy vs. benignity was possible in 89% of cases obviating unnecessary exploratorythoracotomy for diagnostic purpose, Five cases were misinterpreted and eight cases were non-diagnostic oncytology. Inadequate materail was obtained in two cases; one was due to hardness of the mass, which, later,confirmed as chondrosarcoma, and the other was too samll(0.8x1.0cm) to be visible on lateral view. Theobainenability of the tissue was 98%. 14(14%) patients developed pneumothorax; one of them required treatment andthe reminder showed spontaneous resporption. (Transient neglibigle blood tinged sputum was found in 16(16%)cases.) The method, problems and complications are discussed. Authors recommend the percutaneous needle aspirationand biopsy as the initial procedure in diagnostic work-up of pulmonary coin lesions, especially when they aresmaller, more peripheral and metastatic neoplasm is neoplasm is suspected.
Biopsy ; Biopsy, Needle ; Chondrosarcoma ; Hardness ; Humans ; Methods ; Needles ; Pneumothorax ; Solitary Pulmonary Nodule ; Sputum ; Tolnaftate