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

Emphysematous cholecystitis is an uncommon condition which may mimic acute cholecystitis. But it differs from acute cholecystitis in its relatively greater frequency in men and diabetics and has graver prognosis. The condition is diagnosed by demonstration of air in lumen,wall of gallbladder and/or pericholecystic space using a variety of radiographic techniques: simple abdominal radiography, ultrasonography and CT scanning. One illustrative case is presented herein and the pertinent literature is reviewed.
Cholecystitis, Acute ; Emphysematous Cholecystitis* ; Gallbladder ; Humans ; Male ; Prognosis ; Radiography, Abdominal ; Tomography, X-Ray Computed ; Ultrasonography

PURPOSE: Emphysematous pyelonephritis is a rare, life threatening infection of kidney and the pennephric space, characterized by the production of gas within the renal parenchyma. The aim of this study is to analyze the clinical and radiologic characteristics of emphysematous pyelonephritis. MATERIALS AND METHODS: We reviewed 7 cases of the emphysematous pyelonephritis. Six patients had plain abdominal radiographs, ultrasonograms and abdominal CT scans. Only one patient had plain radiograph and ultrasonogram. In 5 operated cases, CT findings were compared with surgical records. RESULTS: Plain radiographs showed characteristic diffuse mottling of gas in renal fossa. On sonogram, intrarenal gas was identified as echogenic loci with dirty shadows. CT scan showed inflammatory mass with gas and fluid levels in adjacent to the kidney. CT findings corresponded relatively wall with the surgical findings in regard to disease extent. CONCLUSION: lntrarenal gas in appropriate clinical setting is highly specific for emphysematous pyelonephritis. CT is the most sensitive method for demonstrating the disease extent as well as specific diagnosis.
Diagnosis ; Humans ; Kidney ; Pyelonephritis* ; Tomography, X-Ray Computed ; Ultrasonography

PURPOSE: Emphysematous pyelonephritis is a rare, life threatening infection of kidney and the pennephric space, characterized by the production of gas within the renal parenchyma. The aim of this study is to analyze the clinical and radiologic characteristics of emphysematous pyelonephritis. MATERIALS AND METHODS: We reviewed 7 cases of the emphysematous pyelonephritis. Six patients had plain abdominal radiographs, ultrasonograms and abdominal CT scans. Only one patient had plain radiograph and ultrasonogram. In 5 operated cases, CT findings were compared with surgical records. RESULTS: Plain radiographs showed characteristic diffuse mottling of gas in renal fossa. On sonogram, intrarenal gas was identified as echogenic loci with dirty shadows. CT scan showed inflammatory mass with gas and fluid levels in adjacent to the kidney. CT findings corresponded relatively wall with the surgical findings in regard to disease extent. CONCLUSION: lntrarenal gas in appropriate clinical setting is highly specific for emphysematous pyelonephritis. CT is the most sensitive method for demonstrating the disease extent as well as specific diagnosis.
Diagnosis ; Humans ; Kidney ; Pyelonephritis* ; Tomography, X-Ray Computed ; Ultrasonography

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

No abstract available.
Child* ; Humans ; Maxillary Sinus* ; Maxillary Sinusitis*

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