Purpose: To report a case of multiple retinal capillary hemangioma associated with neurofibromatosis type 1 and resulting neovascularglaucoma.Case summary: A 13-year-old boy was admitted with complaint of visual disturbance and dull pain in his left eye which had beguntwo weeks prior. Lisch nodules were observed in his left iris and corneal opacity with neovascularization of the iris and anglewere detected in the same eye. Multiple retinal capillary hemangiomas with increased tortuosity and congestion of feeding retinalvessels were observed on the upper equator of the left retina; in addition, vitreous hemorrhage was observed. There were noother abnormalities except iris mammillations in the right eye. Numerous café au lait macules were observed on the patient’s entirebody. He also exhibited axillary freckling. On brain magnetic resonance imaging, T2-weighted signal intensity was increasedin the basal ganglia, left thalamus, and cerebellar white matter; however, no vessel abnormalities were observed on magneticresonance angiography. High intraocular pressure (IOP) persisted despite the use of IOP lowering agents and IOP was normalizedafter trabeculectomy with mitomycin C administration. Conclusions This case shows that multiple retinal capillary hemangiomas can be accompanied by neurofibromatosis type 1,which may result in neovascular glaucoma.

We observed clinical features of 18 bronchopulmonary dysplasia (BPD) patients who were admitted to the neonatal intensive care unit in the Severance Hospital of Yonsel University College of Medicine from January 1. 1987 to June 30. 1991. Eight patients in whom ventilator settings were unchanged for more than 5 days because of lack of improvement in pulmonary function. The effects of dexamethasone in ventilator-dependent were included in the short-term dexamethasone therapy. 1) Eighteen BPD patients consisted of 15 premature infants (83%), 1 full-term infant, and 2 post-term infants, The mean gestational age of the patients were 30 weeks and the mean birth weight was 1,420gm. And there were 13 male and 5 female infants. 2) The underlying conditions which necessitated ventilatory support support were hyaline membrane disease in 13 patients (72%), apnea in 2 (11%), and meconium aspiration syndrome in 3. There patients with meconium aspiration syndrome were either full-term or post-term infants, of whom 2 had neonatal persistent pulmonary hypertension. 3) The mean age at the start of ventilator care was 8 hours and the mean PIP was 32 cm H2O. The mean duration of ventilator care of oxygen therapy and of high O2 requirement (FiO2>0.8) were 39 days, 75 days and 20 days, respectively. 4) Patent ductus arteriosus developed in 8 patients during mechanical ventilation, but they were all closed with the use of mefenamic acid, There were also 4 cases of pneumothorax, 2 cases of pulmonary parenchymal emphysema, and 1 case each of pneumomediastinum and pneumoperitoneum. 5) The mean gestational age of the dexamethasone-treated group was 30 weeks and the mean birth weight was 1,320gm, The mean age at which dexamethasone therapy was started was 39 days after birth, and in only 3.3 days 6 patients were successfully weaned from ventilator, In 5 cases the first trial of dexamethasone therapy was enough but the rest needed the 2nd trial for ventilator weaning. 6) In 6 BPD patient who were weaned from the ventilator after dexamethasone therapy, there were significant decreases in MAP (11.1 vs 8.0 cmH2O), and FiO2 (0.73 vs 0.61), but a significant increase in the urine output (2.56 vs 3.7ml/kg/hr) for the pretreatment (5 days prior to therapy) versus posttreatment period (first day of therapy). 7) The complications of dexamethasone treatment were transient hypertension and hyperglycemia in 3 patients, and systemic candidiasis and gastrointestinal bleeding in 2 patients who failed to be weaned after dexamethasone therapy. Our results suggest that the short-term dexamethasone therapy in bronchopulmonary dysplasia patients who are dependent on mechanical ventilation enables weaning in a short period of time. The inspiratory oxygen concentration and the mean airway pressure may be decreased and the urine output may be increased from the first day of medication, improves pulmonary function and decreases pulmonary interstitial edema.
Apnea ; Birth Weight ; Bronchopulmonary Dysplasia* ; Candidiasis ; Dexamethasone* ; Ductus Arteriosus, Patent ; Edema ; Emphysema ; Female ; Gestational Age ; Hemorrhage ; Humans ; Hyaline Membrane Disease ; Hyperglycemia ; Hypertension ; Hypertension, Pulmonary ; Infant ; Infant, Newborn ; Infant, Premature ; Intensive Care, Neonatal ; Male ; Meconium Aspiration Syndrome ; Mediastinal Emphysema ; Mefenamic Acid ; Oxygen ; Parturition ; Pneumoperitoneum ; Pneumothorax ; Respiration, Artificial ; Ventilator Weaning ; Ventilators, Mechanical ; Weaning

To elucidate some DNA adducts as a biological marker for workers of chromate pigment, the effects of chromium exposure on the formation of 8-hydroxydeoxyguanosine(8-OH-dG) and sister chromatid exchanges(SCEs) frequency in 38 workers of a pigment plant in Bucheon which utilized lead chromates, were examined. The chromium contents of venous blood and urine were measured as working environmental exposure level. The concentrations of 8-OH-dG in DNA isolated from lymphocytes were determined with high performance liquid chromatography and electrochemical detector and denoted as a molar ratio of 8-OH-dG to deoxyguanosine(dG). The SCEs frequency were analyzed in DNA isolated from lymphocytes. A significant correlation was found between creatinine adjusted urine chromium concentration and the molar ratio of 8-OH-dG to dG(r=0.47, p<0.01). After adjusting the current smoking habit, the correlation coefficient was increased(r=0.62, p<0.05). However, there was no significant correlation between the SCE frequency and chromium exposure. This significant results between molar ratio of 8-OH-dG to dG and chromium exposure are in good agreement with in vitro studies that support the importance of DNA adduct formation for the carcinogenic effect of chromium.
Biomarkers ; Chromates ; Chromatids ; Chromatography, Liquid ; Chromium* ; Creatinine ; DNA ; DNA Adducts ; Environmental Exposure ; Gyeonggi-do ; Humans ; Lymphocytes ; Molar ; Plants ; Siblings* ; Sister Chromatid Exchange* ; Smoke ; Smoking

BACKGROUND: It has been well known that absolute and interpeak latencies of brainstem auditory evoked potentials (BAEP) are usually prolonged in diabetics. However, Its etiology is still controversial. We tried to identify whether the cause is structural or metabolic in origin by performing BAEP and brain MRI in the diabetic patients. METHODS: BAEP were performed in both the diabetic patients (DM) group (16 males and 15 females) and the normal control group (25 males and 33 female). A brain MRI was performed in the DM group on those who showed abnormal BAEP and com-pared the results of BAEP of the DM group with those of the control group. RESULTS: 7 patients (22.6%) showed abnor-mal BAEP (male; 6, female; 1, unilateral; 4, bilateral; 3) when abnormal BAEP was defined as being larger than two and a half standard deviations of the control group BAEP results. Two males of the DM group who showed abnormal structural lesions of the pons in their brain MRI were not included in the statistical analysis. The remaining 14 diabetic male patients (mean age: 58.7 +/-9.1 years, mean disease duration: 6.1 +/-4.7 years, mean hemoglobin (Hb) A1C: 7.7 +/- 2.0%) and 15 diabetic female patients (mean age: 60.6 +/-10.8 years, mean disease duration: 5.4 +/-5.1 years, mean HgA1C: 7.8 +/-2.1%) were stastistically analyzed. Interpeak latencies of I-III, III-V, and I-V were found to be signifi-cantly prolonged in the DM group. The prolongation of interpeak latencies of I-III and I-V were found to be signifi-cantly correlated with the disease duration only in the diabetic female patients, but not with age and HbA1c. CONCLUSIONS: These findings suggest that both metabolic derangement and structural lesion contribute to prolonging the central conduction time on BAEP pathway in diabetics.
Brain ; Brain Stem* ; Diabetes Mellitus ; Evoked Potentials, Auditory, Brain Stem* ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Pons

A case of Wegener's granulomatosis is described, with special attention focused on the typical histologic findings and involvement of both middle ear and lung. The patient is a 37-year-old man presented with four-month history of cough and sputum. He had a past history of surgery of both ears because of otitis media followed by left facial palsy. Chest radiographs showed variable sized ill defined nodules in both lower lobes with internal airspace consolidation. Histologic preparations of the open lung biopsy specimens demonstrated a diffusely scattered palisading micro and macrogranulomas with central focus of neutrophils and necrotic collagen surrounded by histiocytes, histiocytic giant cells. Fibrinoid necrosis involved blood vessels and lung parenchyma. Chronic inflammation, diffuse granulation tissue formation and irregular fibrosis are also found in the lung parenchyma. The histologic findings of middle ear which was previously biopsied showed scattered palisading ill defined microgranulomas mixed with fibrotic tissue.
Adult ; Biopsy ; Blood Vessels ; Collagen ; Cough ; Ear ; Ear, Middle* ; Facial Paralysis ; Fibrosis ; Giant Cells ; Granulation Tissue ; Granuloma ; Histiocytes ; Humans ; Inflammation ; Lung* ; Necrosis ; Neutrophils ; Otitis Media ; Radiography, Thoracic ; Sputum ; Vasculitis ; Wegener Granulomatosis*

A 26-year-old man with a one-year history of asthma and sinusitis presented with bilateral pleural effusions, patch basilar infiltrates on a chest x-ray and a pericardial effusion on an echocardiogram. The peripheral blood showed marked eosinophilia. An obstructive pattern was also observed during the pulmonary fuction test, which was responsive to bronchodilator inhalation. Nerve conduction studies showed right sural neuropathy. Thoracentesis yielded an acidotic exudative effusion with low glucose, low C3 and eosinophilia. An open lung biopsy revealed an eosinophilic interstitial pneumonitis associated with a necrotizing eosinophilic vasculitis, and granulomatous inflammation foci. In the literature, pleural effusions were reported in 29 percent of Churg-Strauss patients, but the number of effusions was low and their characteristics have not been well described. This report describes the characteristic findings of pleural fluid and its histologic features in a case of classical Churg-Strauss syndrome.
Adult ; Asthma ; Biopsy ; Churg-Strauss Syndrome* ; Eosinophilia ; Eosinophils ; Glucose ; Humans ; Inflammation ; Inhalation ; Lung ; Lung Diseases, Interstitial ; Neural Conduction ; Pericardial Effusion ; Pleural Effusion* ; Sinusitis ; Thorax ; Vasculitis

Auditory agnoia is defined as a disability to recognize spoken languages and/or nonverbal environmental sounds and music despite adequate hearing while spontaneous speech, reading and writing are preserved. Usually, either bilateral or unilateral temporal lobe especially transverse gyral lesions are responsible for auditory agnosia. Subcortical lesions without cortical damage else rarely causes auditory agnosia. We pesent a 65-year-old right-handed male with generalized auditory agnosia caused by bilateral subcorcal lesions. After the two attacks of stroke, he could not understand and recognize spoken words and meet of non verbal sounds. Repetition and dictation were impossible but spontaneous speech was fluent and comprehensible even though mild paraphasic error was noted. Naming was normal. He could understand and read aloud the written words and phrases. Clinical examination of the ears was normal as were audiometry and brainstem auditory evoked potential. Brain magnetic resonance images showed a large cavitary lesion due to an old intracerebral hematoma in right subcortical temporoparietal lobe with preserved superior temporal gyrus and a slit like subcortical lesion in the left insula.
Aged ; Agnosia* ; Audiometry ; Brain ; Ear ; Evoked Potentials, Auditory, Brain Stem ; Hearing ; Hematoma ; Humans ; Male ; Music ; Stroke ; Temporal Lobe ; Writing

No abstract available.
Bone Density* ; Estrogens* ; Female ; Humans

BACKGROUND: Osteoporosis is developed by progressive decrease of bone rnass from decreased collagen content of bone. Accurate measurement of bone collagen is necessary for the diagnosis of osteoporosis and it is possible by bone biopsy, however bone biopsy is not easy in clinical practice. Skin collagen is consist with type I collagen which is same type of bone collagen and progressive decrease of bone collagen is reflected by decrease of skin collagen. Since skin thickness reflect skin collagen amount, skin thickness measurement may be a useful method for the evaluation of osteoporosis. So ultrasonic skin thickness measurement was developed for the evaluation of osteoporosis. METHODS: A randomly selected 200 women aged fram 30 to 71 years old were asked to have their skin thickness measured as well as lumbar vertebral DXA(Norland, USA) bone densitometry. Except for the two women who failed to complete the study, 45(22.7%) of these women were diagnosed as normal, 74(37.4%) were osteopenic and 79(39.9%) were diagnosed as osteoporosis patients using the WHO criteria. Skin thickness was measured using 20MHz Osteoson DCIII (Minhorst, Germany) at the medial side of the upper arm. A minimal of Two scans were measured and the mean value was cakulated automatically. RESULTS: The correlation coefficient of skin thickness and age was -0.121(ns), DXA BMD(bone mineral density) and age was -0.420(P<0.01), skin thickness and DXA BMD L2-L4 was 0.181(P<0.05). Skin thickness was significantly correlated with body weight(correlation coefficient 0.254, P<0.01) and BMI(correlation coefficient 0.195, P<0.01). Furthermore, the mean and standard deviation of skin thickness in normal BMD group was 0.94+-0.021mm, osteopenic group was 0.92+-0.006mm, and osteoporotic group was 0.89+-0.018mm. There was statistically significant difference in the mean values of skin thickness between the three groups even adjusted with age and BMI(P<0.05). The mean and standard deviation of skin tbickness of healthy 20-40 year old women was 1.11+-0,023mm and their mean and standard deviation of L2-L4 mean BMD was 1.17+-0.145mg/cm2. The diagnostic predictability of skin thickness less than 1mm as the risk of osteoporosis(BMD T score less than -1.0) was evaluated. The sensitivity and the specificity of skin thickness less than 1mm being osteoporotic were 78,2% and 57.8% respectively. The positive and negative predictive value of the skin thickness less than 1mm being osteoporotic were 82.2% and 36.5% respectively. CONCLUSION: This study indicate that the skin thickaess measured with the ultrasound method show good correlatian with the bone density measured with conventional DXA at the lumbar vertebra and the skin thickness less than 1mm on the medial side of the opper arm is relatively sensitive in diagnosing osteoporosis risk in Korean women. The authors suggested that a large randomized control study to define the relationship between the skin thickness and the other determinants of bone turnover in the near future.
Aged ; Arm ; Biopsy ; Bone Density ; Collagen ; Collagen Type I ; Densitometry ; Diagnosis* ; Female ; Humans ; Osteoporosis ; Osteoporosis, Postmenopausal* ; Sensitivity and Specificity ; Skin* ; Spine ; Ultrasonics ; Ultrasonography

No abstract available.
Posterior Cruciate Ligament* ; Tendons*