Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

BACKGROUND: Bronchoscopy is an essential procedure for identifying the bleeding site and evaluating cause of hemoptysis. However, it is controversial regarding to the timing of bronchoscopy in patients with hemoptysis. Early bronchoscopy, which was performed during hemoptysis or with 48hour after cessation of bleeding, was better for identifying the site of bleeding compared with delayed bronchoscopy, which was performed 48 hours after cessation of bleeding. The diagnostic yield of identifying the bleeding site by bronchoscopy was variable in reported literature and the safety of early bronchoscopy was not mentioned in previous literature. Therefore, we evaluated the efficacy and safety of early bronchoscopy in patients with hemoptysis. METHOD: From October 1994 to August 1996 in Samsung Medical Center, bronchoscopy was performed in patients with hemoptysis. Early bronchoscopy was performed prospectively during hemoptysis or within 48 hours after cessation of bleeding from May 1995 to August 1996. Delayed bronchoscopy group included patients who did not recieved early bronchoscopy at the same period or in whom bronchoscopy was performed 48 hour after cessation of bleeding from October 1994 to May 1995. RESULTS: Early bronchoscopy group was performed 73 times in 71 patients. Delayed bronchoscopy was performed in 57 times in 55 patients. There was no difference as to amount and underlying cause of hemoptysis between both groups. Indentification of bleeding site by visualizing active bleeding was significantly higher in early bronchoscopy(38.3%) than delayed bronchoscopy group (8.7%) (p<0.05). Indentification of bleeding site by bleeding after clot removal was 8 in early and 10 in delayed bronchoscopy. Indentification of bleeding site by visualizing active bleeding and bleeding after clot removal was 36 in early and 15 patients in delayed bronchoscopy(p>0.05). Causes of hemoptysis was found in 18 patients in early and 16 patients in delayed bronchoscopy group. patients who had early bronchoscopy underwent surgery. We diagnosed the site of bleeding in 4 patients preoperatively. In 3 patients we made a treatment plan promptly right after bronchoscopy. Among early bronchoscopy group, bleeding over 100cc during bronchoscopy occurred in 2 patients. In early bronchoscopy group there was no other major complication during bronchoscopy. CONCLUSION: In patients with hemoptysis, early bronchoscopy which performed within 48 hours after cessation of bleeding was more effective procedure for indentifying the bleeding site than delayed bronchoscopy which was performed after 48 hour cessation of bleeding.
Bronchoscopy* ; Hemoptysis* ; Hemorrhage ; Humans ; Prospective Studies

OBJECTIVES: Although bronchoscopy is an important diagnostic tool for lung disease, patients compliance is low due to discomfort. Recently, midazolam which has a favorable anterograde amnesia effect and short action duration, has been used to relieve patients discomfort during bronchoscopy. Midazolam was investigated in order to see the beneficial effect and safety during bronchoscopy. METHODS: The study design was single blind, randomized, prospective. 102 patients were included, in whom bronchoscopy was performed between June, 19% and October, 1995 at Samsung Medical Center. They were categorized into midazolam group and control group. Patients were asked about the amnesic effect, discomfort of procedure and the willingness to repeat procedure. The consciousness level of patients during procedure, patient cooperation during procedure and ease of procedure were also reported by bronchoscopists. RESULTS: 1) The difference of oxygen saturation between two groups: There was no significant difference in oxygen saturation between midazolam group and control group before and after bronchoscopy. During procedure, however, mean oxygen saturations in midazolam group (90+/-6.4%) was significantly lower than in control group (93+/-4.7%)(p<0.05). 2) Evaluations by patients (1) Effect of amnesia: 41 patients (82%) in midazolam group could not recall the procedure but 52 patients (100%) recalled the entire procedure in control group. A favorable amnesic effects could be found in midazolam group(p<0.05). {2) The discomfort during the procedure: 43 patents(86%) did not experience discomfort from procedure in midazolam group but 25 patients(48%) complained of discomfort in control group (p<0.05). (3) Most patients except two(96%) were willing to repeat fiberoptic bronchoscopy in midazolam group but 13 patients (25%) answered that they would never repeat bronchoscapy. There was a statistically significant difference between two groups in the willingness to repeat bronchocopy (p<0.05). 3) The evaluations by bronchoscopists Cooperations of the patients and ease of procedure were not different between two groups. The patients in midazolam group except eight could not respond to verbal stimuli but most patients were awakened during procedure in control group(p<0.05). CONCLUSION: Midazolam is a good sedative agent for a patient to give a favorable amnesia, reduction of discomfort during bronchoscopy. We concluded that midazolam is a safe and useful sedative agent and midazolam may be used routinely during bronchoscopy. Monitoring of oxygen saturation, however, is essential to prevent severe hypoxia during procedure.
Amnesia ; Amnesia, Anterograde ; Anoxia ; Bronchoscopy* ; Compliance ; Consciousness ; Humans ; Lung Diseases ; Midazolam* ; Oxygen ; Patient Compliance ; Prospective Studies*

No abstract available.
Gastrointestinal Tract* ; Radionuclide Imaging*

BACKGROUND: The objective responses of cisplatin and etoposide (PVP) combination chemotherapy as second-line therapy following CAV was high (40~50%) and, in several reports, PVP yields survival results that are at least as good as those obtained with cyclophosphamide or doxorubicin-based regimens and with less host-related toxicity in chemotherapy-naive patients. We conducted a phase II study to evaluate the effect of a combination of cisplatin and etoposide as a first-line therapy in patients with small cell lung cancer. METHODS: Sixty-one previously untreated small cell lung cancer patients with measurable lesion(s) received cisplatin(30 mg/m2 IV, day 1~3) and etoposide(100 mg/m2 IV, day 1~3). In patients with limited disease, after completion of 6 cycles of PVP chemotherapy, chest and prophylatic brain irradiation was performed in case of complete responder, chest irradiation only in partial responder. RESULTS: 1) Of 55 evaluable patients, 13(24%) had a complete response and 29(53%) had a partial response. 2) The median survival time was 55.8 weeks for all patients(N=55), 61.1 weeks for limited disease(N=31), 51.3 weeks for extensive disease(N=24). 3) The response duration was 29.1 weeks for responders(N=42). 4) There was no significant prognostic factors iufluencing response rates. 5) The toxicity was tolerable and there was no treatment-related deaths. CONCLUSION: The PVP combination chemotherapy as a first-line therapy was effective and well-tolerated in patients with small cell lung cancer.
Brain ; Cisplatin* ; Cyclophosphamide ; Drug Therapy ; Drug Therapy, Combination* ; Etoposide* ; Humans ; Small Cell Lung Carcinoma* ; Thorax

Exclusively dopamine producing retroperitoneal paragangliomas are extremely rare. We have experienced the first Korean case managed successfully based on the proper evaluation. A 26-year-old female patient came to our attention after the accidental detection of an adrenal mass. She had no symptoms and denied any family history. Laboratory evaluations were normal but serum dopamine (425 ng/L) and 24-hour urine dopamine levels (1,565.3 microg/day) were elevated. She underwent laparoscopic right adrenalectomy. Histopathological diagnosis was a paraganglioma. After operation, dopamine levels in serum and 24-hour urine dropped to 0.09 ng/L and 388.4 microg/day. Dopamine producing paraganglioma elicit no clinical symptoms. Only the dopamine level is elevated in serum and 24-hour urine samples. Surgical resection without using preoperative alpha blockage is the treatment of choice. The prognosis for patients with this tumor tends to be poor because the diagnosis is usually delayed due to lack of symptoms.
Adrenal Glands ; Adrenalectomy ; Adrenergic alpha-Antagonists ; Adult ; Dopamine ; Female ; Humans ; Korea ; Paraganglioma ; Pheochromocytoma ; Porphyrins ; Prognosis

Acute eosinophilic pneumonia(AEP) is one of uncommon causes of fatal respiratory failure. Since first reported in 1979, AEP is considered as different disease entity from chronic eosinophilic pneumonia. Recently, consensus is evolving in regard to diagnostic criteria. We experienced a 21-year old male patient presenting typical clinical and radiological characteristics of AEP. Bronchoalveolar lavage showed eosinophilia and transbronchial lung biopsy revealed infiltration of eosinophils and lymphocytes in interstitium and alveoli. This patient completely recovered within three weeks without corticosteroid. We report this case with brief review of literature.
Biopsy ; Bronchoalveolar Lavage ; Consensus ; Eosinophilia ; Eosinophils* ; Humans ; Lung ; Lymphocytes ; Male ; Pulmonary Eosinophilia* ; Respiratory Insufficiency ; Young Adult

A 73-year-old man who had undergone a right pneumonectomy and open window thoracostomy due to tuberculous empyema, presented with purulent discharge from the previous operation site. The computed tomography of the chest showed diffuse pleural thickening and a low attenuated lesion, with air bubbles in a dependent portion of the right hemithorax. These air bubbles were revealed to be due to 7 pieces of retained surgical gauze by flexible bronchoscopy. The patient showed marked clinical improvement with diminished purulent discharge after removal of the foreign bodies.
Aged ; Bronchoscopy ; Empyema, Tuberculous ; Foreign Bodies ; Humans ; Pneumonectomy ; Thoracostomy* ; Thorax

BACKGROUND: Bronchioloalveolar carcinoma (BAC) has been reported to diveres spectrum of chinical presentations and radiologic patterns. The three representative radiologic patterns are followings; 1) a solitary nodule or mass, 2) a localized consolidation, and 3) multicentric or diffuse disease. While, the localized consolidation and solitary nodular patterns has favorable prognosis, the multicentric of diffuse pattern has worse prognosis regardless of treatment BAC presenting as a solitary pulmonary nodule is often misdiagnosed as other benign disease such as tuberculoma. Therefore it is very important to make proper diagnosis of BAC with solitary nodular pattern, since this pattern of BAC is usually curable with a surgical resection. METHODS: We reviewed the clinical and radiologic features of patients with pathologically-proven BAC with solitary nodular pattern from January 1995 to September 1996 at Samsung Medical Center. RESULTS: Total 11 patients were identified. 6 were men and 5 were women. Age ranged from 37 to 69. Median age was 60. Most patients with BAC with solitary nodular pattern were asymptomatic and were detected by incidental radiologic abnormality. The chest radiograph showed poorly defined opacity or nodule and computed tomography showed consolidation, ground glass appearance, internal bubble-like lucencies, air bronchogram, open bronchus sign, spiculated margin or pleural tag in most patients. The initial diagnosis on chest X-ray were pulmonary tuberculosis in 4 patients, benign nodule in 2 patients and malignant nodule in 5 patients. The FDG-positron emission tomogram was performed in eight patients. The FDG-PET revealed suggestive findings of malignancy in only 3 patients. The pathologic diagnosis was obtained by transbronchial lung biopsy in 1 patient, by CT guided percutaneous needle aspiration in 2 patients, and by lung biopsy via video-assited thoracocopy in 8 patients. Lobectomy was performed in all patients and postoperative pathologic staging were T1N0M0 in 8 patients and T2N0M0 in 3 patients. CONCLUSION: Patients of BAC presenting with solitary nodular pattern were most often asymptomatic and incidentally detected by radiologic abnormality. The chest X-ray showed poorly defined nodule or opacity and these findings were often regarded as benign lesion. If poorly nodule or opacity does not disappear on follow up chest X-ray, computed tomography should be performed. If consolidation, ground glass appearance, open bronchus sign, air bronchogram, internal bubble like lucency, pleural tag or spiculated margin are found on computed tomography, further diagnostic procedures, including open thoracotomy, should be performed to exclude the possiblity of BAC with solitary nodular pattern.
Adenocarcinoma, Bronchiolo-Alveolar* ; Biopsy ; Bronchi ; Diagnosis ; Female ; Follow-Up Studies ; Glass ; Humans ; Lung ; Male ; Needles ; Prognosis ; Radiography, Thoracic ; Solitary Pulmonary Nodule* ; Thoracotomy ; Thorax ; Tuberculoma ; Tuberculosis, Pulmonary

Intrauterine devices (IUDs) are highly effective, long-term methods of contraception. Although evidences of direct association between IUD use and pelvic inflammatory disease are rare, the frequency of inflammatory complications associated with the use of IUDs ranges from 2% to 8%. Gynecological surgeries on the account of purulent, inflammatory disease associated with IUD are 4-7%. We report one case with spontaneous perforation of uterus due to acute gangrenous myometritis in an old woman with IUD for 40 yrs in pelvic cavity, followed by a review of the literature.
Contraception ; Female ; Gynecologic Surgical Procedures ; Humans ; Intrauterine Devices ; Pelvic Inflammatory Disease ; Uterine Perforation* ; Uterus