No abstract available.
Hemoptysis*

Supravalvular aortic stenosis may be defined as an obstructive congenital deformity of the ascending aorta which originates just distal to the level of the origins of the coronary arteries. It may be localized or diffuse, and includes a wide spectrum of pathologic changes. A 16 years old school boy was admitted because of exertional dyspnea for 6 years. Clinical diagnosis of supravalvular and valvular aortic stenosis with bicuspid aortic valves and myocardial hypertrophy was made by echocardiography and angiocardiography. Surgical correction was performed successfully. We presented a case of supravalvular and valvular aortic stenosis with a review of literatures.
Adolescent ; Angiocardiography ; Aorta ; Aortic Stenosis, Supravalvular ; Aortic Valve ; Aortic Valve Stenosis* ; Bicuspid ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Dyspnea ; Echocardiography ; Humans ; Hypertrophy ; Male

Deliberately induced hypotension reduces bleeding in operative fields, therby facilitating the surgical manipulation of a highly vascularized lesion and enabling a better dissection to be made. Hypocapnia is a technique by which the regional cerebral blood flow is reduced, effecting a decrease in the intracranial volume. The monitoring of end-tidal CO2 tension(PECO2) is widely done since the amount of end-tidal CO2 tension reflects indirectly the value of the degree of arterial CO2 tension(PaCO2). During hypotension, increased physiologic dead space my produce the widened PaCO2-PECO2 gradient and this large gradient makes PECO2 an unreliable indication of PaCO2. There are many reports on hypotensive agents and techniques. Induced hypotension with halothane has been reported to be a relatively safe and useful method by Murtagh(1960) and Schettini, et al (1967). We reported 100 cases of halothane induce hypotensive anesthesia for intracranial aneurysm surgery in 1979. The present study reports concerning the hypotensive anesthesia for 259 cases of intracranial aneurysm surgery, which were performed at Severance Hospital of the Yonsei University College of Medicine from 1972 to 1985. We evaluated prospectively the PaCO2-PECO2 gradient with modern infrared capnographs during the induced hypotension of 25 cases, which was performed for intracranial aneurysm surgery at this hospital. The result of the retrospective and prostpective studies were as follows. A. Retrospective study 1) Halothane and enflurane were used as the primary anesthetics in 246 and 13 cases, respectively. Hydralasine, nitroprusside, and trimetaphan were supplementarily used for inducing hypotension in 29, 19 and 15 cases, respectively. 2) The mean arterial blood pressure of the lowest blood pressure in the induced hypotension group was 57.2+/-9.3 mmHg, and the mean arterial blood pressure of the highest blood pressures during the induction fo anesthesia was 111.3+/-20.8mmHg. 3) There was no significant difference in the perioperative hemoglobins, hematocrits, and serum electrolytes. 4) In the introperative period gas analysis revealed respiratory alkalosis(arterial CO2 tension and pH were 29.7+/-6.7mmHg and 7.485+/-0.078, respectively). In other values there was no significant change. 5) The mortality rate in 259 cases of intracranial aneurysm surgery was 6.2%. B. Prospective study There was no significant difference in the PaCO2-PECO2 gradients between in the hypotensive period (5.5+/-3.8 mmHg) and in the normotensiveperiod(4.3+/-3.4mmHg). In conclusion, the technique of using induced hypotension with the inhalation anesthetics, halothane or enflurane, is a safe and useful one to use in performing surgery for intracranial aneurysm, and end-tidal carbon dioxide tension can be used as an indirect measure of arterial carbon dioxide during the induced hypotension.
Anesthesia* ; Anesthetics ; Anesthetics, Inhalation ; Arterial Pressure ; Blood Pressure ; Carbon Dioxide ; Electrolytes ; Enflurane ; Halothane ; Hematocrit ; Hemorrhage ; Hydrogen-Ion Concentration ; Hypocapnia ; Hypotension ; Intracranial Aneurysm* ; Mortality ; Nitroprusside ; Prospective Studies ; Retrospective Studies ; Trimethaphan

No abstract available.
Malignant Atrophic Papulosis*

Some sexual differentiation disorders are associated with gonadal neoplasia and increased incidence of testicular tumors has been discribed in the patients with, XY gonadal dysgenesis. The incidence of testicular tumors in infants and children are rare, representing only 1% of all pediatric solid tumors. In general, gonadal stromal tumors are one of the most characteristic endocrine tumors of the testis, endocrine activity occurs in at least 10-20%, among them Leydig cell tumors and Sertoli cell tumors are clinically important. Although the exact pathogenesis is unknown, endocrine activity due to estrogen secretion can be manifested clinically with gynecomastia or precocious puberty. We experienced and reported a child who visited for sexual precocity and had XY gonadal dysgenesis with Sertoli cell tumor.
Child ; Disorders of Sex Development ; Estrogens ; Gonadal Dysgenesis ; Gonads ; Gynecomastia ; Humans ; Incidence ; Infant ; Leydig Cell Tumor ; Male ; Puberty, Precocious ; Sertoli Cell Tumor* ; Testicular Neoplasms ; Testis

BACKGROUND: The "timing principle" utilises a single bolus of nondepolarizing neuromuscular blocking drug followed by an induction drug at the onset of clinical weakness. The purpose of this study was to compare the intubating conditions after succinylcholine or rocuronium and after rocuronium using the timing principle. METHODS: Forty patients were randomly allocated into four groups. Patients in group I received rocuronium 0.6 mg/kg using the timing principle. At the onset of clinical weakness, anesthesia was induced with the thiopental 4-5 mg/kg. Patients in group II, III, and IV received rocuronium 0.6, 0.9 mg/kg, and succinylcholine 1.5 mg/kg respectively using the usual technique. The trachea were intubated 60 s after thiopental induction. Accelerographic response to train-of-four (TOF) stimulation of the ulnar nerve was used for neuromuscular monitoring. Intubating conditions were assessed according to a grading scale. RESULTS: The twitch depression immediately before tracheal intubation in group I, II, III, and IV were 14.5, 28.2, 11.1, and 6.8%, respectively. The TOF count showed no significant differences between groups. The duration of action in group III (45.3 +/- 12.1 min) was significantly prolonged compared to that in group I (31.2 +/- 6.4 min). Intubation conditions were either good or exellent in all patients except one in group II. In group I, three patients recalled shortness of breath or general weakness. CONCLUSIONS: It is concluded that the use of rocuronium 2 X ED95 using the timing principle did not provide additional benefits compared to rocuronium 3 X ED95 using the usual technique except in duration.
Anesthesia ; Depression ; Dyspnea ; Humans ; Intubation* ; Neuromuscular Blockade ; Neuromuscular Monitoring ; Succinylcholine ; Thiopental ; Trachea ; Ulnar Nerve

A case of unilateral multicystic renal dysplasia was reported in view of rarity, and a review of literature 23-year-old female was admitted to Kyung Hee University Hospital with a complaint of urinary incontinence. An excretory urogram revealed non-visualization of the right kidney. The right ureter had opening at the 2 cm above the vaginal introitus. The resected right kidney revelaed multilobulated outer surface with multiloculated cystic struture on cut surface. The associated anomaly was uterine didelphy. The condition was treated by nephroureterectomy.
Female ; Humans

A case of unilateral multicystic renal dysplasia was reported in view of rarity, and a review of literature 23-year-old female was admitted to Kyung Hee University Hospital with a complaint of urinary incontinence. An excretory urogram revealed non-visualization of the right kidney. The right ureter had opening at the 2 cm above the vaginal introitus. The resected right kidney revelaed multilobulated outer surface with multiloculated cystic struture on cut surface. The associated anomaly was uterine didelphy. The condition was treated by nephroureterectomy.
Female ; Humans

The magnetic resonance images (MRI) of 30 patients with surgically or biochemically confirmed pituitary adenomas (20 macroadenomas, 10 microadenomas) were retrospectively evaluated. Ten patients had hyperprolactinemia, another eight had acromegaly, another eight had nonfunctioning adenoma and four had Cashing disease. The examination were performed at a 1.5-T superconducting MR system using a multisection spin-echo technique with 3mm-thick sections and a 256×224 matrix. TI weighted sagittal and coronal images were obtained before and within 30 minutes after the administration of Gd-DTPA (0.1mmol/kg). Analysis of the MRI was focused on the signal intensity and enhamcement patterns of the pituitary adenoma before and after Gd-DTPA administration. Compared with endocrinologic diagnosis, macroadenoma showed heterogeneous enhacement in 55%, rim enhancement in 35% and homogeneous enhancement in 10%, Conclusively, the enhancement patterns of the pituitary adenoma did not correlate with the subtypes made according to hormone production.
Acromegaly ; Adenoma ; Diagnosis ; Gadolinium DTPA ; Humans ; Hyperprolactinemia ; Magnetic Resonance Imaging* ; Pituitary Neoplasms* ; Retrospective Studies

PURPOSE: We analyzed disease free survival and the prognostic factors of liposarcoma in the extremity. MATERIALS AND METHODS: Between 1994 and 2005, of 44 patients who were diagnosed and treated for liposarcoma of the extremity, 40 patients were restrospectively analysed. 13 out of 40 patients got postoperative radiotherapy. We examined local recurrence, distant metastasis and disease free 5-year survival rate. We also analyzed clinical prognostic factors, such as age, gender, size of tumor, prior unplanned excision, histologic type, surgical excision margin and postoperative radiotherapy respectively. RESULTS: There were 3 cases of local recurrence and 4 cases of distant metastasis. The disease free 5-year survival rate was 85.0%. 26 patients presented with myxoid, 8 well differentiated, 4 round cell, 1 pleomorphic and 1 dedifferentiated histology. The disease free 5-year survival rate of mixoid, well differentiated and round cell liposarcoma were 100.0%, 84.6% and 75.0% (p=0.419). The 5-year disease free survival rate was 90.6% in negative surgical margin (n=25) and 62.5% in positive surgical margin (n=15) (p=0.003). CONCLUSION: Our study suggests that surgical excision margin is significant prognostic factor for 5-year disease free survival rate.
Disease-Free Survival ; Extremities ; Humans ; Liposarcoma ; Neoplasm Metastasis ; Recurrence ; Survival Rate