An autopsy case of male pseudohermaphrodite associated with Bochdalek diaphragmatic hernia is reported. This newborn infant showed a large clitoris and hypospadic urethra which was the only opening in the perineum except for the anus. There were urethrovaginal fistula and uterus didelphys. The adrenals were slightly larger than normal, but microscopically did not show any features of cortical hyperplasia. Well formed testes were found and were histologically unremarkable. In addition, Bochdalek type of diaphragmatic hernia was present, associated with severe left lung hypoplasia and massively herniated abdominal organs into the chest cavity, shifting the mediastinum considerably and eventually led to death in a few minutes after birth. The pathogenesis of genital anomaly and diaphragmatic hernia is briefly discussed and it is suggested that this type of genital abnormality might be due to defect of testicular M llerian inhibitor factor and can be categorized as incomplete male pseudohermaphroditism.
Infant ; Male ; Female ; Infant, Newborn ; Humans

Oncocytic lipoadenoma is a rare tumor, with only 18 cases having been reported since the first in 1998. We encountered a case of oncocytic lipoadenoma presenting as a slowly growing parotid mass in a 71-year-old man. This tumor is characteristically comprised of a mixture of oncocytes and adipocytes. The present case is one of five reported cases of oncocytic lipoadenoma showing sebaceous differentiation. The results of immunohistochemical study with DOG1 antibody supported the origination of this tumor in the striated duct.
Adipocytes ; Aged ; Humans ; Oxyphil Cells ; Parotid Gland*

No abstract available.
Cholecystectomy, Laparoscopic*

Glycogen storage diseases(GSD) are inherited disorders affecting glycogen metabolism and type I GSD is due to the absence or deficiency of glucose-6-phosphatase(G6Pase) enzyme in the liver, kidney, and intestinal mucosa. The defect leads to inadequate hepatic conversion of G6P to glucose and thus make affected individuals susceptible to fasting hypoglycemia, and the accumulation of glycogen occurs in the liver and other organs. Type Ia is the most common form of GSD and clinically growth retardation may manifest of GSD itself rather than growth hormone deficiency(GHD), but we experienced a case of type I GSD with GHD in a 14-year-o1d male. The height was 125 cm, compatible with 50 th percentile of height of 8 years of age. He has doll-like face with fat cheek, relatively thin extremities, and metabolic acidosis, hyperuricemia, hypoglycemia, hyperlipidemia. GH stimulation test with clonidine and L-dopa revealed that the patient had decreased GH secretion. After laboratory work up including liver biopsy, he was diagnosed as type I GSD. Hypoglycemia was managed with frequent feeding with high starch diet(uncooked cornstarch). Metabolic acidosis and hyperuricemia were treated with sodium bicarbonate, allopurinol and probenecid. The patient is being followed at out-patient clinic with clinical improvement after of diet therapy and GH administration.
Acidosis ; Allopurinol ; Biopsy ; Cheek ; Clonidine ; Diet Therapy ; Extremities ; Glucose ; Glycogen Storage Disease* ; Glycogen* ; Growth Hormone* ; Humans ; Hyperlipidemias ; Hyperuricemia ; Hypoglycemia ; Intestinal Mucosa ; Kidney ; Levodopa ; Liver ; Male ; Metabolism ; Outpatients ; Probenecid ; Sodium Bicarbonate ; Starch

Objectives: The early local vasodilator response to local warming is predominantly dependent on neural reflexes. However, it is suggested that systemic vasomotor activities are unaffected by a local warming at early stage. The purposes of this study were to assess the hypothesis that systemic vasomotor activities might make an adjustment at early stage of hand warming. Materials and Methods: Thirty-nine young volunteers who were healthy were recruited. Each participant received a right hand bathing at 40°C for 10 minutes. Doppler ultrasound technique was used to monitor the brachial artery mean blood velocity (aMBV) at the heated arm, and changes in aMBV were used to evaluate local vasodilator response to hand warming. Photoplethysmographic technique was used to monitor digital volume pulse (DVP) at the unheated finger, and changes in the DVP derived peak amplitude (DVPampl), reflection index (DVPRI), stiffness index (DVPSI), and heart rate (HR) were used to evaluate vascular distensibility of the unheated upper limb, small artery tone in the lower body, large artery stiffness, and cardiac regulation respectively. The data collected in each minute were averaged as an interval. Comparisons of aMBV, DVPRI, DVPSI, and HR at each interval with their respective baseline values were performed using one-way ANOVA. Results: Plots of one-minute intervals versus aMBV, DVPampl, DVPRI, DVPSI, and HR were shown in Figure. aMBV values presented significant increases with a early peak at the third minute. DVPampl presented significant decrease in the first minute and then reversed to a significant increase at the sixth minute. DVPRI presented a significant increase in the first three minutes, and then returned to the level of baseline. DVPSI and HR did not present any significant changes. Conclusion: At the period of early local vasodilator response to hand warming, systemic vasomotor activities did make a significant adjustment by decreasing vascular distensibility of the unheated upper limb and increasing small artery tone of the lower body, though the activities in heart rate and large artery tone were unaffected. Later, vascular distensibility of the unheated upper limb made a reverse adjustment and reached a significant increase.

PURPOSE: The purpose of this study is to assess the effect of Transcatheter Arterial Embolization(TAE) according to anglographic findings in hepatocellular carcinoma. MATERIALS AND METHOD: We retrospectively reviewed 50 cases who received TAE for unresectable hepatocellular carcinoma. We analyzed the anglographic findings which were correlated with the effect of TAE. The common anglographic findings of the hepatocellular carcinoma were tumor staining, neovascularity and enlargement of feeding artery. These anglographic findings were classified into grade 0, +1, +2. Effect of TAE were classified into five patterns;good response, partial response, minimal response, no response and more aggravation. RESULT:In grading of tumor staining, among 50 cases, the grade 0, +1, +2 were seen in 1 case(2%), 14 cases(28%), 35 cases(70%) each. In grading of enlargement of feeding artery, the grade 0, +1, +2 were seen in 7 cases(14%), 19 cases(38%), 24 cases(48%) each. In grading of neovascularity, the grade 0, +1, +2 were seen in 6 cases(12%), 15 cases(30%), 29 cases(58%) each. This study showed that the higher grade of anglographic finding, the better effect of TAE. A statistically significant differance was found (p<0.005). But the TAE was not effective in some cases (the maximum diameter of mass is over 10cm, portal vein thrombosis or ateriovenous shunt) in spite of high grade. CONCLUSION: We believe that these angiogr. aphic findings (tumor staining, enlargement of feeding artery, neovascularity) are one of important indices for anticipating the effect of TAE in patients with unresectable hepatocellular carcinoma.
Arteries ; Carcinoma, Hepatocellular* ; Humans ; Retrospective Studies ; Venous Thrombosis

Objectives: The purposes of this study were to determine whether men and women differ in risk factors for acute myocardial infarction (AMI) during the four seasons of the year. Materials and methods: Medical records of 2,086 (women: 693) patients hospitalized with a confirmed AMI were reviewed retrospectively from the region’s only medical center in eastern Taiwan. The onset date of 544 patients (women: 178) was in spring, 493 patients (women: 165) in summer, 474 patients (women: 155) in autumn, and 575 patients (women: 195) in winter. Risk factors of age, percentage of smoking (smoking%), percentage of diabetes (diabetes%), percentage of hypertension (hypertension%), total cholesterol, and body mass index were assessed. In each season, logistic regression model was used to calculate the odds ratio (OR) and 95% conference interval (CI) of women compared to men by risk factors. Results: In spring, women presented significantly higher values in age (OR 1.022; 95%CI 1.012-1.031 ) and diabetes% (OR2.373；95%CI 1.554-3.625), significantly lower in smoking% (OR 0.187；95%CI 0.145-0.240). In summer, women presented significantly lower values in body mass index (OR 0.907；95%CI 0.856-0.960) and smoking% (OR 0.222; 95%CI 0.134-0.367). In autumn, women presented significantly higher values in age (OR 1.033；95%CI 1.012-1.053) and total cholesterol (OR 1.009；95%CI 1.004-1.013), significantly lower in smoking% (OR 0.168；95%CI 0.098-0.289). In winter, women presented significantly higher values in diabetes% (OR 1.845；95%CI 1.250-2.725), hypertension% (OR1.550；95%CI 1.001-2.402), and total cholesterol (OR 1.008；95%CI 1.004-1.012), significantly lower in smoking% (OR 0.188；95%CI 0.119-0.297). Conclusion: The differences between women and men in risk factors for AMI did present seasonal variation in eastern Taiwan. This finding would provide further insight into medical climatology in preventing serious cardiovascular events.

Objective: The Schizophrenia Cognition Rating Scale (SCoRS) is an interview-based assessment tool for evaluating the cognitive deficit and daily functioning of patients with schizophrenia. Methods: Sixty-eight patients with schizophrenia and 68 age- and sex-matched healthy individuals were recruited to validate the Chinese version of SCoRS in this study. All participants underwent cognitive assessment using the SCoRS, which was verified by the Brief Assessment of Cognition in Schizophrenia (BACS), and the UCSD Performance-based Skills Assessment, Brief Version (UPSA-B). Patients with schizophrenia were additionally assessed using the Positive and Negative Syndrome Scale (PANSS). Results: SCoRS ratings reported by patients (SCoRS-S), those reported by the interviewer (SCoRS-I), and SCoRS global scores (SCoRS-G) showed significant correlation with all subscales of the BACS and the UPSA-B. On receiver operating characteristic curve analysis, SCoRS-S, SCoRS-I, and SCoRS-G significantly differentiated patients with schizophrenia from healthy controls. Moreover, SCoRS-S and SCoRS-I ratings showed positive correlation with the negative symptoms and general symptoms of PANSS. Conclusion The Chinese version of SCoRS showed good discriminant, concurrent, and external validity, suggesting that it is a useful and convenient tool for assessment of cognitive function among Mandarin-speaking patients with schizophrenia in clinical practice.

PURPOSE: Bronchial artery embolization has been effective in the treatment of massive hemoptysis. The purpose of this study was to report the effectiveness of bronchial artery embolization in patients with chronic recurrent hemoptysis intractable to medical treatment. MATERIALS AND METHODS: This study included 25 patients who were admitted for treatment of chronic recurrent hemoptysis with bronchial artery embolization. Chronic recurrent hemoptysis was defined as condition intractable to medical treatment persistently and occuring over two times per two months. The target vessels for embolization were selected in consideration of the results of aortography as well as the finding of chest radiography and bronchoscopy. After selective arteriography for embolization by using 5-French Simmons catheter, embolic agents(mainly polyvinyl alcohol(PVA) and additionally gelfoam and coils) were released through the catheter. The results of the embolization were assessed with the review of medical records. RESULT: The causes of the hemoptysis were pulmonary tuberculosis(n=12,48%), bronchiectasis(n=6,24%), aspergilloma(n=3,12%), chronic obstructive pulmonary disease(n=2,8%), chronic bronchitis(n=I,4%) and lung abscess(n=1,4%). Selective embolization was carried out in 49 sites(42 in bronchial artery and 7 in nonbronchial systemic collaterals). Early success rate within 2 months was 96%. After long-term follow up study (6-30 months, average 15 months), complete remission was 72%, partial remission 12% and recurrence 12% respectively. During and after embolization, major complications such as spinal cord injury or bronchial wall necrosis was not found. Minor complications were chest pain, shoulder pain and chilling sense, which were relieved spontaneously within a few days. CONCLUSION: High success rate and relatively low recurrence with no significant complication were achieved with bronchial artery embolization in the patients complaining of chronic recurrent hemoptysis.
Angiography ; Aortography ; Bronchial Arteries* ; Bronchoscopy ; Catheters ; Chest Pain ; Follow-Up Studies ; Gelatin Sponge, Absorbable ; Hemoptysis* ; Humans ; Lung ; Medical Records ; Necrosis ; Polyvinyls ; Radiography ; Recurrence ; Shoulder Pain ; Spinal Cord Injuries ; Thorax

PURPOSE: Steatocystoma is a cyst that originates embryologically from hair follicles and usually occurs on the face, chest, back and axillae as multiple lesions and rarely occurs as a solitary lesion. We experienced a case of steatocystoma simplex that developed in the orbit and present our case with a brief review of the literature. CASE SUMMARY: A 44-year-old male presented with a history of palpable mass in the right inferomedial periorbital area that had been slowly growing for a few months. Physical examination revealed a non-tender, soft and round mass. Magnetic resonance imaging of the orbit showed 2.0 x 2.0 x 2.0 cm-sized mass in the right inferomedial orbit. We performed excisional biopsy of the orbital mass using transconjunctival approach. The pathological diagnosis was steatocystoma. There was no local recurrence during the postoperative follow-up. CONCLUSIONS: We experienced a rare case of steatocystoma simplex that developed in the orbit. Base on our results, steatocystoma simplex should be considered when diagnosing an orbital mass.
Adult ; Axilla ; Biopsy ; Diagnosis ; Follow-Up Studies ; Hair Follicle ; Humans ; Magnetic Resonance Imaging ; Male ; Orbit* ; Physical Examination ; Recurrence ; Thorax