Apert syndrome is an uncommon, congenital disorder characterised by malformation of the skull, most often acrocephaly or oxycephaly, in association with symmetrical syndactyly of both hands and feet. It is due to disturbance in the growth of bone and soft tissue affecting principally the head, the hands and the feet. The original description was presented by Troquart in 1886, and acrocephaloyndactyly was named by Apert in 1906. Since then, more than 200 cases have been reported in the world upto 1970. Recently, we have experienced three for typical Apert syndrome and made a brief related literature review
Acrocephalosyndactylia* ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; Craniosynostoses ; Foot ; Hand ; Head ; Skull ; Syndactyly

No abstract available.
Diabetic Ketoacidosis* ; Drug Therapy* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Remission Induction*

Kallmann syndrome is characterized by hypogonadotropic hypogonadism resulting from insufficient release of GnRH and associated with anosmia or hyposmia. We experienced two cases of Kallmann syndrome with abnormal brain MRI findings(olfactory bulb aplasia) & secondary sexual dysfunction.
Brain ; Gonadotropin-Releasing Hormone ; Hypogonadism ; Kallmann Syndrome* ; Magnetic Resonance Imaging ; Olfaction Disorders

Kallmann syndrome is characterized by hypogonadotropic hypogonadism resulting from insufficient release of GnRH and associated with anosmia or hyposmia. We experienced two cases of Kallmann syndrome with abnormal brain MRI findings(olfactory bulb aplasia) & secondary sexual dysfunction.
Brain ; Gonadotropin-Releasing Hormone ; Hypogonadism ; Kallmann Syndrome* ; Magnetic Resonance Imaging ; Olfaction Disorders

Verumontanum mucosal gland hyperplasia (VMGH) is a relatively common benign proliferative lesion which was first described by Gagucas et al in 1995. VMGH is usually found in radical prostatectomy or transurethral resection specimens and rarely in needle biopsy specimens. The histologic feature of VMGH is characterized by well-circumscribed proliferation of small glands and thus VMGH may mimic low grade adenocarcinoma. We report a case of VMGH from a 61-year-old man. The lesion coexisted with prostatic adenocarcinoma on radical prostatectomy specimen. The lesion was a well circumscribed microacinar proliferation which was present between the openings of ejaculatory ducts. The acini consisted of two cell layers with inner secretory cuboidal epithelium and outer basal cell. Typically, the lumen contained many corpora amylacea. Nuclear pleomorphism, prominent nucleolus, or mitotic figure was not identified. Because of small gland proliferation of VMGH, this lesion can be confused with other small gland proliferative lesions, such as low grade adenocarcinoma, atypical adenomatous hyperplasia, basal cell hyperplasia, mesonephric hyperplasia, and nephrogenic adenoma. To avoid misdiagnosis of VMGH as carcinoma, one should be familiar with this lesion.
Adenocarcinoma ; Adenoma ; Biopsy, Needle ; Diagnostic Errors ; Ejaculatory Ducts ; Epithelium ; Humans ; Hyperplasia* ; Male ; Middle Aged ; Prostate ; Prostatectomy

On 6 healthy adult males aged 20-30 years, oxygen pulse was observed during carrying sand bags weighting 10, 20 and 30kg on a level treadmill running with speeds of 3,4 and 5 km/hr. The results obtained were as follows. 1. Oxygen pulse during load carrying on a level treadmill was proportionately increased with an increase of work load, average oxygen uptake per minute. Regression equation of oxygen pulse (y: ml/beat) on the average oxygen uptake (x:l/min) required for each grade of work was expressed as y=3.34x + 5.99, sy.x=0.858. 2. Oxygen pulse reached the highest value 2-5 minutes after the start of load carrying, and thereafter it decreased gradually to some extend as the load carrying was continued. Rate of decrease in the oxygen pulse was gerater in lighter works. 3. In lighter works requiring less than 2.0 l/min of oxygen uptake, oxygen pulse was larger in case of carrying a losd by embrace, in both hands and on head than on back or on shoulder.
Adult ; Hand ; Head ; Humans ; Male ; Oxygen* ; Running ; Shoulder ; Silicon Dioxide

On 6 healthy adult males aged 20-30 years, oxygen pulse was observed during carrying sand bags weighting 10, 20 and 30kg on a level treadmill running with speeds of 3,4 and 5 km/hr. The results obtained were as follows. 1. Oxygen pulse during load carrying on a level treadmill was proportionately increased with an increase of work load, average oxygen uptake per minute. Regression equation of oxygen pulse (y: ml/beat) on the average oxygen uptake (x:l/min) required for each grade of work was expressed as y=3.34x + 5.99, sy.x=0.858. 2. Oxygen pulse reached the highest value 2-5 minutes after the start of load carrying, and thereafter it decreased gradually to some extend as the load carrying was continued. Rate of decrease in the oxygen pulse was gerater in lighter works. 3. In lighter works requiring less than 2.0 l/min of oxygen uptake, oxygen pulse was larger in case of carrying a losd by embrace, in both hands and on head than on back or on shoulder.
Adult ; Hand ; Head ; Humans ; Male ; Oxygen* ; Running ; Shoulder ; Silicon Dioxide

No abstract available.
*Carcinoma, Neuroendocrine/diagnosis/pathology ; Female ; Human ; *Liver Neoplasms/diagnosis/pathology ; Middle Aged

PURPOSE: Forward pulmonary blood flow may be absent in some neonates with Ebstein's anomaly by anatomical or functional pulmonary atresia in association with the elevated pulmonary vascular resistance, patent ductus arteriosus and tricuspid regurgitation. We reviewed the presentation and outcomes of symptomatic neonates with Ebstein's anomaly focusing on the pulmonary atresia. METHODS: Clinical presentation and outcome of 15 symptomatic neonates with Ebstein's anomaly seen at Asan medical center from 1998 to 2004 were reviewed. RESULTS: Ten (67%) of 15 patients showed no forward pulmonary blood flow and 6 of them had functional pulmonary atresia. O2 saturation and pH were lower and cardiothoracic (CT) ratio in chest radiography was more increased in the patients with pulmonary atresia than in the patients without pulmonary atresia (P< 0.05). pH and CT ratio were not different between the anatomical and functional pulmonary atresia group, but O2 saturation was lower in functional atresia group (P< 0.05). 13 patients (87%) were managed with PGE1. 4 of 6 patients with functional pulmonary atresia were treated with inhaled nitric oxide. Surgery was performed in 1 of 5 patients without pulmonary atresia and in 8 of 10 patients with pulmonary atresia during follow-up period (mean 37 months). 3 patients (20%) died and none of patients without pulmonary atresia died. CONCLUSION: We found that most symptomatic neonates with Ebstein's anomaly had functional or anatomical pulmonary atresia. The neonates with Ebstein's anomaly who had no forward pulmonary blood flow were more symptomatic and needed surgery earlier. Further studies will be needed to distinguish effectively functional and anatomical pulmonary atresia and to manage appropriately neonates with functional atresia.
Infant, Newborn ; Humans

PURPOSE: The purpose of this study was to describe role conflict and job satisfaction of Neurosurgery Clinical Nurse Specialist(NCNS) and to identify associated factors. METHOD: The target populations was 77 NCNSs from 30 general hospitals. Data were collected with self-administered questionnaires and analyzed using t-test and ANOVA. RESULTS: The overall mean scores of role conflict and job satisfaction were 3.60(+/-0.54) and 3.04(+/-0.46) respectively. Significant variables affecting role conflict were the working period as NCNS, the number of colleague NCNSs in working hospital, and assignment of prescription rights. The significant variable affecting job satisfaction was assignment of prescription rights. There was no statistically significant correlation between job and role conflict. CONCLUSION: The variable effecting both role conflict and job satisfaction was the extent of prescription rights. In order to improve the quality of patient care and to protect Neurosurgery Nurse Clinical Specialist, the enactment of law on prescription practice is needed.
Health Services Needs and Demand ; Hospitals, General ; Human Rights ; Humans ; Job Satisfaction* ; Jurisprudence ; Neurosurgery* ; Nurse Clinicians* ; Patient Care ; Prescriptions ; Specialization ; Surveys and Questionnaires