OBJECTIVE: Linkage analysis is a very useful method for prenatal diagnosis of Hemophilia B, especially when a mutation was not identified. Seven polymorphic markers were studied in Korean populations to evaluate the efficiency for prenatal and carrier diagnosis. METHODS: Subjects of this study was 100 healthy Korean women (200 X-chromosomes). Polymerase chain reacton-restriction fragment length polymorphism (PCR-RFLP) method was used to detect SalI, MseI, NruI, DdeI, XmnI, TaqI and HhaI polymorphisms. RESULTS: SalI (-) allele showed the frequency of 0.355 and SalI(+) allele 0.645. MseI(-) allele was 0.645 in frequency and MseI(+) allele was 0.355. SalI and MseI polymorphisms were in complete linkage disequilibrium. And no increase was expected in overall heterozygosity with these two polymorphisms. NruI(-) allele frequency was 0.855 and NruI(+) was 0.145. There was no polymorphism of DdeI, XmnI and TaqI marker systems in Korean population. In HhaI polymorphism, allele frequencies were estimated that HhaI(-) is 0.82 and HhaI(+) is 0.18. CONCLUSION: Only SalI, NruI and HhaI polymorphisms are useful for the diagnosis of hemophilia B in Korean population. Expected heterozygosity for above 3 poylmorphic markers was estimated to be 0.723, and 71 of 100 female subjects were heterozygous for at least one marker system. Korean population showed relatively low extent of polymorphisms compared to Caucasians, Blacks and Japanese. For the effective prenatal diagnosis of hemophilia B with linkage analysis, other polymorphic markers should be evaluated.
African Continental Ancestry Group ; Alleles ; Asian Continental Ancestry Group ; Diagnosis ; Factor IX* ; Female ; Gene Frequency ; Hemophilia B ; Humans ; Linkage Disequilibrium ; Prenatal Diagnosis

Dermatofibroma with granular cells is composed mainly of spindle shaped cells mixed with in-conspicuous foamy cells that contain granular cytoplasm. We present a case of dermatofibroma with granular cells in a 36-year-old male who visited our clinic to evaluate a slow-growing nodule on the medial side of the left lawer leg. He have had the nodule for five years and sometimes felt an itching sensation. Biopsy of the nodule identified a dermal nodular tumor showing cellular spindle cell proliferation admixed with peripheral foamy histiocytes. With the immunohistochemical staining, the granular cell was positive for alpha-1-antitrypsin and CD68, and negative for S-100. A few electron-dense granules were seen in the cytoplasm on the electronmicroscopic study. These results suggested that the granular cells originated from histiocytes.
Adult ; Biopsy ; Cell Proliferation ; Cytoplasm ; Histiocytes ; Histiocytoma, Benign Fibrous* ; Humans ; Leg ; Male ; Pruritus ; Sensation

PURPOSE: The purpose of this study was to investigate the relation between satisfaction in major, career decision-making self-efficacy and career identity of nursing students. METHODS: Data were collected from 543 students by means of self reported questionnaires about satisfaction in major, career decision-making self-efficacy and career identity from November 26 to 30, 2012. RESULTS: The average score of satisfaction in major, career decision-making self-efficacy and career identity of nursing students was 3.56 on a 5 point-scale, 3.28 on a 5 point-scale, and 2.65 on a 4 point-scale respectively. There were significant correlation between satisfaction in major, career decision-making self-efficacy and career identity of nursing students. Career identity could be explained 26.2% by satisfaction in major, career decision-making self-efficacy, admission motivation, and school grade. CONCLUSION: Career identity was correlated with satisfaction in major and career decision-making self-efficacy in nursing students. Therefore, it is necessary to develop an effective intervention program to improve nursing students's satisfaction in major, career decision-making self-efficacy and career identity.
Humans ; Motivation ; Nursing ; Surveys and Questionnaires ; Self Report ; Students, Nursing*

Broncho-gastric fistula caused by benign gastric ulcer perforation after esophagectomy is very rare. In general anesthesia of a patient with broncho-gastric fistula, in spite of hyperventilation, leakage of the anesthetic gases through fistula may make the patient hypercapneic, and positive pressure ventilation may increase the risk of the pulmanary aspiration by the regurgitation of gastric fluid by stomach distension. For that reason, in this patient, denitrogenation was performed during patient's voluntary respiration with 100% oxygen for 5 minutes, and induction was performed without positive pressure ventilation, and one lung ventilation was carried out. Hypoxemia was followed by one lung ventilation because his pulmonary function was moderate obstructive type and his lung was damaged by aspiration of gastric fluid via broncho-gastric fistula. A low level of continuous positive airway pressure (CPAP) has no significant hemodynamic effect and can maintain the patency of nonventilated lung, so hypoxemia induced by one lung ventilation may be reduced. Thus we carried out one lung ventilation with CPAP (10 cmH2O) in nonventilated lung and blocked broncho-gastric fistula with a bronchial blocker for prevention of both regurgitation of gastric fluid and leakage of anesthetic gases. One lung anesthesia was performed without any problem in this case.
Anesthesia ; Anesthesia, General* ; Anesthetics, Inhalation ; Anoxia ; Continuous Positive Airway Pressure ; Esophagectomy ; Fistula* ; Hemodynamics ; Humans ; Hyperventilation ; Lung ; One-Lung Ventilation ; Oxygen ; Positive-Pressure Respiration ; Respiration ; Stomach ; Stomach Ulcer

Spondylometaphyseal dysplasia (SMD) is an extremely rare, which affects the spine and metaphy-ses of the tubular bones on terms of enchondrogenesis. Children who had Kozlowski dwarfism, type of SMD are not recognized until they reach school age since they have normal clinical feature, weight and size in early childhood. Authors experienced a typical case of Kozlowski type of SMD in a 10 years old male who had i) generalized platyspondyly with anterior tapering of vertebrae ii) generalized metaphyseal dysplasia iii) minimal changes in the carpal and tarsal bones. This case is to be reported with review of references.
Child ; Dwarfism ; Humans ; Male ; Spine ; Tarsal Bones

BACKGROUND: More than 90% of acute stroke patients have measurable cardiovascular sequelae, but we have been often overlooked in formal discussions of treatment. If we estimate the incidence of myocardial injury in patient with spontaneous SAH, we may figure the possibility of cardiac dysfunction in such patients. This study was designed to investigate the incidence of myocardial injury in patients with spontaneous SAH using cardiac troponin I(cTnI). METHODS: A prospective single emergency center study was performed to determined preoperative incidence of unrecognized cardiac injury in patients suffering spontaneous SAH. We include the spontaneous SAH patients who underwent serum measurements of the cardic troponin I immediately upon admission last six month period. ECG, CK, CK-MB and myoglobin were also performed at admission. We excluded the spontaneous SAH patients who had past history of myocardial ischemia and ECG abnormality. RESULTS: Fifty-two patients(34 females, 18 males) with spontaneous SAH were studied prospectively. 18 patients(34.6% of the total study population) had cTnI level above 0.5ng/ml. ECG was performed in 52 patients and was abnormal in 15 of the 52 patients(28.8%). CONCLUSION: The measurement of cTnI has provided physicians with a myocardial marker that has a cardiac sensitivity for cardiac injury equal to that of CK-MB yet with greater specificity. So, cardiac troponin I is useful to estimate the incidence of myocardial injury in patients with spontaneous SAH. And we may estimate the possibility of cardiac dysfunction in such patients. This knowledge will hopefully aid in the care and improve the outcome.
Electrocardiography ; Emergencies ; Female ; Humans ; Incidence* ; Myocardial Ischemia ; Myoglobin ; Prospective Studies ; Sensitivity and Specificity ; Stroke ; Troponin I* ; Troponin*

No abstract available.
Endoderm* ; Endodermal Sinus Tumor*

Purpose: This study aimed to assess the feasibility of the Apple AirPods Pro with the headphone accommodation feature as a hearing assistive device for patients with mild to moderate hearing loss (HL). Materials and Methods: The study included a total of 35 participants with mild to moderate HL. To determine the degree of HL in the participants, a screening test using pure-tone audiometry was conducted prior to the main tests of functional gain, word recognition score (WRS), and sentence recognition in noisy environments. The study employed two hearing devices: the Bean (a personal sound amplification product, PSAP) and the AirPods Pro. Results: Regarding functional gain, there were no significant differences between the Bean and the AirPods Pro at all frequencies, except 8 kHz. In terms of WRS, both the Bean and the AirPods Pro had higher scores than the unaided condition. In sentence recognition, both the Bean and the AirPods Pro had higher scores than the unaided condition. During real-ear measurement, the Bean demonstrated consistent frequency responses, while the AirPods had a deviation exceeding 10 dB SPL at 6 kHz in the left ear.This deviation was absent for all other frequencies. Conclusion This study shows that the Apple AirPods Pro, with its headphone accommodation feature, performed similarly to a validated PSAP and improved hearing compared to unaided conditions.

Proliferating trichilemmal tumor is one of rare benign tumors of the skin appendages, considering as hamartoma of the terminal hair follicle, isthmic segment of the outer root sheath. We report a case of numerous proliferating trichilemmal tumors admixed with ordinary trichilemmal cysts with femilial history in 64-year-old man. He has total 157 lesions, which are chiefly located in scalp (48), back (32), and also face, neck, trunk, extremities. Histologically, the tumor is composed of irregularly arranged and ansatomosed trabeculae, lobules, or sheets of proliferated trichilemmal squamous epithelium with peripheral palisading of the basaloid cells. Several layers of squamoid or ovoid cells have PAS-positive clear cytoplasm. And it also shows abrupt amorphous, trichilemmal keratinization. Some squamous eddies with mild cellular atypism are associated, but definite invasion or other evidence of the malignancy is not found. usual multiple trichilemmal cysts are admixed with tumor and occasionally exhibit connection between them.
Cysts ; Hamartoma

BACKGROUND: Video assisted thoracic sympathectomy or sympathicotomy is a safe and effective therapy for the treatment of palmar hyperhidrosis with immediate symptomatic imporvement. However the degree of satisfaction may diminish with time due to cmpensatory sweating or excessive hand dryness. Therefore by comparing and assessing the degree of symptomatic improvement or compensatory sweating following sympathectomy or sympathicotomy at various levels we aim to determine the optimal level of sympathetic nerve block which will result in minimal side effects and maximal benefit. MATERIAL AND METHOD: Among 194 patients having undergone video assisted thoracic sympathectomy or sympathicotomy between January 1996 and June 1999, 137 patients who responded to either telephone interview or questionnaire were included in the current study. The patients were divided into three groups. Group I(n=61) ; patients having undergone T2,3,4 sympathectomy group II(35) ; patients having undergone T2 sympathicotomy and group III(41) ; patients having undergone limited T2 sympathicotomy which consist of block of interganglionic neuronal fiber on the third rib. The parameters studied comprised of pre- and post-operative palmar temperature change treatment satisfaction the degree of compensatory sweating or discomfort from palmar dryness postoperative complication and changes in plantar sweating. RESULT: There was no difference in age and sex among the groups and the mean postoperative elevation in palmar temperature was 21.59degrees C without any differences among the groups. Patients expressing satisfaction were 65.6%, 62.9% and 90.24% in groups I, II and III, respectively(p<0.05) Moderate to severe compensatory sweating was present in 65.6% 51.4%, and 24.39%, in group I, II, and III, respectively (p<0.05) Slight but comfortable amount of palmar humidness was expressed in decreasing order group III(41.6%) group I(24.6%) and group II(5.7%) (p<0.05) Ineffectiveness or recurrence was present in 5patients in group I(8.2%) 1 patient in group II(2.9%) and none in group III. With regards to plantar sweating decrease in sweating was expressed in 43 patients(31.4%) while similar degree of sweating in 61 patients(44.5%) and increase in sweating in another 33 patinets(24.1%). CONCLUSION: Limited T2 sympathicotomy resection of the lower interganglionic neuronal fiber of the second sympathetic ganglion on the third rib showed immediate effect in palmar hyperhidrosis and caused lesser compensatory sweating and hand dryness.
Autonomic Nerve Block ; Ganglia, Sympathetic ; Hand ; Humans ; Hyperhidrosis* ; Interviews as Topic ; Neurons ; Postoperative Complications ; Surveys and Questionnaires ; Recurrence ; Ribs ; Sweat ; Sweating ; Sympathectomy