Menkes disease is an X-linked recessively inherited neurodegenerative disorder of copper metabolism leading to death in early childhood. This disease is characterized by low serum, liver and brain Cu levels, whereas the Cu content in nearly all other organs is increased. The main clinical features are irregular kinky hair, characteristic face, progresssive degeneration of the central nervous system, bone changes, arterial rupture and thrombosis, and instability of temperature. We have experienced a case of Menkes disease in a eight months old male patient, who manifested by seizure, developmental delay, hypotonia, characteristic hair and face along with low levels of serum copper and ceruloplasmin. We report the characteristic findings of cranial MRI and MR angiography in this Menke's patient. In cranial MRI, he showed progressive brain atrophy with subdural hemorrhage and effusion. MR angiography revealed tortuosity of vessels clearly obviating necessity of more invasive conventional arteriography in diagnosis of this disease. This study suggested that cranial MRI and especially MR angiography might be helpful in making early diagnosis of this disease.
Angiography ; Atrophy ; Brain ; Central Nervous System ; Ceruloplasmin ; Copper ; Diagnosis ; Early Diagnosis ; Hair ; Hematoma, Subdural ; Humans ; Liver ; Magnetic Resonance Imaging ; Male ; Menkes Kinky Hair Syndrome ; Metabolism ; Muscle Hypotonia ; Neurodegenerative Diseases ; Rupture ; Seizures ; Thrombosis

Repeat cesarean section is one of the leading causes to make increasing a rate of cesarean section. Trial of labor TOL after cesarean section is attempt to reduce the rate of repeat cesarean section. Trial of labor has been well established as a safe alternative in carefully selected women with transverse scars in the lower uterine segment. This study was based on 104 cases of delivery with prior cesarean birth at the Chonbuk National University Hospital from January, 1991 to June, 1997. The results were as follows: 1) Among 1400 cases with previous cesarean delivery, trial of labor was done in 104 cases (7.4%). Among 104 cases, vaginal birth was successfully completed in 96 cases (92.3%). 2) Among 28 cases with PGE2, vaginal tablet, vaginal delivery was done in 23 cases (82.1%). 3) Among 22 cases with more than 4 of Bishop score, vaginal delivery was done in 21 cases (95.5%), and among 6 cases with less than 3 of Bishop score, vaginal delivery was done only 2 cases (33.3%). 4) There was no matemal death or uterine rupture in the cases of trial of labor, But in 2 cases, uterine dehiscence was observed in 4 cases and postpartal bleeding (more than 400ml) was developed. 5) In the cases of cesarean section, mean of hospital day was 7 days and medical fee was about 400,000 won. In the cases of vaginal birth after cesarean section, mean of hospital day was 2.6 days and medical fee was about 100,000 won. In condusion, vaginal birth after cesarean section is safe and effective alternative to elective repeat cesarean section and also the use of PGE, vaginal tablet is so effective to increase success rate of vaginal delivery. After all, positive these trials might decrease cesarean rate and increase maternal health and quality of medical care.
Cesarean Section ; Cesarean Section, Repeat ; Cicatrix ; Dinoprostone ; Fees, Medical ; Female ; Hemorrhage ; Humans ; Jeollabuk-do ; Maternal Health ; Parturition ; Pregnancy ; Prostaglandins E ; Trial of Labor ; Uterine Rupture ; Vaginal Birth after Cesarean* ; Vaginal Creams, Foams, and Jellies

PURPOSE: This study was a descriptive research to assess the level of and the relationship of related factors in the performance of end-of-life care by intensive care unit (ICU) nurses. METHODS: Participants were 238 ICU nurses from university and general hospitals. Data were collected, using structured questionnaires, and collected data were analyzed using t-test, ANOVA, Scheffé test, Pearson correlation coefficients, and multiple regression analysis with SPSS/WIN 22.0. RESULTS: There were significantly positive effects between performance of end-of-life care and end-of-life care attitudes. Performance end-of-life care was negatively associated with end-of-life care stress and obstacles. Factors that significantly influenced ICU nurses' performance of end-of-life were end-of-life care stress, medical team in obstacles related to end-of-life care, and end-of-life care attitudes, which explained about 53% of the variance in the performance of end-of-life care. CONCLUSION: Findings indicate that hospital organizations should carry out stress management and counseling programs in order to lower ICU nurses' end-of-life care stress, and to enhance end-of-life care attitudes. In addition, concern needs to be given to the medical team which was one of the obstacles to end-of-life care. It is also necessary to decrease the heavy workload and increase the communication with medical workers.
Counseling ; Hospitals, General ; Intensive Care Units ; Terminal Care

Mucin-hypersecreting biliary neoplasm excretes excessive mucin that fills the biliary tree and results in marked dilatation of the bile ducts and obstructive jaundice. In these neoplasm, the mucin produced by the tumor rather than the tumor itself plays an important role in clinical course and radiologic patterns. The purpose of this paper is to report characteristic radiologic patterns of mucin-hypersecreting biliary neoplasms in two cases. These neoplasms were characterized by not only multilocular cystic hepatic mass or extra-hepatic bile duct mass resulting in marked biliary dilatation distal to the mass on US or CT, but also change of shape and extent of amorphous filling defects in the markedly dilated bile duct on serial cholangiograms.
Bile Ducts ; Biliary Tract ; Dilatation ; Jaundice, Obstructive ; Mucins

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*

Heterotopic pregnancy, simultaneous intrauterine and extrauterine gestations, is a relatively rare conditon with an estimated incidence of 1 in 30,000 pregnancies in a natural cycle. But the incidence of heterotopic pregnancies has been increased because of rising incidence of pelvic inflammatory disease, pelvic surgery, intrauterine device, the use of various ovulation induction and assisted reproductive technologies, such as in vitro fertilization and embryo transfer, gamate intrafallopian insemination. The early diagnosis of heterotopic pregnancy is very difficult. So there is a higher maternal morbidity, mortality and fetal loss. Thus careful pelvic examination, combined with serial beta hCG determinations and transvaginal sonography to evaluate the adnexal region are important. We experienced a case of 34-years-old multiparous women with heterotopic pregnancy in natual cycle confirmed by surgical removal of ruptured right tubal pregnancy and sonographic finding of the intrauterine pregnancy, which carried the intrauterine pregnancy to term delivery without complication at 39+5 weeks of gestation, and report this case with a brief review of the literature.
Early Diagnosis ; Embryo Transfer ; Female ; Fertilization in Vitro ; Fertilization* ; Gynecological Examination ; Humans ; Incidence ; Insemination ; Intrauterine Devices ; Mortality ; Ovulation Induction ; Pelvic Inflammatory Disease ; Pregnancy ; Pregnancy, Heterotopic* ; Pregnancy, Tubal ; Reproductive Techniques, Assisted ; Ultrasonography

Hajdu-Cheney syndrome (HCS) is a rare skeletal dysplasia that is characterized by acroosteolysis of the distal phalanges, distinctive craniofacial and skull changes, dental abnormalities and generalized osteoporosis. The clinical and radiologic characteristics are variable and these characteristics progress with age. This syndrome shows autosomal dominant inheritance with sporadic cases. The genetic defects or molecular pathogenesis of HCS are still unknown. We experienced a case of Hajdu-Cheney syndrome in a 20-year-old man who had generalized osteoporosis with multiple non-traumatic spine compression fractures. He had acroosteolysis of the hands and feet, wormian bones in the skull, facial dysmorphism (mid-facial flattening, micrognathia and bushy eyebrows), a high arched palate, malocclusion and short dental alveolar processes. HCS was diagnosed based on the clinical and radiologic evidence. For the differential diagnosis, we excluded the other possible causes of the acroosteolysis and wormian bones, including hyperparathyroidism, osteogenesis imperfecta, hypophosphatemia and mandibuloacral dysplasia. The specific treatment of HCS is unknown, but case reports with bisphosphonate treatment have been reported.
Acro-Osteolysis ; Alveolar Process ; Diagnosis, Differential ; Foot ; Fractures, Compression ; Hajdu-Cheney Syndrome ; Hand ; Humans ; Hyperparathyroidism ; Hypophosphatemia ; Malocclusion ; Osteogenesis Imperfecta ; Osteoporosis ; Palate ; Skull ; Spine ; Wills ; Young Adult

To date, very few cases with intraneural ganglion cyst of the peroneal nerve has been reported. The common symptoms include localized pain and various degrees of motor and sensory deficits. Though electrodiagnostic study has been useful in lesion localization, recent imaging studies, such as ultrasonography and magnetic resonance imaging, should be used in establishing differential diagnosis and extent of a lesion preoperatively. Treatment can be achieved by microsurgical removal of the cyst. We had a 74 year old female with right foot drop for 3 months and we diagnosed that she had intraneural ganglion of the peroneal nerve using physical examination, electrodiagnostic study, ultrasonography, and magnetic resonance imaging. Although there was no specific symptom on the left side, there was a similar lesion like that of right intraneural ganglion, that was detected by ultrasonography. However, the patient's neurologic symptoms have not improved after operation.
Aged ; Diagnosis, Differential ; Electrodiagnosis ; Female ; Foot ; Ganglion Cysts* ; Humans ; Magnetic Resonance Imaging ; Neurologic Manifestations ; Paralysis* ; Peroneal Nerve* ; Physical Examination ; Ultrasonography

PURPOSE: To evaluate the efficacy of CT imaging in differentiating pheochromocytoma from primary adrenalcarcinoma and metastasis. MATERIALS AND METHODS: The authors retrospectively reviewed CT imagings of 24 patients(9 males, 15 females, mean age 49) with 27 surgically or clinically proven adrenal neoplasms larger than 3cm indiameter. These neoplasms included pheochromocytomas(n=11), adrenal carcinomas(n=6), and adrenal metastases(n=10,bilateral : 3). Two radiologists visually inspected CT features with respect to tumor size, shape, intratumoral hypodensity, and calcification, and agreed upon their evalvation. These results were compared with the final histopathologic and clinical diagnosis. RESULTS: Ten of eleven pheochromocytomas(91%) were observed to be round or ovoid; this shape was rarely seen in primary carcinomas(0/6) or metastases(2/10). in contrast, mostcarcinomas(6/6, 100%) were lobulated or irregular in shape, as were most metastases(8/10, 80%). smooth, round orovoid intratumoral hypodensity was noted in all pheochromocytomas. Stellate or linear, irregular intratumoral hypodensity was present in all carcinomas and metastases. However, there were no significant differences in CTdensity of calcification. CONCLUSION: Tumor shape and intratumoral hypodensity are very useful CT findings for differentiating adrenal pheochromocytomas from primary carcinomas and metastases.
Adrenal Gland Neoplasms ; Diagnosis ; Female ; Humans ; Male ; Neoplasm Metastasis* ; Pheochromocytoma* ; Retrospective Studies

This study was conducted to derive core and detailed competencies of dental hygienists to be utilized as educational targets to be reached by graduation as well as basic data that can be reflected in the development and improvement of dental hygiene curricula. This study analyzed publication reports from the Korean Dental Hygienists Association, the International Federation of Dental Hygienists, the Commission on Dental Accreditation, and the American Dental Education Association. Based on the academic classification system for dental hygiene studies, the components of core and detailed competencies of dental hygiene school at the time of graduation were extracted and developed through expert panel discussions. This study defined competencies at the graduation level of dental hygiene school and derived eight core competencies and their 52 detailed competencies to serve as educational objectives from four areas: professionalism, communication, clinical practice, and community and health promotion. In the future, it will be necessary to conduct self-assessments of competencies based on those developed in this study, at time of the graduation from dental hygiene school, as well as to continuously develop competency-based curricula according to entry level, knowledge level, and graduation level. Thus, it is urgently necessary to develop a system that can evaluate the competencies of dental hygienists after graduation and put this system into practical use.
Accreditation ; Classification ; Competency-Based Education ; Curriculum ; Dental Hygienists ; Education, Dental ; Health Promotion ; Humans ; Oral Hygiene* ; Professionalism ; Publications ; Self-Assessment