Marfan's syndrome is a widespread disturbance of mesodermal tissue throughout the body. The main disturbances are characteristic skeletal anomaly, cardiopathy and eye disturbance such as lens dislocation, glaucoma, etc. Marfan originally described it under the name of dolichostenomelie in 1896. Since then, many cases have been reported in the literature. Authors experienced 3 cases of Marfan's syndrome and 2 cases were operated. The result of surgical treatment. other clinical findings, and brief review of literature are reported as following.
Glaucoma ; Lens Subluxation ; Marfan Syndrome* ; Mesoderm

BACKGROUND: Osteoporosis is developed by progressive decrease of bone rnass from decreased collagen content of bone. Accurate measurement of bone collagen is necessary for the diagnosis of osteoporosis and it is possible by bone biopsy, however bone biopsy is not easy in clinical practice. Skin collagen is consist with type I collagen which is same type of bone collagen and progressive decrease of bone collagen is reflected by decrease of skin collagen. Since skin thickness reflect skin collagen amount, skin thickness measurement may be a useful method for the evaluation of osteoporosis. So ultrasonic skin thickness measurement was developed for the evaluation of osteoporosis. METHODS: A randomly selected 200 women aged fram 30 to 71 years old were asked to have their skin thickness measured as well as lumbar vertebral DXA(Norland, USA) bone densitometry. Except for the two women who failed to complete the study, 45(22.7%) of these women were diagnosed as normal, 74(37.4%) were osteopenic and 79(39.9%) were diagnosed as osteoporosis patients using the WHO criteria. Skin thickness was measured using 20MHz Osteoson DCIII (Minhorst, Germany) at the medial side of the upper arm. A minimal of Two scans were measured and the mean value was cakulated automatically. RESULTS: The correlation coefficient of skin thickness and age was -0.121(ns), DXA BMD(bone mineral density) and age was -0.420(P<0.01), skin thickness and DXA BMD L2-L4 was 0.181(P<0.05). Skin thickness was significantly correlated with body weight(correlation coefficient 0.254, P<0.01) and BMI(correlation coefficient 0.195, P<0.01). Furthermore, the mean and standard deviation of skin thickness in normal BMD group was 0.94+-0.021mm, osteopenic group was 0.92+-0.006mm, and osteoporotic group was 0.89+-0.018mm. There was statistically significant difference in the mean values of skin thickness between the three groups even adjusted with age and BMI(P<0.05). The mean and standard deviation of skin tbickness of healthy 20-40 year old women was 1.11+-0,023mm and their mean and standard deviation of L2-L4 mean BMD was 1.17+-0.145mg/cm2. The diagnostic predictability of skin thickness less than 1mm as the risk of osteoporosis(BMD T score less than -1.0) was evaluated. The sensitivity and the specificity of skin thickness less than 1mm being osteoporotic were 78,2% and 57.8% respectively. The positive and negative predictive value of the skin thickness less than 1mm being osteoporotic were 82.2% and 36.5% respectively. CONCLUSION: This study indicate that the skin thickaess measured with the ultrasound method show good correlatian with the bone density measured with conventional DXA at the lumbar vertebra and the skin thickness less than 1mm on the medial side of the opper arm is relatively sensitive in diagnosing osteoporosis risk in Korean women. The authors suggested that a large randomized control study to define the relationship between the skin thickness and the other determinants of bone turnover in the near future.
Aged ; Arm ; Biopsy ; Bone Density ; Collagen ; Collagen Type I ; Densitometry ; Diagnosis* ; Female ; Humans ; Osteoporosis ; Osteoporosis, Postmenopausal* ; Sensitivity and Specificity ; Skin* ; Spine ; Ultrasonics ; Ultrasonography

Acute mountain sickness (AMS) occurs commonly in hikers who are rapidly exposed to high altitude environments. Despite the numerous reports of AMS, few studies have reported pallidal lesions associated with altitude sickness. A previously healthy 49-yr-old Korean patient, after ascent to 4,700 m, suffered symptoms consistent with AMS. After returning home, the patient showed changes in personality characterized by abulia, indifference, and indecisiveness. T2 weighted brain magnetic resonance imaging showed high signal lesions involving bilateral globus pallidus. Our case suggests that globus pallidus injury should be included in the differential diagnosis of patients with personality or cognitive change after recovery from AMS.
Altitude ; Altitude Sickness/*complications ; Behavior ; Brain/pathology ; Brain Damage, Chronic/*etiology/pathology ; Environment ; Globus Pallidus/*pathology ; Humans ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Mountaineering

PURPOSE: Intussusception is one of the most common causes of childhood intestinal obstructions. Early diagnosis is essential to avoid treatment delays, which can increase morbidity and mortality. In this study, authors have tried to analyze the risk factors associated with intussusception in infants and children to establish an early diagnosis to prevent unwanted outcomes. METHODS: We reviewed 44 cases involving operations for childhood intussusception from 1997 to 2001. These were divided into two groups and compared retrospectively using medical records. Group I included 34 cases of manual reduction (77.3%), and group II consisted of 10 cases of bowel resection (22.7%). RESULTS: There were no significant differences in age, sex, and clinical symptoms between the 2 groups. The anatomic location of the intussusceptum, and fever seemed to vary, but this was of no statistical significance. Factors significantly contributing to increase in the rate of bowel resection were the duration of the symptoms (more than 48 hours), and the leukocyte count (more than 15,000/mm3). CONCLUSION: This study revealed that only the duration of symptom and the leukocyte count contributed to an increase in the rate of bowel resection. Further prospective studies would be needed to reveal more risk factors to accurately predict the prognosis of the disease.
Child ; Early Diagnosis ; Fever ; Humans ; Infant ; Intestinal Obstruction ; Intussusception* ; Leukocyte Count ; Medical Records ; Mortality ; Prognosis ; Retrospective Studies ; Risk Factors*

PURPOSE: Epilepsy, a typical chronic disease, may cause severe of psychosocial difficulties for all family members, including stigmatization. The present study focused on how children with epilepsy affect their parents' perceived stigma. METHODS: Participants were 87 parents(21 fathers and 66 mothers) whose children were diagnosed as and treated for epilepsy at the pediatric epilepsy clinic in Dongguk University Hospital. To obtain data, the parental perceived stigma scales with 3 questionnaires were developed by modifying Jacoby's stigma scales. Data were analyzed with the SPSS 10.0 program using oneway ANOVA and multiple regression. RESULTS: The results of the analysis showed that 22 parents(25.3%) have perceived stigma. Oneway ANOVA showed significant differences of the parental perceived stigma level according to the parental academic background, the number of antiepileptic drugs, the combined disabilities, the educational institution of epileptic children, and the desire for support. Multiple regression showed that the parental academic background and the existence of combined disabilities are the factors which influence significantly on the parental perceived stigma level. CONCLUSION: These results suggest that the parents of epileptic children who have low academic background and who care for the epilepsy-plus children may have more perceived stigma. These findings may help healthcare providers prepare for the epilepsy family programs in terms of relieving their social stigma.
Anticonvulsants ; Child* ; Chronic Disease ; Epilepsy ; Fathers ; Health Personnel ; Humans ; Parents* ; Surveys and Questionnaires ; Social Stigma ; Stereotyping ; Weights and Measures

No abstract available.
Thrombocytopenia*

Diabetic nephropathy is a common and serious complication in diabetic patients. Renal diseases other than diabetic nephropathy (non-diabetic nephropathy) can occur in diabetic patients with nephrosis. The presence of non-diabetic nephropathy is noted in type 2 diabetes patients, but no data exists for type 1 diabetes. In this report we describe the case of a 15-year-old girl with type 1 diabetes mellitus, who presented with an acute elevation of urinary microalbumin excretion, general edema, and liver enzyme elevation. She had shown microalbuminuria about 3 years earlier, as well as an uncontrolled hemoglobin A1c level, but she had no diabetic retinopathy and neuropathy. A renal biopsy was conducted, and she was diagnosed with primary focal segmental glomerulosclerosis. She was treated with corticosteroids and an angiotensin converting enzyme inhibitor.
Adolescent ; Adrenal Cortex Hormones ; Biopsy ; Diabetes Mellitus, Type 1 ; Diabetic Nephropathies ; Diabetic Retinopathy ; Edema ; Glomerulosclerosis, Focal Segmental ; Hemoglobins ; Humans ; Liver ; Nephrosis ; Peptidyl-Dipeptidase A

False aneurysm is one of the complications of untreated arterial injury. Most traumatic aneurysms in the extremities are false, and they are usually caused by penetrating trauma. The pathogenesis of false aneurysm is initiated by a damaged artery that allows hemorrhage into surrounding soft tissue, an encapsulated hematoma is then formed, which undergoes organization, with invasion of fibrin and con-nective tissue, and the endothelialization of its central cavity, which communicates with the arterial defect, forming the false aneurysm. We experienced two cases of traumatic false anerysm of a left anterior tibial artery and a right radial artery, which were treated successfully by end-to-end anastomosis in the former and a saphenous vein graft in the latter.
Aneurysm ; Aneurysm, False* ; Arteries ; Extremities ; Fibrin ; Hematoma ; Hemorrhage ; Radial Artery ; Saphenous Vein ; Tibial Arteries ; Transplants

BACKGROUND: Retrograde amnesia (RA) refers to the failure to recall events that occurred before a brain injury. RA is known to be associated with brain lesions involving the hippocampus, entorhinal cortex and the frontal lobe. Anterior thalamic lesion often causes anterograde amnesia but rarely causes RA. The aim of the present study is in two parts . First, we discuss the neuroanatomical perspectives of RA based on our case with severe RA after a right anterior thalamic infarction. Second, we introduce a test for RA termed the "Korean Public Events Recall Test (K-PERT)", which was developed based on famous Korean public events from 1966 to 1997. METHODS: A 62-year-old woman with transient RA after a left anterior thalamic infarction 4 years ago presented severe and persistent RA following a right anterior thalamic infarction. We followed up the patient with neuropsychological tests. We also performed the K-PERT on the patient as well as on 14 women of the same age and education. RESULTS: Neuropsychological tests showed severe impairment in autobiographical memory with frontal lobe dysfunction. On K-PERT, the normal controls scored 13.7 +/- 3.7 in recall and 21.2 +/- 3.1 in recognition out of a maximum score of 30, whereas the patient obtained only 3/30 and 4/30, respectively. CONCLUSIONS: In our case, RA might have resulted from damage to the pathway that retrieves old memories, which are stored in the frontal lobe. Thus, anterior thalamus might be viewed as the gate of memory engram. Further studies are needed to elaborate the usefulness of K-PERT as an objective tool for investigating remote memory.
Amnesia, Anterograde ; Amnesia, Retrograde* ; Brain ; Brain Injuries ; Education ; Entorhinal Cortex ; Female ; Frontal Lobe ; Hippocampus ; Humans ; Infarction* ; Memory ; Memory, Episodic ; Memory, Long-Term ; Middle Aged ; Neuropsychological Tests ; Thalamus

We examined predictors of suicide attempts in clinically depressed adolescents in Korea and gender differences in suicidal behavior. In total, 106 adolescents diagnosed with depressive disorder were recruited in South Korea. We assessed various variables that might affect suicide attempts, and used a structured interview for the diagnosis of depression and comorbidities and to evaluate suicidality. Demographic and clinical characteristics of the subjects were compared between suicide attempt and non-suicide attempt groups and we examined significant predictors of suicide attempts. Gender differences in suicidal ideation and suicidal behavior were also analyzed. Among 106 depressed participants, 50 (47.2%) adolescents were classified in the suicide attempt group. Generally, the suicide attempt and non-suicide attempt group shared similar clinical characteristics. The suicide attempt group had more females, more major depressive disorder diagnoses, more depressive episodes, and higher suicidal ideation than the non-suicide attempt group. Suicidal ideation was the only significant predictor of suicidal attempt, regardless of gender. Higher suicidal ideation frequency scores and more non-suicidal self-injurious behaviors were shown in the female suicide attempt group than the male suicide attempt group. It is recommended that suicidal ideation be assessed regularly and managed rigorously to decrease suicide risks in depressive adolescents.
Adolescent* ; Comorbidity ; Depression ; Depressive Disorder ; Depressive Disorder, Major ; Diagnosis ; Female ; Humans ; Korea ; Male ; Self-Injurious Behavior ; Suicidal Ideation ; Suicide* ; Suicide, Attempted