No abstract availalbe.
Genetic Therapy*

Being a rare disease entity, there have been few references about the cleft hand in Korea. We evaluated the postoperative cosmetic results of 17 cleft hands in 14 patients, who were treated surgically at the Department of Orthopedic Surgery of Seoul National University Hospital, from 1982 to 1993. Among 14 patients, 8 were males. Bilateral cleft hands were in five patients and unilateral in 9. Three of five patients who had bilateral cleft hands, were treated surgically on both hands. Remaining two patients had an operation on one hand which affected more severely. Average age at the time of operation was 2 years and 5 months(5 months-10years). Average duration of follow-up was 34 months(1 year 1 month-7years 5 months). By Lange's classification, typical patterns were 16 hands and atypical pattern one. By Flatt's classification, there were 8 hands of I b, 7 I c, 1 I a, 1 II. One patient had family history whose father had both cleft hands and feet. Congenital anomalies other than cleft hand were associated in 10 of 14 patients. Two patients had 3 associated anomalies, 4 patients 2, and 4 patients one. Syndactyly was the most common associated anomaly of affected cleft hand. Central cleft was closed by Snow-Littler technique in 4 hands(24%), Barsky method in 12(71%) and others in 1(6%). Metacarpal bone was treated by intermetacarpal ligament reconstruction in 1 hand(6%), excision only 7(41%), transfer of 2(rd) metacarpal to 3(rd) metacarpal base after excision 5(29%), and 2(rd) metacarpal corrective osteotomy 3(18%). Complications were in 2 hands; one wound dehiesence of cleft, and one partial necrosis of skin. The cosmetic results of surgical treatment were graded into good, fair or poor by the parents-Good ; when parents were satisfied with the result, Fair; improved cosmesis but not satisfied, and Poor; no improvement after surgery. Good was in 16 hands and fair in one.
Classification ; Fathers ; Follow-Up Studies ; Foot ; Hand ; Humans ; Korea ; Ligaments ; Male ; Methods ; Necrosis ; Orthopedics ; Osteotomy ; Parents ; Rare Diseases ; Seoul ; Skin ; Syndactyly ; Wounds and Injuries

Two cases of abnormalities of the left ventricular wall(left ventricular aneurysm or diverticulum) are presented. A saccular deformity of the left ventricle may be and aneurysm or a diverticulum. In one case, the defect seems to be subcalvular aneurysm(or fibrous diverticulum) or aneurysm of the membranous ventricular septum; this lesion seems to be a natural consequence of spontaneous closure of a defect of the membranous septum. The other case, it seems that the defect is ventricular aneurysm with syndrome of myocardial infarction and normal coronary arteries, or double or accessory chambered left ventricle. The thromboembolic phenomenon was noted on a left frontoparietal lobe of brain. Both cases have the diagnosis supported by cardiac catheterization and angiography. The clinical, angiographic and pathologic characteristic of diverticulum and aneurysm of the heart are reviewed, and an attempt is made to clarify the concept of aneurysm and diverticulum of the heart.
Aneurysm* ; Angiography ; Brain ; Cardiac Catheterization ; Cardiac Catheters ; Congenital Abnormalities ; Coronary Vessels ; Diagnosis ; Diverticulum* ; Heart ; Heart Ventricles* ; Myocardial Infarction ; Ventricular Septum

A 27-year-old woman developed Wemicke's encephalopathy in the 16th week of her first pregnaney. She had thyroid storm and abnormal liver function. Her thyrotoxic symptom and abncemal liver function was recovered after medication of antithyroid drug, steroid, hepatotonic drug and administration of thiamine(fursulthiamin), but the fetus was lost, Thereafter her thyroid function returned to normal and euthyroid state was maintained without medication of antithyroid drug, but her neurological defect was remained. We suggest that severe hyperemesis gravidarum is a possible risk factor of the thyroid storm and Wemicke's encephalopathy in patients with hyperthyroidism, and consider the check of the thyroid function. The need for parenteral thiamine supplementation and medication of antithyroid drug is warranted in patients with severe hyperemesis gravidarum which lasts longer than 3 weeks and abnormal thyroid function.
Adult ; Female ; Fetus ; Humans ; Hyperemesis Gravidarum* ; Hyperthyroidism ; Liver* ; Pregnancy ; Risk Factors ; Thiamine ; Thyroid Crisis* ; Thyroid Gland* ; Wernicke Encephalopathy*

BACKGROUND: Many physicians hesitate to discuss do-not-resuscitate (DNR) orders with patients or family members in critical situations. In the intensive care unit (ICU), delayed DNR decisions could cause unintentional cardiopulmonary resuscitation, patient distress, and substantial cost. We investigated whether the timing of DNR designation affects patient outcome in the medical ICU. METHODS: We enrolled retrospective patients with written DNR orders in a medical ICU (13 bed) from June 1, 2014 to May 31, 2015. The patients were divided into two groups: early DNR patients for whom DNR orders were implemented within 48 h of ICU admission, and late DNR patients for whom DNR orders were implemented more than 48 h after ICU admission. RESULTS: Herein, 354 patients were admitted to the medical ICU and among them, 80 (22.6%) patients had requested DNR orders. Of these patients, 37 (46.3%) had designated DNR orders within 48 hours of ICU admission and 43 (53.7%) patients had designated DNR orders more than 48 hours after ICU admission. Compared with early DNR patients, late DNR patients tended to withhold or withdraw life-sustaining management (18.9% vs. 37.2%, p = 0.072). DNR consent forms were signed by family members instead of the patients. Septic shock was the most common cause of medical ICU admission in both the early and late DNR patients (54.1% vs. 37.2%, p = 0.131). There was no difference in in-hospital mortality (83.8% vs. 81.4%, p = 0.779). Late DNR patients had longer ICU stays than early DNR patients (7.4 ± 8.1 vs. 19.7 ± 19.2, p < 0.001). CONCLUSIONS: Clinical outcomes are not influenced by the time of DNR designation in the medical ICU. The late DNR group is associated with a longer length of ICU stay and a tendency of withholding or withdrawing life-sustaining treatment. However, further studies are needed to clarify the guideline for end-of-life care in critically ill patients.
Advance Directives ; Cardiopulmonary Resuscitation ; Consent Forms ; Critical Illness* ; Hospital Mortality ; Humans ; Intensive Care Units ; Resuscitation Orders* ; Retrospective Studies ; Shock, Septic

PURPOSE: We previously reported that concurrent reactivation of latent Epstein-Barr virus (EBV) in children with hepatitis A virus (HAV) infection is common and EBV reactivation with HAV infection adversely affects the clinical features of hepatitis. However, the incidence of concurrent reactivation was not accurate because the detection of EBV reactivation was based on serologic methods. Therefore, we studied the effects of polymerase chain reaction (PCR)-proven EBV reactivation, thus a more precise concurrence, on acute HAV infection in children. METHODS: PCR were conducted in 34 patients, who had enrolled previous study and diagnosed with acute HAV infection between January 2008 and June 2010. Their medical records were reviewed. RESULTS: Among 34 patients with acute HAV infection, 12 patients (35.3%) had EBV reactivation which was proven using serologic and molecular biologic techniques. There were significant differences in the peak levels of AST and ALT between the reactivated and non-reactivated groups (p=0.001 and p<0.001, respectively). The duration of full recovery from hepatitis was more prolonged in the reactivated group (p<0.001). Clinical parameters, such as serum protein (p<0.001) and albumin concentrations (p<0.001), atypical lymphocyte count (p=0.001), prothrombin time-international normalized ratio (PT-INR, p<0.001), and splenomegaly (p<0.001), showed significant differences. The clinical features in the reactivated sub-group >10 years of age revealed more liver dysfunction compared to the non-reactivated sub-group. A comparison with a previous study was performed. CONCLUSION: PCR-proven reactivation of latent EBV in children with HAV infection is common and EBV reactivation with HAV infection adversely affects the clinical features of hepatitis, especially in older children.
Child ; Hepatitis ; Hepatitis A ; Hepatitis A virus ; Herpesvirus 4, Human ; Humans ; Incidence ; Liver Diseases ; Lymphocyte Count ; Medical Records ; Polymerase Chain Reaction ; Prothrombin ; Splenomegaly

No abstract available.
Child* ; Humans ; Purpura, Thrombocytopenic, Idiopathic*

Backgrounds: Thyroid hormones play a fundamental role in the initiation and maintenance of somatic growth in mammalian species, and the insulin-like growth factors(IGFs) occupy a position of central importance in the growth of all tissues. To evaluate the changes in serum insulin-like growth factor-I(IGF-I) and insulin-like growth factor binding proteins in hyperthyroid and hypothyroid patients, sera was obtained from 19 hyperthyroid patients, 9 hypothyroid patients, and 10 healthy volunteers. Methods: IGF-I concentration was determined by radioimmunoassay, and changes in IGFBPs were assesed by Western Ligand Blotting. To evaluate the binding pattern of IGF-I & IGFBPs, autoradiographs were obtained. Results & Conclusion: IGF-I levels were increased significantly in hyperthyroid patients(mean ±SE, 267.88±9.80 ng/ml, p<0.05) and decreased significantly in hypothyroid patients(154.81±1.43 ng/ml, p<0.01) compaired to healthy control group(209.45±.60 ng/ml). Autoradiograph of serum IGFBPs from patients with hyperthyroidism and hypothyroidism did not show any change in the intensity of IGFBP-3 bands(40-45 KD) and IGFBP-1 bands, but in hyperthyroid patients, it showed increased intensity of IGFBP-2 band compared to healthy control group and hypothyroid patients.
Equidae ; Healthy Volunteers ; Humans ; Hyperthyroidism ; Hypothyroidism ; Insulin-Like Growth Factor Binding Protein 1 ; Insulin-Like Growth Factor Binding Protein 2 ; Insulin-Like Growth Factor Binding Protein 3 ; Insulin-Like Growth Factor Binding Proteins ; Insulin-Like Growth Factor I ; Radioimmunoassay ; Thyroid Hormones

Atrial Septal Defect(ASD) is usually suspected clinically but requires a confirmative diagnostic procedure before surgical repair. Conventional transthoracic echocardiography has relatively high sensitivity and specificity for ASD, but difficulty in visualizing the ASD occasionally. Transesophageal echocardiography has special advantages for investigating the posteriorly located cardiac structures, including the atrial septum, which is imaged perpendicularly at a relatively short distance. We describe a case in which ostium secundum ASD was not visualized by conventional transthoracic echocardiography, but was diagnosed confidently by transesophageal echocardiography. It is concluded that transesophageal echocardiography appears to be a promising diagnostic tools for the evaluation of ASD on the basis of its ability to provide excellent imaging of the entire atrial septum and related posterior cardiac structures.
Atrial Septum ; Echocardiography ; Echocardiography, Transesophageal* ; Heart Septal Defects, Atrial* ; Sensitivity and Specificity

Angiomyofibroblastoma is a distinctive, hitherto uncharacterized, benign soft tissue tumor of the vulva with histology similar to an aggressive pelvic angiomyxoma. It can be distinguished from an aggressive angiomyxoma by its circumscribed borders, higher cellularity, more numerous blood vessels, the frequent presence of plump stromal cells, minimal stromal mucin, and rarity of erythrocyte extravasation. We experienced a case of angiomyofibroblastoma of the vulva occurring in a 45-year-old woman. The lesion was a well-defined but not encapsulated mass, 4.5x4.2 cm. Histologically the mass was characterized by alternating hypercellular and hypocellular edematous zones in which abundant blood vessels were irregularly distributed. Immunohistochemically, the spindled, plump spindled, and oval stromal cells were reactive for vimentin and desmin, but not for cytokeratin, or S-100 protein.
Female ; Humans