Craniosysostosis syndrome is caused by premature fusion of bones of skull and face during fetal development. It is related to Fibroblast growth factor receptor gene and most common craniosynostosis syndromes are Apert, Pfeiffer and Crouzon. Apert syndrome is one of the severe type of craniosynostosis syndromes which shows mutations in the Fibroblast growth factor receptor 2 (FGFR2) gene. Pfeiffer syndrome is also related with FGFR 1 or 2 gene mutation. We experienced two patients with craniosynostosis syndromes, Apert syndrome and Pfeiffer syndrome. The first baby was a in-born female baby presented with syndactly of the hands and feet and facial dysmorphism including shallow orbit with deep crease above eye brow. Apert syndrome was confirmed by the presence of a mutation in FGFR2. The second patient visited our developmental delay clinic due to developmental delay at seven month old age. He showed facial dysmorphism including cloverleaf-shaped skull, micrognathia, low set ears, low nasal bridge and high-arched palate, but there were no syndactly or limb anomalies. He was suspected of Pfeiffer syndrome, however his FGFR2 gene study was normal. These patients need multidisciplinary team management and regular follow up for visual, auditory, and cognitive development functions Pediatricians have important role on recognizing the patients with facial dysmorphism, planning to evaluate accompanying anomalies and making appropriate decisions about the timing of surgical management to minimize growth and cognitive impairments.
Acrocephalosyndactylia ; Craniosynostoses ; Ear ; Extremities ; Female ; Fetal Development ; Follow-Up Studies ; Foot ; Hand ; Humans ; Orbit ; Palate ; Receptor, Fibroblast Growth Factor, Type 2 ; Receptors, Fibroblast Growth Factor ; Skull

PURPOSE: Since 1997, private postnatal care facilities (San-hu-jo-ri-won in Korean) have emerged to take the role of the family. As a result, neonates are now exposed to many people and are very vulnerable to infection. However, there has been no study on the influence of postnatal care facilities on neonatal infection. The aim of this study was to determine the risk factors of neonatal infection in full-term babies in Korea. MATERIALS and METHODS: We followed up 556 pregnant women and their babies for 4 weeks after their births at 2 hospitals in Seoul and Daejeon from October 2004 to September 2005. Among 512 full-term babies, 58 had infectious diseases. To determine the risk factors for infection, 53 infected neonates at 4-28 days of life and 413 healthy neonates were compared. RESULTS: The incidence of neonatal infection at 4 to 28 days after birth was 10.5%. After adjusting the related factors, the number of siblings (OR = 2.05, 95% CI = 1.13-3.71 for 1 or more) and postnatal care facilities or home aides (OR = 1.91, 95% CI = 1.07-3.45) were significant risk factors. Formula or mixed feeding (OR = 1.66, 95% CI = 0.91-3.04) increased the risk of neonatal infection but it was not statistically significant. CONCLUSION: When the newborns had siblings, stayed at postnatal care facilities, or were cared for by home aides, the risk of neonatal infections significantly increased. Further research on the feeding effect on neonatal infection and evaluation of prevention efforts are needed.
Communicable Diseases/*epidemiology ; Female ; Humans ; Infant, Newborn ; Korea/epidemiology ; Mothers ; Risk Factors ; *Term Birth

No abstract available.
Blood Pressure* ; Humans ; Infant, Newborn*

OBJECTIVE: To investigate the response of hyperplastic endometrium to Medroxyprogesterone acetate according to the histologic types such as simple typical, complex typical and atypical hyperplasia. METHODS: A total of 79 patients with histologically proved endometrial hyperplasia were enrolled into this prospective study between March 1996 and May 1998. Patients without atypia were placed on a regimen of cyclic therapy with 10mg MPA orally, each day for 14days per month for 3 months. In the cases in which hyperplasia did not regress , MPA was increased to 20mg. Patients with atypical hyperplasia received continuous MPA therapy, 20mg orally each day for 3 month. All patients were followed up for a minimum of 3 months and a maximum of 1 year(mean 7 months). RESULTS: In patients with simple typical hyperplasia, 45 patients(80.4%) had regression, 11 patients(19.6%) had persistence and none had progression. In patients with complex typical hyperplasia, 10 patients(83.3%) had regression, 2 patients(16.7%) had persistence and none had progression. But, in patients with atypical hyperplasia 5 patients(45.4%) had regression, 4 patients(36.4%) had persistence and 2(18.2%) patients had well differentiated endometrial adenocarcinoma. There was no recurrence during the follow up. CONCLUSION: This data suggest that most women with typical hyperplasia respond to progestin therapy, but there is high failure rate of response to progestin therapy and risk of endometrial cancer in patients with atypical hyperplasia. If the young patient desires to preserve her fertility, then progestin therapy may be considered as primary treatment in patients with atypical hyperplasia. But older patients in whom fertility is not an issue, hysterectomy should be selected as treatment of choice for atypical lesion.
Adenocarcinoma ; Endometrial Hyperplasia* ; Endometrial Neoplasms ; Endometrium ; Female ; Fertility ; Follow-Up Studies ; Humans ; Hyperplasia ; Hysterectomy ; Medroxyprogesterone Acetate ; Medroxyprogesterone* ; Prospective Studies ; Recurrence

BACKGROUND: Disialoganglioside GD2 is a tumor-associated antigen that is overexpressed on tumor cells of neuroectodermal origin, such as melanoma, small cell lung carcinoma and neuroblastoma. Immunity against GD2 has anti-tumor activities, but GD2 is poorly immunogenic. Anti-idiotypic antibodies that mimic GD2 may induce more effective immune responses than GD2 antigen itself, because they are protein antigens and are known to be able to break immune tolerance. In our previous study, we produced anti-idiotypic antibodies mimicking GD2 (3A4 and 3H9), which induced humoral immunity. However, cellular immunity is essential to eradicate tumor cells in vivo as well as humoral immunity. In the present study, we investigated whether these anti-idiotypic antibodies 3A4 and 3H9 could induce cellular immunes responses. METHODS: BALB/C mice were immunized with anti-idiotypic antibody 3A4 or 3H9, or normal mouse IgG as a negative control. Lymphoproliferative responses, cytokine production responses, and delayed-type hypersensitivity reactions were measured in mice immunized with the anti-idiotypic antibodies. RESULTS: Both the anti-idiotypic antibody 3A4 and 3H9 induced GD2-specific lymphoproliferative responses and IFN-gamma production of lymph node lymphocytes in BALB/C mice. Only anti-idiotypic antibody 3H9 induced significant GD2-specific delayed-type hypersensitivity in the mice. CONCLUSION: These results show that anti-idiotypic antibodies 3A4 and 3H9 have the potentiality of inducing GD2-specific cellular immune responses that cannot be induced by the native antigen GD2 itself.
Animals ; Antibodies, Anti-Idiotypic* ; Hypersensitivity ; Immune Tolerance ; Immunity, Cellular* ; Immunity, Humoral ; Immunoglobulin G ; Lymph Nodes ; Lymphocytes ; Melanoma ; Mice ; Neural Plate ; Neuroblastoma ; Small Cell Lung Carcinoma

In air leak syndrome, a significant portion of the volume delivered during a positive pressure breath can be lost through the leak. HFOV can achieve adequate ventilation at lower peak and/or mean intrapulmonary pressure than conventional mechanical ventilation (CMV) and has been an effective treatment of already established air leak syndrome. We report a 1-day-old male infant with severe respiratory failure from pneumothorax and pneumomediastinum, who was refractory to CMV with chest tube drainage. HFOV was applied to this patient for 114 hours, and improvement of oxygenation and ventilation as well as significant reduction of pneumothorax followed.
Chest Tubes ; Drainage ; Humans ; Infant ; Male ; Mediastinal Emphysema ; Oxygen ; Pneumothorax ; Respiration, Artificial ; Respiratory Insufficiency ; Ventilation*

The purpose of this study was the presence of accessory tendon and its anatomical variation of insertion of the abductor pollicis longus and extensor pollicis brevis. 1. Among 46 cases, all had one or more accessory tendon except one which inserted into the base of the first metacarpal bone on its anterolateral surface with a single tendon. 2. Among 45 cases (which had one or more accessory tendon), the abductor pollicis longus tendon inserted into the trapezium in 30 cases(66.6%), and thenar muscles in 38 cases (84.4%). 3. Among 46 cases, the extensor pollicis brevis tendon inserted into the proximal phalanx in 30 cases(65%) and into the distal phalanx with extensor pollicis longus in 8 cases (18%) and into both in 8 cases (18%). 4. Among 22 cadavers, symmetry of insertion of the abductor pollicis longus noticed in 16 cases (88 and extensor pollicis brevis in 21 cases (95%).
Cadaver ; Muscles ; Tendons

This study was performed to evaluate the semiquantitative food frequency questionnaire used to obtain information about. dietary patterns of adults in a rural county, Yang-pyeung Gun. The study population was 1311 adults aged 17~85 years. And well-trained interviewers interviewed subjects in 1990, 1992, 1993. The results are as follows. 1) Nutrients intake calculated from each questionnaire showed significant difference, because the number of question was not same(fifty questions in 1990, eighty questions in 1992, 1993). 2) Estimated nutrients intake from separate food items was significantly different when food items are combined in one question. 3) The agreement of nutrients intake level except vit C were high when the subjects are cross-classified by their ranks on the nutrients intake level from items in both questionnaires and on the nutrient intake level from items in each questionnaire. 4) And analysing this study data by multiple stepwise regression, we selected twenty seven food items whic could explained over 85% of inter-individual variation of nutrients(energy, carbohydrate, fat, protein, vit A, vit B1, vit B2, vit C, Ca). From above results, we suggested that it is neccessary to standardize questionnaire and perform validity studies.
Adult ; Humans ; Korea* ; Rural Population* ; Surveys and Questionnaires

Adenomatoid tumor is extremely rare benign neoplasm occurring in the genital tract. They are mesothelial origin tumors and frequently found in the testis, epididymis, tunica vaginalis, or spermatic cord in male. But most adenomatoid tumors are found near or in epididymis. While in female they are usually located in the fallopian tube, uterus, and ovary. Adenomatoid tumors are usually small, firm or solid, and asymptomatic, these are clinically found or routine physical examination. They are usually less than 2cm in diameter, but may measure up to 5cm, Histologically the tumor consists of tubules and gland like spaces lined with cuboidal or flattened in a fibrous stroma. The tumor was first described and adenomyoma by Sakaguchi in 1915. Since that time there have been numerous case reports of similar tumors. But the term, adenomatoid tumor, introduced by Golde and Ash in 1945 seems to have been even more widely used. Recently we experienced a case of adenomatoid tumor of epididymis, which was found incidentally during physical examination and confirmed by excisional biopsy in a patient who complained lower abdominal pain.
Abdominal Pain ; Adenomatoid Tumor* ; Adenomyoma ; Biopsy ; Epididymis* ; Fallopian Tubes ; Female ; Humans ; Male ; Ovary ; Physical Examination ; Spermatic Cord ; Testis ; Uterus

No abstract available.
Bony Callus* ; Coinfection* ; Epidermal Cyst*