Antiphospholipid syndrome (APS) is a noninflammatory autoimmune disorder characterized by the association of arterial and/or venous thrombosis, recurrent fetal loss and elevated titres of antiphospholipid antibodies, namely lupus anticoagulant (LAC) and/or anticardiolipin antibodies (aCL). It can either occur as a free-standing condition (primary APS) or be associated with another autoimmune disease (secondary APS), mainly systemic lupus erythematosus. The precise pathogenesis of thrombosis in APS in unknown. For children with unexplainable venous or arterial thrombosis, APS should be considered. The diagnosis of APS in children requires a clinical event including venous or arterial thrombosis or immune thrombocytopenia and a laboratory abnormality including positive LAC test or positive aCL antibody test, moderate or high titer IgG. The laboratory abnormality should persist for at least 2 months. We report a 7-year-old girl who had pulmonary embolism and multiple arterial thrombosis caused by primary APS. The laboratory abnormality was positive LAC and beta2-glycoprotein I. She was treated successfully with low molecualr weight heparin (LMWH). After recovery, she was continuously treated with subcutaneous LMWH for another 3 months and switched to oral warfarin. She was symptom-free through 3 years of follow-up.
Antibodies, Anticardiolipin ; Antibodies, Antiphospholipid ; Antiphospholipid Syndrome* ; Autoimmune Diseases ; beta 2-Glycoprotein I ; Child ; Diagnosis ; Female ; Follow-Up Studies ; Heparin ; Heparin, Low-Molecular-Weight ; Humans ; Immunoglobulin G ; Lupus Coagulation Inhibitor ; Lupus Erythematosus, Systemic ; Pulmonary Embolism ; Pulmonary Infarction* ; Thrombocytopenia ; Thromboembolism* ; Thrombosis ; Venous Thrombosis ; Warfarin

Infantile fibrosarcoma is a rare malignant soft tissue tumor occurring especially in newborn and young children under 2 years. We experienced three cases of infantile fibrosarcoma presenting in the neonatal period. Case 1 presented with a multiseptated cystic mass on his left thigh at birth that was diagnosed as lymphangioma. After picibanil injection, we noted the size of the mass doubled and a solid lesion was prominent in the magnetic resonance image. Case 2 was found to have a reddish mass on his lower back mimicking hemangioma. Over 2 weeks, the mass grew rapidly with internal hemorrhaging. Case 3 was noted to have an encircling mass around the splenic flexure, which developed into congenital bowel obstruction. All of the tumors were resected completely, but microscopic resection margin was not clear in two patients. The two patients received adjuvant chemotherapy and all patients are well without evidence of recurrence.
Chemotherapy, Adjuvant ; Child ; Colon, Transverse ; Fibrosarcoma ; Hemangioma ; Humans ; Infant, Newborn ; Lymphangioma ; Magnetic Resonance Spectroscopy ; Parturition ; Picibanil ; Recurrence ; Thigh

Clear cell sarcoma is a rare malignant rumor of the kidney which occurs in children and is differentiated from Wilms' tumor by its different clinicopathologic features and natural history. Previous studies indicate that this tumor may be of mesenchymal cell origin; however, this has not been proven conclusively. Further accumulation and study need to be conducted in order to clarify the histogenesis of this tumor. We report two cases of clear cell sarcoma of the kidney which occurred in a 2 and a half-year old and a 2-year old boy. This report places special emphasis on the clinicopathologic characteristics of these two cases including electron microscopic and immunohistochemical findings. Attempts were also made to differentiate the clinicopathologic aspects of clear cell sarcoma from Wilms' tumor and speculate on the histogenesis of this rumor.
Child ; Male ; Female ; Humans

The lack of sufficient oral mucosa available for intra-oral reconstruction has been dealt with by the use of skin or oral mucosa grafts harvested from donor sites but grafts requires more than one surgical procedures and could cause donor site morbidity. Many investigators have attempted to increase available soft tissue by tissue engineered skin or oral mucosa replacements for clinical applications. But, reconstructed mucosa by several methods have low physical properties such as rolling and contraction. The aims of this study were to develope an in vitro experimental model that maintains an epithelial-mesenchymal interaction by organotypic raft culture, and to characterize biologic properties of three-dimensionally cultured oral mucosa embedded with Polydioxanone mesh by histological and immunohistochemical analysis. The results were as follows; 1. Oral mucosa reconstructed by three-dimensional organotypic culture revealed similar morphologic characteristics to equvalent normal oral mucosa in the point that they show stratification and differentiation. 2. The expression of cytokeratin 10/13 and involucrin in the cultured tissue showed the same pattern with normal oral mucosa suggesting that organotypic co-culture condition is able to induce cellular differentiation. 3. After insertion of polydioxanone mesh, increased tensile strength were observed. These results suggest that three-dimensional organotypic co-culture of the oral mucosa cell lines with the dermal equvalent consisting type I collagen and fibroblasts reproduce the morphologic and immunohistochemical characteristics similar to those in vivo condition. And increased physical properties by use of polydioxanone mesh will helpful for clinical applications.
Cell Line ; Coculture Techniques ; Collagen Type I ; Fibroblasts ; Humans ; Keratins ; Models, Theoretical ; Mouth Mucosa* ; Mucous Membrane ; Polydioxanone* ; Research Personnel ; Skin ; Tensile Strength ; Tissue Donors ; Transplants

No abstract available.
Gastroschisis* ; Hernia, Umbilical*

No abstract available.
Gastric Emptying*

PURPOSE: To compare intraocular straylight in normal and cataractous eyes as the morphology and to compare straylight as the result of subjective symptoms in early cataract cases using the C-quant straylight meter, the only tool to measure light scattering in media. METHODS: Straylight values were measured in 217 normal eyes and 138 cataractous eyes. Cataractous eyes were classified into posterior subcapsular opacity, anterior subcapsular opacity and nucleosclerosis. Straylight values of each group were measured. The 56 early cataractous eyes were categorized into two groups, depending on the presence of subjective symptoms, and each straylight value was measured. The preoperative and postoperative straylight values of early cataracts were also compared. RESULTS: The mean straylight values of normal and cataractous eyes were 1.34 and 2.46, respectively. The value of posterior subcapsular opacity (2.81) was significantly higher than that of anterior subcapsular opacity (2.33) and nucleosclerosis (1.99). The straylight values of early cataracts were significantly higher in the group with subjective symptoms (2.02) than in the group without subjective symptoms (1.56). The postoperative straylight value decreased to 1.42. CONCLUSIONS: The posterior subcapsular cataract showed significantly high intraocular straylight, indicating that light scattering occurred to a greater extent in this group. Light scattering occurred more in early cataractous eyes with subjective symptoms than in eyes without symptoms, and light scattering was reduced after surgery. The C-quant straylight meter, which measures the light scattering in media, can be a useful tool to determine the time of cataract surgery and to evaluate the quality of vision.
Cataract ; Eye ; Light ; Vision, Ocular

PURPOSE: The purpose of this study was to examine how child development knowledge, child rearing knowledge, and use of healthcare services after discharge affect maternal confidence among mothers of premature infants. METHODS: Participants in this study were 55 mothers who were involved in internet communities for mothers with premature infants and 30 mothers who visited hospitals for follow up care after having their babies discharged from Neonatal Intensive Care Units (NICU) in South Korea. RESULTS: Presence of operation history, child development and rearing knowledge, and use of healthcare service explained 12.2% of maternal confidence. Presence of operation history (β=.32, p<.05) and child development knowledge (β=.52, p<.05) were significant predictors for maternal confidence. CONCLUSION: The results of this study suggest that mothers with premature infants need further education especially on motor development, developmental knowledge, and knowledge related to operations for mothers whose child had surgery in the NICU. Also information about services provided from community health services for premature infants need to be advertised and distributed.
Child ; Child Development* ; Child Rearing ; Child* ; Community Health Services ; Delivery of Health Care* ; Education ; Follow-Up Studies ; Humans ; Infant, Newborn ; Infant, Premature* ; Intensive Care Units, Neonatal ; Internet ; Korea ; Mothers* ; Premature Birth

OBJECTIVES: This study was performed to identify the relations between the old's participation in voluntary service activities and health-related quality of life. METHODS: The subjects of the study were 318 elderly people aged over 65 years. They were interviewed with the use of a questionnaire from May 1 to June 30, 2012 to collect data. To verify the data collected, this study carried out a linear regression analysis. RESULTS: The health-related quality of life of the elderly who were participating in voluntary service activities was statistically significantly higher than that of those who were not. CONCLUSIONS: This study has meaning in that it discovered that the elderly people's participation in voluntary service activities had a positive effect on their health-related quality of life.
Aged* ; Humans ; Linear Models ; Quality of Life*

In epilepsy-aphasia spectrum (EAS) disorders, mutations in the glutamate receptor ionotropic N-methyl-D-aspartate type subunit 2A (GRIN2A) have become important for screening the disease. Research into the phenotypic variability of several types of neurologic impairment involving these mutations is in progress. However, the non-neurological problems related to these mutations are poorly understood. EAS disorders usually have epileptic, cognitive, or behavioral manifestations. In this case report, we present a female patient with epilepsy, delay in expressive language and social development, and intellectual disability with low intelligence quotient and memory quotient, but normal motor development. Through genetic analysis, she was found to have a missense and a nonsense mutation in GRIN2A (c.1770A>C; p.Lys509Asn and c.3187G>T; p.Glu1063*, respectively) and we consider the nonsense mutation as ‘pathogenic variant’. She was also discovered to have congenital hypothyroidism, growth hormone deficiency and Rathke’s cleft cyst in the brain, which were previously unknown features of GRIN2A mutation. Our findings should widen understanding of the spectrum of GRIN2A phenotypes, and emphasize the need for more research into the association between GRIN2A mutations and non-neurologic clinical presentations.