Asphyxiating thoracic dysplasia(ATD;Jeunes's syndrome) is a rare variety of short limb dwarfism. It is characterized by an extremely small thorax when compared to the ab-dominal circumference, which frequently results in respiratory distress. Other anomalies as-sociated with Jeune's syndrome are pelvic bone malformations and renal dysplasia. It was first described and namely by Jeune et al. in 1954. Jeune's syndrome is an autosomal rece-ssive trait and has a 25% recurrence risk. These patients died at early age due to respirat-ory insufficiency. Death due to uremia has occurred in number of children surviving infan-cy, following progressive renal failure, hypertension and hepatic failure. About 50 cases have been reported in the world literature. We experienced a case of small thorax with short limb dwarfism on antenatal ultraso- und examination and then the baby was delivered by cesarean section. The diagnosis was confirmed to Asphyxiating thoracic dysplasia by clinical features, radiological findings and pathological findings. We reported a case of Asphyxiating thoracic dysplasia with review of literatures.
Cesarean Section ; Child ; Diagnosis ; Dwarfism ; Extremities ; Female ; Humans ; Hypertension ; Liver Failure ; Pelvic Bones ; Pregnancy ; Recurrence ; Renal Insufficiency ; Thorax ; Uremia

PURPOSE: Feeding problems are common for typically developing children and have negative effects on physical, behavioral, and cognitive development. The purpose of the current study was to examine factors related to childhood feeding problems for typically developing children via parental reports. METHODS: The feeding questionnaire developed by the authors and the Childhood Eating Behavior Inventory (CEBI) were administered to 796 parents of children who recruited from 2 pediatric outpatient clinics (n=379) and community (n=417). Problem eaters (PE) were identified by parental report and frequency analysis was conducted for types of feeding problems and its severity, problem behaviors during mealtime, the feeding methods of the parents, and the need for treatment. RESULTS: The CEBI scores were significantly different between the PE and non-problem eaters (NPE), which suggests that the parental reports were reliable. The younger children had more feeding problems than the older children. The most frequent and severe feeding problems were selective eating and longer mealtimes across all age groups. One-half of the children had more than one problem behaviors during mealtime. Most parents of PE used ineffective methods to deal with children's behaviors during meal time, such as cajoling, which was related to their increased level of stress. Many parents reported their children need professional help for the feeding problems. CONCLUSION: Feeding problems are prevalent among children, especially younger children. Children with feeding problems showed a number of problem behaviors during mealtime and parental coping methods appeared to be ineffective. The need for treatment was considerable, thus the characteristics of this population must be acknowledged for providing proper treatment and advice.
Ambulatory Care Facilities ; Child ; Eating ; Feeding Behavior ; Feeding Methods ; Humans ; Meals ; Parents ; Surveys and Questionnaires

Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.
Actins ; Central Nervous System ; Cerebellum* ; Child ; Diagnosis ; Drug Therapy ; Facial Paralysis ; Humans ; Infant ; Molecular Biology ; Mucin-1 ; Muscle, Smooth ; Paralysis ; Phrenic Nerve ; Pneumonia ; Prognosis ; Radiotherapy ; Vimentin

Wilson s disease is an autosomal recessive disorder characterized by degenerative changes in the brain, liver, and cornea. Treatment includes D-penicillamine, trientine, and zinc sulfate. D-penicillamine has been used frequently as first line therapy for Wilson s disease. However, nephrotoxicity can occur after D-penicillamine treatment. Among them membranous glomerulopathy is the most common histological abnormality but minimal change lesions have also been reported. Nephrotic syndrome is a late complication of D-penicillamine treatment but very rarely can occur within 2 months after treatment of D-penicillamine. We report the early development of minimal change nephrotic syndrome in a 3-year-old girl with Wilson s disease 3 weeks after initiation of D-penicillamine.
Brain ; Child, Preschool ; Cornea ; Female ; Glomerulonephritis, Membranous ; Humans ; Liver ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Penicillamine* ; Trientine ; Zinc Sulfate

PURPOSE: The purpose of this study was to evaluate the effects of a devised posture management program based on the Theory of Planned Behavior in adolescents with mild idiopathic scoliosis. METHODS: A quasi-experimental study was conducted. It involved a nonequivalent comparison group design with pretest and posttest. Forty-four female adolescents with mild idiopathic scoliosis participated; data from 35 participants (20 for the test group, 15 for the control group) were used for the final analyses. The devised posture management program ran for 6 weeks. Posture management behavioral determinants (attitude, subjective norms, perceived behavioral control, and behavioral intention) as cognitive outcomes and muscular strength and flexibility as physical outcomes were measured three times: at baseline, week 6 and week 8. Cobb's angle as another physical outcome was measured twice: at baseline and week 8. Descriptive analysis, repeated measures analysis of variance and t test were used for data analyses. RESULTS: Attitude, perceived control, and behavioral intention were consistently enhanced by the posture management program. The intervention increased flexibility and muscular strength and decreased Cobb's angle, which reduced spinal curvature. Frequency of posture management exercise showed a gradual increase in the test group. CONCLUSION: The results indicate that the posture management program is effective in maintaining posture management behavior in adolescents with mild idiopathic scoliosis for both cognitive and physical outcomes. The posture management program should be helpful in expanding the role of school nurses in improving the health status of adolescents with mild idiopathic scoliosis.
Adolescent* ; Health Behavior ; Humans ; Intention ; Pliability ; Posture* ; Scoliosis* ; Spinal Curvatures

No abstract available.
Encephalitis ; Thymoma

The possible association of colorectal adenomatous polyps, a precursor lesion for colorectal cancer, with risk factors including cigarette smoking, alcohol consumption, coffee consumption, family history and dietary factors was investigated in a case-control study. Between January 1993 and March 1995, 143 cases of patients with pathologically confirmed incident colorectal adenomatous polyps and 301 control subjects were collected from Our Lady of Mercy Hospital. Information on exposure was obtained by structured interviews. After adjustment for age and sex, statistically significant odds ratio (highest-lowest tercile) were found for cigarette smoking(5.53; 95% confidence interval CI, 3.26 9.38), coffee consumption (2.01 ; 95% CI 1. 15-3.53). For dietary factors, significant odds ratio were found for yogurt consumption (0.35 ; 95% CI 0.14-0.85) and milk consumption (0.34; 95% CI 0.17-0.70). No significant associations were observed for total fat intake and total fiber intake after adjustment for age, sex and total calorie. Odds ratio of family history of colorectal cancer and/or polyp was 3.05(95% CI 0.84-11.05). Cigarette smoking, coffee consumption, appears to be a significant risk factor, and milk consumption, yogurt consumption appears to be a significant protective factor for colorectal adenomatous polyps.
Adenomatous Polyps* ; Alcohol Drinking ; Case-Control Studies* ; Coffee ; Colorectal Neoplasms ; Humans ; Milk ; Odds Ratio ; Polyps ; Risk Factors* ; Smoking ; Tobacco Products ; Yogurt

We sought to determine whether early amniocentesis is a safe and acceptable method of genetic evaluation in early pregnancy. During the 20-month period from February 1994 to September 1995, 80 consecutive early amniocentesis were performed transabdominally at 12(+3)-14(+6) weeks of gestation and 305 consecutive mid-second-trimester transabdominal amniocenteses were performed at 16(+0)-18(+0) weeks of gestation. All amniotic fluid samples were cultured using flask method. There were no significant differences between the early and mid-second-trimester amniocenteses in failed sampling, ambiguous results, pregnancy loss within 4 weeks after the procedure, pregnancy loss from 4 weeks after procedure to 28 weeks of gestation, preterm birth, and perinatal death. We may conclude that early amniocentesis is a safe and acceptable method for prenatal diagnosis.
Amniocentesis* ; Amniotic Fluid ; Female ; Pregnancy ; Premature Birth ; Prenatal Diagnosis

PURPOSE: The purpose of this study was to explore the current status of education for nursing management in Korea. METHODS: A descriptive study was done using a questionnaire developed by the investigators. The data were collected by survey from 96 out of 129 nursing schools, between December 2010 and February 2011. RESULTS: For 22.9% of nursing schools, there was no faculty for nursing management. The credits and subjects included in nursing management varied among the nursing schools. Lectures in subjects related to nursing management were given not only by nursing management faculty but also by faculty with other majors. There were more faculty and credits for nursing management courses in 4-year nursing schools than in 3-year schools. CONCLUSION: To improve the quality of education in nursing management, there is a need to standardized courses and provide nursing faculty who have majored in nursing management.
Faculty, Nursing ; Humans ; Lectures ; Surveys and Questionnaires ; Research Personnel ; Schools, Nursing

PURPOSE: We investigated systemic risk factors for clinically significant macula edema (CSME) within 1 year after pan-retinal photocoagulation in patients with proliferative diabetic retinopathy. METHODS: A retrospective chart review was performed on 171 patients who received pan-retinal photocoagulation at our hospital from January 2010 to December 2016. The patients were divided into Group Ⅰ with CSME (85 eyes) and Group II without CSME (86 eyes). The associations between presence of CSME and glycated hemoglobin (HbA1c), duration of diabetes, systolic and diastolic blood pressure (BP), body mass index (BMI), lipid status, sex, and estimated glomerular filtration rate (eGFR) were evaluated. RESULTS: In the present study of 171 patients, there was no significant difference in age and gender distribution between the two groups. Duration of diabetes, total serum cholesterol, serum low density lipoprotein, HbA1c, and eGFR were significantly higher in patients with CSME (p < 0.05). Serum high-density lipoprotein, triglycerides, BMI, and systolic and diastolic BP showed no correlation with CSME. Multiple logistic regression analysis showed that total serum cholesterol and HbA1c values had significantly high odds of developing CSME. CONCLUSIONS: HbA1c, total serum cholesterol, and eGFR are important risk factors associated with CSME in patients with proliferative diabetic retinopathy secondary to pan-retinal photocoagulation. Thus, early detection of these risk factors and their control have significant roles in preventing the development and progression of maculopathy and thereby preventing severe visual loss.
Blood Pressure ; Body Mass Index ; Cholesterol ; Diabetic Retinopathy* ; Edema ; Glomerular Filtration Rate ; Hemoglobin A, Glycosylated ; Humans ; Light Coagulation ; Lipoproteins ; Logistic Models ; Retrospective Studies ; Risk Factors ; Triglycerides