Malrotation is a congenital anomaly that becomes symptomatic more frequently during infancy. The indication for surgical treatment at that age is straightforward. In older children, the diagnosis may be more difficult because of chronic and vague complaint. The aim of this study is to compare the symptoms, rate of volvulus and surgical findings in children younger and older than one year. A retrospective study of 40 patients in a a single medical center diagnosed with malrotation from April 1996 to May 2010 was performed. There were 20 (50%) boys and 20 (50%) girls. At the time of operation, 27 (67.5%) patients were younger and 13 (32.5%) were older than 1 year. Vomiting was seen in 20 cases (74.1%) of the younger group compared to 2 cases (15.4%) of the older group. Abdominal sonography and upper gastrointestinal series showed a sensitivity of 100%. Operative findings: 12 (44.4%) of the younger group presented with volvulus compared to none of the older group. The Ladd's procedure was routinely performed with appendectomy in all cases and bowel resection was requires when volvulus included bowel necrosis or other anomalies were found. After definite procedures, surgical correction for adhesive obstruction was necessary in 5 menbers (18.5%) of the younger group and 1 patient (7.7%) in the older group. There was 1 death due to respiratory failure and pneumonia. Abdominal pain was more frequent symptom and bilious vomiting was less frequent. Volvulus did not occur in the older group. Malrotation should be diagnosed promptly in children over 1 year of age by upper gastrointestinal series and abdominal ultrasonography even though symptoms are not as clear cut as in infants.
Abdominal Pain ; Adhesives ; Appendectomy ; Child ; Humans ; Infant ; Intestinal Volvulus ; Necrosis ; Pneumonia ; Respiratory Insufficiency ; Retrospective Studies ; Vomiting

Down's syndrome (trisomy 21) has been frequently associated with thyroid disease, mainly subclinical hypothyroidism (12.5-32.5%). The occurrence of Downs syndrome in conjunction with hyperthyroidism is rare (0.6-2.5%). The mechanism that Down's syndrome was frequently associated with autoimmune thyroid disease is not clear, but T cell maturation defects and overexpression of chromosome 21 products in Down's syndrome have been suggested. A 19-year-old female was transferred because of generalized weakness. She was born to a 42-year-old mother, She had been suffered from heat intolerance, weight loss, palpitation, dyspnea on exertion and neck swelling and had intermittently taken some medication since her age 9. She had mental retardation (IQ 41) and underdeveloprnent. Exophthalmos, upward-outward slant of palpabral fissures, epicanthal folds, lowset ears, and large, protruding, fissured tongue were identified. Short fifth middle phalanges, clinodactyly and small-sized interventricular septal defect were also detected. Thyroid gland was diffusely enlarged four times the normal size, firm in consistency and had a bruit. Serum T concentration was 7.8ug/dL, T2 306ng/dL, and TSH 0.01ulU/mL. She was positive for thyroid autoantibodies (antimicosomal antibody 1,867 IU/mL, antithyroglobulin antibody 106 IU/mL, and TBII 79.6%). Twenty-four hours radioactive iodine uptake was 64%. Chromosomal analysis with T cell culture stimulated by phytohemagglutinin revealed 47XX, 21 trisomy. Pituitary hormones except TSH were fully stimulated by combined pituitary stimulation. She was finally diagnosed as Down's syndrome with Graves' disease and controlled with use of methimazole.
Adult ; Autoantibodies ; Cell Culture Techniques ; Chromosomes, Human, Pair 21 ; Down Syndrome* ; Dyspnea ; Ear ; Exophthalmos ; Female ; Graves Disease* ; Hot Temperature ; Humans ; Hyperthyroidism ; Hypothyroidism ; Intellectual Disability ; Iodine ; Methimazole ; Mothers ; Neck ; Pituitary Hormones ; Thyroid Diseases ; Thyroid Gland ; Tongue, Fissured ; Trisomy ; Weight Loss ; Young Adult

Background: Despite advancements in heart transplantation for pediatric patients in Korea, the waiting list mortality has not been reported. Therefore, we investigated the waiting list mortality rate and factors associated with patient mortality. Methods: We reviewed the medical records of pediatric patients who were registered for heart transplantation at three major hospitals in Korea from January 2000 to January 2020.All patients who died while waiting for heart transplantation were investigated, and we identified the waiting list mortality rate, causes of mortality and the median survival periods depending on the variable risk factors. Results: A total of 145 patients received heart transplantations at the three institutions we surveyed, and the waiting list mortality rate was 26%. The most common underlying diseases were cardiomyopathy (66.7%) and congenital heart disease (30.3%). The leading causes that contributed to death were heart failure (36.3%), multi-organ failure (27.2%), and complications associated with extracorporeal membrane oxygenation (ECMO) (25.7%). The median survival period was 63 days. ECMO was applied in 30 patients. The different waiting list mortality percentages according to age, cardiac diagnosis, use of ECMO, and initial Korean Network of Organ Sharing (KONOS) level were determined using univariate analysis, but age was the only significant factor associated with waiting list mortality based on a multivariate analysis. Conclusion The waiting list mortality of pediatric heart transplantation candidates was confirmed to be considerably high, and age, underlying disease, the application of ECMO, and the initial KONOS level were the factors that influenced the survival period.

PURPOSE: In radiotherapy for cervix cancer, both 3-dimensioal radiotherapy (3D-CRT) and intensity-modulated radiation therapy (IMRT) could reduce the dose to the small bowel (SB), while the small bowel displacement system (SBDS) could reduce the SB volume in the pelvic cavity. To evaluate the effect of the SBDS on the dose to the SB in 3D-CRT and IMRT plans, 3D-CRT and IMRT plans, with or without SBDS, were compared. MATERIALS AND MEHTODS: Ten consecutive uterine cervix cancer patients, receiving curative radiotherapy, were accrued. Ten pairs of computerized tomography (CT) scans were obtained in the prone position, with or without SBDS, which consisted of a Styrofoam compression device and an individualized custom-made abdominal immobilization device. Both 3D-CRT, using the 4-field box technique, and IMRT plans, with 7 portals of 15 MV X-ray, were generated for each CT image, and prescribed 50 Gy (25 fractions) to the isocenter. For the SB, the volume change due to the SBDS and the DVHs of the four different plans were analyzed using paired t-tests. RESULTS: The SBDS significantly reduced the mean SB volume from 522 to 262 cm3 (49.8% reduction). The SB volumes that received a dose of 10~50 Gy were significantly reduced in 3D-CRT (65~80% reduction) and IMRT plans (54~67% reduction) using the SBDS. When the SB volumes that received 20~50 Gy were compared between the 3D-CRT and IMRT plans, those of the IMRT without the SBDS were significantly less, by 6~7%, than those for the 3D-CRT without the SBDS, but the volume difference was less than 1% when using the SBDS. CONCLUSION: The SBDS reduced the radiation dose to the SB in both the 3D-CRT and IMRT plans, so could reduce the radiation injury of the SB.
Cervix Uteri* ; Female ; Humans ; Immobilization ; Prone Position ; Radiation Injuries ; Radiotherapy ; Uterine Cervical Neoplasms*