PURPOSE: The study aimed to compare the physical activity of hematopoietic stem cell transplantation (HSCT) patients with healthy people (HP). METHODS: The research design of this study was cross sectional and, descriptive study. It was conducted from January 26th to May 22nd, 2013. We matched HSCT patients to HP based on age and gender, and measured physical activity using a pedometer. Patient's clinical information were gathered by the medical record reviews. RESULTS: The number of steps was significantly less in HSCT patients (HSCT: 214 (7~3,373) vs HP: 7,921 (3,180~17,539), p<.001). Physical activity time was significantly less in HSCT patients (HSCT: 840 (680~1,320)min vs HP: 990 (540~1,090)min, p<.001). Step count showed a significant relationship with platelet count (r=.40, p=.004). CONCLUSION: HSCT patients perform very low intensity of physical activities. The study shows the necessity to apply programs to promote physical activities in isolation rooms for patients undergoing HSCT.
Environment, Controlled ; Exercise Test ; Hematologic Diseases ; Hematopoietic Stem Cell Transplantation* ; Humans ; Medical Records ; Motor Activity* ; Platelet Count ; Research Design

PURPOSE: To evaluate the effect of different doses of intravitreal triamcinolone acetonide on diffuse diabetic macular edema. METHODS: In a retrospective study, 44 eyes with diffuse diabetic macular edema were treated with an intravitreal injection of 4 mg (n=12 eyes), 8 mg (n=17) or 25 mg (n=15) of triamcinolone acetonide (TA). Optical coherence tomography, best-corrected logMAR visual acuity and Goldmann tonometry were performed at baseline, 1 week, and 1, 3, 6, 9 and 12 months after treatment. Mean follow-up was 9.8 months (standard deviation=2.3) with a range of 5-12 months. RESULTS: The duration of intravitreal TA effects on macular thickness and visual acuity increased with increasing dosage. An observed increase in intraocular pressure induced by TA was not significantly associated with dosage. CONCLUSIONS: In patients with diffuse diabetic macular edema who receive intravitreal TA, effects may last longer after a dosage of 25 mg, than after lower doses of 8 mg or 4 mg.
Adult ; Aged ; Aged, 80 and over ; Diabetic Retinopathy/*complications/drug therapy/pathology ; Dose-Response Relationship, Drug ; Female ; Follow-Up Studies ; Glucocorticoids/*administration & dosage ; Humans ; Injections ; Intraocular Pressure ; Macular Edema/diagnosis/*drug therapy/etiology ; Male ; Middle Aged ; Retrospective Studies ; Time Factors ; Tomography, Optical Coherence ; Treatment Outcome ; Triamcinolone Acetonide/*administration & dosage ; Visual Acuity ; Vitreous Body

PFAPA (periodic fever, aphthous stomatitis, pharyngitis, cervical adenitis) syndrome is one of the causes of periodic fever in pediatrics with unknown etiology. It is characterized by abrupt onset of fever, malaise, aphthous stomatitis, pharyngitis and cervical adenitis without long-term sequelae. Laboratory findings of this sporadic and nonhereditary syndrome are so non-specific that the diagnosis is based on clinical findings. Oral prednisolone is quite effective in controlling the symptoms. We report a case of a 6-year-old girl who was diagnosed as having PFAPA syndrome after 2 years of episodes, by excluding other disease entities with similar clinical features. The patient was treated with oral prednisolone and her symptoms improved dramatically.
Child ; Diagnosis ; Female ; Fever* ; Humans ; Lymphadenitis ; Pediatrics ; Pharyngitis* ; Prednisolone ; Stomatitis, Aphthous* ; Yemen

OBJECTIVES: The purpose of this study was to evaluate the relationship of nonalcoholic fatty liver and cardiovascular risk factors. METHODS: This study was conducted to investigate the association of nonalcoholic fatty liver and cardiovascular risk factors for adult men (n=2976) and women (n=2442) who were over 19 years old, after excluding the HBsAg(+) or anti-HCV(+) patients and the men and women with increased alcohol intake (men: 40 g/week, women: 20 g/week). RESULTS: Compared with the normal liver subjects, the nonalcoholic fatty liver subjects showed a significantly increased frequency of abnormal systolic blood pressure (> or =120 mmHg), fasting blood sugar (> or =100 mg/dL), total cholesterol (> or =200 mg/dL), triglyceride (> or =150 mg/dL), high density lipoprotein cholesterol (<40 mg/dL), low density lipoprotein cholesterol (> or =130g m/dL) and abdominal obesity in men, and all these measures were significantly increased in the women except for abnormal HDL cholesterol. After adjusting for the body mass index, age, smoking, exercise and a nonalcoholic liver, the odds ratios of an abnormal waist hip ratio were 1.35(95% Confidence Interval=1.05-4.72) in the mild fatty liver, 1.61(1.19-2.18) in the moderate fatty liver, 2.77(1.57-4.92) in the severe fatty liver compared with a normal liver. The adjusted odds ratios for abnormal fasting blood sugar were 1.26(1.03-1.53) in the mild fatty liver, 1.62(1.27-2.06) in the moderate fatty lliver and 1.77(1.12-2.78) in the severe fatty liver. The adjusted odds ratios for abnormal triglyceride were 1.38(1.11-1.72) in the mild fatty liver, 1.73(0.33-2.24) in the moderate fatty liver and 1.91(1.17-3.10) in the severe fatty liver of men. Adjusted odds ratios for abnormal triglyceride were 1.50(1.04-2.15) in mild, 1.71(1.07-2.68) in moderate, 1.81(0.69-4.38) in severe fatty liver of women. CONCLUSIONS: The nonalcoholic fatty liver subjects had more cardiovascular risk factors compared with the normal liver subjects. Thus, prevention and treatment of the nonalcoholic fatty liver is necessary by lifestyle modifications such as restriction of alcohol intake, no smoking, exercise and adequate eating habits.
Adult ; Blood Pressure ; Body Mass Index ; Cardiovascular Diseases/*epidemiology/etiology ; Cholesterol/blood ; Cross-Sectional Studies ; Data Interpretation, Statistical ; Fatty Liver/*complications ; Female ; Humans ; Male ; Middle Aged ; Risk Factors ; Waist-Hip Ratio

BACKGROUND: A correct estimate of the tracheal tube insertion depth can prevent complications, including endobronchial intubation and vocal cord trauma. We evaluated a new topographical method for endotracheal tube positioning relative to the carina, using a well-known prior topographical method for comparison. METHODS: One hundred adult (male 50, female 50) patients were studied. The comparison topographic length (in cm) was measured by adding the distance between the right mouth corner and the right mandibular angle to the distance between the right mandibular angle and the center of the sternal manubrium. The new endotracheal tube insertion depth (in cm) was determined by adding the distance between the right mouth corner and the vocal cords, measured with the endotracheal tube itself, to the distance between the thyroid prominence and the manubriosternal joint, and then subtracting 4 cm. After intubation, the endotracheal tube was positioned properly at the right mouth corner and the endotracheal tube tip was evaluated using a fiberoptic bronchoscope at the carina. RESULTS: The distances from the tip of the endotracheal tube to the carina were not significantly different between the methods in the same gender. However, our method allowed endotracheal tube tip placement between 3 cm and 5 cm, above the carina more frequently than the prior method in males. CONCLUSIONS: The new topographical method can be used as a guide to positioning the endotracheal tubes.
Adult ; Bronchoscopes ; Female ; Humans ; Intubation ; Joints ; Manubrium ; Mouth ; Thyroid Gland ; Vocal Cords

PURPOSE: This study aimed to evaluate the clinical course of childhood immune thrombocytopenia (ITP) and to assess the risk factors for developing chronic ITP. METHODS: The records of 64 children diagnosed with ITP from November 2005 and December 2014 at single center were retrospectively analyzed. RESULTS: The median age at diagnosis and the median platelet count were 1 year (range, 1 month to 15 years) and 9×10⁹/L (range, 0–84×10⁹/L), respectively. No patient experienced severe bleeding. Nineteen children (29.7%) spontaneously recovered their platelet count to ≥100×10⁹/L at a median of 10 days. In total 45 patients (70.3%) received intravenous immunoglobulin (IVIG) as first-line therapy, and showed platelet recovery at 1 week. The final diagnosis of 55 (85.9%) and 9 patients (14.1%) was acute and chronic ITP, respectively. Older age, absence of prior infection and insidious onset of symptoms were significantly associated with the development of chronic ITP. Among the patients who received IVIG, those with platelet count <45×10⁹/L at 1 month after IVIG showed a significantly higher incidence of chronic ITP compared to those with platelet count ≥45×10⁹/L (88.8% vs. 44.4%, P<0.01). CONCLUSION: In most patients, ITP runs a benign course and approximately 86% of them recover within 1 year of their initial diagnosis. The potential impact of the risk factors of chronic ITP on clinical practice needs to be explored and further studies are warranted to determine whether IVIG influences the course of ITP.
Blood Platelets ; Child ; Diagnosis ; Hemorrhage ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Incidence ; Platelet Count ; Purpura, Thrombocytopenic, Idiopathic* ; Retrospective Studies ; Risk Factors

PURPOSE: Long-term survival of biliary atresia patients after Kasai's portoenterostomy is being increasingly reported. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. The authors investigated 5-year survival rates and prognostic factors for survival after Kasai's portoenterostomy using univariate and multivariable methods. METHODS: The authors analyzed 5-year survival rates and prognostic factors in 56 patients who underwent Kasai's portoenterostomy during the period from 1991 to 2005 by the Kaplan-Meier model and Cox proportional hazards model, respectively. Both clinical factors and histological features of the liver and periportal fibrous mass were analyzed as prognostic factors. RESULTS: Younger ages of less than 90 days at surgery, clearance of jaundice and absence of post-Kasai cholangitis in 6 months were predictive of a favorable outcome. A significant difference between the live and the dead was found with regard to an average of 7 or more bile ductules in periportal fibrous mass in 200 HPF (P=0.013). The external diameter of the longest bile ductule in the periportal fibrous mass is not correlated to the prognosis (P=0.49). Independent factors for good prognosis were jaundice-free in 6 months and over 7 bile ductules density in periportal fibrous mass. CONCLUSION: Favorable outcome after Kasai's portoenterostomy for biliary atresia is determined by bilirubin clearance in 6 months after Kasai operation and seven or more bile ductules in periportal fibrous mass. The 5-year post-Kasai survival rate is 77.2%.
Bile ; Biliary Atresia ; Bilirubin ; Cholangitis ; Humans ; Jaundice ; Liver ; Prognosis ; Proportional Hazards Models ; Survival Rate

Interchromosomal insertion of Y chromosome heterochromatin in an autosome was identified in a fetus and a family. A fetal karyotype was analyzed as 46,XX,dup(7)(?q22q21.1) in a referred amniocentesis at 16 weeks of gestation for advanced maternal age. In the familial karyotype analyses for identification of der(7), the mother, the first daughter and the maternal grandmother showed the same der(7) as the fetus's. CBG-banding was positive at 7q22 region of der(7) that indicated inserted material was originated from heterochromatin. The origin of heterochromatic insertion region in der(7) of the fetus and the mother was found in Yq12 region by fluorescent in situ hybridization with a DYZ1 probe. In the specific analysis of Y chromosomal heterochromatic region of ins(7;Y) of the mother, 15 sequence tagged sites from Yp11.3 region including SRY to Yq11.223 region was not detected. Final karyotypes of the mother, the first daughter and the maternal grandmother were reported as 46,XX,der(7)ins(7;Y)(q21.3;q12q12). All female carriers of ins(7;Y) in the family showed normal phenotype and the mother and the maternal grandmother were fertile. A healthy girl was born at term. We report a rare case of familial interchromosomal insertion of Y chromosome heterochromatin detected only in female family members with normal phenotype that was diagnosed prenatally.
Amniocentesis ; Female ; Fetus ; Grandparents ; Heterochromatin* ; Humans ; In Situ Hybridization, Fluorescence ; Karyotype ; Maternal Age ; Mothers ; Nuclear Family ; Phenotype ; Pregnancy ; Prenatal Diagnosis* ; Sequence Tagged Sites ; Y Chromosome*

Recent developments in genome editing technology using meganucleases demonstrate an efficient method of producing gene edited pigs. In this study, we examined the effectiveness of the transcription activator-like effector nuclease (TALEN) system in generating specific mutations on the pig genome. Specific TALEN was designed to induce a double-strand break on exon 9 of the porcine α1,3-galactosyltransferase (GGTA1) gene as it is the main cause of hyperacute rejection after xenotransplantation. Human decay-accelerating factor (hDAF) gene, which can produce a complement inhibitor to protect cells from complement attack after xenotransplantation, was also integrated into the genome simultaneously. Plasmids coding for the TALEN pair and hDAF gene were transfected into porcine cells by electroporation to disrupt the porcine GGTA1 gene and express hDAF. The transfected cells were then sorted using a biotin-labeled IB4 lectin attached to magnetic beads to obtain GGTA1 deficient cells. As a result, we established GGTA1 knockout (KO) cell lines with biallelic modification (35.0%) and GGTA1 KO cell lines expressing hDAF (13.0%). When these cells were used for somatic cell nuclear transfer, we successfully obtained live GGTA1 KO pigs expressing hDAF. Our results demonstrate that TALEN-mediated genome editing is efficient and can be successfully used to generate gene edited pigs.
Animals ; Antigens, CD55/genetics ; Cell Line ; DNA Breaks, Double-Stranded ; Exons/genetics ; Galactosyltransferases/*genetics ; Gene Editing/*veterinary ; Gene Knockout Techniques ; Humans ; Nuclear Transfer Techniques ; Swine ; Transcription Activator-Like Effector Nucleases/*genetics/*metabolism

PURPOSE: This study was designed to confirm whether the paracentric inversions of fetuses and parents may be harmless. MATERIALS AND METHODS: We report 10 cases (0.14%) with paracentric inversions among 7,181 prenatal cases observed during prenatal diagnosis performed at Cheil General Hospital between January 2009 and June 2013. We used cytogenetic GTL- and RBG-banding techniques. RESULTS: Of the 10 cases, nine cases were transmitted from each of the parents, and one case was de novo. Nine cases were phenotypically normal up to one month of age after birth. One case was lost to follow-up. We present prenatal diagnosis and follow-up examination of the fetuses with paracentric inversion. CONCLUSION: Based on our cases, most paracentric inversions are considered to be harmless. The precise identification of paracentric inversions might be clinically important and helpful for genetic counseling.
Amniocentesis ; Chorionic Villi Sampling ; Cytogenetics ; Female ; Fetus ; Follow-Up Studies ; Genetic Counseling ; Hospitals, General ; Humans ; Lost to Follow-Up ; Parents ; Parturition ; Pregnancy ; Prenatal Diagnosis*