PURPOSE: To quantify the incidence of asymptomatic incidental trochlear calcifications and to describe their clinical features. METHODS: We retrospectively reviewed orbital computed tomography (CT) scans of 216 patients to identify the presence of trochlear calcifications. We analyzed the prevalence, age distribution, and gender preponderance of trochlear calcifications. We also examined age-specific prevalence rates for trochlear calcifications, as well as their relationship to systemic disease. RESULTS: The mean age of patients was 26.8 years. Trochlear calcifications were observed in 35 (16%) of the 216 patients, and 18 of the 35 patients had bilateral calcifications. The rate of trochlear calcification was higher in males; 32 (20.9%) of 153 male patients had trochlear calcifications, compared with 3 (4.8%) of 63 female patients. Age, hypertension, diabetes mellitus, and thyroid disease were not significantly associated with the incidence of trochlear calcifications. CONCLUSIONS: Incidental asymptomatic orbital calcification is more commonly observed on CT images than we expected and occurs predominantly in male patients. Understanding this to be a relatively common, benign finding may help us to rule out foreign bodies and other pathologic conditions.
Adult ; Calcinosis/*epidemiology/radiography ; Female ; Humans ; Incidence ; Male ; Orbital Diseases/*epidemiology/radiography ; Republic of Korea/epidemiology ; Retrospective Studies ; Sex Distribution ; Tomography, X-Ray Computed

PURPOSE: To evaluate the clinical course of visual acuity and foveal thickness in the idiopathic epiretinal membrane (ERM) after a vitrectomy with the use of triamcinolone. METHODS: We retrospectively reviewed the records of 30 patients (30 eyes) with ERM that were treated by vitrectomy from 2004 to 2008. Visual acuity and foveal thickness from optical coherence tomography imaging was obtained preoperatively and at every postoperative follow-up visit. RESULTS: Visual acuity improved by two or more lines of vision in 30%, 50%, 60%, and 70%, and stayed the same within +/-1 line in 47%, 50%, 40%, and 30% at one month, three months, five months, and seven months after surgery. Twenty-three percents of the subjects deteriorated by two or more lines of vision within one month after surgery. None of the subjects had reduced vision three months after surgery. Foveal thickness decreased significantly after surgery. The mean thickness was 409.7+/-107.9 microm before surgery and 288.6+/-66.1 microm seven months after surgery. Parameters which were significantly correlated with the final visual acuity included preoperative visual acuity (0.683), preoperative foveal thickness (0.544), and final foveal thickness (0.643) (p<0.005). CONCLUSIONS: Foveal thickness and visual acuity improved until seven months after the vitrectomy in patients with idiopathic ERM. Preoperative visual acuity, foveal thickness, and final foveal thickness had a significant correlation with the final visual acuity.
Aged ; Epiretinal Membrane/pathology/*surgery ; Female ; Follow-Up Studies ; Fovea Centralis/*pathology ; Humans ; Male ; Middle Aged ; Postoperative Period ; Retrospective Studies ; Tomography, Optical Coherence ; Treatment Outcome ; Visual Acuity ; Vitrectomy/*methods

Objectives: This study examined the level of depression and anxiety and the related factors, especially the knowledge and attitudes towards dementia, of people caring for a family member with dementia. Methods: Data on the demographics, care burdens, and clinical characteristics of dementia patients and their family caregivers were collected. The Center for Epidemiologic Studies Depression Scale (CES-D) and Korean Screening Tool for Anxiety disorders (K-ANX), Dementia Knowledge Scale (DKS), and Dementia Attitudes Scale (DAS) were performed. This study investigated whether depression and anxiety of caregivers differed according to the caregivers, patients, and the burden of caregivers. Multiple regression analysis was performed to investigate the potential factors that may influence the psychological symptoms in family caregivers. Results: There were 135 respondents. The scores of CES-D and K-ANX were 19.18±12.05 (probable depression) and 11.48±8.88 (mild anxiety), respectively. There was a significant difference in the degree of depression according to the level of education (F=4.14, p<0.05), the severity of dementia (F=3.63, p<0.05), and cohabitation with patients with dementia (t=2.07, p<0.05). On the other hand, the difference in the degree of anxiety was not significant depending on the stratified potential factors. The degree of depression in caregivers was positively associated with severe dementia (β=0.252, p<0.01) and negatively associated with the DAS score (β= -0.392, p<0.001). Anxiety was only inversely affected by the DAS score (β=-0.369, p<0.001). Conclusion This study shows that family caregivers of people with dementia experience high levels of depression and anxiety, which are influenced by the patient’s severity of dementia and the caregiver’s attitude toward dementia.

PURPOSE: Liver function test abnormalities have been reported frequently in patients receiving total parenteral nutrition (TPN). In adults, it is known that liver complications decrease with the use of cyclic parenteral nutrition (CPN), especially if the shift to cycling was not too late. However, there are few studies about the effects of cycling on liver injury in children beyond the neonatal period. The aim of this study is to evaluate the effect of the early use of CPN on total parenteral nutrition induced hepatic dysfunction. METHODS: Twelve sets of CPN in 11 children (2 months to 17 years) were included in this study. Data on underlying diseases, age, length of time on TPN, macronutrient intake, complications, and biochemical parameters were collected from clinical records. All children had received CPN in the early period of persistent transaminase elevation or cholestasis complicated by previous continuous PN. The duration of infusion off-time in CPN was 2 hours in patients less than 3 months of age and 4 hours in the older children. RESULTS: All 12 cases showed elevated aminotransferase and 5 of them also showed cholestasis. Serum total bilirubin concentration was normalized in all 5 cases with median periods of 8 days (p<0.05) after initiation of CPN. ALT either decreased significantly or was normalized in all cases with median periods of 30 days (p<0.05) on CPN. The CPN was well tolerated without significant complication except for one case of hyperglycemia. CONCLUSION: The early use of cyclic parenteral nutrition had a beneficial effect in improving hepatic dysfunction complicated by TPN in children.
Adult ; Bilirubin ; Child ; Cholestasis ; Humans ; Hyperglycemia ; Liver ; Liver Function Tests ; Parenteral Nutrition* ; Parenteral Nutrition, Total*

PURPOSE: A prospective, controlled trial was conducted to evaluate growth, bone mineralization, and nutritional status receiving preterm human milk supplemented with a newly formulated Maeil human milk fortifier. METHODS: Twenty five fortified human milk-fed and preterm formula-fed infants with a birth weight < 1, 800 g and gestational age <35 weeks, who were born at Ajou University Hospital from March, 2003 through August, 2004 were studied. Growth, biochemical indices of bone mineralization, feeding tolerance, morbidity and wrist X-ray were assessed serially. Total body bone mineral density was measured by dual energy X-ray absorptiometry at 2 and 5months of age. RESULTS: There were no differences in growth, including weight, height and head circumference, between two groups. Serum Ca, P, ALP and other biochemical indices were similar. Although low grade rickets (grade I and II) were occasionally found on wrist X-ray, the rate of occurrence and severity were similar. The bone mineral densities of both group showed no difference. CONCLUSION: The fortified human milk-fed infants and preterm formula-fed infants showed no difference in growth, and bone mineralization. This newly formulated Maeil human milk fortifier can be safely used in preterm infants.
Absorptiometry, Photon ; Birth Weight ; Bone Density ; Calcification, Physiologic* ; Gestational Age ; Head ; Humans* ; Infant ; Infant, Newborn ; Infant, Premature* ; Milk, Human* ; Nutritional Status ; Prospective Studies ; Rickets ; Wrist

PURPOSE: To evaluate the efficacy of transcatheter arterial embolization(TAE) in children with blunt splenicinjury. MATERIALS AND METHODS: The results of transcatheter splenic arterial embolization in nine children whosuffered splenic injury after blunt abdominal trauma were retrospectively studied. This injury was demonstrated byCT, and the findings were evaluated according to the classification of Mirvis et al. ; two patients were grade 3and seven were grade 4. All were carefully observed in intensive care before embolization. TAE was performed if apatient satisfied the following criteria : (1) transfusion and/or fluid replacement required to maintainhemodynamic stability ; or (2) rapid Hb/Hct decrease ; or (3) both. Splenic function was subsequently estimatedaccording to the results of 99mTc-sulfur colloid scintigraphy and/or CT scanning. RESULT: TAE was suscessful inall nine children. Two were embolized with a coil only, three with gelfoam, and four with gelfoam and a coil.Seven were embolized in the main trunk of the splenic artery and others in both the main trunk and its branches.Splenic function was preserved in all nine children, during follow-up, none suffered rebleeding. CONCLUSION: TAEof the splenic artery can be a safe and effective nonsurgical approach to the management of blunt splenic injuryin children, and can preserve splenic function.
Child* ; Classification ; Colloids ; Follow-Up Studies ; Gelatin Sponge, Absorbable ; Humans ; Critical Care ; Radionuclide Imaging ; Retrospective Studies ; Splenic Artery ; Tomography, X-Ray Computed

Biliary atresia (BA) is the major cause of cholestasis and the leading indication for liver transplantation (LT). However, the incidence of BA in Korea has not been reported. The aim of this study was to investigate the incidence and clinical outcomes of BA in Korea. We used the Korean universal health insurance database and extracted data regarding BA patients younger than 18 years of age admitted between 2011 and 2015. The incidence of BA was calculated by dividing the number of BA patients by the number of live births. Two hundred forty infants were newly diagnosed with BA. A total of 963 BA patients younger than 18 years of age were followed up for 5 years. The overall incidence of BA was 1.06 cases per 10,000 live births. The incidence of BA was 1.4 times higher for female patients than for male patients. Additionally, significant seasonal variation was observed; in particular, the incidence of BA was 2 times higher from June through August than from December through February. Congenital anomalies were found in 38 of 240 patients (15.8%). Congenital heart diseases were major associated congenital anomalies (6.3%). Several complications developed during the study period, including cholangitis (24.0%), varix (6.2%), and gastrointestinal bleeding (4.4%). Three hundred and one of the 963 BA patients under 18 years of age (31.3%) received LT for BA. The incidence of BA is higher in Korea than that in Western countries. We also report significant gender-associated differences and seasonal variation with respect to the incidence of BA.

Acrodysostosis is an extremely rare disorder characterized by short fingers and toes with peripheral dysostosis, nasal hypoplasia, and mental retardation. We report a 16-year-old Korean boy with acrodysostosis who had characteristic clinical features and cervical spine stenosis manifested by neurologic symptoms. On presentation, he complained of difficulty in raising his arms, and suffered from intermittent pain and weakness in both upper extremities. He had short stature and dysmorphic facial features, including a broad, depressed nasal bridge, small, upturned nose, bilateral epicanthal folds, and mild hypertelorism. Moderate mental retardation and sensorineural hearing loss in both ears were also present. Radiological findings included broad, short metacarpals and phalanges with cone-shaped epiphyses, bilateral Madelung deformities, hypertrophied first metatarsals, and thickening of the calvarium. Magnetic resonance imaging findings included stenosis of the cervical spine, platybasia with compression into the cervicomedullary junction, and downward displacement of the cerebellar tonsils. Here, we report a case of acrodysostosis with symptoms and signs of cervical spinal stenosis first in Korea. If it is diagnosed in the early stages, possible life-threatening complications, including spinal canal stenosis, can be managed properly and permanent neurologic sequelae might be avoided. Therefore, it is important to consider acrodysostosis in the differential diagnosis of peripheral dysostosis.
Adolescent ; Arm ; Congenital Abnormalities ; Constriction, Pathologic ; Diagnosis, Differential ; Displacement (Psychology) ; Dysostoses ; Ear ; Epiphyses ; Fingers ; Growth Disorders ; Hearing Loss, Sensorineural ; Humans ; Hypertelorism ; Intellectual Disability ; Korea ; Magnetic Resonance Imaging ; Metacarpal Bones ; Metatarsal Bones ; Neurologic Manifestations ; Nose ; Osteochondrodysplasias ; Palatine Tonsil ; Platybasia ; Skull ; Spinal Canal ; Spinal Stenosis ; Spine ; Toes ; Upper Extremity

Multiple epiphyseal dysplasia (MED) is a clinically and genetically heterogeneous chondroplasia, characterized by delayed development of the ossification centers and, deformities of the extremities that involve only the epiphysis and result in mild short stature. Mutations in the cartilage oligomeric matrix protein (COMP) gene are most commonly found, and most of the mutations are located in the calmodulin-like repeats and the C-terminal domain. We report a Korean kindred of?12 family members with MED in four generations who were found to have a novel mutation in the COMP gene. A pedigree showed early onset osteoarthritis requiring arthroplasty that was an autosomal dominant inherited trait. Radiological examinations demonstrated the presence of osteochondral defects in the medial femoral condyles, and the knee and hip joints showed variable degrees of precocious degenerative changes. Mutation analysis of the COMP gene in the proband and five other affected family members identified a novel missense mutation, c.1280G>C (p.Gly427Ala) in exon 12, which was not found in three unaffected family members. Direct sequencing of the COMP gene may yield pathogenic mutations in dominantly inherited MED cases, and may provide opportunities of carrier detection among high-risk family members, leading to genetic counseling for early diagnosis and intervention before the onset of complications.
Achondroplasia ; Arthroplasty ; Cartilage ; Congenital Abnormalities ; Early Diagnosis ; Epiphyses ; Exons ; Extracellular Matrix Proteins ; Extremities ; Family Characteristics ; Genetic Counseling ; Glycoproteins ; Hip Joint ; Humans ; Knee ; Mutation, Missense ; Osteoarthritis ; Osteochondrodysplasias ; Pedigree

The effects of deliberate hypotension by sodium nitroprusside (SNP) on the cardiovascular system and dosage of SNP were studied in 10 patients undergone Cotrel-Dubousset instrumentation for the operative treatment of scoliosis, spinal stenosis or herniated intervertebral disc. Mean arterial pressure decreased significantly (p<0.05), but cardiac output, central venous pressure and sytemic vascular resistance showed no significant change, The correlations between average dose of SNP and age, body weight, and age to weight ratio were r= -0.4 (p>0.05), r= -0.2 (p>0.05), and r= - 0.25 (p>0.05) respectively. The amount of whole blood transfused during the operation was 5. 3+/-1 .7 units. Hemoglobin and hematocrit decreased significantly (p<0.05) from 11.3+/-1.3g/dl and 33.7+/-4.1% to 10.1+/-1.5g/dl and 30+/-4.1% during operation.
Anesthesia* ; Arterial Pressure ; Body Weight ; Cardiac Output ; Cardiovascular System* ; Central Venous Pressure ; Halothane* ; Hematocrit ; Humans ; Hypotension* ; Intervertebral Disc ; Nitroprusside* ; Scoliosis ; Sodium* ; Spinal Stenosis ; Vascular Resistance