We describe two cases of primary thyroid lymphoma, involving 64-and 74-year-old women who presented with arapidly growing palpable mass in the anterior neck. In both patients, plain radiographs of this region re-vealedtracheal displacement and soft tissue mass, and CT scans demonstrated the presence of a large soft tis-sue masswith homogeneously low attenuation, predominantly located in the unilateral lobe of the thyroid g-land. Within themasses there was no calcification, necrosis, or hemorrhage, though in one patient the trachea was involvedposteriorly by the mass Both masses appeared as cold nodules on RI scan, and in both patients, the final diagnosiswas diffuse large cell type non-Hodgkin's lymphoma with a background of Hashimoto's thyroiditis.
Aged ; Female ; Hemorrhage ; Humans ; Lymphoma* ; Lymphoma, Non-Hodgkin ; Neck ; Necrosis ; Thyroid Gland ; Thyroiditis ; Tomography, X-Ray Computed ; Trachea

Here, we report early treatment outcomes of gene therapy for early onset retinitis pigmentosa (RP) (Leber congenital amaurosis) associated with biallelic RPE65 mutation in a 30-year-old female patient. Initially, her visual acuity (VA) was 20/200, and her visual field (VF) was severely constricted to the center in the left eye. Her electroretinography showed nearly extinct signals. Full-field stimulus threshold test (FST) revealed diminished dark-adapted light sensitivity. Voretigene neparvovec-rzyl (VN) is the first in vivo viral gene therapy agent to be approved. At 3 months after subretinal injection of VN in the left eye, VA, VF, and FST showed sustained improvement. She did not exhibit any signs of adverse effects from the treatment. Gene therapy for RP proved to be an effective and safe treatment in an advanced case of RPE65-associatied early onset RP.

Trisomy 1 is a rare chromosomal anomaly and has never been reported in Korea. Clinical features of trisomy 1 include macrocephaly, prominent forehead, flat nasal bridge, low set ears, and micrognathia, all of which result in a very distinguishable facial structure. A child with trisomy 1 also suffers from mental retardation and/or developmental delays. In this case report, the child was diagnosed with de novo trisomy 1 without receiving any treatment until visiting our hospital. The child suffered from foot and ankle deformities, leading her unable to stand independently. Here we report the surgical treatment and rehabilitation treatment that enabled the child to walk independently.
Ankle ; Child* ; Congenital Abnormalities ; Ear ; Foot ; Forehead ; Humans ; Intellectual Disability ; Korea ; Megalencephaly ; Micrognathism ; Orthopedic Procedures ; Rehabilitation* ; Trisomy

OBJECTIVE: To analyze speech and linguistic features in children with articulation disorder characterized by consonant and vowel phonological errors. METHODS: Between February 2007 and June 2015, 117 children who showed articulation disorder were selected for the study. Based on comprehensive speech and language assessments, the subjects were classified into articulation dysfunction (AD), or AD overlapping with language delay. Detailed information of articulation, including percentage of consonants correct (PCC) and normal percentage of variable consonants derived from the Assessment of Phonology and Articulation for Children test, were compared between the two groups. RESULTS: Totally, 55 children were diagnosed as AD and 62 as AD with language delay. Mean PCC was not significantly different between the two groups. In both groups, the acquisition order of consonants followed the universal developmental sequence. However, differences were observed in the nasal & plosive consonants abnormality between the two groups. When adjusted to their delayed language level in AD with language delay group, 53% of children had appropriate articulation function for their expressive language level. CONCLUSION: Speech and linguistic characteristics in children with articulation disorder were variable. Therefore, comprehensive assessment is required in children with inaccurate pronunciation, and a proper treatment plan based on the results of assessment should be followed.
Articulation Disorders* ; Child* ; Humans ; Language Development Disorders ; Linguistics*

OBJECTIVE: To investigate motor and cognitive developmental profiles and to evaluate the correlation between two developmental areas and assess the influencing factors of the developmental process in children with Down syndrome (DS). METHODS: Seventy-eight children with DS participated in this study. The medical history was taken and motoric milestone achievements recorded. The Korean Wechsler Preschool and Primary Scale of Intelligence (K-WPPSI) and Bayley Scales of Infant Development-II (BSID-II) were administered. Subjects were divided into severe motor delay group (severe group) and typical motor delay group (typical group). RESULTS: Children with DS follow the same sequence of motor development and generally displayed double times of acquisition of developmental milestones compared with healthy children. Furthermore, having surgery for associated complications showed negative influence to the motor development. Almost of all children with DS showed moderate degree of intellectual disability and motor and cognitive development do not seem to correlate one another. CONCLUSION: Surgery of associated complications can be negatively related to motor development. However, early motor development did not have any significant effects on the achievement of later cognitive functioning.
Child* ; Cognition ; Down Syndrome* ; Humans ; Infant ; Intellectual Disability ; Intelligence ; Weights and Measures

OBJECTIVE: To investigate the disability registration state of children with cerebral palsy (CP) in Korea. METHODS: Based on the National Health Information Database, the disability registration state was examined for brain lesion disability and other possible complicated disabilities accompanying brain disorder in children diagnosed with CP aged up to 5 years old who were born between 2002 and 2008. RESULTS: Of children diagnosed with CP, 73.1% were registered as having brain lesion disability for the first time before they turned 2 years old. The younger the children, the more likely they will have 1st and 2nd degree disability. However, when the age of children is increased, such likelihood is decreased. The percentage of children registered as having overlapping disabilities was 7%–20%. CONCLUSION: It is important to establish a more accurate standard to rate disability and provide national support systems for children with CP with various severities and multiple disabilities. By reorganizing the current disability registration system for pediatric brain lesions, the system could serve as a classification standard to provide medical and social welfare services.
Brain ; Brain Diseases ; Cerebral Palsy* ; Child* ; Classification ; Disabled Children ; Humans ; Korea* ; Registries ; Social Welfare

The purpose of this study was to evaluate the viability of periodontal ligament cells of rat teeth after low-temperature preservation under high pressure by means of MTT assay, WST-1 assay. 12 teeth of Sprague-Dawley white female rats of 4 week-old were used for each group. Both side of the first and second maxillary molars were extracted as atraumatically as possible under tiletamine anesthesia. The experimental groups were group 1 (Immediate extraction), group 2 (Slow freezing under pressure of 3 MPa), group 3 (Slow freezing under pressure of 2 MPa), group 4 (Slow freezing under no additional pressure), group 5 (Rapid freezing in liquid nitrogen under pressure of 2 MPa), group 6 (Rapid freezing in liquid nitrogen under no additional pressure), group 7 (low-temperature preservation at 0degrees C under pressure of 2 MPa), group 8 (low-temperature preservation at 0degrees C under no additional pressure), group 9 (low-temperature preservation at -5degrees C under pressure of 90 MPa). F-medium and 10% DMSO were used as preservation medium and cryo-protectant. For cryo-preservation groups, thawing was performed in 37degrees C water bath, then MTT assay, WST-1 assay were processed. One way ANOVA and Tukey HSD method were performed at the 95% level of confidence. The values of optical density obtained by MTT assay and WST-1 were divided by the values of eosin staining for tissue volume standardization. In both MTT and WST-1 assay, group 7 (0degrees C/2 MPa) showed higher viability of periodontal ligament cells than other group (2-6, 8) and this was statistically significant (p < 0.05), but showed lower viability than group 1, immediate extraction group (no statistical significance). By the results of this study, low-temperature preservation at 0degrees C under pressure of 2 MPa suggest the possibility for long term preservation of teeth.
Anesthesia ; Animals ; Baths ; Cryopreservation ; Dimethyl Sulfoxide ; Eosine Yellowish-(YS) ; Female ; Freezing ; Humans ; Molar ; Nitrogen ; Periodontal Ligament ; Rats ; Tiletamine ; Tooth

Effect of shift work on worker's health, family and social life was investigated at a automobile manufacturing plant in Inchon. In total, 2488 shift workers and 599 non-shift workers completed self administered questionnaire in their sleep pattern, subjective gastrointestinal symptoms, prevalent chronic disease status, general well-being schedule, family and social life. More shift workers complained of sleep disturbance, poor sleep quality, tiredness at awakening, and sleepiness in work than non-shift workers. More shift workers also complained of gastrointestinal disturbance than non-shift workers. Worker's mental health was assessed by General Well-Being Schedule(GWB) questionnaire developed for the U.S. Health and Nutrition Examination Surveys(HANES I). The percentage of severely distressed shift workers was significantly higher than that of non-shift workers(p<0.01). Among subscores in General Well-Being Schedule, anxiety, depression, positive well-being, vitality, and general health subscale of shift workers were lower than those of non-shift workers (p<0.05). In terms of family and social life, there was no significant difference between two groups. Multiple logistic regression analysis was done for discrete variables which showed statistically significant difference between shift and non-shift group. The variables included in analysis were sleep disturbance symptoms, gastrointestinal complaints, and distress level calculated from GWB score. Age, tenure, smoking, alcohol drinking, and exercise were adjusted as confounding factors and odds ratios for above symptoms due to shift work were calculated. Odds ratios (ORs) for sleep disturbance symptoms ranged from 0.52 to 3.59. ORs for gastrointestinal complaints ranged from 1.19 to 1.34. OR for distress level was 1.31. We concluded that shift workers are suffered from physical and psychological ailments due to shift work and interventional methods for preventing worker's health from adverse effects of shift work are needed.
Alcohol Drinking ; Anxiety ; Appointments and Schedules ; Automobiles* ; Chronic Disease ; Depression ; Family Health* ; Humans ; Incheon ; Logistic Models ; Mental Health ; Odds Ratio ; Plants* ; Questionnaires ; Smoke ; Smoking

Astigmatism ; Coma ; Corneal Topography ; Corneal Wavefront Aberration ; Humans ; Linear Models ; Lotus ; Myopia ; Refractive Surgical Procedures ; Retrospective Studies ; Visual Acuity

BACKGROUND: The aim of this study was to investigate the current state of the assistive devices and appliances provision system for cerebral palsy patients registered with brain disability. METHODS: From 2003 to 2013, we analyzed the records of cerebral palsy patients who had assistive devices and appliances provisioned at least once in their lives based on National Health Information Database. Patients with cerebral palsy were divided into three groups: infants and toddlers, school age and adolescence, and adults. RESULTS: Data on short leg plastic orthoses, ankle joint orthoses, and wheelchair were mainly analyzed. The types of ankle joint orthoses divided into three categories: limited, 90° limited, and Klenzac. Limited ankle joint orthoses was most frequently supported of the three in all age groups. Powered wheelchair and scooter were most supported to adult patients. When the re-supply duration was evaluated, the duration was suitable to the duration on guideline of regulation of re-supplement according to the related laws in adult patients but not in infants/toddlers and school age/adolescence as the actual re-supplement duration was much shorter than the reference value. CONCLUSION: This study confirmed the pattern of assistive devices and appliances supply differed depending on the age of cerebral palsy patients.
Adolescent ; Adult ; Ankle Joint ; Brain ; Cerebral Palsy ; Foot Orthoses ; Humans ; Infant ; Jurisprudence ; Korea ; Leg ; Orthotic Devices ; Plastics ; Reference Values ; Self-Help Devices ; Wheelchairs