• Archives of Craniofacial Surgery
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• v.14 no.1
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• pp.69-72
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• 2013

Lateral eyebrow mass with primary skull lesion are rare in pediatric population. Although epidermoid cyst and dermoid cyst are the most commonly encountered skull lesions in pediatric population, Langerhans cell histiocytosis (LCH) is rarely reported. We report a case of LCH arising from the lateral eyebrow with osteolytic lesion involving the frontal bone. A 5-year-old boy was presented with a hard, fixed mass in his lateral eyebrow. Contrast magnetic resonance imaging revealed inhomogeneous enhancement of the mass with direct invasion of the frontal bone and adjacent dura mater. Under general anesthesia, linear incision at the lateral eyebrow region was made. Intraoperative evaluation revealed hard, fixed and well-defined soft tissue mass. The final extirpated mass was $2.5{\times}2.4cm$ in size, and was accompanied by a $1{\times}1cm$ sized defect on the frontal bone with intact dura mater. The surgical wound was closed primarily by a layer-by-layer fashion. Histologic examination was later performed for definite diagnosis. The histologic examination revealed abnormal proliferation of Langerhans cell with granuloma formation. Radionuclide bone scan and positron emission tomography was taken and revealed free of multi-organ involvement. At 3 months after surgery, natural looking contour at the lateral eyebrow region was observed with no tumor recurrence. Differential diagnosis of the hard and fixed mass at the lateral eyebrow region affecting the primary skull lesion from pediatric population includes epidermoid cyst, dermoid cyst and LCH. Generally, brief physical examination with plain X-ray view can be performed for clinical evaluation, but for a definite diagnosis, contrast MRI may be helpful.

• Journal of Korean Medical Science
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• v.33 no.51
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• pp.324.1-324.6
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• 2018

Oxysterol $7{\alpha}$-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of $3{\beta}$-monohydroxy-${\Delta}^5$-bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol $7{\alpha}$-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.

• Laboratory Medicine Online
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• v.7 no.2
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• pp.83-87
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• 2017

Pseudohypoparathyroidism (PHP) is a rare disorder caused by genetic and epigenetic aberrations in the GNAS complex locus resulting in impaired expression of stimulatory G protein ($Gs{\alpha}$). PHP type Ib (PHP-Ib) is characterized by hypocalcemia and hyperphosphatemia due to renal resistance to the parathyroid hormone, and is distinguished from PHP-Ia by the absence of osteodystrophic features. An 11-yr-old boy presented with poor oral intake and cramping lower limb pain after physical activity. Laboratory studies revealed hypocalcemia, hyperphosphatemia, and increased parathyroid hormone levels. The GNAS complex locus was evaluated using the methylation-specific multiplex ligation-dependent probe amplification (MS-MLPA) assay. Gain of methylation in the NESP55 domain and loss of methylation in the antisense (AS) transcript, XL, and A/B domains in the maternal allele were observed. Consequently, we present a case of PHP-Ib diagnosed using MS-MLPA.

• Journal of health informatics and statistics
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• v.43 no.4
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• pp.282-289
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• 2018

Objectives: The purpose of this study was to investigate factors related to nutrients intakes among young children aged 1-5 years and their mothers. Methods: The study subjects were 621 young children and their mothers from the data of 2nd year (2014) and 3rd year (2015) of the 6th National Health and Nutrition Survey. To find a difference of Recommended Nutrient Intake in young children and their mothers, independent t-test, ANOVA, and Duncan test as a post-hoc analysis were used. For the factors influencing young children Recommended Nutrient Intakes, hierarchical multiple regression analysis was used. Results: The mean score of Recommended Nutrient Intakes was $6.92{\pm}2.17$ in young children and $4.74{\pm}2.06$ in mothers. The Recommended Nutrient Intakes of young children was increased according to gender (boy) (p=0.001), increasing young children age ($p{\leq}0.001$), young children breakfast frequency (p<0.001), mothers age (p<0.001), education level (p=0.020) and mothers lunch frequency (p=0.002) and family income (p=0.007). The Recommended Nutrient Intakes of mothers was increased according to their age (p=0.005), family income (p=0.020) and having breakfast (p=0.038). The factor that influenced young children Recommended Nutrient Intakes was their mothers Recommended Nutrient Intakes (${\beta}=0.21$, p<0.001). Conclusions: Given the results, it is necessary to establish a plan or nutrition education for young children's menu differently depending on their age and gender. Since mothers' Recommended Nutrient Intakes influence their young children's Recommended Nutrient Intakes, it is necessary to provide mothers' with nutrition education about the importance of mothers' nutrition management and right nutrient intakes, and to make customized nutrition education guidelines in consideration of mothers' characteristics.

• Journal of the Korean Society of Mineral and Energy Resources Engineers
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• v.55 no.6
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• pp.564-575
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• 2018

Recently, the possibility of promoting inter-Korean economic cooperation is increasing because the tension between South and North Korea is being reduced. Consequently, the interest in North Korea's mine development projects is growing as one of the aspects of inter-Korean economic cooperation. In the promotion of cooperation in mining development, mine hazard risk management should be considered. However, there is a lack of information pertaining to mine hazards in North Korea. To this end, this study was performed to determine the status of mining-related hazards in 12 mines in North Korea by using the image analysis feature of Google Earth. From the results obtained, we observed some mining-related hazards such as tailing dam failures, yellow boy phenomenon, and land subsidence.

• Childhood Kidney Diseases
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• v.23 no.2
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• pp.111-115
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• 2019

Vitamin D dependent rickets type 1A (VDDR1A) is an autosomal recessive disorder caused by mutations in CYP27B1. Clinical findings are growth retardation, hypotonia, muscle weakness, hypocalcemic seizures, and radiological features of rickets. We aimed to present the VDDR1A case with a genetic study of CYP27B1. The 14-month-old boy was admitted to the hospital due to a seizure. Serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone (PTH), 25(OH) vitamin D, and 1,25(OH)2 vitamin D values were 5.1 mg/dL, 3.7 mg/dL, 705 IU/L, 429 pg/mL, 24.9 ng/mL, and 8.8 pg/mL, respectively. Radiological study showed cupping and fraying of the distal ulna and radius. The molecular genetic study revealed that the patient had a compound heterozygous mutation, $Phe443Profs^*24$ and c.589+1G>A, in CYP27B1. Genetic analysis of the family members presented that the mother was heterozygous for the mutation c.589+1G>A, and that the father was heterozygous for $Phe443Profs^*24$. The patient was treated with calcium lactate and calcitriol. Until now, six Korean patients with VDDR1A have been studied. Including this case, Korean patients with VDDR1A were found to have only three different mutations in 14 alleles, indicating that the mutation in the CYP27B1 gene is homogeneous in the Korean population.

• Korean Journal of Human Ecology
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• v.6 no.2
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• pp.1-12
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• 2003

This study is designed to analyse the difference between consciousness and behaviors for environmental preservation dependant on independent variables and to identify factors that affect the behaviors of environmental preservation. For this study, a questionnaire that included measurements of environmental preservation consciousness and behaviors was produced as a studying tool. Targets were 520 boy and girl students in the second grade of two high schools in Gwangju and three high schools in small districts of Chonnam Province and 482 questionnaires were distributed for the analysis. The data collected was analysed using SAS Program Package to obtain frequency, percentage, standard deviation, and correlation and T-Test One-way ANOVA, Duncan's M411ip1e Range Test, Multiple Regression were performed. The results of the analyses are as follows : 1. Overall, environmental preservation consciousness is higher than preservation behaviors. Interest in air pollution is the highest in both environmental preservation consciousness and behaviors and it is low in recycling. 2. The environmental preservation consciousness has a significant difference dependant on sex, regions, order of birth, educational background of parents and income level. the performing degree of environmental education, consciousness about kinds of environmental preservation, and whether the learning of theory and practice of environmental education is performed. 3. The environmental preservation behaviors has a significant difference dependant on regions, educational background of mother, mother's job, income level, acquiring of information, performing degree of environmental education and consciousness of kinds of environmental education. 4. Variables that affect environmental preservation behavior are environmental preservation consciousness, acquiring information, performing degree of environmental education and awareness of environmental preservation. Their power of explanation is 15%.

• Clinical and Experimental Reproductive Medicine
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• v.48 no.3
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• pp.268-272
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• 2021

Bone marrow-derived cell (BMDC) therapy has numerous applications as potential biological cells for use in regenerative medicine. Here, we present an original case of endometrial atrophy associated with genital tuberculosis in a woman who achieved a live birth with BMDC. This 27-year-old woman came to our center with endometrial atrophy and primary infertility. She had a past history of genital tuberculosis and amenorrhea. Her husband's semen quality was normal. The patient was counseled for hysteroscopy due to thin endometrium and advised in vitro fertilization (IVF) with donor eggs in lieu of poor ovarian reserve. Several attempts of IVF with hormone replacement therapy (HRT) were made, but the desired thickness of the endometrium was not achieved. Uterine artery injection of BMDC through interventional radiology was given, followed by HRT for three months, which resulted in improved endometrium. This was subsequently followed by IVF with donor egg. The treatment resulted in the conception and delivery of a 3.1-kg baby boy through lower segment caesarean section with no antenatal, intranatal or postnatal complications. Recently, there has been massive interest in stem cells as a novel treatment method for regenerative medicine, and more specifically for the regeneration of human endometrium disorders like Asherman syndrome and thin endometrium, which was the reason behind using this strategy for treatment.

• Journal of The Korean Society of Integrative Medicine
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• v.9 no.2
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• pp.105-108
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• 2021

Background : Hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome is a rare, autosomal recessive metabolic disorder which is caused by genetic mutations that disrupt the urea cycle. It is characterized by variable clinical presentation and the age of onset. Patients may present with gait disturbance and progressive paraplegia and muscle tightness in the lower extremities. The use of botulinum toxin in metabolic disease has rarely been discussed. We describe a case of a 14-year-old-boy with HHH syndrome, who presented with a several - month history of gait disturbance and lower extremity weakness. Case presentation : A 14-year old male had a history of recurrent upper respiratory tract infections, occasional vomiting, loss of appetite, and general weakness, all of which started since he was 10 months old. He was diagnosed with HHH syndrome at one year of age. At the age of 14, he was referred for the assessment and treatment of his gait disturbance and aggravated weakness of the lower extremities. Brain MRI, electrodiagnostic study and blood test were performed to exclude any lesions related to neurologic dysfunction. Botulinum toxin type A were injected into muscles of adductor longus, adductor magnus, lateral and medial hamstring, and lateral and medial gastrocnemius muscle heads under needle electromyography guidance to reduce lower limb spasticity. Intensive physical therapy including gait training and stretching exercise of adductor and calf muscles were also provided. After intensive physical therapy and botulinum toxin injection to reduce lower limb spasticity, he was able to ambulate for 20 meters independently without any walking aids. There were no adverse events after the injection. Conclusion : Botulinum toxin injection is a safe and effective therapy for patients with HHH syndrome who suffer from gait disturbance.

• The Mathematical Education
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• v.58 no.3
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• pp.337-346
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• 2019

The purpose of this paper is to investigate the differences of the mathematics anxiety and mathematical achievement of high school students according to gender and grade, and to find out which mathematics anxiety causes have more influence on mathematical achievement and how much it is. The subjects of this study consist of 459 students selected for a class of unit, in high schools located in Seoul, Korea. Huh(1996)'s Mathematics Anxiety Scale was used. The collected data were analyzed by using the 24.0 SPSS program. The data were also tested by using the t-test, correlation and multiple regression. The major results of this study were as follows: Firstly, there was no difference in mathematics score according to gender, but mathematics anxiety was higher in girl students. Mathematics score and mathematics anxiety have significantly related each other. Boy students' mathematics scores were significantly explained by interest, Mathematical Achievement factor, and mathematical confidence factor. For girl students, mathematics achievement factor, interest were the significant predictors. Secondly, mathematical anxiety and mathematics scores were correlated in the first and third grades, and the variables that predict mathematics scores significantly in all grades were interest.