• Clinical and Experimental Pediatrics
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• v.53 no.10
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• pp.872-879
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• 2010

Obstructive sleep apnea (OSA) in children is a frequent disease for which optimal diagnostic methods are still being defined. Treatment of OSA in children should include providing space, improving craniofacial growth, resolving all symptoms, and preventing the development of the disease in the adult years. Adenotonsillectomy (T&A) has been the treatment of choice and thought to solve young patient's OSA problem, which is not the case for most adults. Recent reports showed success rates that vary from 27.2% to 82.9%. Children snoring regularly generally have a narrow maxilla compared to children who do not snore. The impairment of nasal breathing with increased nasal resistance has a well-documented negative impact on early childhood maxilla-mandibular development, making the upper airway smaller and might lead to adult OSA. Surgery in young children should be performed as early as possible to prevent the resulting morphologic changes and neurobehavioral, cardiovascular, endocrine, and metabolic complications. Close postoperative follow-up to monitor for residual disease is equally important. As the proportion of obese children has been increasing recently, parents should be informed about the weight gain after T&A. Multidisciplinary evaluation of the anatomic abnormalities in children with OSA leads to better overall treatment outcome.

• Clinical and Experimental Pediatrics
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• v.57 no.3
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• pp.101-109
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• 2014

The use of glucocorticoids (GCs) in the perinatal period is suspected of being associated with adverse effects on long-term neurodevelopmental outcomes for preterm infants. Repeated administration of antenatal GCs to mothers at risk of preterm birth may adversely affect fetal growth and head circumference. Fetal exposure to excess GCs during critical periods of brain development may profoundly modify the limbic system (primarily the hippocampus), resulting in long-term effects on cognition, behavior, memory, co-ordination of the autonomic nervous system, and regulation of the endocrine system later in adult life. Postnatal GC treatment for chronic lung disease in premature infants, particularly involving the use of dexamethasone, has been shown to induce neurodevelopmental impairment and increases the risk of cerebral palsy. In contrast to studies involving postnatal dexamethasone, long-term follow-up studies for hydrocortisone therapy have not revealed adverse effects on neurodevelopmental outcomes. In experimental studies on animals, GCs has been shown to impair neurogenesis, and induce neuronal apoptosis in the immature brains of newborn animals. A recent study has demonstrated that dexamethasone-induced hypomyelination may result from the apoptotic degeneration of oligodendrocyte progenitors in the immature brain. Thus, based on clinical and experimental studies, there is enough evidence to advice caution regarding the use of GCs in the perinatal period; and moreover, the potential long-term effects of GCs on brain development need to be determined.

• The Journal of Internal Korean Medicine
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• v.10 no.1
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• pp.53-64
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• 1989

In an attempt to investigate the current of clinical researches on spleen yang or vital energy deficiency syndrome, the results were as follows. 1. It is possible to occure spleen deficiency syndrome which come from genetic factor. 2. The absorption disturbance in spleen deficiency syndrome can be likely caused by gastrointestinal mucosa injury, disorder of vagus nerve funtion and impairment of excretion of exocrine gland in pancreas. 3. Owing to the failure of tansporting and converting funtion of spleen, minerals, hematogenic substance and nutritional substance are scanty and then imbalanced metabolism state which heat production is decreasing is appeared. 4. By the failure of vital energy and blood growth, decreasement of $O_2$ transportation ability of RBC, disoder of blood coagulation, immune system disturbance which humoral immunity is enhanced and cellular immunity is decreased, are noted. 5. While there is not still an attemt to study the spleen deficiency sydrome in muscle disease or disease of four extremities, but it is likely suggested that spleen-stomach supplyment thereapy is very excellent effect on muscle disease and disease of four extremities.

• Journal of Genetic Medicine
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• v.17 no.2
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• pp.79-82
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• 2020

In epilepsy-aphasia spectrum (EAS) disorders, mutations in the glutamate receptor ionotropic N-methyl-D-aspartate type subunit 2A (GRIN2A) have become important for screening the disease. Research into the phenotypic variability of several types of neurologic impairment involving these mutations is in progress. However, the non-neurological problems related to these mutations are poorly understood. EAS disorders usually have epileptic, cognitive, or behavioral manifestations. In this case report, we present a female patient with epilepsy, delay in expressive language and social development, and intellectual disability with low intelligence quotient and memory quotient, but normal motor development. Through genetic analysis, she was found to have a missense and a nonsense mutation in GRIN2A (c.1770A>C; p.Lys509Asn and c.3187G>T; p.Glu1063∗, respectively) and we consider the nonsense mutation as 'pathogenic variant'. She was also discovered to have congenital hypothyroidism, growth hormone deficiency and Rathke's cleft cyst in the brain, which were previously unknown features of GRIN2A mutation. Our findings should widen understanding of the spectrum of GRIN2A phenotypes, and emphasize the need for more research into the association between GRIN2A mutations and non-neurologic clinical presentations.

• Journal of TMJ Balancing Medicine
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• v.12 no.1
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• pp.7-14
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• 2022

Objectives: Dementia is a disease in which a person maintains a normal intellectual level during the growth period, but has acquired cognitive impairment and personality change. In this study, we tried to check whether the network pharmacology analysis method is useful in the search for herbal medicine resources for the treatment of dementia. Methods: The Traditional Chinese Medicine Systems Pharmacology Database and Analysis Platform (TCMSP) database is a database frequently used in Chinese medicine research. We used the TCMSP to identify herbal medicines and their molecular targets that can be used for dementia by using network pharmacology research methods. Results: It was possible to select 28 types of components that are expected to be active by applying them to the living body, and 75 types of targets that these components act on were secured. In addition, 16 kinds of drugs were identified by checking the drugs containing 28 kinds of ingredients, and it was found that Radix Salviae contained 2 kinds of the selected 28 kinds of ingredients. Conclusions: Through this study, we were able to identify ingredients, drugs, and targets that can be used for basic and clinical research on dementia.

• Archives of Plastic Surgery
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• v.41 no.2
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• pp.163-170
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• 2014

Background The septal cartilage is the most useful donor site for autologous cartilage graft material in rhinoplasty. For successful nasal surgery, it is necessary to understand the developmental process of the nasal septum and to predict the amount of harvestable septal cartilage before surgery. Methods One hundred twenty-three Korean patients who underwent three-dimensional (3D) facial bone computed tomography (CT) were selected for evaluation of the midsagittal view of the nasal septum. Multiple parameters such as the area of each component of the nasal septum and the amount of harvestable septal cartilage were measured using Digimizer software. Results The area of the total nasal septum showed rapid growth until the teenage years, but thereafter no significant change throughout the lifetime. However, the development of the septal cartilage showed a gradual decline due to ossification changes with aging after puberty in spite of a lack of change in the total septal area. The area of harvestable septal cartilage in young adults was $549.84{\pm}151.26mm^2$ and decreased thereafter with age. Conclusions A 3D facial bone CT scan can provide valuable information on the septal cartilage graft before rhinoplasty. Considering the developmental process of the septal cartilage identified in this study, septal surgery should not be performed until puberty due to the risk of nasal growth impairment. Furthermore, in elderly patients who show a decreased cartilage area due to ossification changes, septal cartilage harvesting should be performed carefully due to the risk of saddle nose deformity.

• BMB Reports
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• v.50 no.2
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• pp.60-70
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• 2017

Glaucoma is characterized by a slow and progressive degeneration of the optic nerve, including retinal ganglion cell (RGC) axons in the optic nerve head (ONH), leading to visual impairment. Despite its high prevalence, the biological basis of glaucoma pathogenesis still is not yet fully understood, and the factors contributing to its progression are currently not well characterized. Intraocular pressure (IOP) is the only modifiable risk factor, and reduction of IOP is the standard treatment for glaucoma. However, lowering IOP itself is not always effective for preserving visual function in patients with primary open-angle glaucoma. The second messenger cyclic adenosine 3',5'-monophosphate (cAMP) regulates numerous biological processes in the central nervous system including the retina and the optic nerve. Although recent studies revealed that cAMP generated by adenylyl cyclases (ACs) is important in regulating aqueous humor dynamics in ocular tissues, such as the ciliary body and trabecular meshwork, as well as cell death and growth in the retina and optic nerve, the functional role and significance of cAMP in glaucoma remain to be elucidated. In this review, we will discuss the functional role of cAMP in aqueous humor dynamics and IOP regulation, and review the current medications, which are related to the cAMP signaling pathway, for glaucoma treatment. Also, we will further focus on cAMP signaling in RGC growth and regeneration by soluble AC as well as ONH astrocytes by transmembrane ACs to understand its potential role in the pathogenesis of glaucoma neurodegeneration.

• Journal of Korean Neurosurgical Society
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• v.42 no.3
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• pp.159-167
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• 2007

Vestibular schwannoma (VS) is a benign tumor typically originated in the schwann cell of vestibular nerve and usually accompany hearing symptom. Microsurgical removal and radiosurgery have a great role for the treatment of VS. Recently radiosurgery has been considered as an alternative or primary treatment for VS with the tremendous increase of patients who were treated with gamma knife radiosurgery (GKS) though microsurgery still takes the premier. By many published results, it is proved that GKS is a effective and noninvasive technique for VS, especially small sized tumors with satisfactory tumor control rate. The authors assumed that GKS can be expected to achieve satisfactory tumor control rate for small VS under 5cc in volume. A major interest regarding radiosurgery nowadays is to determine the optimal radiation dose for hearing preservation to improve the quality of life of patients. The more high radiation dose are used for effective tumor growth control, the more radiation-related complications like as hearing deficit, the impairment of other cranial nerve function are increased. Since 1990's the mean radiation dose for tumor margin was more than 18 Gy, but there were high complication rate in spite of good tumor growth control. After the year of 2000, under the influence of advanced neuro-imaging techniques and radiosurgical planning system which enable clinicians to do more precise planning, marginal dose for VS has been decreased to 12-13 Gy and the radiation-related complications has been reduced. But because there may be a unexpected radiation induced complications as time goes by after the latency period, optimal radiation dose for VS should be established on the basis of more long term follow-up observation.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.13-17
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• 2018

Methylmalonic acidemia is an autosomal recessive disorder caused by complete (mut0) or partial (mut-) deficiency of methylmalonyl-CoA mutase (MUT) or by defects in the synthesis of adenosylcobalamin (cblA, cblB, cblD variant 2). Long term complications of methylmalonic acidemia include tubulointerstitial nephritis with progressive renal failure, intellectual impairment, pancreatitis, and growth failure. We report a case of methylmalonic acidemia in a girl who diagnosed at 6 days after birth. She has developed recurrent metabolic crises with hyperammonemia and metabolic acidosis. In addition, she suffered from the chronic complications including tubulointerstitial nephritis, electrolyte imbalance associated with renal dysfunction, growth failure and fracture of femur shaft. At the age of 10 years, hypercalcemia and severe osteoporosis were noted, and pamidronate therapy was given for two years, which relieved hypercalcemia and osteoporosis.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.3
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• pp.246-250
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• 2004

Uni- or bilateral mandibular hypoplasia can be associated with various syndromes or is acquired after early traumatic or inflammatory disease in the temporomandibular joint(TMJ). Early treatment is necessary to avoid consequent impairment of midfacial growth. The standard treatment of these malformations consists of the application of bone grafts which can lead to unpredictable growth, but the new procedure of bone lengthening which was presented by McCarthy et al. represents a limited surgical intervention and therefore open up a new perspective of treatment, especially in younger children with severe deformities. Patients with hemifacial microsomia and facial asymmetry have a vertically short maxilla, a tilted occlusal plane, and a short mandible. A 14-years-old boy with facial asymmetry, who was fractured on both condyle and mandibular symphysis before 8 years ago, was treated by mandibular ramus lengthening, symphysial widening and surgically assisted rapid palatal expansion with corticotomy. After allowing 1 week for the healing of the periosteum, the distraction was performed at the rate of 0.5-1.0mm per day for 7 days on maxilla and 14 days on mandible. The device was maintained on maxilla and mandible for 12 weeks following distraction. The difference in ramus and mandibular transverse deficiency were corrected and facial asymmetry was improved with complex distraction osteogenesis.