• Clinical and Experimental Pediatrics
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• 제60권2호
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• pp.31-37
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• 2017

Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.

• 대한한의학회지
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• 제29권1호
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• pp.15-24
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• 2008

Objectives : The purpose of this study is to reexamine current oriental medical treatment fees based on valuation of input resources which are the treating time, the treatment's difficulty, and the material cost. Methods : This study obtained the following results by reviewing the answers given by 172 oriental medical doctors from March 1 to April 15, 2006. To investigate material cost, we took inventory of treatment materials used by 2 oriental hospitals. Results : The current system does not reflect well enough the treating time and treatment's difficulty. Considering current oriental treatment fees, material costs are too much of doctors' fees. Especially, Wang-ssuk-dduem, which is another form of moxibustion treatment, was spent as much as 74.6% on materials cost. Conclusions : The findings suggest the current oriental treatment fees should be revised to reflect the actual input resources into oriental medical doctor activities and to avoid a distortion of physicians' behavior.

• Childhood Kidney Diseases
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• 제25권2호
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• pp.92-111
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• 2021

Purpose: Nephrotic syndrome (NS) is the most common form of glomerulopathy in children. Most pediatric patients respond to glucocorticosteroid treatment (steroid-sensitive NS, SSNS), while approximately 10-15% will remain unresponsive or later become steroid-resistant. There has been a long-standing effort to find biomarkers that may predict steroid responsiveness. Methods: We systematically reviewed current studies which investigated clinically relevant biomarkers for predicting steroid responsiveness in pediatric NS. We performed a PubMed and EMBASE search to identify eligible articles. We collected data on urinary markers, blood/serum markers (including cellular phenotypes and mRNA expression), genotypes and HLA allele frequency. Results: A total of 659 articles were identified following electronic and manual searches. After reviewing the titles, abstracts, and full texts, 72 eligible articles were finally included. Vitamin D-binding protein (VDBP) seemed to be significantly elevated in SRNS than in SSNS, in both serum and urine specimen, although further validation is required. Conclusions: The present paper narratively illustrates current understandings of potential biomarkers that may help predict steroid responsiveness. Further investigation and collaboration involving a larger number of patients are necessary.

• 동의생리병리학회지
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• 제28권1호
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• pp.113-121
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• 2014

With the development of information technology, knowledge information-oriented and information systems are being rapidly paced. In addition, doctor's needs of the system that assist decision making is gradually increasing. Because the complex process of decision-making should be a lot. We propose a web based system for supporting medical treatment based on Korean medicine ontology. There are three kinds of processes. First, a pattern is decided for patient' symptoms, a formula for the pattern is selected and medicinal materials constituting the formula is added or removed. Second, a formula is decided for patient' symptoms, medicinal materials constituting the formula is added or removed. Third, a Treat method is decided for patient' symptoms, medicinal materials constituting the formula is added or removed. We have designed and implemented the clinical decision support system that supports flexible processes and necessary information and functions. The system shows the appropriate form of ontology knowledge as interrelated and provide analysis and processing, does not show simply search. The system is one of the systems utilizing ontology and a web based system that can be used in anywhere. Therefore, This system Will be useful as for doctors to make decision.

• Journal of mucopolysaccharidosis and rare diseases
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• 제5권1호
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• pp.29-33
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• 2021

Prader-Willi Syndrome (PWS) is a neurodevelopmental genomic imprinting disorder involving a lack of gene expression from the paternal chromosome 15q11-q13 region. This is typically due to paternal 15q11-q13 deletions (in approximately 60% of cases), maternal uniparental disomy 15, or when both 15s are from the mother (about 35% of cases). An imprinting center controls the expression of imprinted genes in the chromosome 15q11-q13 region. PWS is a neurodevelopmental disorder characterized by mental retardation and distinct physical, behavioral, and psychiatric features. Characteristic behavioral disturbances in PWS include excessive interest in food, skin picking, difficulty with a change in routine, temper tantrums, obsessive and compulsive behaviors, and mood fluctuations. Individuals with PWS typically have intellectual disabilities (borderline to mild/moderate mental retardation) and exhibit a higher overall level of behavior disturbances compared to individuals with similar intellectual disabilities. This condition severely limits social adaptations and quality of life. Different factors have been linked to the intensity and form of these behavioral disturbances, but there is no consensus regarding the cause. Consequently, there is still controversy surrounding management strategies and there is a need for new data. PWS is a multisystem disorder. Family members, caregivers, physicians, dieticians, and speech-language pathologists all play an important role in the management and treatment of symptoms in an individual with PWS. Here we analyze behavioral problems in children and adults with PWS by age and review appropriate management and treatment strategies for these symptoms.

• 한방안이비인후피부과학회지
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• 제17권2호
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• pp.153-158
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• 2004

Background and Objective: Erythema multiforme(EM) is related disorders of skin and mucous membranes which is typically associated with antecedent medications or infections. etc. EM is an erythematous maculopapular cutaneous eruption of variable form. However, the pathophysiology of the EM remains obscure. Treatment at present is symptomatic and supportive. There is no report on EM treated with oriental medical treatment. Methods: We conducted one patient with EM seen at Won-Kwang University Kwang-Ju Oriental Medical Hospital in 2004. We treated EM in a twenty-two years old man by a herbal medicine(Joa-Chang-Bang), a herbal medicine for external use, acupunture, moxa, COTDE-made cosmetics, and P-Tx(carbon). Conclusions. We had a significant results. So we report this case to estimate the efficiency of oriental medical treatment and management on EM.

• 한국환경과학회지
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• 제29권3호
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• pp.299-306
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• 2020

This study analyzed the changes in the concentrations of the pollutants of constructed treatment wetlands which come from the discharge water of a sewage treatment plant. According to the results of budgets in constructed wetlands, the net production of the organic carbon, nitrogen and phosphorus were 368 kgC/month, 306 kgN/month and -49 kgP/month, respectively. The high particle form of pollutants are mostly removed due to settlement and absorption when passing through wetlands, but because a low processing efficiency for pollutants was shown when sewage treatment plant wastewater flows in, there is a need for a water management system that can reduce the organic matter load through monitoring. The low removal efficiency of constructed wetlands were caused by both structural and operational problems. Therefore, to enable to play a role as a reduction facility of pollutants, an appropriate design and operation manuals for constructed wetlands is urgently needed.

• Journal of mucopolysaccharidosis and rare diseases
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• 제4권2호
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• pp.37-41
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• 2018

Mucopolysaccharidosis III (MPS III, Sanfilippo syndrome) is a rare autosomal recessive disease caused by a deficiency of one of four enzymes involved in the degradation of glycosaminoglycan (GAG). The resultant cellular accumulation of GAG causes various clinical manifestations. MPS III is divided into four subtypes depending on the deficient enzyme. All the subtypes show similar clinical features and are characterized by progressive degeneration of the central nervous system. A number of genetic and biochemical diagnostic methods have been developed. However, there is no effective therapy available for any form of MPS III, with treatment currently limited to clinical management of neurological symptoms. Main purpose of the treatment for MPS III is to prevent neurologic deterioration. Because conventional intravenous enzyme replacement therapy (ERT) has a limitation due to inability to cross the blood-brain barrier, several innovative therapeutic approaches for MPS III are being developed. This review covers the currently developing new therapeutic options for MPS III including high dose ERT, substrate reduction therapy, intrathecal or intraventricular ERT, fusion protein delivery using bioengineering technology, and gene therapy.

• 한방안이비인후피부과학회지
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• 제19권2호
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• pp.313-319
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• 2006

Background and Objective : Localized Pustular Psoriasis(LPP) is a rare form of psoriasis characterized by erythematous papules, plaques studded with pustules(usually on palms or soles) However, the pathophysiology of the LPP remains obscure. Treatment at present is symptomatic and supportive. There is no report on LPP treated with oriental medical treatment. Methods : We conducted one patient with LPP seen at Won-Kwang University Ik-San Oriental Medical Hospital in 2003. We treated LPP in a forty-nine years old man by a herbal medicine, acupuncture, moxa, etc Conclusions : We had a significant results. So we report this case to estimate the efficiency of oriental medic at treatment and management on LPP

• 성인간호학회지
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• 제14권4호
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• pp.491-500
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• 2002

Purpose: To exam how functional status varied according to age, gender, stage, treatment protocols, and pathologic types of cancer in lung cancer patients. Method: A Cross-sectional and descriptive study was used. Functional status was measured with the Medical Outcome Study Short Form-36. A total of 106 lung cancer patients participated. Their mean age was 61 years. Majorities were male, in advanced stages, and receiving chemotherapy. Result: Functional status of the subjects were relatively low compared to the results of previous studies. Women had more severe functional limitations in Role-Emotion(t=2.17, p <.05). Generally, older patients(> 60 yrs.) had relatively more severe limitations in all subcategories, but the difference was not statistically significant. Subjects in late lung cancer stage(stage III & IV) had more severe functional limitation in all subcategories. But the statistical difference was found only in General Health(t=2.10, p<.05). In terms of treatment protocol, no-current treatment group had lower General Health than those of the chemotherapy group(F=3.42, p<.01). There were no statistical differences in functional status among pathological cancer cell types. Conclusion: The results of this study suggest that effective management may be achieved when these factors are considered on individual basis in the clinical management of lung cancer patients.