• 대한영상의학회지
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• 제84권3호
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• pp.745-749
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• 2023

Gerstmann-Strssler-Scheinker (이하 GSS) disease는 드문 유전성 프라이온 질환으로 초기에 발생해 진행하는 소뇌실조와 후기에 발생하는 인지기능 저하가 특징적이다. 저자들은 진행하는 보행장애와 5개월 후 발생한 구음 장애, 인지 기능 저하를 주소로 내원한 39세 남성 환자의 증례를 보고하고자 한다. 뇌 MRI에서 양측 대뇌피질과 기저핵, 시상에 확산 저하를 동반한 T2 강조영상에서의 고신호 강도 병변이 관찰되었다. 환자는 모두 40-50대에 비슷한 증상을 호소하였던 가족력을 동반하였으며 PRNP 유전자 검사를 통해 GSS로 확진되었다.

• 대한치과의사협회지
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• 제36권11호통권354호
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• pp.794-799
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• 1998

Many references report that abnormal diastema except temporary diastema existing in mixed dentition period is caused by maxilary heavy labial frenum, malocclusion, progressive periodontal disease, and loss of posterior teeth. We can diagnose patient as diastema caused by periodontal disease, especially, in case of accompanying progressively destructed anterior maxillary alveolar bone defect, and interseptal bone defect. We report Multiple disciplinary approach for diastema associated with periodontal disease. Periodontal treatment(Guided Tissue -Regeneration, alveoloplasty, bone graft), or thodontic treatment (space closure, redistribution), and the final proshodontic restoration for retention were used.

• 대한유전성대사질환학회지
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• 제14권1호
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• pp.42-47
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• 2014

Gaucher disease is a multisystemic disorder arising from a deficient activity of the lysosomal enzyme glucocerebrosidase, which leads to accumulation of glycosylceraide and other glycolipids in the regiculoendothelial system. The characteristics of Gaucher disease are anemia, thrombocytopenia, hepatosplenomegaly, and skeletal disease. Enzyme replacement therapy (ERT) has been proven to prevent progressive manifestations of Gaucher disease and effective in improving anemia, thrombocytopenia, bone markers and biomarkers. However, some patient needs still remain unmet because of the inaccessibility of certain sites including brain, bone and various organs. ERT could not Improve the irreversible lesion such as liver fibrosis, hepatopulmonary syndrome, and necrosis or infarction of bone and other organs. Adult patients with Gaucher disease should be screened for longterm complication such as bone disease, pulmonary hypertension, gallstone, and cancer, especially in patients with splenectomy. Parkinsonism and polyneuropathy was also reported among patients with type 1 Gaucher disease, but ERT does not improve neurological function. We need to review the benefits and unmet needs of ERT in Gaucher disease.

• Journal of Hospice and Palliative Care
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• 제23권1호
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• pp.27-38
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• 2020

Purpose: The purpose of this study was to compare differences in spiritual needs (SNs) and factors influencing SNs between patients with progressive terminal kidney disease and their family caregivers. Methods: An explorative comparative survey was used to identify the SNs of patients (N=102) with progressive terminal kidney disease undergoing hemodialysis and their family caregivers (N=88) at a general hospital located in Seoul, South Korea. The data were analyzed using descriptive statistics, the chi-square test, the independent t-test, one way analysis of variance, the Scheffe test, and multiple regression with dummy variables. Results: The SNs among family caregivers were higher than in the patient group. SNs were higher among those who were religious in both groups. Loving others was the highest-ranked subdimension in the patient group, followed in descending order by maintaining positive perspective, finding meaning, Reevaluating beliefs and life, asking "why?", receiving love and spiritual support, preparing for death, and relating to God. In the family group, the corresponding order was maintaining positive perspective, loving others, finding meaning, receiving love and spiritual support, preparing for death, relating to God, and asking "why?". The factors that had a negative influence on the level of SNs were not being religious in the patient group and having only a middle school level of education in the family group. Conclusion: The results of this study may serve as evidence that spiritual care for non-cancer patients' family caregivers should be considered as an important part of hospice and palliative care.

• Annals of Clinical Neurophysiology
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• 제2권2호
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• pp.107-113
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• 2000

Background & Objectives : Motor evoked potentials(MEPs) to magnetic trans cranial stimulation were performed to evaluate upper motor neuron involvement and relationship to lower motor neuron involvement in motor neuron disease patients. Method : MEPs were obtained in the 17 consecutive patients with motor neuron disease. These patients were divided into three group based on clinical evidence of upper and lower motor neuron involvement, bulbar symptom; amyotrophic lateral sclerosis(ALS), progressive muscular atrophy(PMA), progressive bulbar palsy(PBP). MEPs were recorded from abductor pollicis brevis and abductor hallucis muscles. Abnormal MEPs were defined by delayed central motor conduction time or absent MEP. Results : MEPs were abnormal in 64%(11/17) of patients; 100%(7/7) in ALS, 64%(4/7) in PMA, 0%(0/3) in PBP respectively. In 68 total recording muscles, 34 muscles had evidence of motor weakness and showed abnormal responses in 59%(20/34). Whereas 34 muscles with normal strength, only 3%(1/34) of muscles showed abnormal response. Conclusion : MEPs are well correlated with upper motor neuron signs in ALS and may detect masking upper motor neuron signs in PMA. The muscles with lower motor neuron sign(weakness) usually relate with abnormal MEPs reponses.

• 대한치과마취과학회지
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• 제7권1호
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• pp.18-21
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• 2007

Pelizaeus-Merzbacher disease (PMD) can be defined as an X-linked recessive leukodystrophy that is caused by a mutation in the proteolipid protein gene on chromosome Xq22. PMD is one of a group of progressive, degenerative disorders of the cerebral white matter known as the leukodystrophies. Due to the progressive nature of the disorders and their devastating effects on the central nervous system, these children frequently require anesthesia during imaging procedures such as MRI or during various surgical procedures. Anesthetic concerns in theses cases include high prevalence of seizure disorders, gastroesophageal reflux with the risk of aspiration, airway complications related to poor pharyngeal muscle control and copious oral secretions, and mental retardation. We report a successful anesthetic management in a patient with PMD for dental procedures.

• Clinical and Experimental Pediatrics
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• 제54권5호
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• pp.192-196
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• 2011

Respiratory syncytial virus (RSV) is a main cause of hospitalization for bronchiolitis and pneumonia in infants worldwide. Children with hemodynamically significant congenital heart disease (HS-CHD), as well as premature infants are at high risk for severe RSV diseases. Mortality rates for CHD patients hospitalized with RSV have been reported as about 24 times higher compared with those without RSV infection. Recently with advances in intensive care, mortality rates in CHD patients combined with RSV have decreased below 2%. The requirements of intensive care and mechanical ventilation for CHD patients with RSV infection were still higher than those without RSV infection or with non-CHD children. RSV infection has frequently threatened CHD infants with congestive heart failure, cyanosis, or with pulmonary hypertension. As a progressive RSV pneumonitis in those infants develops, the impairment of oxygen uptake, the breathing workload gradually increases and eventually causes to significant pulmonary hypertension, even after the operation. Preventing RSV infection as much as possible is very important, especially in infants with HS-CHD. A humanized monoclonal antibody, palivizumab, has effective in preventing severe RSV disease in high-risk infants, and progressive advances in supportive care including pulmonary vasodilator have dramatically decreased the mortality (<1%). Depending on the global trend, Korean Health Insurance guidelines have approved the use of palivizumab in children <1 year of age with HS-CHD since 2009. Korean data are collected for RSV prophylaxis in infants with CHD.

• Tuberculosis and Respiratory Diseases
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• 제43권3호
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• pp.455-460
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• 1996

폐기포는 폐 내부에 공기를 포함하는 공간으로 보통 1cm 이상의 크기와 2mm이하의 모발선 모양의 기포벽을 갖는다. 자연경과상 지속적으로 약화되는 양상을 보이고 자연소실 또는 호전되는 경우는 매우 드문 것으로 알려져 있다. 저자들은 임신중 발생한 속립성 결핵 환자에서 성인형 호흡곤란 증후군의 경과 후 발생한 다발성 폐기포가 자연소실된 1예를 관찰하였기에 보고하는 바이다.

• 대한한의정보학회지
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• 제13권1호
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• pp.26-38
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• 2007

Interstitial lung disease (ILD) is characterized by progressive scarring of the lung leading to restriction and diminished oxygen transfer. Clinically, the presenting symptoms of ILD are nonspecific (cough and progressive dyspnea on exertion) and are often attributed to other diseases, thus delaying diagnosis and timely therapy. In this study, I analyzed the 10 chinese papers of interstitial lung diseases(ILD). The etiology are body resistance weakness(本虛) and pathogenic factor prevailing(標實). The body resistance weakness(本虛) including deficiency of the lung(肺虛), deficiency of the kidney(腎虛), deficiency of the spleen(脾虛), deficiency of Qi and Yin(氣陰兩虛), pathogenic factor prevailing(標實) including stagnation of phlegm(痰濁), blood stasis(瘀血), noxious heat(熱毒). As an treatment aim at supplementing lung and kidney(益肺腎), resolving phlegm and blood stasis(化痰瘀).

• International Journal of Advanced Culture Technology
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• 제11권3호
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• pp.9-17
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• 2023

Parkinson's disease is a complex neurodegenerative disease characterized by the progressive loss of dopamine-producing neurons in the substantia nigra, resulting in a variety of motor and non-motor symptoms. This study aimed to provide a comprehensive overview of Parkinson's disease, including classification of Parkinson's disease, impairment due to impairment, how disability is assessed, and how it is treated. Major symptoms of Parkinson's disease include tremors, stiffness, bradykinesia, and postural instability, and treatment methods include rehabilitation through drugs, surgical procedures, physical therapy, and occupational therapy. Early diagnosis, individualized treatment interventions, and comprehensive treatment involving a multidisciplinary medical team will be essential to manage Parkinson's disease and improve patients' quality of life. In conclusion, this study will provide comprehensive information on the complex nature of Parkinson's disease and serve as a useful guide for healthcare providers designing treatment plans for Parkinson's patients.