• 수면정신생리
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• 제30권1호
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• pp.3-8
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• 2023

Periodic limb movement disorder (PLMD) is a sleep-related movement disorder characterized by involuntary, rhythmic limb movements during sleep. While PLMD itself is not considered life-threatening, its association with certain underlying health conditions raises concerns about mortality risks. PLMD has been found to be associated with cardiovascular diseases such as hypertension and cardiovascular disease. The fragmented sleep caused by the repetitive limb movements and associated arousals may contribute to sympathetic activation, chronic sleep disruption, sleep deprivation, and subsequent cardiovascular problems, which can increase mortality risks. The comorbidities and health factors commonly associated with PLMD, such as obesity, diabetes, and chronic kidney disease, may also contribute to increased mortality risks. PLMD is often observed alongside other neurological disorders, including restless legs syndrome (RLS) and Parkinson's disease. The presence of PLMD in these conditions may exacerbate the underlying health issues and potentially contribute to higher mortality rates. Further research is needed to elucidate the specific mechanisms linking PLMD to mortality risks and to develop targeted interventions that address these risks.

• 대한유전성대사질환학회지
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• 제18권2호
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• pp.50-54
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• 2018

Jehovah's Witnesses do not accept blood transfusions, because of their particular interpretation of the Old and New Testaments. When people with such religious convictions are in need of medical care, their faith and belief may become an obstacle for proper treatment, and pose legal, ethical, and medical challenges for the health care providers. We report two inherited metabolic disorder cases in South Korea where the infants died whilst under medical care because of parental refusal of blood transfusions for religious reasons. Case 1 had methylmalonic acidemia, Down syndrome and associated congenital cardiac anomalies requiring surgery. Case 2 had anemia and methylmalonic acidemia requiring dialysis to treat hyperammonemia and metabolic acidosis. For effective medical management, they needed life-saving blood transfusions. As a part of alternative treatment, Erythropoietin was administered in both cases. As a result, two babies died from their extremely low hemoglobin and hematocrit. The hemoglobin concentrations below 2.7 g/dL without cardiac problem and 5.4 g/dL with cardiac anomaly complicated by pulmonary hypertension are considered life-threatening hemoglobin threshold. The medical professional must respect and accommodate religious beliefs of the patients who can make informed decisions. However, when parents or legal guardians oppose medical treatment of their babies and incompetent care receivers on cultural and religious grounds, the duty to assist and save persons exposed to serious danger, particularly life-threatening events must come first.

• Journal of Medicine and Life Science
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• 제16권3호
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• pp.55-59
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• 2019

Cerebral vessels are functionally and structurally specialized to provide adequate blood flow to brain which shows high metabolic rates. Cerebral hemorrhage or ischemic infarction due to cerebrovascular injury or occlusion can cause the immediate brain damage, and if not treated rapidly, can lead to serious or permanent brain damages, and sometimes life-threatening. Unlike these popular cerebrovascular diseases, there are diseases caused by genetic problems. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is one of them. CADASIL does not show the high incidence, but it is considered to be significantly affected by regional obstructiveness such as islands and therefore, to be an important genetic disease in Jeju. This paper aims to summarize the possibility of animal model research that can provide preclinical data for CADASIL disease research and to evaluate its applicability in future research plans.

• 대한두개안면성형외과학회지
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• 제21권3호
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• pp.206-209
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• 2020

Candida osteomyelitis affecting maxillofacial bones has been scantly documented in the literature. Infantile osteomyelitis is an uncommon and life-threatening disease. Candida osteomyelitis causes significant morbidity. The present report describes a case of a 9-month-old infant with infantile osteomyelitis secondary to candida infection. This report describes its presentation and the management of palatal fistula in an infant.

• Neonatal Medicine
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• 제28권3호
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• pp.139-142
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• 2021

Macrophage activation syndrome (MAS) is a potentially life-threatening complication in many autoimmune diseases. Early recognition and intervention are essential for a favorable outcome. Neonatal lupus, an acquired autoimmune disease in neonates caused by the transplacental passage of maternal autoantibodies, is rare and usually self-limited. Herein, we report a case of MAS in a patient with neonatal lupus, which improved with intravenous immunoglobulin.

• 대한기관식도과학회지
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• 제10권2호
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• pp.5-8
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• 2004

Stenosing airway disease is classified as intraluminal obstruction, extrinsic compression, and malacia by the anatomical site of the lesion. Stenting therapy is indicated for symptomatic relief of life-threatening dyspnea caused by the last two types. Airway stents are made with metal mesh and/or silicone rubber, and currently more than 20 kinds of stent are available. Among many kinds of silicone stent, the Dumon stent is mostly widely used for benign and malignant airway stenoses, but general anesthesia and rigid bronchoscopy are needed for insertion. It can be removed when the stenosing airway disease subsides completely. In many clinical studies, most patients $(85-90\%)$ improved immediately after stenting, and procedure-related mortality was low $(<3\%)$ in experienced centers. Stent displacement, mucus impaction, and granulation tissue formation are potential complications. Stenting is one of many effective therapeutic modalities for stenosing central airway disease. Careful patient selection, experiences, and continuous development of new technology will bring better results.

• Tuberculosis and Respiratory Diseases
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• 제70권5호
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• pp.371-383
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• 2011

The number of immunocompromised patients has increased over the past decades due to HIV infection, solid and stem cell transplantation, intensified chemotherapy and treatment of autoimmune disease. Pneumonia is a major cause of both morbidity and mortality in immunocompromised patients. Clinical management of pneumonia is difficult, since differential diagnosis in this setting is broad and includes both infectious and noninfectious processes. Because the development of pneumonia in immunocompromised patients is frequently life threatening, early therapeutic and diagnostic intervention is essential to obtain better outcomes.

• 대한유전성대사질환학회지
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• 제17권1호
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• pp.24-30
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• 2017

Gorham-Stout disease (GSD)는 골용해와 함께 혈관 및 림프조직의 비정상적인 증식으로 골조직 파괴가 유발되는 매우 드문 질환이다. 아직까지 GSD의 정확한 병인 및 기전은 밝혀지지 않았다. 악성종양이나 신경병증, 감염과의 연관성은 불명확하며, 골조직이 있는 신체 어디에서든 기형적 혈관-림프관 증식이 발생할 수 있다. GSD 중 약 20%에서 유미흉을 동반하는데, 림프관 형성이상이나 가슴 림프관 손상에 의해 이차적으로 발생한다. 급격한 호흡부전으로 이어질 수 있어 불량한 예후인자로 알려져 있지만, 질환 자체의 희귀성 때문에 현재까지 확립된 표준치료법은 없다. 본 증례는 유미흉을 동반한 생명을 위협하는 GSD 환자에서 적극적인 외과적 중재술 후 mTOR inhibitor 및 beat-blocker 복합요법을 적용하여 치료에 성공한 보고이다. 환자는 가슴림프관 결찰술 및 흉막유착술을 시행 받았으나, 일시적 증상호전 이후로 유미흉 및 호흡곤란의 재악화 반복되었다. 양측 흉막유착제거 및 폐쇄 흉강삽관술과 함께, beta-blocker와 mTOR inbititor 경구투약을 시작했다. 약물투약 1개월 후 유미흉 재발없이 호흡 안정적으로 유지되어 산소 보조치료 없이 퇴원하였다. 현재 11개월째 지속적으로 약물 투약 중으로, 약물 부작용 및 추가적인 입원치료 없이 정상적인 일상생활을 유지하고 있다. 추후 유미흉을 동반한 GSD 환자의 치료에서, 적극적인 외과적 중재술과 함께 경구 mTOR inhibitor 및 beta-blocker 복합요법을 고려해 볼 수 있겠다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제35권2호
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• pp.107-111
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• 2013

Careful examination of the oral cavity may reveal findings indicative of an underlying systemic condition, and allow for early diagnosis and treatment. Examination should include evaluation for mucosal changes, periodontal inflammation and bleeding, and general condition of the teeth. A 12-year-old man visited for molar pain during 3 months. He was diagnosed with having a possibility of hematopoietic malignancy, showing the loss of lamina dura, destruction of bony crypt, and high attenuation in the bone marrow. He was referred to department of pediatrics, additional study, including peripheral cell morphology and bone marrow exam, were performed, and diagnosed as acute lymphoblastic anemia. Despite chemotherapy to cure leukemia, he was expired 8 months after initial diagnosis. The purpose of this report is to promote and evoke the awareness regarding an initial examination of the dentist to make an effort to acquire accurate knowledge and information about life-threatening disease in usual dental practice.

• Journal of Chest Surgery
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• 제29권5호
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• pp.530-535
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• 1996

폐국균증은 Aspergillus fulmigatus라는 곰팡이에 의 해 폐공동에 유발되는 위 협적 인 기 회 감염으로 외 과적 관심의 대상이 되어 왔다. 부산대 학교병 원 흉부외과에서는 1984년 1월부터 1994년 8월라지 폐국균종으로 진단된 12명의 환자에 대해 수술을 시행하였다. 평균 연령은 38.8세이었으며 주증상은 9례에서 객혈이었고 그중 2례에서 생명 을 위협하는 다량객혈이 있었다 9레에서 기존의 공동성 폐질환을 가지고 있었고그주원인은폐결핵이 었다. 가장 흔히 시 행된 술식은 폐엽절제술(6례)이 었다. 주요 술후 합병증으로는 대량출혈 (2례), 폐렴 (2 례), 기관지흥막루를 동반한 농흥(1례)이 발생하였으며 1례의 환자가 사망하여 수술사망률은 8.3%였다 술후 추적관찰기 간동안 증상의 재발례는 1례도 없었다. 결론적으로 폐국균종으로 진단된 경우 증상이 경미하더라도 초기에 적극적인 수술을 시행함으로써 높은 완치효과를 기대할 수 있을 것으로 사료된다.