• Sleep Medicine and Psychophysiology
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• v.30 no.1
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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.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.2
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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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• v.16 no.3
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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.

• Archives of Craniofacial Surgery
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• v.21 no.3
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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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• v.28 no.3
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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.

• Korean Journal of Bronchoesophagology
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• v.10 no.2
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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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• v.70 no.5
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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.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.24-30
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• 2017

Gorham-Stout disease is a rare disorder characterized by lymphovascular proliferation and destruction of osseous matrix. The etiology of this condition remains poorly understood. Chylothorax as a consequence of lymphatic leakage in thoracic cage may cause a severe life-threatening complication, accompanying respiratory difficulty. Currently, there is no standard management for this extremely rare condition. Here we describe a patient affected by Gorham-Stout disease successfully managed by the combined treatment of mTOR inhibitor and beta-blocker. A previously healthy 11-year-old female developed dyspnea and chest pain with a massive pleural effusion. The ligation of right thoracic duct and bilateral pleurodesis temporarily decreased her pleural effusion, which was aggravated repetitively and required frequent admission and tube thoracotomies. Along with bilateral pleural adhesiolysis with thoracotomy, the combined treatment of oral beta-blocker and mTOR inhibitor was commenced. After 1 month of oral medication, her pleural effusion was not increased and she was free of respiratory difficulty on room air without chest tubes. Over eleven months of treatment, no serious adverse reaction was noted and her condition has been stable with no further admission required.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.35 no.2
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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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• v.29 no.5
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• pp.530-535
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• 1996

Saprophytic aspergillosis (aspergilloma), a potentially life-threatening disease from the colonization of lung cavities by the ubiquitous fungus Aspergillus fumigatus, has been of surgical concern. Twelve patients with symptomatic aspergilloma underwent operative procedures at Pusan national University Hospital between January 1, 1984 and August 31, 1994. The mean age of the patients was 38.8 years and nine of them had hemoptysis; in two it was massive and life-threatening. Wine of the patients had underlying causes of cavitary lung disease and tuberculosis was the most common cause. Lobectomy was the most commonly performed procedure (in six) . Several major complications including excessive bleeding (2), and pneumonia (2), and empyema with bronchopleural fistula (1) occurred .and one patient died (operative mortality=8.3%). There were no recurrent symptoms in any of the 11 operative survivors during the follow-up period. It is concluded that early, aggressive surgical intervention can provide definite cure in established aspergilloma, even with minimal symptoms.