• Neonatal Medicine
• /
• 제25권4호
• /
• pp.137-143
• /
• 2018

Purpose: This study was performed to determine the clinical features of full-term infants with hypoxemia detected by pulse oximetry and to establish the diagnosis of critical congenital heart disease (CCHD). Methods: We retrospectively reviewed the medical records of neonates who had been admitted to the neonatal intensive care unit within 2 weeks of birth at Korea University Ansan Hospital between January 2013 and October 2017 (n=450). We classified these neonates based on the presence of hypoxemia at admission and investigated neonatal characteristics, initial symptoms, echocardiographic findings, and final diagnosis associated with hypoxemic diseases. Results: Of 450 term infants, 265 infants (58.9%) were identified hypoxemia by pulse oximetry at admission. The most common symptoms of them were cyanosis and tachypnea. Among them, 80.1% of infants (214/265) were diagnosed with respiratory tract disease and 8.3% of infants (22/265) had congenital heart disease. Thirteen infants (13/265, 4.9%) had CCHD and were treated with urgent surgery or transcatheter intervention within 28 days of birth. Majority of infants with respiratory tract disorder were transferred from hospital immediately after birth, but 46.1% of infants (6/13) with CCHD remained asymptomatic after birth and were admitted after 48 hours after birth. In addition, other hypoxemic illnesses were identified as neonatal infectious and neurological diseases. Conclusion: This study showed the importance of assessment in neonates with hypoxemia, including those diagnosed with CCHD. The possibility of CCHD should be considered in the differential diagnosis in neonates demonstrating hypoxemia after 48 hours of birth. A larger prospective study is needed to assess the effectiveness and outcomes of pulse oximetry for neonatal screening in Korea.

• 동의신경정신과학회지
• /
• 제31권4호
• /
• pp.269-278
• /
• 2020

Objectives: Yukmijihwang-won (Liuweidihuang-wan in Chinese) is a frequently used medicinal herbal formula. It is used as Yin tonic in Korea and China to recover patients from Yin deficiency. However, the scientific evidence on this drug has not revealed the beneficial effect or mechanism of its effects on the neurological disorder. We designed this study to examine the antidepressive and analgesic effects of Yukmijihwang-won (YJ-01) and the minor modification of YJ-01, YJ-06 on the reserpine-induced pain-depression dyad mice model. Methods: Reserpine (1 mg/kg) was administered subcutaneously once a day for three consecutive days to induce pain and depression-like behavior. The oral administration of YJ-01 and YJ-06 (100, 200, or 300 mg/kg) was performed once daily from three days after the reserpine injection. Results: Repeated administration of the YJs significantly reduced the immobility time in a forced swimming test and increased the moved distance and number of crossings in the open field test. In the von-Frey filament test, the oral administration of YJs remarkably suppressed the increase in paw withdrawal frequency. Conclusions: The results of this study suggest that YJ-01 and 06 may be good candidates to treat the pain-depression dyad.

• Journal of mucopolysaccharidosis and rare diseases
• /
• 제5권1호
• /
• pp.17-21
• /
• 2021

Hunter syndrome or mucopolysaccharidosis type II (MPS-II) (OMIM 309900) is a rare lysosomal storage disorder caused by deficiency in the activity of the enzyme iduronate-2-sulfatase. This enzyme is responsible for the catabolism of the following two different glycosaminoglycans (GAGs): dermatan sulfate and heparan sulfate. The lysosomal accumulation of these GAG molecules results in cell, tissue, and organ dysfunction. Patients can be broadly classified as having one of the following two forms of MPS II: a severe form and an attenuated form. In the severe form of the disease, signs and symptoms (including neurological impairment) develop in early childhood, whereas in the attenuated form, signs and symptoms develop in adolescence or early adulthood, and patients do not experience significant cognitive impairment. The involvement of the skeletal-muscle system is because of essential accumulated GAGs in joints and connective tissue. MPS II has many clinical features and includes two recognized clinical entities (mild and severe) that represent two ends of a wide spectrum of clinical severities. However, enzyme replacement therapy is likely to have only a limited impact on bone and joint disease based on the results of MPS II studies. The aim of this study was to review the involvement of joints in MPS II.

• 한국임상약학회지
• /
• 제31권1호
• /
• pp.35-43
• /
• 2021

Background: Migraine is a common neurological disorder that affects the quality of life and causes several health problems. Preventive migraine treatment can reduce migraine frequency, headache severity, and health care costs. This study aimed to estimate the utilization of migraine preventive therapy and associated factors in eligible patients. Methods: We studied 534 patients with migraine who were eligible for migraine preventive therapy using 2017 National Patient Sample (NPS) data from the Health Insurance Review and Assessment Service (HIRA). We estimated the migraine days by calculating the monthly average number of defined daily dose (DDD) of migraine-specific acute drug. Patients with a monthly average number of DDD of 4 or more were considered as subjects for preventive treatment. Chi-square test and multiple logistic regression analysis were used to determine the association between the preventive therapy and the influencing variables. Results: Less than half of the eligible patients for prophylaxis (n=234, 43.8%) were prescribed preventive therapy. Multiple logistic regression results show that migraine preventive therapy was influenced by age, the type of migraine, and some comorbidities. Patients over the age of 50 tend to receive less prophylactic treatment than under the age of 40. On the other hand, migraine patients with epilepsy or depression were more likely to receive preventive therapy. Sumatriptan was the most preferred medication for acute treatment, and propranolol was the most commonly prescribed drug for prevention. Conclusions: More than half of the patients who were candidates for migraine prophylaxis were not receiving suitable preventive treatment. Positive factors affecting the use of migraine prevention were the presence of comorbidities such as epilepsy and depression.

• Biomolecules & Therapeutics
• /
• 제29권5호
• /
• pp.498-505
• /
• 2021

Amyotrophic lateral sclerosis (ALS) is a lethal neurological disorder characterized by the deterioration of motor neurons. The aim of this study was to investigate alteration of cationic amino acid transporter (CAT-1) activity in the transport of lysine and the pretreatment effect of lysine on pro-inflammatory states in an amyotrophic lateral sclerosis cell line. The mRNA expression of cationic amino acid transporter 1 was lower in NSC-34/hSOD1^G93A (MT) than the control cell line (WT), lysine transport is mediated by CAT-1 in NSC-34 cell lines. The uptake of [³H]L-lysine was Na⁺-independent, voltage-sensitive, and strongly inhibited by inhibitors and substrates of cationic amino acid transporter 1 (system y⁺). The transport process involved two saturable processes in both cell lines. In the MT cell line, at a high-affinity site, the affinity was 9.4-fold higher and capacity 24-fold lower than that in the WT; at a low-affinity site, the capacity was 2.3-fold lower than that in the WT cell line. Donepezil and verapamil competitively inhibited [³H]L-lysine uptake in the NSC-34 cell lines. Pretreatment with pro-inflammatory cytokines decreased the uptake of [³H]L-lysine and mRNA expression levels in both cell lines; however, the addition of L-lysine restored the transport activity in the MT cell lines. L-Lysine exhibited neuroprotective effects against pro-inflammatory states in the ALS disease model cell lines. In conclusion, studying the alteration in the expression of transporters and characteristics of lysine transport in ALS can lead to the development of new therapies for neurodegenerative diseases.

• 대한의용생체공학회:의공학회지
• /
• 제42권6호
• /
• pp.295-303
• /
• 2021

Idiopathic normal-pressure hydrocephalus (INPH) is considered a potentially treatable neurological disorder by shunt surgery and characterized by a triad of symptoms including gait disturbance, cognitive impairment and urinary dysfunction. Although disorders of white matter are generally viewed as the principal pathological features of INPH, analysis of cortical features are important since the destruction of neural tracts could be associated with cortical structural changing. The aim of the study was to determine whether there was any relationship between gait parameter and structural features of cerebral cortex in INPH patients. Gait parameters were measured as follows: step width, toe in/out angle, coefficient of variation (CV) value of stride length, CV value of stride time. After obtaining individual brain MRI of patients with INPH and hemispheric cortical surfaces were automatically extracted from each MR volume, which reconstructed the inner and outer cortical surface. Then, cortical thickness, surface area, and volume were calculated from the cortical surface. As a result, step width was positively correlated with bilateral postcentral gyrus and left precentral gyrus, and toe in/out was positively correlated with left posterior parietal cortex and left insula. Also, the CV value of stride length showed positive correlation in the right superior frontal sulcus, left insula, and the CV value of stride time showed positive correlation in the right superior frontal sulcus. Unique parameter of cerebral cortical changes, as measured using MRI, might underline impairments in distinct gait parameters in patients with INPH.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제25권3호
• /
• pp.180-193
• /
• 2022

Pediatric inflammatory bowel disease (PIBD) is a multisystem disorder characterized by intestinal and extraintestinal manifestations and complications. Cerebrovascular events (CVE) are rare extraintestinal complications in patients with PIBD. Statistics show that 3.3% patients with PIBD and 1.3-6.4% adult patients with inflammatory bowel disease (IBD) experience CVE during the course of the disease. Therefore, this study aimed to review the records of children with IBD who developed CVE during the course of the disease. We retrospectively reviewed 62 cases of PIBD complicated by CVE. The mean patient age at the time of thrombotic events was 12.48±4.13 years. The incidence of ulcerative colitis was significantly higher than that of Crohn's disease (43 [70.5%] vs. 13 [21.3%] patients). Most patients (87.93%) were in the active phase of IBD at the time of CVE. The mean time interval between the onset of IBD and CVE was 20.84 weeks. Overall, 11 (26.83%) patients showed neurological symptoms of CVE at disease onset. The most frequent symptom on admission was persistent and severe headaches (67.85%). The most common site of cerebral venous thrombosis was the transverse sinuses (n=23, 53.48%). The right middle cerebral artery (n=3, 33.34%) was the predominant site of cerebral arterial infarction. Overall, 41 (69.49%) patients who were mostly administered unfractionated heparin or low-molecular-weight heparin (56.09%) recovered completely. Patients with IBD are at a risk of thromboembolism. CVE may be the most common type of thromboembolism. Based on these findings, the most common risk factor for CVE is IBD flares. In patients with CVE, anticoagulant therapy with heparin, followed by warfarin, is necessary.

• 대한영상의학회지
• /
• 제84권5호
• /
• pp.1152-1157
• /
• 2023

총경동맥 폐쇄는 비교적 드물고 거의 연구되지 않은 질병이다. 총경동맥 폐쇄는 여러 신경학적 증상을 유발하지만 다양한 문합의 발달 덕분에 때때로 무증상일 수 있다. 저자들은 문합을 통한 혈류로 인한 무증상 총경동맥 폐쇄 환자의 증례를 보고하고자 한다. 환자는 경대퇴동맥 뇌혈관 조영술을 시행하여 총경동맥 폐쇄와 후두 동맥-척추 동맥 연결을 포함한 두 가지 측부 경로가 있는 것으로 확인되었다. 저자들은 총경동맥 폐쇄가 의심되는 경우 경대퇴동맥 뇌혈관 조영술 시행이 중요함을 강조하고, 총경동맥 폐쇄의 유형 및 문합 경로를 문헌고찰하였다.

• 동의신경정신과학회지
• /
• 제35권3호
• /
• pp.257-267
• /
• 2024

Objectives: Parkinson's disease is a degenerative neurological disorder caused by dopamine neuron damage, leading to various motor and non-motor symptoms, including depression. While several clinical studies on Traditional Korean Medicine (TKM) have addressed this issue, no comprehensive review has been conducted. This study aimed to summarize and analyze TKM treatments for depression associated with Parkinson's disease. Methods: This study reviewed research focused on treatment of Parkinson's-related depression, particularly examining traditional therapies such as acupuncture and herbal medicine. Data on treatment methods were analyzed to compare approaches of control and experimental groups. Specific use of acupuncture points and other TKM treatments was analyzed. Results: Out of 57 studies, 17 involving 649 participants were selected. Various treatment methods were evaluated, primarily using the Unified Parkinson's Disease Rating Scale (UPDRS). Studies predominantly focused on acupuncture targeting specific points (such as LI 4, GB 20, and ST 36) and the use of various herbal medicine combinations. Conclusions: Of the 17 studies, 13 focused on effects of acupuncture, suggesting that acupuncture might play a significant role in alleviating depression associated with Parkinson's disease. Specifically, the use of acupuncture points such as LI 4, GB 20, and ST 36 showed potential therapeutic effects. Additionally, 13 studies demonstrated that TKM could significantly alleviate depression, indicating the potential for an integrative approach combining Eastern and Western therapies. Herbal medicine and Qigong dance therapy also showed promising effects in improving depression.

• 대한유전성대사질환학회지
• /
• 제21권1호
• /
• pp.15-21
• /
• 2021

목적: 요소회로대사 질환은 선천성 대사이상질환으로, 요소 회로 각 단계의 특정 효소의 결핍에 따라 다양한 질환과 임상적 증상이 나타난다. 본 연구의 목적은 요소회로대사 질환의 다양한 종류에 따른 장기적 임상 경과를 조사하는 것이다. 방법: 부산대학교 어린이병원에 등록된 22명의 요소회로대사 질환 환자들의 임상적 양상과 생화학적, 유전학적 검사 결과들을 포함한 의무기록을 후향적으로 조사하였다. 결과: 본 연구에서 진단된 요소회로대사 질환은, OTCD 10명(45.5%), ASSD 6명(27.3%), CPS1D 3명, HHHS 2명 ARG1D 1명으로 확인되었다. 진단 시 평균연령은 32.7±66.2개월(범위 0.1-228.0개월) 이었다. 요소회로대사 질환 환자 8명(36.4%)에서 성장 장애가 동반되었다. 또한 요소회로대사 질환 환자11명(50%)에서는 신경학적 후유증이 관찰되었다. 분자유전학적 분석 결과 새로운 돌연변이 14개를 포함해 37개의 서로 다른 돌연변이 유형(과오돌연변이 14개, 넌센스 6개, 결실변이 6개, 스플라이싱변이 6개, 결실삽입변이 3개, 삽입변이 1개, 중복변이 1개)이 확인됐다. 진행성 성장 장애와 나쁜 신경학적 결과는 혈장 이소루이신과 루이신 농도와 각각 관련이 있었다. 결론: 단백의 제한과 같은 조치나 과다한 질소를 제거하는 약제의 복용에도 불구하고, 요소회로대사 질환의 예후는 아직까지 만족스럽지 못하다. 가지사슬아미노산의 보충이 요소회로대사 질환 환자들의 성장부전과, 대사성 위기 또는 신경학적 예후에 효과를 보일지에 관해서는 전향적인 추가 연구가 필요하겠다.