• 제목/요약/키워드: Intermediate syndrome

검색결과 40건 처리시간 0.032초

경련을 동반한 신생아 부신백질이영양증 (Neonatal Adrenoleukodystrophy Presenting with Neonatal Seizure)

  • 신영림;유한욱
    • 대한유전성대사질환학회지
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    • 제2권1호
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    • pp.15-19
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    • 2002
  • Disorders resulting from defects in peroxisomal biogenesis include Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. The three diseases are now considered as a continuum of clinical features. Neonatal adrenoleukodystrophy is intermediate between Zellweger syndrome and infantile Refsum disease in severity, and is characterized by profound hypotonia, intractable seizures and premature death. We report a cases of neonatal adrenoleukodystrophy presenting with neonatal seizure and hypotonia. At the age of 43 months, she had clinical evidence of adrenal insufficiency with skin hyperpigmentation and electrolyte imbalance. She was diagnosed having neonatal adrenoleukodystrophy based on abnormally high levels of plasma very long-chain fatty acids, pipecolic acid and phytanic acid.

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Intermediate syndrome after dermal exposure to organophosphate insecticide

  • Lee, Su Bin;Ryu, Seung Ho;Park, Doo Yong;Park, Jong-Ho;Kim, Jee Young
    • Annals of Clinical Neurophysiology
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    • 제20권1호
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    • pp.41-43
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    • 2018
  • ntermediate syndrome (IMS) typically occurs at 24-96 hours following organophosphate (OP) poisoning, after an acute cholinergic crisis, but before OP-induced delayed polyneuropathy. It is characterized by proximal muscle weakness and respiratory insufficiency, which is a major contributing factor of OP-related morbidity and mortality. We report an atypical IMS case showing rapid-onset ascending paralysis and respiratory disturbance with an acute cholinergic crisis occurring 4-5 days after skin exposure to OP.

지역사회 획득 메치실린 내성 포도알균에 의한 포도알균 열상 피부 증후군의 임상적 고찰 (A clinical review of community acquired methicillin resistant staphylococcal scalded skin syndrome)

  • 허순영;송윤정;김성준;박선영;강두철;마상혁
    • Pediatric Infection and Vaccine
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    • 제14권1호
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    • pp.83-90
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    • 2007
  • 목 적 : 포도알균 열상 피부 증후군(Staphylococcal Scalded Skin Syndrome)은 포도알균의 피부박탈성 독소에 의하여 전신성 수포와 낙설을 일으키는 포도알균의 피부 감염증의 한 형태이다. 환자들의 임상양상과 치료의 결과, 합병증 등을 알고 자 본 연구를 시행하였다. 방 법 : 2002년 2월부터 2005년 12월까지 특징적인 피부병변을 보여 포도알균 열상 피부 증후군으로 진단된 53명의 환자들을 대상으로 의무기록을 후향적으로 검토하였고 임상형을 전신형, 중간형, 부전형 등으로 분류하고 2003년 9월이후 부터 발생한 환자의 비강 내 검체를 이용하여 원인 균 및 독소를 규명하였다. 결 과 : 1) 연구기간 동안 4S로 진단되어 치료한 환자는 총 53명이었다. 발병연령은 생후 20일부터 7세까지 분포하였으며, 평균연령은 2.8세였다. 성별은 남자가 35명이었고 여자는 18명으로 남여 성비는 1.9:1이었다. 2) 임상적인 유형은 전신형이 6례(11%), 중간형이 29례(55%), 부전형이 18례(34%)로 중간형이 가장 많았다. 대부분의 환자에서 혈액 검사 상 백혈구 증다증이나 C반응단백의 증가는 없었다. 3) 2003년 9월 이후에 15명 환자의 비강에서 얻은 검체 중 14명의 환자에서 얻은 검체에서 포도알균을 분리하였고 피부박탈성 독소는 독소 B의 유전자를 확인하였고 Panton-Valentine leukocidin의 유전자는 음성으로 나왔다. 4) 입원하여 치료받은 환자의 경우 평균 입원 기간은 7.3일이었으며 사용한 항생제는 amoxacillin/clavulanate, ampicillin/sulbactam, cefuroxime, vancomycin 등이었다. 모든 환자에서 전신적인 낙설이 나타났으며 임상형이나 사용한 항생제 종류에 관계없이 모든 환자가 특별한 합병증이 없이 회복이 되었다. 결 론 : 창원과 인근 지역에서 단기간 내에 지역사회 획득 메치실린 내성 포도알균감염으로 인한 다수의 4S 환자가 연속적으로 발생하였으며 원인은 피부박탈성 독소 B이었다.

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1,2-Dichloropropane (1,2-DCP)-Induced Angiogenesis in Dermatitis

  • Jin, Meiying;Hong, Youngeun;Lee, Hyunji;Tran, Quangdon;Cho, Hyeonjeong;Kim, Minhee;Kwon, So Hee;Kang, Nak Heon;Park, Jisoo;Park, Jongsun
    • Toxicological Research
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    • 제35권4호
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    • pp.361-369
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    • 2019
  • 1,2-Dichloropropane (1,2-DCP) has been used as an industrial solvent and a chemical intermediate, as well as in soil fumigants. Human exposure may occur during its production and industrial use. The target organs of 1,2-DCP are the eyes, respiratory system, liver, kidneys, central nervous system, and skin. Repeated or prolonged contact may cause skin sensitization. In this study, 1,2-DCP was dissolved in corn oil at 0, 2.73, 5.75, and 8.75 mL/kg. The skin of mice treated with 1,2-DCP was investigated using western blotting, hematoxylin and eosin staining, and immunohistochemistry. 1,2-DCP was applied to the dorsal skin and both ears of C57BL/6J mice. The thickness of ears and the epidermis increased significantly following treatment, and the appearance of blood vessels was observed in the dorsal skin. Additionally, the expression of vascular endothelial growth factor, which is tightly associated with neovascularization, increased significantly. The levels of protein kinase-B (PKB), phosphorylated PKB, mammalian target of rapamycin (mTOR), and phosphorylated mTOR, all of which are key components of the phosphoinositide 3-kinase/PKB/mTOR signaling pathway, were also enhanced. Taken together, 1,2-DCP induced angiogenesis in dermatitis through the PI3K/PKB/mTOR pathway in the skin.

대동맥관 폐쇄부전을 동반한 상행대동맥의 외과적 치료 (Results with Total Replacement of the Ascending Aorta and Reimplantation of the Coronary Arteries)

  • 안혁;노준량
    • Journal of Chest Surgery
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    • 제24권4호
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    • pp.352-356
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    • 1991
  • From April, 1981, to April, 1990, 20 male and 7 female patients ranging in age from 17 to 63, were operated on for aortic insufficiency with an aneurysm of the ascending aorta. Ten patients were in New York Heart Association functional class II, 7 in class III, and ten in class IV. The surgical treatment in all cases consisted of total replacement of the ascending aorta with composite graft containing a prosthetic aortic valve and reimplantation of the coronary arteries by an intermediate tube graft. In 15 patients an uncomplicated annulo-aortic ectasia existed, and in 12 an aortic dissection; three of the latter group were operated during the acute phase. 17 patients showed typical Marfan syndrome, and 3 patients showed severe ascending aortic aneurysm secondary to the aortic valve disease. The overall operative mortality was 7%[2 deaths]. Those 2 deaths occurred following emergency operation due to associated aortic dissection, but no death during elective operation. All survivors have been followed-up during a period ranging 1 to 108 month[average 34 months]. There was no late mortality. Among the survivors, clinical improvement is readily apparent[2,3 in class I, 2 in class II ]. In conclusion, the treatment of aortic insufficiency associated with an aneurysm of the ascending aorta by insertion of a composite graft and reimplantation of the coronary arteries through an intermediate Dacron tube is a reliable method with low mortality and excellent results.

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Hemostatic Dysfunction in a Dog with Mammary Gland Carcinoma

  • Rankyung Jung;Hyeona Bae;ARom Cho;Young Ju Kim;Yeseul Jun;Minji Kim;Sumin Cha;Min-Jeong Kang;Tae-Sung Hwang;Hee-Chun Lee;Kyu-Woan Cho;Dong-In Jung;Dae Young Kim;DoHyeon Yu
    • 한국임상수의학회지
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    • 제39권6호
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    • pp.373-377
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    • 2022
  • Hypercoagulability is the most common coagulopathy seen in dogs with neoplasia, whereas a hypocoagulable state is relatively rare. A 16-year-old spayed female miniature Schnauzer presented with bilateral epistaxis, bilateral hindlimb swelling, and lameness. Previously, the dog was diagnosed with intermediate grade solid carcinoma after mastectomy, and then showed generalized ecchymoses on the abdomen and bilateral hindlimbs on presentation. Laboratory tests revealed a hypocoagulable state, including thrombocytopenia, hypofibrinogenemia, and delayed prothrombin and activated partial thromboplastin time. Thromboelastography demonstrated a prolonged K time with a decreased alpha angle and low maximal amplitude, reflecting a decrease in clot strength. The hypocoagulable state of disseminated intravascular coagulopathy was speculated based on the presence of an underlying tumor, clinical symptoms, and laboratory results of the hypocoagulable state. This case shows the incidence of hemostatic dysfunction as a paraneoplastic syndrome in a dog with mammary gland carcinoma.

Effects of Sleep Pattern, Stress, Menstrual Attitude, and Behavior That Reduces Exposure to Endocrine Disrupting Chemicals on Premenstrual Syndrome in Adolescents

  • Kim, Hye Jin;Choi, So Young;Min, Haeyoung
    • 여성건강간호학회지
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    • 제25권4호
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    • pp.423-433
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    • 2019
  • Purpose: The study aimed to examine the effects of sleep pattern, stress, and attitude towards menstruation, and behavior that causes a reduction in exposure to endocrine disrupting chemicals (EDCs) in the course of premenstrual syndrome (PMS) in female adolescents. Method: In total, 200 girls attending middle school in K city were recruited via convenience sampling between December 11 and 31, 2018, and 192 subjects were selected for the analysis. Data were analyzed using descriptive statistics, an independent t-test, ANOVA, Pearson's correlation coefficient, and multiple regression. Results: Overall, 9 (4.7%), 152 (79.2%), and 31 (16.1%) students had morning-, intermediate-, and evening-type sleep pattern, respectively. The mean scores for school-related stress were highest. Scores for stress regarding relationships with friends were the lowest. The mean score for attitudes toward menstruation was 72.33 out of 147. The mean score for behavior that led to a reduction in EDCs exposure was 56.57 out of 85. The mean scores for PMS severity were 25.30 and 38.39 out of 60. Analysis of factors affecting PMS revealed that severity of menstrual pain (β=.28), use of analgesics during menstruation (β=.17), family history of PMS (β=-.16), stress (β=.19), and behavior that reduces EDCs exposure (β=-.17) exerted significant effects on PMS. The explanatory power of these variables was 37%. Conclusion: The findings propose the necessity of a health intervention program including educational information, which can reduce PMS in adolescents, induce the behaviors which can decrease exposure to EDCs, and activities for managing the stress levels.

Evaluation of YouTube videos as sources of information about complex regional pain syndrome

  • Altun, Aylin;Askin, Ayhan;Sengul, Ilker;Aghazada, Nazrin;Aydin, Yagmur
    • The Korean Journal of Pain
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    • 제35권3호
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    • pp.319-326
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    • 2022
  • Background: As the internet usage becomes easily accessible, the patients are more frequently searching about diseases and medical/non-medical treatments. Considering that complex regional pain syndrome (CRPS) is a debilitating disease, it is important to check the information that patients are accessing. Therefore, this study aimed to investigate the reliability, sufficiency, and accuracy of the YouTube videos about CRPS. Methods: This study is a descriptive research which is derived by searching videos using the keyword 'complex regional pain syndrome' on YouTube. Relevance-based sequencing was used to sort the videos. Sources and video parameters were documented. To evaluate the accuracy, reliability and content quality of the videos, Global Quality Score, Journal of American Medical Association Benchmark Criteria and Modified DISCERN Questionnaire scales were used. Results: A total of 167 videos were included in this study. The majority of the videos originated from USA (80.2%, n = 134). The median number of views was 639 and the viewing rate was 73.3. Most of the videos had partially sufficient data and the interaction index viewing rate parameters for videos with high content quality were greater than videos with low content quality (P = 0.010, P = 0.014). Conclusions: Our results showed that videos about CRPS on YouTube mostly had partially sufficient data and include intermediate-high quality contents. Moreover, high-content quality videos had higher viewing rates, interaction indexes, number of likes, longer durations, as well as better reliability and accuracy scores. Videos with high quality and reliable content are needed to reduce misinformation about CRPS.

사상체질에 따른 대사증후군과 수족냉증 분포 차이와 역상관관계 (Inverse Association and Differences in the Distribution of Metabolic Syndrome and Cold Hypersensitivity in the Hands and Feet According to Sasang Constitution)

  • 배광호;박기현;이시우
    • 사상체질의학회지
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    • 제34권1호
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    • pp.1-12
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    • 2022
  • Objectives This study aimed to examine the differences in the distribution of metabolic syndrome(MetS) and cold hypersensitivity in the hands and feet(CHHF) according to Sasang constitution, and to determine whether CHHF and MetS have an inverse association. Methods MetS and its components, CHHF, Sasang constitution data from 1,998 participants in the Korean medicine Daejeon Citizen Cohort study(KDCC) were obtained. The participants were divided into a non-CHHF(n = 1,270, 63.6%), intermediate(n = 220, 11.0%) and CHHF(n = 508, 25.4%) group according to the thermal sensitivity questionnaire. Sasang constitution was diagnosed by Korea Sasang Constitutional Diagnostic Questionnaire(KS-15). One-way ANOVA and the chi-square test were used for participants' general characteristics and thermal sensitivity and MetS related factors. ANCOVA and logistic regression were used to compare the differences and the odds ratios(ORs) for MetS and its components. Results The MetS and CHHF prevalence rates of the Taeeumin, Soeumin, and Soyangin were 27.6%, 3.8%, 7.7%, and 18.3%, 42.3%, 26.4% respectively. The ANCOVA for MetS components showed that the waist circumference was significantly lower in the CHHF group as compared to the non-CHHF group in total and Soyangin. The logistic regression for MetS prevalence showed that CHHF had a significant inverse association in total(OR = 0.611) and Taeeumin(OR = 0.521). Conclusions The MetS prevalence had the highest in Taeeumin, followed by Soyangin and Soeumin, while the prevalence of CHHF was highest in Soeumin, followed by Soyangin and Taeeumin. In addition, it was confirmed that CHHF and MetS had an inverse association independently.

폐경후 여성호르몬 보충요법 (Postmenopausal Hormone Replacement Therapy)

  • 박형무
    • 정신신체의학
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    • 제4권1호
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    • pp.98-108
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    • 1996
  • Korean women are now living almost 1/3 of their life after menopause. Sex-steroid hormone deficiency adversely affect various fields of physical & mental activity and quality of life during this period. Therefore, replacement of deficient hormone is thought to be natural therapeutic modality. Postmenopausal syndrome is recently redefined as an endocrinopathy with both short-and long-term sequelae, as a result of cessation of ovarian function. Hormone replacement therapy taken at or near time of menopause alleviates shot-term acute menopausal symptoms such as vasomotor disturbances and psychological problems. HRT also beneficially affects some of intermediate symptoms such as urogenital atrophy and cutaneous problems. The major benefits of long-term use were reductions in risk of long-term sequelae, that is, total fracture by 50-60%, cardiovascular disease by 50% and cerebrovascular disease by 30-40%, respectively. In addition, HRT may also positively influence Alzheimer's disease, reduce the colorectal cancer risk and increase longevity of the life. In conclusion, all postmenopausal women should consider preventive HRT when there are no contraindications.

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