• Journal of Audiology & Otology
• /
• 제25권1호
• /
• pp.49-54
• /
• 2021

Type II mucopolysaccharidosis (MPS II) commonly known as Hunter syndrome, is a rare X-linked lysosomal storage disorder caused by iduronate-2-sulfatase deficiency, which in turn causes otorhinolaryngological manifestations, including sensorineural hearing loss (SNHL). Previously, the median survival age of patients with MPS was approximately 13.4 years. However, in the era of enzyme replacement therapy and other multidisciplinary care modalities, the life expectancy has increased. Herein, we report a rare case of an adolescent with MPS II who underwent SNHL treatment with cochlear implantation (CI). Based on unexpected findings of mastoid emissary veins and overgrowth of the vessels around the temporal bone, CI was performed using the transmeatal approach instead of the conventional transmastoid method, to avoid damage to the vessels. The average hearing threshold after CI was 35 dB and no surgical complications were encountered. Adolescent MPS II may present vessel abnormalities, which can reduce the success rate of surgery. In patients with MPS II with SNHL, CI should be performed under careful monitoring of vessel overgrowth. Moreover, with regard to feasibility of CI in adolescent patients with MPS II with SNHL, surgical techniques such as the transmeatal approach should be selected based on adequate assessment of the case.

• 상하수도학회지
• /
• 제36권2호
• /
• pp.107-119
• /
• 2022

The frequent detection and occurrence of micropollutants (MPs) in aquatic ecosystems has raised public health concerns worldwide. In this study, the behavior of 50 MPs was investigated in three different domestic wastewater treatment plants (WWTPs). Furthermore, the Kruskal-Wallis test was used to assess the geographical and seasonal variation of MPs in the WWTPs. The results showed that the concentrations of 43 MPs ranged from less than 0.1 to 237.6 ㎍ L^-1, while other seven MPs including 17-ethynylestradiol, 17-estradiol, sulfathiazole, sulfamethazine, clofibric acid, simvastatin, and lovastatin were not detected in all WWTPs. Among the detected MPs, the pharmaceuticals such as metformin, acetaminophen, naproxen, and caffeine were prominent with maximum concentrations of 133.4, 237.6, 71.5, and 107.7 ㎍ L^-1, respectively. Most perfluorinated compounds and nitrosamines were found at trace levels of 1.2 to 55.3 ng L^-1, while the concentration of corrosion inhibitors, preservatives (parabens), and endocrine disruptors ranged from less than 0.1 to 4310.8 ng L^-1. Regardless of the type of biological treatment process such as MLE, A2O, and MBR, the majority of pharmaceuticals (except lincomycin, diclofenac, iopromide, and carbamazepine), parabens (except Methyl paraben), and endocrine disruptors were removed by more than 80%. However, the removal efficiencies of certain MPs such as atrazine, DEET, perfluorinated compounds (except PFHxA), nitrosamines, and corrosion inhibitors were relatively low or their concentration even increased after treatment. The results of statistical analysis reveal that there is no significant geographical difference in the removal efficacy of MPs, but there are temporal seasonal variations in all WWTPs.

• 한국안광학회지
• /
• 제20권1호
• /
• pp.51-60
• /
• 2015

목적: 시판되고 있는 소프트콘택트렌즈용 다목적용액이 사람의 각막상피세포에 미치는 세포독성을 이미지 분석법을 이용하여 평가하고자 하였다. 방법: 각막상피세포를 6종류의 다목적용액(A~F)이 0.05~50% 포함된 배양액에서 각각 2시간, 12시간, 24시간, 48시간 동안 배양하였다. 배양 후 각막상피세포를 고정한 다음 Draq 5로 염색하고 공초점현미경과 ImageXpress $Ultra^{TM}$를 이용하여 세포형태를 관찰하고 세포생존율과 세포자살(apoptosis)을 비교하였다. 결과: 각막상피세포 생존율과 세포자살은 다목적용액을 2시간 처리한 경우에는 모든 제품에서 대조군과 차이가 없었으나, 12시간 이상 처리한 경우에는 B~F 제품에서 대조군의 52~75% 수준으로 감소하였고, 세포자살은 모든 제품에서 차이가 없었다. 24~48시간 처리한 경우에는 생존율이 29~73% 수준으로 감소하였고(p<0.05), 세포자살은 199~526% 증가하였다. 제품별로는 다목적용액 D, E, F가 A보다 각막상피세포 생존율을 감소시켰고 세포자살을 증가시켰다(p<0.05). 결론: 저농도의 다목적용액은 각막상피세포 독성에 영향을 미치지 않지만 고농도의 다목적용액은 각막상피세포의 자살을 유도하고 세포생존율을 저하시킬 수 있으므로 다목적용액의 성분은 소독기능이 우수하고 독성이 없는 성분으로 개발되어야 할 것으로 사료된다.

• 한국해양생명과학회지
• /
• 제6권1호
• /
• pp.31-37
• /
• 2021

Larvae from the marine medaka fish Oryzias javanicus were exposed with polystyrene microplastics (MPs) for 24 h. Exposure to waterborne fluorescent MPs showed clear ingestion and egestion in feces. Under constant MPs, the concentration of dissolved oxygen significantly decreased in 24 h compared to the control. Significant intracellular reactive oxygen species and malondialdehyde contents were detected in larvae, indicating oxidative stress and lipid peroxidation. Significant elevations in mRNA expressions of heat shock protein 70 and antioxidant defense system genes (glutathione reductase, glutathione peroxidase, catalase, and superoxide dismutase) were measured with increases in enzymatic activity of oxidative stress-related proteins. Taken together, the alterations to the molecular and biochemical components suggested that waterborne MPs had an oxidative stress effect on marine medaka larvae.

• 한국수산과학회지
• /
• 제57권3호
• /
• pp.227-238
• /
• 2024

This study aimed to evaluate the effects of polyethylene microplastic (PE-MPs) via measuring the growth performance, hematological parameters, and antioxidant responses in Korean rockfish Sebastes schlegelii exposed to waterborne polyethylene microplastic with dimensions of 22-71 ㎛. S. schlegelii (mean weight, 34.55±5.82 g; mean length, 12.59±0.79 cm) were exposed to PE-MPs at concentrations of 0, 400, 800, 1,600 and 3,200 ㎍/L for 10 and 20 days. PE-MPs significantly affected growth performance, hematological parameters, plasma components, and antioxidant responses in a concentration-dependent manner. At a concentration ≥1,600 ㎍/L, PE-MPs significantly decreased body weight gain and specific growth rate, and significantly increased the hepatosomatic index. Hematological parameters showed a significant decrease in total red blood cell count and hemoglobin levels. Plasma components showed a significant increase in glucose, aspartate aminotransferase, and alanine transaminase levels, whereas total protein, calcium, and magnesium levels significantly decreased. Exposure to ≥1,600 ㎍/L PE-MPs also induced reactive oxygen species generation in the gill and liver, significantly increasing superoxide dismutase and catalase activity. These findings suggest that exposure to ≥1,600 ㎍/L PE-MPs could significantly change growth performance, hematological parameters, plasma components, and antioxidant responses, resulting in physiological toxicity.

• The Korean Journal of Pain
• /
• 제14권1호
• /
• pp.32-36
• /
• 2001

Background: There is no reliable objective test for the diagnosis of myofascial pain syndromes. The aim of this study was to evaluate the usefulness of a pressure algometer for the diagnosis of the trigger points and for the evaluation of the treatment in myofascial pain syndromes (MPS). Methods: Twenty female patients with clinical MPS of shoulder were included in this study. Pressure pain thresholds were measured by a pressure algometer at three different sites including the trapezius, supraspinatus and infraspinatus before, and then the 1st, 3rd and 7th days after TPI. Results: Mean pressure pain thresholds were lower in patients with MPS in than normal volunteers in all the examined skeletal muscles. Mean pressure pain thresholds in patients with MPS were increased significantly after TPI in all the examined skeletal muscles. Conclusions: Pressure algometer can be used as relatively objective diagnostic tool for locating trigger points and to quantify the effect of TPI in MPS. However, more investigation is necessary.

• 한국환경과학회지
• /
• 제18권10호
• /
• pp.1103-1114
• /
• 2009

In this study, the environmental behavior of malodor pollutants (MPs: $H_2S$, $CH_3SH$, DMS, and DMDS) was investigated around areas influenced by strong anthropogenic processes based on observations and modeling study (a CALPUFF dispersion model). The MP emission concentrations were measured from 8 industrial source regions (tire plants (S1-S3), waste water disposal plant (S4), and oil refinery (S5) in an urban center area and paper mill/incineration plant (S6) and livestock feedlots (S7-S8) in Ungsang area) in Yangsan city during a fall period in 2008 (21 October 2008). Overall, the most MPs emitted from the urban center area were found to affect the malodor pollution in their downwind areas during early morning (06:00 LST) and nighttime (18:00 and 21:00 LST), compared with those in the Ungsang area. For malodor intensity, the most MPs in the urban center area (especially S1 and S2) were found to be a significant contributor, whereas $CH_3SH$ and $H_2S$ in the Ungsnag area (especially S6) were the dominant contributor. The model study showed agreement in the spatial distributions of simulated MPs with those of the observations. The largest impact of MPs in the urban center area on the malodor pollution in its residential areas occurred at S1, S2, and S3 sites during nighttime, while that of MPs in the Ungsang area occurred at S6 and S8 sites. This may be caused mainly by the high MP emissions and in part by wind conditions (prevailing northeasterly winds with low wind speeds of 2-3 m/s).

• 한국방재학회:학술대회논문집
• /
• 한국방재학회 2009년도 정기 학술발표대회
• /
• pp.56.1-56.1
• /
• 2009

• Clinical and Experimental Pediatrics
• /
• 제48권10호
• /
• pp.1132-1138
• /
• 2005

목 적 : 뮤코 다당체 침착증은 glycosaminoglycan을 분해하는 라이소솜 효소의 유전적 결핍에 의해 라이소솜에 전구 물질이 축적되는 질환군이다. 임상 양상은 매우 다양하지만, 일반적으로 만성적이고 진행되는 경과를 보이며 투박한 얼굴 모양, 관절의 경직, 간비 비대, 성장 지연, 신경학적 퇴화를 특징으로 한다. 최근 뮤코 다당체 침착증 I형의 효소 대체 요법이 가능하며 곧 II형에서도 실용화될 전망이다. 임상 증상이 나타나기 전에 효소 치료를 시작함으로써, 뮤코 다당체 침착증 환자에서 보이는 신경학적 및 근골격계 퇴행의 예방이 가능할 것으로 기대하고 있으며, 이에 조기 진단이 더욱 강조되고 있는 상황이다. 저자들은 본원 뮤코 다당체 침착증 환아 모임에 가입된 환아 80명을 대상으로 임상 양상을 분석하여, 국내 뮤코 다당체 환아들의 임상양상을 연구하고자 하였다. 방 법 : 1995년 2월부터 2004년 12월까지 삼성서울병원 소아과를 방문한 환아 중, 피부 섬유아세포 배양 효소 검사에 의해 뮤코 다당체 침착증이 확진된 환아 80명을 대상으로 하였다. 입원기록 및 외래기록을 검토하여 진단시 연령과 성별, 가족력, 이학적 특징, 방사선 검사, 이비인후과 검사, 안과 검사, 지능검사, 언어 평가에 대한 결과를 분석하였다. 결 론 : 유형별로 II형이 51명(64%), III형이 14명(17.5%), I형이 12명(15%), IV형이 3명(3.8%)으로 II형의 빈도가 월등히 높았다. 진단시 연령은 1세부터 20까지 있었으며, 평균 5.5세였다. 남녀비는 4.7:1이였고, II형 헌터 증후군 51명은 모두 남아였다. 부모가 환아에서 이상을 느낀 첫 징후는 발달 지연이 12례(17%)로 가장 많았고, 그 외에 언어 발달지연(17%), 외모의 이상(16%), 관절 경직(14%) 등이 있었다. 방사선 검사상 전형적인 골격계 변화가 45례(83%)에서 관찰되었다. 55례에서 심장 초음파 검사를 시행했는데 판막의 비후와 경한 역류 소견이 많았고 특히 승모판막의 비후와 역류가 각각 46례(82%), 31례(55%)로 가장 빈번하였다. 이비인후과 평가를 받은 63례 중 46례(73%)에서 중이저류가 관찰되었고, 28례의 환아는 환기관 삽입을 시행 받았다. 33례(82%)에서 중등도 이상의 청력소실이 있었고, 특히 II형 환아들의 ABR 역치 평균이 66.9로 가장 높았다. 지능검사가 가능했던 35례의 환아 중에서 중등도 이상의 정신 지체가 14례(56%)였다. II형 51례의 환아 중에서 4명이 HLA 일치되는 형제로부터 골수이식을 받았다. 그 중 1명은 이식편대 숙주 반응 합병증으로 사망하였고, 나머지 3명에서도 신경학적 퇴행을 예방하지 못하였다. 현재 5명의 I형 환아들에서 효소 대체 치료를 시작하였고 이들의 임상 경과를 주목하고 있다. 결 론 : 본 연구에서는 80명의 뮤코 다당체 침착증 환자를 대상으로 임상 양상을 분석하였고 유형별로는 II형 헌터 증후군의 빈도가 64%로 외국 연구에 비해 월등히 높았으며 이는 인구학적 차이를 반영한다고 사료된다. 발달 지연, 저신장, 근골격계 변화, 심장판막 변화, 정신 지체, 청력 소실 등 뮤코 다당체 침착증의 전형적인 증상들이 환아의 대부분에서 관찰되었다. 저자들은 국내 뮤코 다당체 침착증 환아들의 임상적 양상을 연구함으로써 조기 진단과 적절한 치료를 하는데 도움이 되고자 하였다.

• Clinical and Experimental Pediatrics
• /
• 제55권11호
• /
• pp.430-437
• /
• 2012

Purpose: Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is rare lysosomal storage disorder caused by N-acetylgalactosamine-6-sulfatase (GALNS) deficiency. Only a few MPS IVA cases have been reported in the Korean literature; there is a paucity of research about clinical or radiologic findings for this disorder. Therefore, we studied clinical findings, radiological features, and genetic data of Korean MPS IVA patients for determining factors that may allow early diagnosis and that may thus improve the patients' quality of life. Method: MPS IVA was confirmed via assay for enzymatic activity of leukocytes in 10 patients. The GALNS gene was analyzed. Patients' charts were retrospectively reviewed for obtaining clinical features and evaluated for radiological skeletal surveys, echocardiography, pulmonary function test, and ophthalmologic test results. Result: Nine patients had severe clinical phenotype, and 1 had an intermediate phenotype, on the basis of clinical phenotype criteria. Radiologic findings indicated skeletal abnormalities in all patients, especially in the hips and extremities. Eight patients had an odontoid hypoplasia, and 1 showed mild atlantoaxial subluxation and cord myelopathy. Genetic analysis indicated 10 different GALNS mutations. Two mutations, c.451C>A and c.1000C>T, account for 37.5% (6/16) and 25% (4/16) of all mutations in this samples, respectively. Conclusion: An understanding of the clinical and radiological features involved in MPS IVA may allow early diagnosis of MPS IVA. Adequate evaluations and therapy in the early stages may improve the quality of life of patients suffering from skeletal abnormalities and may reduce life-threatening effects of atlantoaxial subluxation.