Malignancy in Patients With Inborn Errors of Immunity Beyond Infectious Complication: Single Center Experience for 30 Years

( Doo Ri Kim ),( Kyung-ran Kim ),( Hwanhee Park ),( Joon-sik Choi ),( Yoonsun Yoon ),( Sohee Son ),( Hee Young Ju ),( Jihyun Kim ),( Keon Hee Yoo ),( Kangmo Ahn ),( Hee-jin Kim ),( Eun-suk Kang ),( Ju 대한소아감염학회 2023 Pediatric Infection and Vaccine Vol.30 No.3

목적: 선천면역장애 환자들은 감염에 취약할 뿐만 아니라, 면역이 정상인 사람들에 비해 암 발생률도 높은 것으로 알려져 있다. 본 연구는 단일 기관에서 추적 중인 선천면역장애 환자들에서의 암 발생을 조사하여 보고자 하였다. 방법: 1994년 11월부터 2023년 9월까지 삼성서울병원에서 선천면역장애 진단 하에 추적하는 환자를 대상으로 후향적으로 의무기록을 리뷰하였다. 선천면역장애 환자 중에서 암으로 진단된 환자를 확인하였다. 결과: 총 194명의 선천면역장애 환자 중, 7명(3.6%)의 환자에서 암이 진단되었다. 5명의 환자가 림프종으로 진단받았으며 그 중 4명의 환자는 Epstein-Barr 바이러스 연관 림프종이었다. 나머지 암은 위암과 다발 골수종이었다. 암 진단 당시 나이는 중앙값 18세 (범위, 1세-75세)였다. 암이 발생한 환자들의 면역결핍 질환은 X-linked lymphoproliferative disorder-1 (XLP-1) 3명, activated phosphoinositide 3-kinase delta disease (APDS) 2명, cytotoxic T-lymphocyte antigen 4 (CTLA-4) haplo-insufficiency 2명이었다. 개별 질환별로 분석하였을 때, XLP-1 환자의 75.0%, APDS 환자의 40.0%, CTLA-4 환자의 50.0%에서 암이 발생하였다. XLP-1 환자는 APDS 및 CTLA-4 haplo-insufficiency 환자에 비해 더 이른 나이에 암이 발생하였다 (중앙연령 5세, P<0.001). 한 명은 조혈모세포 이식 치료 중 사망하였다. 결론: 국내 단일 기관에서 진료받는 선천면역장애 환자들의 3.6%에서 암이 발생하였다. 선천면역장애 환자들을 진료하는 의료진들은 이들 환자에서 감염이나 염증 등의 문제외에도 암 발생의 가능성, 특히 Epstein-Barr 바이러스 감염과 연관된 암의 비중이 높은 것에 대한 인식을 갖는 것이 중요하다 Purpose: Cancer incidence is known to be higher in patients with inborn errors of immunity (IEI) compared to the general population in addition to traditionally well-known infection susceptibility. We aimed to investigate cancer occurrence in patients with IEI in a single center. Methods: Medical records of IEI patients treated at Samsung Medical Center, Seoul, Korea were retrospectively reviewed from November 1994 to September 2023. Patients with IEI and cancer were identified. Results: Among 194 patients with IEI, seven patients (3.6%) were diagnosed with cancer. Five cases were lymphomas, 4 of which were Epstein-Barr virus (EBV)-associated lymphomas. The remaining cases included gastric cancer and multiple myeloma. The median age at cancer diagnosis was 18 years (range, 1-75 years). Among patients with cancer, underlying IEIs included X-linked lymphoproliferative disease-1 (XLP-1, n=3), activated phosphoinositide 3-kinase delta syndrome (APDS, n=2), and cytotoxic T-lymphocyte antigen 4 (CTLA-4) haplo-insufficiency (n=2). Seventy-five percent (3/4) of XLP-1 patients, 40.0% (2/5) of APDS patients, and 50.0% (2/4) of CTLA-4 haplo-insufficiency patients developed cancer. Patients with XLP-1 developed cancer at earlier age (median age 5 years) compared to those with APDS and CTLA-4 (P<0.001). One patient with APDS died during hematopoietic cell transplantation. Conclusions: Cancer occurred in 3.6% of IEI patients at a single center in Korea. In addition to infectious complications and inflammation, physicians caring for IEI patients should be aware of the potential risk of cancer, especially in association with EBV infection

Severe Human Rhinovirus Lower Respiratory Tract Infections in Young Children

( Doo Ri Kim ),( Kyung-ran Kim ),( Hwanhee Park ),( Esther Park ),( Joongbum Cho ),( Jihyun Kim ),( Hee Jae Huh ),( Kangmo Ahn ),( Nam Yong Lee ),( Yae-jean Kim ) 대한소아감염학회 2023 Pediatric Infection and Vaccine Vol.30 No.3

목적: 리노바이러스의 감염은 하기도 감염을 일으키기도 한다. 본 연구에서는 리노바이러스에 의한 중증 하기도 감염을 보이는 소아환자의 특성을 알아보고자 하였다. 방법: 2016년부터 2020년까지 삼성서울병원 소아청소년과에 리노바이러스 하기도감염으로 입원한 환자의 의무기록을 후향적으로 분석하였다. 입원 시 연령이 생후90일 이상, 5세 미만인 소아 환자를 대상으로 하였다. 다른 호흡기 병원체와의 동시 감염이 확인된 환자는 제외하였다. 리노바이러스에 의한 중증 하기도감염은 고유량 산소요법 치료가 필요한 경우, 기계 호흡이 필요한 경우 또는 중환자실 입원하는 경우로 정의하였다. 결과: 해당 기간 동안 총 115건의 리노바이러스 하기도 감염 입원이 확인되었다. 연령 중앙값은 17개월 (범위, 3-56개월) 이었으며, 입원 일수 중앙값은 4일 (범위, 2-31일) 이었다. 115 건 중 18건의 입원 (15.7%)은 중증 리노바이러스 하기도 감염 그룹으로 분류되었다. 중증 경과 그룹 환자의 연령 중앙값은 그렇지 않은 그룹에 비해 연령 중앙값이 낮았다 (9.5개월 vs. 19.0 개월, P=0.001). 18명의 중증 리노바이러스 하기도 감염 그룹 환자 중 11명 (61.1%)는 기저질환을 가지고 있었으며, 만성 폐질환이 가장 많은 비율을 차지하였다 (63.6%). 여섯 명의 환자는 (33.3%) 기계 호흡을 필요로 하였다. 일곱 명의 기저질환이 없는 환자도 중증 리노바이러스 하기도 감염 그룹에 포함되어 있었다. 이들 일곱 명의 환자 중 네 명은 추후에 천식으로 진단되었다. 115건의 입원을 기저질환이 없는 환자군 (n=60)과 기저질환이 있는 환자군 (n=55)으로 나누어 분석하였을 때, 리노바이러스에 의한 중증 하기도 감염을 보이는 비율은 각각 11.7% 와 20.0%였다 (P=0.219). 결론: 리노바이러스 감염은 중증 하기도감염의 원인이 될 수 있으며, 기저질환자 뿐 아니라 건강한 소아에서도 중증 하기도감염을 일으킬 수 있다 Purpose: Human rhinovirus (HRV) infections can result in lower respiratory tract infections (LRTIs). We aimed to investigate the characteristics of severe HRV LRTI in young children. Methods: Medical records were reviewed retrospectively in patients who were hospitalized for HRV LRTIs from 2016 to 2020 at the Samsung Medical Center in Seoul, Korea. Patients aged 90 days or older and younger than 5 years were included. Patients with co-infections with other respiratory pathogens were excluded. Severe HRV LRTI was defined as the following: the need for high-flow oxygenation, mechanical ventilation, or intensive care unit admission. Results: A total of 115 cases were identified. The median age was 17 months (range, 3-56 months) and the median hospital days were 4 days (range, 2-31 days). Of the 115 cases, 18 patients (15.7%) developed severe HRV LRTI. The median age was younger in the severe group compared to the non-severe group (9.5 months vs. 19.0 months, P=0.001). Of 18 patients with severe HRV LRTI, 11 (61.1%) had underlying diseases - chronic lung diseases accounted for the largest proportion (63.6%). Six patients (33.3%) required mechanical ventilation. Of note, 7 previously healthy children were diagnosed with severe HRV LRTI. Of those 7 children, 4 of them were diagnosed with asthma later. When the 115 cases were divided into previously healthy (n=60) and underlying disease (n=55) groups, severe courses of HRV LRTI were observed in 11.7% and 20.0% of children, respectively (P=0.219). Conclusions: HRV can cause severe LRTI even in previously healthy children as well as in children with comorbidities

Myositis as an Initial Presentation of Ulcerative Colitis before Gastrointestinal Symptoms

Doo Ri Kim,DongSub Kim,SangJoon Choi,Yeon-Lim Suh,유소영,Mi Jin Kim,최연호,김예진 대한소아소화기영양학회 2020 Pediatric gastroenterology, hepatology & nutrition Vol.23 No.3

The musculoskeletal system can be involved as an extra-intestinal manifestation of inflammatory bowel disease. Among these, myositis in ulcerative colitis (UC) is very rare. A 14-year-old girl was admitted due to severe shoulder tenderness. She had complained of left jaw pain and swelling for the past 10 days. Inflammatory markers were elevated with no evidence of infectious etiology. Myositis was suspected by shoulder magnetic resonance imaging. Three days after admission, she developed hematochezia. Muscle biopsy and colonoscopy was performed due to worsening left mandibular area pain and persistent hematochezia. Colonoscopy showed consistent findings with UC. She was finally diagnosed with UC with myositis as an extra-intestinal manifestation. She showed a dramatic response to UC treatment. Gastrointestinal symptoms were well-controlled. After 14 months, UC symptoms and muscle pain were aggravated, which were relieved after steroid and cyclosporin treatment. We report a unique case of UC initially presented with myositis, preceding gastrointestinal symptoms.

Fast Microchip Electrophoresis Using Field Strength Gradients for Single Nucleotide Polymorphism Identification of Cattle Breeds

Oh, Doo-Ri,Cheong, Il-Cheong,Lee, Hee-Gu,Eo, Seong-Kug,Kang, Seong-Ho Korean Chemical Society 2010 Bulletin of the Korean Chemical Society Vol.31 No.7

A microchip electrophoresis (ME) method was developed using a programmed field strength gradients (PFSG) for the single nucleotide polymorphism (SNP) based fast identification of cattle breeds. Four different Korean cattle (Hanwoo) and Holstein SNP markers amplified by allele-specific polymerase chain reaction were separated in a glass microchip filled with 0.5% poly(ethyleneoxide) ($M_r$ = 8 000 000) by PFSG as follows: 750 V/cm for 0 - 14 s, 166.7 V/cm for 14 - 31 s, 83.3 V/cm for 31 - 46 s, and 750 V/cm for 46 - 100 s. The cattle breeds were clearly distinguished within 45 s. The ME-PFSG method was 7 times and 5 times faster than the constant electric field ME method and the capillary electrophoresis- PFSG method, respectively, with a high resolving power ($R_s$ = 5.05 - 9.98). The proposed methodology could be a powerful tool for the fast and simultaneous determination of SNP markers for various cattle breeds with high accuracy.

Controlled Assembly of Gold Nanoprism and Hexagonal Nanoplate Films for Surface Enhanced Raman Scattering

Lee, Doo-Ri,Hong, Soon-Chang,Park, Sung-Ho Korean Chemical Society 2011 Bulletin of the Korean Chemical Society Vol.32 No.10

This paper reports a methodology for preparing close-packed two dimensional gold nanoprism films and hexagonal nanoplate films at a hexane/water interface. By controlling the concentration of linker molecules in the hexane layer and the temperature of the colloid solution, highly ordered close-packed nanoplate arrays can be fabricated. These films were investigated to compare their corresponding surface enhanced Raman scattering (SERS) efficiencies. It was demonstrated that the Au nanoprism films resulted in a stronger SERS enhancement than the Au hexagonal nanoplate films. The difference in the SERS enhancement is attributed to the film array difference, demonstrating that Au nanoprism films have a higher line contact density than their Au hexagonal analogues.

백내장 수술에서 수정체낭원형절개와 인공수정체의 중심이탈 및 경사가 임상 결과에 미치는 영향

어두리(Doo Ri Eo),임동희(Dong Hui Lim),현주(Joo Hyun),이주연(Ju Yeon Lee),임한웅(Han Woong Lim),오재응(Jae Eung Oh),정의상(Eui Sang Chung),정태영(Tae Young Chung) 대한안과학회 2016 대한안과학회지 Vol.57 No.4

목적: 백내장 수술 후 수정체낭원형절개와 인공수정체의 중심이탈 및 경사도가 술 후 임상 결과에 미치는 영향을 알아보고자 한다. 대상과 방법: 백내장 수술을 받은 환자 52명 62안을 대상으로 술 전과 술 후 3개월의 나안 및 교정시력, 현성굴절검사를 시행하였다. 술 후 3개월에 전안부 사진을 촬영하여 인공수정체의 중심이탈 정도를 분석하고, 전안부단층촬영을 하여 인공수정체의 경사도를 분석하여, 술 후 나안시력, 교정시력 및 고위수차와의 관련성을 분석하였다. 또한 술 중 수정체낭원형절개의 크기 및 중심이탈을 측정하여 술 후 인공수정체의 위치 변화와의 연관성을 알아보았다. 결과: 술 중 평균 수정체낭원형절개의 크기는 5.40 ± 0.51 mm (4.12-6.24 mm), 중심이탈은 0.30 ± 0.19 mm (0.09-1.21 mm)였으며, 술 후 평균 인공수정체의 중심이탈은 0.23 ± 0.15 mm (0.00-0.71 mm), 경사도는 1.43 ± 0.73° (0.00-4.22°)로 측정되었다. 술 중 수정체낭원형절개의 크기 및 중심이탈 정도는 술 후 인공수정체의 중심이탈과는 유의한 관련성을 보였으나(p=0.01, p<0.001), 술 후 인공수정체의 경사도와는 관련이 없었다(p=0.69, p=0.52). 술 후 인공수정체의 중심이탈 및 경사도는 술 후 시력, 난시 및 고위수차와는 유의한 관련성을 보이지 않았다(p>0.05). 결론: 술 중 수정체낭원형절개의 크기 및 중심이탈은 술 후 인공수정체의 중심이탈에 영향을 미치지만, 인공수정체의 중심이탈과 경사도는 술 후 임상 결과에 큰 영향을 주지 않는 것으로 생각한다. Purpose: To evaluate the effects of continuous curvilinear capsulorhexis, intraocular lens (IOL) decentration and tilt on postoperative clinical outcomes after cataract surgery. Methods: We reviewed 62 eyes of 52 patients who underwent cataract surgery and measured the uncorrected visual acuity, best corrected visual acuity and manifest refraction preoperatively and 3 months postoperatively. IOL decentration on anterior segment photography and IOL tilt on anterior optical coherent tomography were analyzed and correlations of postoperative uncorrected visual acuity, best corrected visual acuity, and higher order aberrations were evaluated. In addition, we inspected the relationship of size and decentration of continuous curvilinear capsulorhexis (CCC) intraoperatively with the change in IOL position postoperatively. Results: The average size of CCC was 5.40 ± 0.51 mm (4.12-6.24 mm) and the average decentration of CCC was 0.30 ± 0.19 mm (0.09-1.21 mm) intraoperatively. The average decentration of IOL was 0.23 ± 0.15 mm (0.00-0.71 mm) and the average IOL tilt was 1.43 ± 0.73° (0.00-4.22°) postoperatively. Intraoperative CCC size and decentration were associated with postoperative IOL decentration (p = 0.01, p < 0.001), but not with IOL tilt (p = 0.69, p = 0.52). There were no significant correlations between IOL decentration and tilt with postoperative visual outcomes and higher order aberrations. Conclusions: The CCC size and decentration can affect the IOL decentration, but IOL decentration and tilt do not have a significant impact on clinical outcomes after cataract surgery.

이식편대숙주반응으로 장기간 전신스테로이드 투여 후 발생한 소아 스테로이드 유발 녹내장 1예

어두리(Doo Ri Eo),한종철(Jong Chul Han),기창원(Chang Won Kee) 대한안과학회 2016 대한안과학회지 Vol.57 No.3

목적: 이식편대숙주반응으로 장기간 전신 스테로이드를 투여 받은 소아에서 발생한 스테로이드 유발 녹내장 1예를 보고하고자 한다. 증례요약: 10세 남자 환아가 지속적인 두통, 구역감으로 뇌압 상승에 의한 시신경 유두부종 유무 검사를 위해 안과에 내원하였다. 환아는 8년 전 재생불량성빈혈을 진단 받고 같은 해에 조혈모세포이식 후 발생한 폐의 이식편대숙주반응으로 2007년 4월부터 2015년 4월까지 8년 동안 총 4,000 mg 정도의 전신스테로이드(oral prednisolone)를 투여 받았다. 내원 당시 최대교정시력은 우안 0.8, 좌안 0.5, 안압은 골드만 압평 안압계로 우안 42 mmHg, 좌안 43 mmHg로 측정되었다. 시신경 유두비는 우안이 0.8, 좌안은 0.7로 커져 있었고, 양안의 상하측 시신경유두테가 얇아지는 등 녹내장성 시신경 변화 소견이 관찰되었다. 안압하강제를 점안하였으나 안압이 정상범위로 조절되지 않아 양안의 미토마이신 C를 사용한 섬유주절제술을 시행하였다. 결론: 전신스테로이드 투여를 받는 소아환자에서도 그 투여기간이 길 경우에 안압이 상승되고, 녹내장 발생이 가능하므로 이에 대한 정기적 관찰이 필요할 것으로 생각된다. Purpose: To report a case of steroid-induced glaucoma in a child who was treated with systemic steroids for a long period due to graft-versus-host disease. Case summary: A 10-year-old male was referred to our ophthalmologic clinic for examination of papilledema due to persistent headache and nausea. He was diagnosed as aplastic anemia 8 years prior and took approximately 4,000 mg of oral prednisolone for 8 years from April 2007 to April 2015 for treatment of lung graft-versus-host disease after hematopoietic stem cell transplantation. His best corrected visual acuity was 0.8 (decimal) in the right eye, 0.5 in the left eye and intraocular pressure (IOP) measured using a Goldmann applanation tonometer was 42 mm Hg in the right eye and 43 mm Hg in the left eye. His cup-to-disc ratio was 0.8 in the right eye and 0.7 in the left eye. Additionally, superior and inferior neuroretinal rim thinning was present in both eyes. Despite using IOP-lowering agents, IOP was not controlled. However, after trabeculectomy with mitomycin C in both eyes, IOP became normalized. Conclusions: In cases of pediatric patients treated with systemic steroids for a long period of time, regular observation is necessary to prevent IOP elevation and steroid-induced glaucoma.

스마트폰 기반의 3D 영상 처리 구현에 대한 연구

한두리(Doo-Ri Han),문승진(Seung-Jin Moon) 한국통신학회 2013 한국통신학회 학술대회논문집 Vol.2013 No.11

Low-ppm-Level colorimetric acid detection using gold nanoparticles with electro-steric stabilization.

Bae, Doo Ri,Lee, You-Jin,Lee, Sung Woo,Han, Young-Kyu,Yoon, Jae-Sik,Lee, Ji-Hyun,Lee, Sang-Gil,Chang, Ki Soo,Yi, Gi-Ra,Lee, Gaehang American Scientific Publishers 2014 Journal of nanoscience and nanotechnology Vol.14 No.12

<P>Electro-sterically stabilized gold suspensions were employed in a colorimetric system for the detection of strong acid in water. Using oleyamine and oleic acid as steric stabilizer in 1,2-dichlorobenzene, hydrophobic gold nanoparticles were first synthesized by a reduction reaction of gold salts and were then transferred into water with a cationic surfactant. When the hydrochlo- ric acid solution higher than critical concentration was injected, particles were quickly aggregated and precipitated, creating a clear solution from the colored suspension. The particles were stable against chemical etching by corrosive ion such as chloride. Critical concentration was dependent of the size and concentration of the particles. The minimum concentration of dramatic color change was at 5 ppm level of hydrochloric acid, in which the largest colloidal gold nanoparticles (54 nm) were used. Furthermore, because of their steric repulsive soft layer on particles, particles could be reused for further detection experiments after regeneration by the simple pH-neutralization and washing process.</P>

Terpyridine-based organic-inorganic hybrid nanofiber and its application as an adsorbent for protein.

Bae, Doo Ri,Cho, Eun Jin,Kang, Jeong Ku,Jung, Jong Hwa American Scientific Publishers 2010 Journal of nanoscience and nanotechnology Vol.10 No.8

<P>A terpyridine-based organic-inorganic hybrid amphiphile possessing a triethoxylsilane moiety was synthesized and following its self-assembly in the presence of Cu2+ ion, its binding ability of histidine and His-tag proteins was observed by UV-VIS spectroscopy, Circular dichroism spectroscopy and confocal fluorescence microscopy. The self-assembled 1 in the absence and the presence Cu2+ ion revealed a helical fiber structure, and the self-assembled 1 formed a 1:1 complex with Cu2+ ion. Histidine bound to the self-assembled 1-Cu2+ ion, but more interestingly, His-tag protein was selectively bound to the surface of the self-assembled 1-Cu2+ nanofiber. The finding indicates that the self-assembled 1-Cu2+ nanofiber selectively recognized the His-tag protein.</P>