• Clinical Endoscopy
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• 제51권6호
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• pp.505-507
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• 2018

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제22권6호
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• pp.594-600
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• 2019

With improving survival of children with complex congenital heart disease (CCHD), postoperative complications, like protein-losing enteropathy (PLE) are increasingly encountered. A 3-year-old girl with surgically corrected CCHD (ventricular inversion/L-transposition of the great arteries, ventricular septal defect, pulmonary atresia, postdouble switch procedure [Rastelli and Glenn]) developed chylothoraces. She was treated with pleurodesis, thoracic duct ligation and subsequently developed chylous ascites and PLE (serum albumin ${\leq}0.9g/dL$) and was malnourished, despite nutritional rehabilitation. Lymphangioscintigraphy/single-photon emission computed tomography showed lymphatic obstruction at the cisterna chyli level. A segmental chyle leak and chylous lymphangiectasia were confirmed by gastrointestinal endoscopy, magnetic resonance (MR) enterography, and MR lymphangiography. Selective glue embolization of leaking intestinal lymphatic trunks led to prompt reversal of PLE. Serum albumin level and weight gain markedly improved and have been maintained for over 3 years. Selective interventional embolization reversed this devastating lymphatic complication of surgically corrected CCHD.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권1호
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• pp.47-53
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• 2001

목 적: 우유 과민성 장병증(cow's milk-sensitive enteropathy, CMSE)은 글루텐 과민성 장병증과는 달리 일시적인 질환으로 알려져 있다. CMSE의 장기 추적관찰을 통하여 우유 및 타 음식에 대한 내성 시기, 발현 증상, 알레르기 질환 동반 유무, 가족력 등을 파악하여 환자에 대한 예후설명, 수유방법의 변화시기와 이유식의 이용 등 향후 식이 조절에 대한 지침을 마련하고자 본 연구를 시행하였다. 대상 및 방법: 1992년 3월부터 1997년 3월까지 대구가톨릭대학병원 소아과에서 성장장애와 설사를 주소로 내원하여, 우유 유발 및 제거시험, 소장 생검조직의 형태학적 계측을 통하여 CMSE로 진단받은 후 추적 관찰과 전화 설문이 가능하였던 21명을 대상으로 하였다. 입원당시 연령은 $30.7{\pm}8.8$ (18~47)일, 설문당시는 $43.4{\pm}23.7$ (16~84)개월이였다. 생후 6개월까지는 절대적으로 단백가수 분해물을 수유하게 하였으며, 추후 2개월 간격으로 유발시험을 시행하는 치료 계획서를 작성 이용하였다. 결 과: 1) 우유를 먹여도 내성을 보였던 최종 연령은 $12.6{\pm}3.7$ (6~24)개월이었다. 2) 나이에 적합한 식이 가능한 모든 식품에 내성을 보였던 연령은 $15.5{\pm}3.7$ (10~24)개월이었다. 3) 우유 유발시험에 따라 관찰된 유해반응 19회 중 구토(31%), 설사(31%), 보챔 혹은 기면(21%), 발진(10%), 복부팽만(5%)의 증세를 보였다. 4) 입원당시 혈중 IgE와 우유 RAST에 양성을 보인 5명과 음성을 보였던 16명을 비교하여 우유 및 타 식품에 대한 내성 시기(p>0.05), 타 알레르기 질환의 발현 유무, 알레르기 가족력에서 의미 있는 차이가 관찰되지 않았다. 결 론: CMSE는 2세 이내에 임상적으로 완전한 회복을 보인다. 우유에 대하여 61%에서 1세까지, 전례에서 2세 전까지 내성을 보여 영유아기의 일시적 질환으로 판단된다. 타 식품에 대한 알레르기 발현은 특정 음식보다는 개인에 따른 다양한 음식물에서 발생하며, 전례에서 2세까지 내성을 보인다. 진단 당시 우유 RAST, 혈중 IgE의 상승과 우유 및 타 식품에 대한 내성의 발현 시기와는 무관하다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제1권1호
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• pp.45-55
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• 1998

목 적: 우유 과민성 장병증(cow's milk-sensitive enteropathy, CMSE)은 우유를 식이 하였을 때 유해 반응과 함께 발생하는 상부 소장 점막의 손상이 병리적 특징으로 관찰되며, 이러한 점막의 손상은 우유를 제거하면 회복될 수 있고, 다시 유발시험을 하면 재발될 수 있다. CMSE 환자에서 소장 점막 손상의 정도를 형태학적 계측을 이용하여 정량적으로 분석하여 보고, 이를 이용한 CMSE의 진단적 가치 및 병리적 특성에 관하여 알아보고자 본 연구를 시행하였다. 대상 및 방법: 1994년 3월부터 1996년 6월까지 만성 설사와 성장 장애를 주소로 대구효성가톨릭 대학병원 소아과에 입원한 환자에서 우유에 대한 유발, 제거시험에 따른 임상적 반응 및 상부위장 관내시경을 시술하여 얻게 된 십이지장부의 생검조직 소견을 통하여 CMSE로 진단된 생후 $30.4{\pm}11.7$(14~56)일된 13명의 환아를 대상으로 하였다. 소장 생검조직의 형태학적 계측을 이용하여 융모 높이(villous height, VH), 음와 영역 깊이(crypt zone depth, CZD), 점막전체 두께(total mucosal thickness, TMT), 음와 영역에 대한 융모의 상대적 높이(VH/CD), 융모 1 mm당 상피세포간 림프구의 수(interepithelial lymphocytes, IEL), 0.5 mm의 점막근육판(muscularis mucosa)에 해당하는 상피 표면의 길이(length of surface epithelium, LSE), 상피층의 호산구 침윤(EOS-EP), 고유층(lamina propria)의 호산구 침윤(EOS-LP)을 측정하고, 대조군 및 회복기 생검 조직과 비교하였다. 결 과: 1) VH는 CMSE 환자군에서 $135{\pm}59\;{\mu}m$으로 대조군 $408{\pm}48\;{\mu}m$에 비하여 유의하게 낮았으며(p<0.001), 전례에서 융모 위축의 소견이 관찰되었다. 2) CZD는 CMSE 환자군에서 $311{\pm}65\;{\mu}m$으로 대조군 $188{\pm}24\;{\mu}m$에 비하여 유의하게 깊었다(p<0.05). 3) TMT는 CMSE 환자군에서 $449{\pm}56\;{\mu}m$으로 대조군 $596{\pm}71\;{\mu}m$에 비하여 유의하게 낮았다(p<0.001). 4) VH/CD는 CMSE 환자군에서 $0.46{\pm}0.28$로 대조군 $2.17{\pm}0.12$에 비하여 유의하게 낮았다(p<0.001). 5) LSE는 CMSE 환자군에서 $889{\pm}231\;{\mu}m$으로 대조군 $2547{\pm}378\;{\mu}m$에 비하여 유의하게 낮았다(p<0.001). 6) IEL은 CMSE 환자군에서 $34.1{\pm}10.5$개로 대조군 $13.6{\pm}3.6$개에 비하여 유의하게 높았다(p=0.001). 7) EOS-EP는 CMSE 환자군에서 $0.47{\pm}0.83$개로 의미 있는 침윤은 관찰되지 않았으며, 대조군 $0.80{\pm}0.84$개와 유의한 차이는 보이지 않았다. EOS-LP는 환자군에서 $2.67{\pm}3.72$개로 의미 있는 침윤은 관찰되지 않았으며, 대조군 $4.0{\pm}2.5$개와 유의한 차이를 보이지 않았다. 8) 회복기의 형태학적 변화는 VH $320{\pm}34\;{\mu}m$, CZD는 $194{\pm}19\;{\mu}m$, TMT $514{\pm}21\;{\mu}m$, VH/CD $1.41{\pm}0.25$, LSE $1840{\pm}210\;{\mu}m$, IEL $10.8{\pm}2.3$개로 관찰되었다. 결 론: 소장 조직의 생검은 상부위장관내시경을 이용하여도 그 임상적 유용성이 높으며, CMSE 환자에서 소장 점막의 손상은 형태학적 계측을 통하여 정량적으로 평가할 수 있으며 진단적 민감도가 높다. CMSE에서 점막의 손상은 빠른 융모 상피의 손상에 대한 음와 세포의 충분한 보상의 실패로 사료되며, 단백질 가수분해물의 수유후 빠른 회복이 관찰되었다.

• Journal of Biosystems Engineering
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• 제37권6호
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• pp.420-428
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• 2012

Purpose: Porcine proliferative enteropathy(PPE), caused by the obligate intracellular bacterium Lawsonia intracellularis, is a widely distributed disease throughout the world causing substantial economic loss. The bacterial pathogen invades the intestinal epithelial cells which causes hyperplasia of the infected cells and leads to the process of disease pathogenesis. For diagnosing PPE in a pig farm in earlier stage, a rapid diagnosing test equipment is needed for farmers. To test the equipment appropriately, we prepare the samples and antibodies for rapid detecting the Lawsonia intracellularis in pig feces. Methods : To prepare the PPE infected samples, we sampled PPE suspected pig feces in a pig farm. To manufacture a anti-Lawsonia intracellularis antibody for capturing the Lawsonia intracellularis, the rabbit-anti LsaA synthetic peptide polyclonal antibody was inoculated to rabbits. To select the couple of antibodies which is most well sandwiched with the bacteria, ELISA test was done with PPE infected ileum samples. Finally, to verify the PPE infected feces which would be used to test the rapid kit, PCR test was done on the sampled PPE suspected feces Results : The rabbit-anti LsaA synthetic peptide polyclonal antibody is developed, and is verified to capture the bacterial well through the fluorescence antibody test. Also, we found that the monoclonal antibody and the polyclonal antibody could be used as couples for sandwiching the bacteria. Finally, through the PCR test for samples of pig feces, we could prepare the 150 PPE positive samples and 50 PPE negative samples. Conclusions : The manufactured polyclonal antibody and the imported monoclonal antibody could be used to capture the bacteria using the sandwich techniques. Also, the prepared PPE infected negative and positive samples could be used to test the performance of the rapid kit to capture the bacterium Lawsonia intracellularis.

• Korean Journal of Radiology
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• 제24권4호
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• pp.305-312
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• 2023

Objective: Chronic enteropathy associated with SLCO2A1 gene (CEAS) is a recently recognized disease. We aimed to evaluate the enterographic findings of CEAS. Materials and Methods: Altogether, 14 patients with CEAS were confirmed based on known SLCO2A1 mutations. They were registered in a multicenter Korean registry between July 2018 and July 2021. Nine of the patients (37.2 ± 13 years; all female) who underwent surgery-naïve-state computed tomography enterography (CTE) or magnetic resonance enterography (MRE) were identified. Two experienced radiologists reviewed 25 and 2 sets of CTE and MRE examinations, respectively, regarding the small bowel findings. Results: In initial evaluation, eight patients showed a total of 37 areas with mural abnormalities in the ileum on CTE, including 1-4 segments in six and > 10 segments in two patients. One patient showed unremarkable CTE. The involved segments were 10-85 mm (median, 20 mm) in length, 3-14 mm (median, 7 mm) in mural thickness, circumferential in 86.5% (32/37), and showed stratified enhancement in the enteric and portal phases in 91.9% (34/37) and 81.8% (9/11), respectively. Perienteric infiltration and prominent vasa recta were noted in 2.7% (1/37) and 13.5% (5/37), respectively. Bowel strictures were identified in six patients (66.7%), with a maximum upstream diameter of 31-48 mm. Two patients underwent surgery for strictures immediately after the initial enterography. Follow-up CTE and MRE in the remaining patients showed minimal-to-mild changes in the extent and thickness of the mural involvement for 17-138 months (median, 47.5 months) after initial enterography. Two patients required surgery for bowel stricture at 19 and 38 months of follow-up, respectively. Conclusion: CEAS of the small bowel typically manifested on enterography in varying numbers and lengths of abnormal ileal segments that showed circumferential mural thickening with layered enhancement without perienteric abnormalities. The lesions caused bowel strictures that required surgery in some patients.

• Journal of Biosystems Engineering
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• 제38권2호
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• pp.121-128
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• 2013

Purpose: Porcine proliferative enteropathy (PPE), caused by the obligate intracellular bacterium Lawsonia intracellularis, is a widely distributed disease throughout the world causing substantial economic loss. In order to diagnose PPE rapidly, the rapid kit was developed and tested. Methods: In this study, a rapid kit was developed to screen the PPE rapidly at the pig farm. Also, occult blood test with fecal occult blood (FOB) kit was done for detecting the blood in pig feces which might be the evident of hemorrhagic PPE. For developing the kit, we tested fecal samples of PPE infected pigs diagnosed by polymerase chain reaction (PCR) method. Results: With the developed rapid kit, Lawsonia intracellularis was detected in high density emulsion of ileum. On the other hand, the test result of detecting Lawsonia in feces showed too high non-specific response. In addition, nevertheless the FOB test result showed that blood evident could be founded in pig feces, the diagnosing result was not fit to PCR test result, which shows blood in pig feces could be from not only hemorrhagic PPE but also many reasons. Conclusions: To deal with the PPE effectively, it will be better for farmers to screen the PPE in earlier stage with easy and rapid diagnosing tool on farm. This study found out that the rapid kit could detect the Lawsonia intracellularis and hemoglobin in pig feces. However, the non-specific response to negative samples of PPE was too high to use at a pig farm. Further research is needed for lowering the non-specific response with the rapid kit.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권2호
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• pp.239-242
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• 2004

저자들은 만성적이고 반복적인 복통을 호소하는 13세 여아에서 호산구성 위장관염 1례를 경험하여 보고하는 바이다.

• 한국임상수의학회지
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• 제21권2호
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• pp.194-196
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• 2004

A 12-year old neutered male Yorkshire terrier dog was presented to the Veterinary Medical Teaching Hospital of Seoul National University with a history of chronic intermittent diarrhea, vomiting, anorexia and weight loss of 2-months duration. On presentation, he was very cachexic and had ascites. Abnormal findings on a complete blood count and chemistry profile included mild anemia, leukocytosis, panhypoproteinemia, hypocholesterolemia, decreased blood urea nitrogen (BUN) and increased serum bile acids. Radiographic findings indicated microhepatica. Peritoneal fluid analysis was consistent with transudates (total protein < 2.5 g/dl, total nucleated cell count = 2,200/ul) and cytologic examination of the fluid revealed neoplastic lymphoblasts. From these findings hepatic dysfunction and protein-losing enteropathy were attributable to abdominal lymphoma. This case suggests that cytologic examination is important in diagnosing underlying diseases of ascites, even if it is transudative effusion.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권5호
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• pp.413-422
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• 2021

Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy. Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia. Determining the extent of the lesion provides direction for treatment options. Focal short-segment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.