• 대한핵의학회지
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• 제27권1호
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• pp.88-97
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• 1993

Anterior abdominal scintigraphy after intravenous injection of $^{99m}Tc$-human serum albumin ($^{99m}Tc$-HSA 20 mCi) was done in 16 patients with connective tissue diseases and 15 healthy control patients. Patients with proteinuria or hepatopathy were excluded. 1) 7 (44%) patients among 16 connective tissue disease patients without the apparent evidence of external protein loss showed abnormal intestinal accumulation of albumin. 6 patients with positive albumin scintigraphy showed bypoalbuminemia. 2) There was no false positive scintigraphic finding in control group. 3) The serum albumin level in connective tissue disease patients ($3.1{\pm}0.6 g/dl$, n=16) was lower than control patients ($3.9{\pm}0.3 g/dl$, n=15) (p<0.0001). 4) The serum albumin level was lower in connective tissue disease patients with positive $^{99m}Tc$-HSA scan ($2.8{\pm}0.6 g/dl$, n=7) than the connective tissue disease patients with negative scan ($3.3{\pm}0.3 g/dl$, n=9) (p<0.05). 5) The hemoglobin level in connective tissue disease patients with positive nan ($10.6{\pm}2.91 g/dl$) was lower than that of the control group ($13.6{\pm}1.5 g/dl$) (p<0.05). Mypoalbuminemia is frequently involved in chronic connective tissue diseases. Protein losing enteropathy (PLE) is also responsible for the majority of the bypoalbuminemia in these patients. But it has been ignored because the conventional method for the diagnosis of PLE was difficult to perform. $^{99m}Tc$-HSA scan also must be validated by more extended study and comparison with the quantitative study such as stool ${\alpha}-1$ antitrypsin measurement. There must be a reevaluation of PLE in various diseases especially in connective tissue diseases with easy, fast, economical, and non-invasive method.

• Korean Circulation Journal
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• 제52권8호
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• pp.606-620
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• 2022

Background and Objectives: Protein-losing enteropathy (PLE) is a devastating complication after the Fontan operation. This study aimed to investigate the clinical characteristics, treatment response, and outcomes of Fontan-associated PLE. Methods: We reviewed the medical records of 38 patients with Fontan-associated PLE from 1992 to 2018 in 2 institutions in Korea. Results: PLE occurred in 4.6% of the total 832 patients after the Fontan operation. After a mean period of 7.7 years after Fontan operation, PLE was diagnosed at a mean age of 11.6 years. The mean follow-up period was 8.9 years. The survival rates were 81.6% at 5 years and 76.5% at 10 years. In the multivariate analysis, New York Heart Association Functional classification III or IV (p=0.002), low aortic oxygen saturation (<90%) (p=0.003), and ventricular dysfunction (p=0.032) at the time of PLE diagnosis were found as predictors of mortality. PLE was resolved in 10 of the 38 patients after treatment. Among medical managements, an initial heparin response was associated with survival (p=0.043). Heparin treatment resulted in resolution in 4 patients. We found no evidence on pulmonary vasodilator therapy alone. PLE was also resolved after surgical Fontan fenestration (2/6), aortopulmonary collateral ligation (1/1), and transplantation (1/1). Conclusions: The survival rate of patients with Fontan-associated PLE has improved with the advancement of conservative care. Although there is no definitive method, some treatments led to the resolution of PLE in one-fourth of the patients. Further investigations are needed to develop the best prevention and therapeutic strategies for PLE.

• 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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• 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.

• 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.

• Journal of Veterinary Science
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• 제24권2호
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• pp.25.1-25.6
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• 2023

An eight-year-old Maltese dog presented with diarrhea and anorexia. Ultrasonography revealed marked focal wall thickening with loss of layering in the distal ileum. Contrast-enhanced computed tomography (CT) revealed a preserved wall layer with hypoattenuating middle wall thickening. In some segments of the lesion, small nodules protruding toward the mesentery from the outer layer were observed. Histopathology revealed focal lipogranulomatous lymphangitis (FLL) with lymphangiectasia. This is the first report to describe the CT features of FLL in a dog. CT features of preserved wall layers with hypoattenuating middle wall thickening and small nodules can assist in diagnosing FLL in dogs.

• Clinical and Experimental Pediatrics
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• 제45권2호
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• pp.199-207
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• 2002

목 적 : 본 연구에서는 기능적 단심실의 치료로 심방-폐동맥 문합술 시행 후 나타난 합병증을 해결하기 위해 총 공정맥-폐동맥 문합술을 시행한 증례를 검토하여 그 필요성과 유용성에 대해 조사하였다. 방 법: 심방-폐동맥 문합술 후 생긴 합병증의 치료를 위해 총 공정맥-폐동맥 문합술로 전환한 환아 6명(남 : 녀=5 : 1)을 대상으로 수술 전, 후 임상 양상 및 혈역학적 검사 소견을 후향적으로 분석하였다. 기능적 단심실 환자에서 변형 폰탄 수술 후 총 공정맥-폐동맥 문합술로 전환까지의 시간은 112개월이었다. 결 과 : 심방-폐동맥 문합술 후 재수술의 적응증이 된 후기 합병증은 단백 소실성 장병증(3례), 폰탄순환 부전(2례) 및 우심방 세동(1례)이었으며, 심방 혈전증과 우심방 확장으로 인한 우폐정맥 수축이 각각 1례에서 병발하였다. 전례에서 임상 증상 및 혈역학적 소견이 호전되었지만 2례에서 단백소실성 장병증이 재발하여 heparin 투여 후 치료되었다. 총 공정맥-폐동맥 문합술전 우심방의 압력은 $18.0{\pm}3.6mmHg$이었으나, 수술 후 $14.4{\pm}3.7mmHg$로 감소하였으며, 폐동맥 형태의 퇴행은 관찰되지 않았다. 총 공정맥-폐동맥 문합술 전환 후 수술 사망률 및 중대한 합병증은 없었다. 결 론: 심방-폐동맥 문합술 후 후기 합병증의 완화를 위하여 총 공정맥-폐동맥 문합술로 전환한 증례들에서 혈역학적인 개선과 함께 예후가 호전됨을 관찰하였으며, 따라서 심방-폐동맥 문합술 후 후기 합병증이 유발된 경우 총 공정맥-폐동맥 문합술로의 전환을 적극적으로 검토할 필요가 있을 것으로 사료된다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제7권2호
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• pp.253-259
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• 2004

저자들은 전신 부종을 주소로 내원한 6세 소아에서 장 조직 검사로 장 단백 소실을 유발하는 원발성 장 림프관 확장증을 확인하였고, 특별한 약물 치료 없이 medium chain triglyceride를 지방 대체 식이로 이용한 저지방, 고단백의 식이요법만으로 장 단백 소실의 호전을 보였고 증상 소실과 지속적인 성장 발달을 유지하였던 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제8권2호
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• pp.101-108
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• 1975

Since 1959 the authors experienced 43 cases of chronic constrictive pericarditis treated surgically at the Department of Thoracic & Cardiovascular Surgery, the National Medical Center in Seonl. Of 43 cases, detailed patients' records could be obtainable in 36 cases, and most of our studies. were made on the basis of these 36 available cases. About 84 per cent of the cases were male with several pediatric cases, and duration of symptoms ranged between 2 months and 10 years. The diagnosis of this condition is not difficult, however, about half of our cases were previously treated under the impression rf various other conditions such as liver cirrhosis or nephrotic syndrome at other hospitals and clinics. Many of our cases showed hepatic functional disturbances and about 89 per cent of the cases showed reversed A/G ratio, and we are sure that some of them had so-called protein losing enteropathy. Three of 36 cases showed normal electrocardiogram, and most peculiar electrocardiographic findings were ST or T changes and low amplitude of QRS complexes. Seven cases showed auricular fibrillation and five had first degree A-V block. Mean preoperative peripheral venous pressure at the antecubital fossa and arm-to-tongue circulation time were 273 mm $H_2O$and 20.2 seconds, respectively, and they were markedly reduced postoperatively to 152 mm $H_2O$ and 13 seconds, respectively. Several different approaches were made with various extents of pericardial decortication according to patients' condition and probably surgeon's preference. In 12 cases we met cardiovascular injuries during decortication and one of them died of massive bleeding through the torn right atrium, and we experienced excellent postoperative result in a grave case operated on just a small pericardial window. Eleven of 35 cases were tuberculous pericarditis and others were non-specific pericarditis histopathologically, and 6 of total 43 cases operated on passed away by various ways with the mortality rate of 13.9 per cent.

• Clinical and Experimental Pediatrics
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• 제55권2호
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• pp.48-53
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• 2012

Purpose: Gaucher disease is caused by a ${\beta}$-glucocerebrosidase (GBA) deficiency. The aim of this study is to investigate the clinical and genetic characteristics according to subtypes of Gaucher disease in the Korean population. Methods: Clinical findings at diagnosis, $GBA$ mutations, and clinical courses were reviewed in 20 patients diagnosed with Gaucher disease. Results: Eleven patients were diagnosed with non-neuronopathic type, 2 with acute neuronopathic type, and 7 with chronic neuronopathic type. Most patients presented with hepatosplenomegaly, thrombocytopenia, and short stature. In the neuronopathic group, variable neurological features, such as seizure, tremor, gaze palsy, and hypotonia, were noted at age $8.7{\pm}4.3$ years. B cell lymphoma, protein-losing enteropathy, and hydrops fetalis were the atypical manifestations. Biomarkers, including chitotriosidase, acid phosphatase, and angiotensin-converting enzyme, increased at the initial evaluation and subsequently decreased with enzyme replacement treatment (ERT). The clinical findings, including hepatosplenomegaly, thrombocytopenia, and skeletal findings, improved following ERT, except for the neurological manifestations. L444P was the most common mutation in our cohort. One novel mutation, R277C, was found. Conclusion: Although the clinical outcome for Gaucher disease improved remarkably following ERT, the outcome differed according to subtype. Considering the high proportion of the neuronopathic form in the Korean population, new therapeutic strategies targeting the central nervous system are needed, with the development of a new scoring system and biomarkers representing clinical courses in a more comprehensive manner.