• Title/Summary/Keyword: Enteropathy

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Protein-losing Enteropathy Induced by 4 Different Etiologies (4가지 다른 병인들에 의해 유발된 단백소실성 장병증 증례)

  • Cho, Kyu-Woan;Moon, Jong-Hyun;Lee, Hee-Chun;Kwon, Do-Hyung;Kang, Byeong-Teck;Sur, Jung-Hyang;Jung, Dong-In
    • Journal of Veterinary Clinics
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    • v.29 no.1
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    • pp.93-97
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    • 2012
  • Four dogs (case 1; 2-year-old spayed female Schnauzer, case 2; 3-year-old spayed female Cocker Spaniel, case 3; 9-year-old castrated male Yorkshire Terrier, and case 4; 9-year-old intact female Shih-tzu) were referred to us with gastrointestinal signs such as diarrhea, vomiting, and anorexia. Results of blood analysis revealed hypoproteinemia and hypoalbuminemia in all dogs. Case 4 showed large circular mass which is connected with small intestine on abdominal ultrasonography and other 3 cases showed no remarkable findings on abdominal radiography and ultrasonography. We performed enterectomy in case 4 and gastrointestinal endoscopic examination with biopsy in other 3 patients. Finally, 4 patients diagnosed to protein losing enteropathy with 4 different etiologies.

Treatment of Protein-Iosing Enteropathy After Fontan Procedure by Conversion to the Total Cavopulmonary Connection with Fenestration (완전 대정맥-폐동맥 연결로 전환에 의한 폰탄술식 후 단백소모성 장질환의 치료)

  • 이현성;박영환;홍유선;송석원;이진구
    • Journal of Chest Surgery
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    • v.34 no.1
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    • pp.85-90
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    • 2001
  • 폰탄술식 후 수술사망률이 감소하고 생존자가 증가함에 따라 대정맥-폐동맥 순환의 부자연스러운 생리와 관련된 합병증이 발생하게 되었다. 이러한 혈역학적인 문제로 인하여 발생한 합병증 중의 하나가 단백소모성 장질환이다. 단백소모성 장질환은 폰탄술식 후에 발생하는 드물지만 매우 위험한 합병증이다. 많은 다른 치료경향이 제안되었지만 성공적인 치료보고는 제한되어 있다. 폰탄술식후 단백소모성 장질환이 발생한 3명의 환아에서 완전 대정맥-폐동맥 연결로 전환을 시행하였다. 완전 대정맥 폐동맥 연결로 전환함이 만족할 만한 치료방법임을 보고하는 바이다.

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Congenital Pyloric Atresia with Junctional Epidermolysis Bullosa-a case report (수포성 표피박리증을 동반한 선천성 유문부폐쇄증 1예)

  • Cho, Ma-Hae;Han, Seok-Joo
    • Advances in pediatric surgery
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    • v.3 no.1
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    • pp.77-82
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    • 1997
  • The association of pyloric atresia and epidermolysis bullosa(EB) in newborn is rare and inheritant as an autosomal recessive trait. We report a newborn girl with pyloric atresia and epidermolysis bullosa. Blisters were noted on her skin at birth, especially in pressure-exposed area, and later on the oral mucosa. Junctional epidermolysis bullosa was confirmed by light microscopy and electron microscopy. Radiography revealed pyloric atresia. Segmental resection of 1.5 cm and gastroduodenostomy were carried out at 4 days of age. Protein loosing enteropathy developed after oral feeding. The frequency of episodes of nonscarred blisters and the severity and duration improved significantly with time. The protein loosing enteropathy was persistent, and at 1 year of age, her growth is markedly retarded.

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Food Protein-induced Enterocolitis Syndrome: an Update on Clinical Approaches and Its Pathophysiology (식품 단백질 유발성 장염 증후군: 임상적 접근과 병태생리의 최신 지견)

  • Hwang, Jin-Bok
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.10 no.2
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    • pp.117-128
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    • 2007
  • Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated hypersensitivity disorder, which is associated with mainly gastrointestinal symptoms and has a delayed onset. The vomiting and/or diarrheal symptoms of FPIES typically begin in the first month of life in association with a failure to thrive, metabolic acidosis, and shock. Therefore, the differential diagnosis of FPIES and neonatal or infantile sepsis-like illnesses or gastroenteritis is difficult. The early recognition of indexes of suspicion for FPIES may help in the diagnosis and treatment of this disorder. The diagnosis of FPIES is generally made through clinical practice and food-specific IgE test findings are typically negative in this condition. Therefore, oral cow's milk challenge (OCC) remains the valid diagnostic standard for FPIES. An investigation of positive OCC outcomes helps to find out a diagnostic algorithm of criteria of a positive challenge in FPIES. Moreover, it has not been clearly determined in infantile FPIES when $1^{st}$ follow up-oral food challenge (FU-OFC) should be performed, with what kind of food protein (e.g., cow's milk, soy), and how much protein should be administered. Hence, to prevent the risk of inappropriate FU-OFC or accidental exposure and achieve appropriate dietary management, it is necessary to identify tolerance rates to major foods under the careful follow up of infantile FPIES patients. On the other hand, small intestinal enteropathy with villous atrophy is observed in FPIES and this enteropathy seems to be in part induced by both of epithelial apoptosis and intercellular junctional complex breakdown. The purpose of this report is to introduce an update on diagnostic and therapeutic approaches in FPIES and suggest the possible histopathological evidences in this disorder.

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Establishment of a diagnostic method for porcine proliferative enteropathy using polymerase chain reaction (중합효소연쇄반응을 이용한 돼지 증식성 장염 진단기법 확립)

  • Lym, Suk-kyung;Lee, Hee-soo;Woo, Sung-ryong;Yoon, Soon-seek;Moon, Oun-kyong;Lee, Yoo-young;Koh, Hong-bum
    • Korean Journal of Veterinary Research
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    • v.39 no.1
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    • pp.118-125
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    • 1999
  • Porcine Proliferative Enteropathy(PPE) is an infectious enteric disease and a major cause of economic loss in swine industry due to weight loss, poor growth and sudden death in growing and finishing pigs at 6 to 20 weeks of age. PPE has been diagnosed by clinical signs, syndrom and lesions in the intestine in Korea. However, the diagnostic method had several problems in the detection of infected or carrier pigs. Therefore, in this study, we established the polymerase chain reaction(PCR) which was a fast, specific and sensitive method for identification of Lawsonia intracellularis (L intracellularis). We designed and synthesized primer on the 16S rDNA and p78 gene encoding L intracellularis. Specificity of the method was confirmed by comparison of the PCR results using other enteric bacteria and the study has shown that PCR method was sensitive to detect 1ng of genomic DNA as a template. Identity of the PCR products was confirmed by comparison of pattern of restriction endonuclease analysis with restriction enzyme Hae III and Pst I. Also, the PCR method was applicable to the naturally affected pigs with PPE. Based on the results from this study, the PCR method could be used as a fast and specific diagnostic tool for PPE.

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Homozygous Missense Epithelial Cell Adhesion Molecule Variant in a Patient with Congenital Tufting Enteropathy and Literature Review

  • Guvenoglu, Merve;Simsek-Kiper, Pelin Ozlem;Kosukcu, Can;Taskiran, Ekim Z.;Saltik-Temizel, Inci Nur;Gucer, Safak;Utine, Eda;Boduroglu, Koray
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.25 no.6
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    • pp.441-452
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    • 2022
  • Congenital diarrheal disorders (CDDs) with genetic etiology are uncommon hereditary intestinal diseases characterized by chronic, life-threatening, intractable watery diarrhea that starts in infancy. CDDs can be mechanistically divided into osmotic and secretory diarrhea. Congenital tufting enteropathy (CTE), also known as intestinal epithelial dysplasia, is a type of secretory CDD. CTE is a rare autosomal recessive enteropathy that presents with intractable neonatal-onset diarrhea, intestinal failure, severe malnutrition, and parenteral nutrition dependence. Villous atrophy of the intestinal epithelium, crypt hyperplasia, and irregularity of surface enterocytes are the specific pathological findings of CTE. The small intestine and occasionally the colonic mucosa include focal epithelial tufts. In 2008, Sivagnanam et al. discovered that mutations in the epithelial cell adhesion molecule (EpCAM, MIM# 185535) were the genetic cause of CTE (MIM# 613217). More than a hundred mutations have been reported to date. Furthermore, mutations in the serine peptidase inhibitor Kunitz type 2 (SPINT2, MIM# 605124) have been linked to syndromic CTE. In this study, we report the case of a 17-month-old male infant with congenital diarrhea. Despite extensive etiological workup, no etiology could be established before admission to our center. The patient died 15 hours after being admitted to our center in a metabolically decompensated state, probably due to a delay in admission and diagnosis. Molecular autopsy with exome sequencing revealed a previously reported homozygous missense variant, c.757G>A, in EpCAM, which was confirmed by histopathological examination.

Fecal Microbiota Transplantation via Commercial Oral Capsules for Chronic Enteropathies in Dogs and Cats

  • Min-Ok Ryu;Soh-Yeon Lee;Se-Hoon Kim;Hwa-Young Youn;Kyoung-Won Seo
    • Journal of Veterinary Clinics
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    • v.41 no.3
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    • pp.150-156
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    • 2024
  • This retrospective case series assessed the effectiveness of commercially available oral fecal microbiota transplantation (FMT) for treating chronic enteropathies in eight animals, five dogs, and three cats, between 2020 and 2023 at the Seoul National University Veterinary Medical Teaching Hospital. Chronic enteropathies, often resistant to conventional therapies, present a significant challenge in veterinary medicine. To assess oral capsule FMT's effectiveness (Doggybiome® one capsule daily for dogs and Kittybiome® one capsule daily for cats) as a universal adjunctive therapy for chronic enteropathies across species not responding to traditional treatments. This retrospective case series applied a uniform evaluation of gastrointestinal symptoms and treatment efficacy, utilizing established scoring systems (Canine Inflammatory Bowel Disease Activity Index [CIBDAI] and Canine Chronic Enteropathy Clinical Activity Index [CCECAI] for dogs, Feline Chronic Enteropathy Activity Index [FCEAI] for cats) before and one month after FMT. This approach ensured consistency in hypothesis testing across the study population. Results revealed significant improvements in clinical indices post-FMT, with notable reductions in the CIBDAI, CCECAI, and FCEAI scores (p < 0.05). Additionally, symptoms such as anorexia, lethargy, diarrhea, vomiting, and weight loss showed marked improvement, with normalization of appetite and activity levels observed in most cases. No adverse effects were reported, indicating the safety and tolerability of this treatment. This study highlights the potential of oral capsule FMT as a viable therapeutic option for dogs and cats with chronic enteropathies unresponsive to conventional treatments, providing a new avenue for clinical management. Further research is warranted to expand these findings and explore the microbiome changes associated with FMT in veterinary patients.

Diagnosis of Protein Losing Enteropathy in Connective Tissue Diseases with $^{99m}Tc$-human Serum Albumin(HSA) (교원병에 동반된 단백소실장질환(Protein Losing Enteropathy)에서의 테크네슘표지 인혈청알부민 스캔)

  • Won, Kyoung-Sook;Oh, Yeong-Seok;Bang, Shin-Ho;Park, Won
    • The Korean Journal of Nuclear Medicine
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    • v.27 no.1
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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.

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