• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.2
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• pp.199-205
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• 2000

Autoimmune enteropathy is a rare chronic diarrheal disease of infancy. Clinicopathologically, this entity is characterized by chronic secretory diarrhea, villous atrophy with crypt hypoplasia of a small intestine and/or associated autoimmune disorders, and absence of severe immunodeficiency. For the confirmation of diagnosis, antienterocyte autoantibody should be delineated. The treatment of choice of this disorder is immunosuppression. We has been experienced a case of autoimmune enteropathy without autoimmune disorders in a 10-month-old male infant. He developed protracted diarrhea from 5 months of his age and has been appeared to be failure to thrive. Antienterocyte autoantibody was demonstrated by immunohistochemistry and western blotting. He was successfully treated with corticosteroid and FK506. This is the first case report of autoimmune enteropathy without autoimmune disorders in Korea.

• 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.

• Journal of Veterinary Science
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• v.21 no.4
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• pp.47.1-47.12
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• 2020

Background: Homocysteine (HCY) was evaluated in healthy and chronic enteropathic dogs, however no studies on dogs with immunosuppressant-responsive enteropathy are available. Objectives: The aim was to evaluate serum HCY concentrations and its prognostic role in dogs with immunosuppressant-responsive enteropathy compared to healthy dogs. Methods: Serum HCY concentration was statistically compared between 24 healthy dogs and 29 dogs with immunosuppressant-responsive enteropathy. Correlation analyses between serum total protein, albumin (ALB), C-reactive protein (CRP), folate and cobalamin, and serum HCY concentration were performed in immunosuppressant-responsive enteropathic dogs. Results: The associations between serum HCY concentration and clinical, histological, endoscopic scores and follow-up were evaluated. Mean serum HCY concentration was higher in immunosuppressant-responsive enteropathic dogs compared to control dogs (30.22 ± 8.67 µmol/L vs. 5.26 ± 2.78 µmol/L; p < 0.0001). No association between serum HCY concentration and total protein, ALB, CRP, folate concentration as well as, clinical score, histological and endoscopic scores was found. A negative correlation between serum HCY concentration and cobalamin was noted (p = 0.0025, r = -0.54). No significant difference in HCY was found between responsive and non-responsive dogs or between survivors and non-survivors. Conclusions: Although, serum HCY concentration was higher in immunosuppressant-responsive enteropathy, its prognostic value remains unclear. However, further prospective, large-scale studies are warranted to better investigate the possible prognostic role of HCY in immunosuppressant-responsive enteropathic dogs.

• Korean Journal of Radiology
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• v.24 no.4
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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.

• 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.

• 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.239-242
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• 2004

Eosinophilic gastroenteritis is a rare condition of unknown etiology characterized by peripheral eosinophilia, eosinophilic infiltration of the gastrointestinal tract, and gastrointestinal symptoms. Eosinophilic gastroenteritis is generally classified according to the Klain classification: predominant mucosal, muscular, and subserosal disease. Mucosal involvement may result in abdominal pain, nausea, vomiting, diarrhea, weight loss, anemia, protein-losing enteropathy, and intestinal perforation. Patients with muscular layer disease generally have obstructive symptoms. Subserosal eosinophilic infiltration may result in development of eosinophilic ascites. Most commonly, the stomach, duodenum, and small bowel are involved. A 13-year-old girl came to our hospital presenting with chronic, intermittent abdominal pain. She showed peripheral eosinophilia and biopsy specimen of the duodenum revealed eosinophilic infiltration of the mucosal layer. We here report a case of eosinophilic gastroenteritis.

• Journal of Veterinary Clinics
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• v.21 no.2
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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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• v.4 no.2
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• pp.238-242
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• 2001

Food allergy is a disease caused by an abnormal immunological reaction to specific food proteins. Whole milk and soy beans are the most frequent causes of food allergy, some studies show that 2.2~2.8% of children aged between 1 and 2 year are allergic to milk. It can be classified to acute (urticaria, asthma, anaphylaxis) or chronic (diarrhea, atopic dermatitis) allergy according to clinical symptoms, or to IgE related or non IgE related allergy by an immunological aspect. Generally, allergies invading only the GI tract are mostly due to a non IgE related reaction. These hypersensitive, immunologic reactions of the GI tract, not related to specific IgE for food, present themselves in many ways such as food protein-induced enteropathy, food protein-induced enterocolitis syndrome (FPIES), celiac disease, food induced protocolitis, or allergic eosinophillic gastroenteritis. FPIES is one kind of non IgE related allergic reaction and is manifested as severe vomiting and diarrhea in infants between 1 week and 3 months. We report a case of FPIES in a 40-day old male infant presenting with 3 times of repeated events of watery diarrhea after cow's milk feeding.

• Journal of Chest Surgery
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• v.8 no.2
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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.