• 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.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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.5
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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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• v.24 no.2
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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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• v.45 no.2
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• pp.199-207
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• 2002

Purpose : Since the successful application of total atrio-pulmonary connection(TAPC) to patients with various types of physiologic single ventricles in 1971, post-operative survival rates have reached more than 90%. However some patients have been shown to present with late complications such as right atrial thrombosis, atrial fibrillation and protein losing enteropathy eventually leading to re-operation to control the long-term complications. The aim of this study is to review the results of total cavo-pulmonary connection(TCPC) in cases with late complications after TAPC. Methods : Between Jan. 1995 and Dec. 2000, 6 patients(5 males and 1 female) underwent cardiac catheterization $11{\pm}3$ months after conversion of previous TAPC to TCPC. We compared the hemodynamic and morphologic parameters before and after TCPC and also assessed the clinical outcomes. The indications for TAPC were tricuspid atresia in 4 cases and complex double-outlet right ventricle with single ventricle physiology in 2 cases. Results : There was no peri-operative mortality and all patients were clinically and hemodynamically improved at a mean follow-up of 11 months(range : 4 to 13). However, protein losing enteropathy recurred in 2 patients; this was were successfully treated with subcutaneous administration of heparin. Right atrial pressure before TCPC was $18.0{\pm}3.6mmHg$, but baffle pressure, corresponding to right atrial pressure decreased to $14.8{\pm}3.6mmHg$ after TCPC. The size of the pulmonary arteries did not regress after TCPC. Conclusion : The conversion of TAPC to TCPC improves clinical and hemodynamic status by decreasing the right atrial pressure and by providing a laminar cavo-pulmonary flow which enhances the effective pulmonary circulation in the so-called Fontan circulation.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.253-259
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• 2004

Primary intestinal lymphangiectasia is a congenital lymphatic disorder in which intestinal lymphatic channels are dilated and ruptured resulting in loss of protein, lipid, and lymphocyte into the intestine or peritoneum. As a result, hypoalbuminemia, generalized edema, diarrhea are clinically manifested. We report a case of primary intestinal lymphangiectasia with generalized edema which occurred in a 7-year old boy who was treated with lipid restriction diet with medium chain triglyceride oil supplement.

• 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.

• Clinical and Experimental Pediatrics
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• v.55 no.2
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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.

• Journal of Chest Surgery
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• v.36 no.2
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• pp.73-78
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• 2003

The Fontan operation has undergone a number of major modifications and clinical results have been improving over time. Nevertheless, during the follow-up period, life-threatening complications develop and affect the long-term outcomes. Surgical interventions for these complications are needed and are increasing. Material and Method: From April 1988 to January 2000, 16 patients underwent reoperations for complications after Fontan operation. The mean age at reoperation was 8.8 :-5.5 years. Initial Fontan operations were atriopulmonary connections in 8 and total cavopulmonary connections in 8. Total cavopulmonary connections were accomplished with intracardiac lateral tunnel in 5 and extracardiac epicardial lateral tunnel in 3. Five patients had variable sized fenestrations. The reasons for reoperations included residual shunt in 6, pulmonary venous obstruction in 3, atrial flutter in 3, atrioventricular valve regurgitation in 2, Fontan pathway stenosis in 1, and protein-losing enteropathy in 1 Result: There were 3 early and late deaths respectively Patients who had residual shunts underwent primary closure of shunt site (n=2), atrial reseptation for separation between systemic and pulmonary vein (n=2), conversion to lateral tunnel (n=1), and conversion to one and a half ventricular repair (n=1). Four patients who had stenotic lesion of pulmonary vein or Fontan pathway underwent widening of the lesion (n=3) and left pneumonectomy (n=1) In cases of atrial flutter, conversion to lateral tunnel after revision of atriopulmonary connections was performed (n=3). For the atrioventricular valve regurgitation (n=2), we performed a replacement with mechanical valve. In one patient who had developed protein-losing enteropathy, aorto-pulmonary collateral arteries were obliterated via thoracotomy. Cryoablation was performed concomitantly in 4 patients as an additional treatment modality of atrial arrhythmia. Conclusion: Complications after Fontan operation are difficult to manage and have a considerable morbidity and mortality. However, more accurate understanding of Fontan physiology and technical advancement increased the possibility of treatment for such complications as well as Fontan operation itself. Appropriate surgical treatment for these patients relieved the symptoms and improved the functional class, Although the results were not satisfactory enough in all patients.

• Clinical and Experimental Pediatrics
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• v.46 no.7
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• pp.661-667
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• 2003

Purpose : This study was performed to find the chief clinical problems associated with the ages of adult patients of tetralogy of Fallot(TOF) who had undergone total correction. Methods : Of the 30 patients who were registered at the Grown-Up Congenital Heart Disease (GUCH) Clinic of Samsung Medical Center for TOF, a retrospective investigation was carried out on 28 patients who underwent total correction. Results : Mean age at retrospective study was 30.8(range : 16-53) years old. Age at total correction was 15.8(range : 2-49) years old. Problems after corrective surgery were assessed. They were arrhythmia, pulmonary valve regurgitation, left pulmonary artery stenosis, residual ventricular septal defect, mitral valve regurgitation, tricuspid valve regurgitation, right ventricle outflow tract obstruction, aortic valve regurgitation, infective endocarditis and protein losing enteropathy. After repair of TOF, such arrhythmias as atrial arrhythmia and AV conduction disturbances were observed in some patients. Cardiomegaly was found significantly in the subjects with arrhythmia(P<0.05), and arrhythmia was less observed in patients who underwent surgery at a young age. Eight patients required a reoperation; the main indications were residual ventricular septal defect, right ventricle outflow tract obstruction and peripheral pulmonary artery stenosis. Conclusion : The majority of the patients seemed to live normal lives after Tetralogy of Fallot repair. However, as residual anatomic and functional abnormalities exist postoperatively, continued careful follow-up is needed to detect and correct structural and functional abnormalities.