• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.2
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• pp.192-198
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• 2002

Alagille syndrome is characterized by paucity of interlobular bile ducts, chronic cholestasis, characteristic facial abnormalities, cardiovascular abnormalities, posterior embryotoxon, vertebral arch defects, skeletal abnormalities, and glomerular renal involvement. We experienced a case of Alagille syndrome in a 10 month-old male presenting with jaundice. He had chronic cholestasis, characteristic face, cardiovascular abnormalities (aortic stenosis, dextrocardia, double chamber of left ventricle), and situs inversus. Histological examination of liver biopsy specimen revealed paucity of interlobular bile ducts with septal fibrosis, cirrhotic transformation and severe cholestasis. He underwent liver transplantation, but died of cardiopulmonary arrest associated with cardiac anomaly.

• Tuberculosis and Respiratory Diseases
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• v.50 no.5
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• pp.607-614
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• 2001

Background : Residual pleural thickening is frequently seen following treatment for tuberculous pleurisy, and pleural decortication is performed occasionally in patients with severe residual pleural thickening. However, predictive factors for the development of residual pleural thickening are uncertain at the initial diagnosis of the tuberculous pleurisy. Therefore, the purpose of this study was to identify the associated factors for residual pleural thickening at initial diagnosis. Methods : We separated 63 patients diagnosed as tuberculous pleurisy into two groups; group 1 consisted of patients without residual pleural thickening and group 2 comprised patients with residual pleural thickening at the end of tuberculous pleurisy treatment. We analyzed the clinical characteristics, radiological findings, pleural biopsy and characteristics of pleural fluid between group 1 and group 2. Results : The study population and clinical symptoms of the two groups were not significantly different and the duration of symptoms before treatment and the peripheral WBC were similar between the two groups. The presence of pulmonary tuberculosis, pleural fluid loculation or the amount of pleural effusion sid not differ significantly between the two groups. The incidence of positive AFB staining(group 1 : 8%, group 2 : 38%) and granuloma(group 1 : 30%, group 2: 62%)on pleural biopsy specimens was significantly higher in group 2 than in group 1. Pleural fluid WBC and differential count, adenosine deaminase level, pH, protein level or glucose level did not differ between the two groups. However, group 2 had higher LDH levels ($1370{\pm}208mg/dL$) than group 1 ($860{\pm}71mg/dL$, p<0.05). Conclusion : In tuberculous pleurisy, patients with residual pleural thickening following treatment demonstrated a higher incidence of positive AFB staining and granuloma on the pleural biopsy specimens or higher LDH level in the pleural fluid than patients without residual pleural thickening From these results, we speculate that the amount of tuberculous bacilli and granuloma are probably correlated with residual pleural thickening in the tuberculous pleurisy.

• Journal of Veterinary Clinics
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• v.27 no.4
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• pp.445-449
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• 2010

A dog with a history of long-term phenobarbital administration presented to Seoul National University Hospital for Animals with clinical signs of footpad ulceration and facial ulcerative dermatitis. Skin lesions (facial/footpad dermatitis), histopathologic signs (parakeratotic hyperkeratosis, edema and hyperplasia of the epidermis), and liver involvement (increased plasma liver enzymes and nodular lesions) suggested a diagnosis of superficial necrolytic dermatitis (SND). The patient died suddenly at home of unknown cause after 8 months medical treatment, but intravenous amino acid supplementation and supportive hepatic medication proved as effective for the skin conditions.

• Tuberculosis and Respiratory Diseases
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• v.59 no.1
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• pp.77-85
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• 2005

Background : According to the 2002 consensus report of the American Thoracic Society/European Respiratory Society (ATS/ERS), idiopathic pulmonary fibrosis (IPF) was classified as biopsy proven or probable IPF. Probable IPF is defined as those with distinctive features that allow for a confident diagnosis of IPF/usual interstitial pneumonia (UIP) within an appropriate clinical setting. The determination of the clinical course of probable IPF, as diagnosed by the ATS/ERS criteria, was studied. Methods : Between March 1995 and August 2002, 36 patients with probable IPF, from two tertiary referral hospitals, were enrolled in this study. The clinical characteristics, prognostic factors and treatment efficacy of these patients were retrospectively evaluated. Results : The mean age of the subjects was $65{\pm}6$ years. The one and 3 year survival rates were 82.4 and 50.3%, respectively, and a median survival period of 42.0 months. The total cell count of bronchoalveolar lavage was higher in the death than the survival group (p<0.05). No survival benefits were found in the cytoxan and steroid treatment groups compared with other treatment groups. Conclusion : These results suggest that the clinical course of probable IPF may be similar to that of biopsy-proven UIP. However, atypical patients must undergo an open lung biopsy for confirmation of the diagnosis.

• Journal of Chest Surgery
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• v.29 no.5
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• pp.542-547
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• 1996

All patients who underwent video-assisted thoracic surgery (VATS) for diagnostic purposes from Jan. 1992 to Aug. 1995 were reviewed. The total number of patients were 111 with 57 male and 54 female, and the mean age was 49 years (range 1 to 74). Multiple biopsies from more than one location were performed in 17 patients , pleural biopsies were performed In 49 patients, lung biopsies in 43 patients, mediastinal mass or Iymph node biopsies in 33 patients, and two pericardium biopsies and one dia- phragm biopsy, for a total of 128 biopsies. Seventeen pleural biopsy cases and one lung biopsy case underwent operation under local anesthesia , the rest were performed under general anesthesia. In patients who underwent lung biopsy, the mean age was 49.1 ye rs (range 22~ 73). The operating time was 40 to 170 minutes (mean 97), intravenous or intramuscular injection for pain control was required 0 to 22 times(mean 4.7), and chest tube was inserted from 1 to 26 days(mean 7). In all patients except two, a diagnosis was obtained from the biopsy and complication was encountered in one patient in whom intraoperative paroxysmal atrial tachycardia was detected. In 7 patients, a thorn- cotomy had to be done due to pleural adhesion or intraoperative bleeding, and 7 patients had postoperative complications associated with the chest tube. In the pleural biopsy group, the mean age was 49 years (range 17~ 74). The operating time was 25 to 80 minutes (mean 49), intravenous or intramuscular injection for pain control was needed 0 to 20 times (mean 3.6), and the chest tube was i.nserted for 0 to 67 days(mean 9.8). In all the patients, a diagnosis was possible. The chest tube was inserted for longer than 7 days in 11 patients. In the Iymph node biopsy roup, the mean age was 44.2 years (range 1 ~ 68). The operating time was )0 to 3)5 minutes(mean 105), pain control was required 0 to 15 times(mean 3.2), and a chest tube was kept in place for 1 to 36 days(mean 6.1). In one patient, a diagnosis was not possible and a chest tube was kept in place for longer than 7 days in 7 patients. In the multiple biopsy group, the mean age was 53.1 years(range 20~ 71). The operating time was 15 to 165 minutes(mean 85), and pain control was done from 0 to 17 times(mean 3.1). The chest tube was kept in place for 1 to 16 days (mean 7.9).

• Clinical and Experimental Pediatrics
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• v.51 no.6
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• pp.650-654
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• 2008

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.2
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• pp.208-214
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• 2004

Purpose: The prognosis of neonates with cholestasis is not clear. Some factors, such as high peak bilirubin levels and liver histologic findings have been claimed to affect the prognosis adversely. Our study aims to define which factors influence the prognosis of neonatal intrahepatic cholestasis. Methods: Retrospective reviews of the medical records were performed in 32 cases with neonatal intrahepatic cholestasis, who were admitted to Department of Pediatrics, Pusan National University Hospital from July 1995 to July 2002. Neonates were classified into 2 groups according to the duration of elevated serum alanine aminotransferase (ALT) levels more or less than 6 months. The data, such as biochemical, histopathologic and radiologic findings, were compared in both groups. Biochemical data included mean peak level of serum ALT, total bilirubin, direct bilirubin, and alkaline phosphatase. Histologic parameters related to lobular architecture, fibrosis, inflammatory infiltration and degenerative features of hepatocytes were arbitrary estimated on a scale of 1 to 3. Results: There were 19 males and 13 females, whose mean age was 48 days (14~77 days). The peak serum ALT levels were higher in the poor outcome group. Ductular proliferation and portoportal bridging were more severe in the poor outcome group. But the degree of multinucleated hepatocytes, hepatocellular swelling and canalicular plug did not appear to be significantly related to the long-term outcome. The DISIDA scintigraphy by visualization time of gall bladder and intestine was not useful in predicting outcome of neonatal intrahepatic cholestasis. Conclusion: Neonates who have intrahepatic cholestasis with high serum ALT levels, severe ductular proliferation and portoportal bridging in the liver biopsy specimen should be carefully followed up because they may have a poor prognosis.

• Journal of Veterinary Clinics
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• v.23 no.1
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• pp.72-76
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• 2006

A 1-year-old, female English cocker spaniel (ECS) dog was presented with 3-month history of vomiting and retaking of the vomitus, and chronic weight loss. The client had noticed mild abdominal distension 10 days before. The dog was diagnosed as chronic hepatitis with hepatic cirrhosis based on complete blood count (CBC), serum chemistry profiles, radiography, ascites assessment, bile acid evaluation, and liver biopsy through exploratory laparotomy and necropsy. CBC and serum chemistry profiles revealed mild anemia, slightly elevated hepatic enzymes (ALT and AST), increased creatinine kinase (CK), hyperammonemia, and hypoproteinemia with hypoalbuminemia. Ascites was transudate according to analysis of components. Bile acid assessment (fasting; $174.4{\mu}mol/L$ and postprandial; $198.4{\mu}mol/L$) showed strongly suspected hepatic insufficiency. On radiological findings, ascites was evident. Atrophied liver (especially left side lobes) and distended mesenteric vasculatures were observed by exploratory laparotomy. Histopathological examination of marginal lesion of left lateral lobe of liver by biopsy revealed the necrosis of hepatic cells, dilation of sinusoids, infiltration of neutrophils in sinusoids, and vacuolation of hepatic cytoplasm. The patient had been managed with careful low protein diet and specific supportive therapy (ursodeoxycholic acid, prednisolone, vitamine E, and interferon). Vomiting and ascites disappeared with medical management. The dog was monitored periodically by CBC, serum chemistry and radiographic examination. The dog survived more 18 months with medical therapy. After spontaneous death, necropsy and histopathologic examination were performed.

• Korean Journal of Adult Nursing
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• v.27 no.2
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• pp.233-241
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• 2015

Purpose: This study examined the effects of e position change upon reported discomfort and bleeding complications during bed rest following a liver biopsy. Methods: The research design for this study was a non-equivalent control group quasi-experimental design. Twenty-nine participants were assigned to the treatment group and twenty seven participants were in the comparison group. Following the biopsy, the treatment group participants had a position change from the supine without compression for two hours followed by compressive right lateral position for two hours. The comparison group maintained continuously the compressive right lateral position with sandbag for four hours. Results: There were statistically significant differences in reported discomfort between the treatment and comparison groups following the intervention. No significant differences were found in bleeding complications between the two groups. Conclusion: The results of the study suggest that the positional change is an effective nursing intervention in reducing discomfort without risk of bleeding following a liver biopsy.

• The Journal of Korean Orthopaedic Ultrasound Society
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• v.6 no.1
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• pp.38-44
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• 2013

The ultrasound can be used primarily to guide exact needle placement for aspirations of fluids, injections of steroid, and biopsies in musculoskeletal field. Recently, ultrasound-guided intervention is widely used because of several advantages such as real-time performance, relatively inexpensiveness, and getting multiple images without additional radiations. However, the modality is operator dependent and requires detailed knowledge of the relevant anatomy and there have been also reported serious complications related to the procedure. So, authors will discuss about the basic techniques, principles and cautions for the use of ultrasound-guided intervention in musculoskeletal field.