• Journal of Chest Surgery
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• v.45 no.5
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• pp.348-350
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• 2012

We herein report a very rare case of adult right-sided Bochdalek hernia accompanied with hepatic hypoplasia and inguinal hernia. A 29-year-old man was admitted with right-sided pneumothorax. A computed tomography was performed and revealed large right sided Bochdalek hernia with hepatic hypoplasia. Under thoracolaparotomy, the defect was closed with Gore-Tex soft tissue patch. After the operation, left-sided inguinal hernia was found. However, it turned out that it had been present during infancy and spontaneously resolved during adolescence. This is the first report of right-sided Bochdalek hernia with hepatic hypoplasia and inguinal hernia in an adult.

• Journal of Veterinary Clinics
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• v.37 no.2
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• pp.88-90
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• 2020

A one year old spayed female Bichon Frise dog presented with gait abnormalities and seizure. Serum biochemical results showed elevated levels of alkaline phosphatase, alanine aminotransferase, and ammonia. Serum bile acid level was also increased to be over 30 μmol/L on preprandial. Urinalysis identified the presence of ammonium urate crystal. Abdominal ultrasonography and CT revealed aberrant, tortuous, and multiple small vessels connected to the caudal vena cava between left kidney and caudal vena cava. Macroscopic specific findings associated with extrahepatic congenital portosystemic shunts (PSS) or other liver diseases were not identified. Liver biopsy was performed. Histopathologic evaluation revealed hepatic lobular hypoplasia with portal arterial duplication and vascular shunts. Based on these finding, this case was diagnosed as multiple acquired PSS secondary to hepatic microvascular dysplasia (HMD) and hepatic encephalopathy. A liver biopsy is recommended to differentiate HMD from other liver diseases and to confirm HMD when a young dog has multiple acquired PSS.

• Neonatal Medicine
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• v.17 no.1
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• pp.136-140
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• 2010

Galactosemia is a group of inherited enzyme deficiencies characterized by increase in the blood galactose levels. This condition may be associated with deficiencies of galactose-1-phosphate uridyl transferase, galactokinase, or uridine diphosphate galactose-4-epimerase. However, the elevated galactose identified by neonatal screening tests has several other possible etiologies, including hepatic hemangioendothelioma, hepatic hemangioma, and patent ductus venosus with hypoplasia of the portal vein. We report a 13-day-old Korean male with hepatic hemangioendothelioma, which was incidentally detected during the evaluation for suspected galactosemia. Laboratory studies revealed that mildly elevated levels of galactose, galactose-1-phosphate and alpha- fetoprotein, at the time of admission, were graduallydecreased to the normal range over the 6 months of observation. Ultrasonography showed a well-defined heterogeneous hypoechoic mass in the liver, and magnetic resonance imaging study showed multiple enhanced mass lesions, which was compatible with the diagnosis of a hepatic hemangioendothelioma. Thus, hepatic imaging, especially ultrasonography, should be performed if neonatal screening suggests galactosemia.

• Journal of the korean academy of Pediatric Dentistry
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• v.27 no.1
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• pp.70-76
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• 2000

Congenital biliary atresia with progressive sclerosis of the intra- and extra-hepatic duct system occurs in 1 : 10,000 live births, and has a poor prognosis with an expected survival of less than 5 years. Etiology of biliary atresia is unclear, however, it is believed a genetic or developmental cause. The clinical characteristics include pronounced jaundice, hepatosplenomegaly, pruritus, steatorrhea, xanthomas, growth retardation, portal hypertension, bleedings, ascites and respiratory infections. Oral manifestations have seldom been reported in patients with biliary atresia, but there may be enamel hypoplasia, delayed tooth eruption, and green teeth. Early diagnosis and surgical intervention have decreased morbidity. returned growth and development to normal and improved the prognosis for survival. Authors report the clinical and radiologic characteristics, proper managements about two cases with biliary atresia.

• Journal of the korean academy of Pediatric Dentistry
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• v.31 no.3
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• pp.400-405
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• 2004

Biliary atresia is defined as a complete obstruction of bile flow owing to destruction or absence of all or part of the extrahepatic bile ducts. This disease is occurring in approximately 1:10,000 live births and moderate predominance of female is noted. The etiology of biliary atresia remained unsolved. The signs and symptoms are hyperbilirubinemla, jaundice, clay-colored stools, steatorrhea, dark yellow urine and hepatomegaly. Currently biliary atresia is best managed by hepatic portoenterostomy with or without liver transplantation. Biliary atresia patients with these cases showed staining of the teeth. The stains ranged in color from yellowish-brown to deep green. Enamel hypoplasia was all erupted teeth present. Patients had poor oral hygiene and rampant caries.