• Title/Summary/Keyword: hepatic encephalopathy

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Intrahepatic Portosystemic Shunt Fixed By Transvenous Coil Embolization in a Samoyed Dog (샤모예드종 개에서 발생한 간내성 문맥-정맥 문합을 경정맥 코일장착으로 치료한 증례)

  • Lee, Moo-Hyun;Lee, Seoung-Jin;Lee, Seung-Gon;Moon, Hyeong-Sun;Lee, Joon-Seok;Choi, Ran;Park, In-Chul;Hyun, Chang-Baig
    • Journal of Veterinary Clinics
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    • v.24 no.3
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    • pp.426-431
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    • 2007
  • A 5-month-old female Samoyed dog was presented with primary complaints including exercise tolerance and neurological sign associated with hepatic encephalopathy. The major findings in clinical examination included an intermittent seizure, anemia, elevated pre- and post-prandial serum bile acid, hypoproteinemia and bilirubinuria. Diagnostic imaging studies revealed an intrahepatic portosystemic shunt (IPSS). The shunted vessel was successfully occluded by transvenous coil embolization. Clinical signs were gradually improved after shunt occlusion. This case is a rare case of IPSS in a large breed dog fixed by transvenous coil embolization.

Novel Mutations in the CPT1A Gene Identified in the Patient Presenting Jaundice as the First Manifestation of Carnitine Palmitoyltransferase 1A Deficiency

  • Choi, Jong Sub;Yoo, Hyeoh Won;Lee, Kyung Jae;Ko, Jung Min;Moon, Jin Soo;Ko, Jae Sung
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.19 no.1
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    • pp.76-81
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    • 2016
  • Carnitine palmitoyltransferase 1A (CPT1A) is an enzyme functioning in mitochondrial fatty acid oxidation (FAO) of the liver. Patients with CPT1A deficiency have impaired mitochondrial FAO and display hypoketotic hypoglycemia and hepatic encephalopathy as typical manifestations. In this report, we present a case of CPT1A deficiency presenting jaundice as the first manifestation. A 1.9 years old boy showed jaundice and elevated levels of free and total carnitine were observed. From direct sequencing analysis of CPT1A, two novel mutations, c.1163+1G>A and c.1393G>A (p.Gly465Arg), were identified. At the age of 2.2 years, hypoglycemia, tachycardia, and altered mental status developed just after cranioplasty for craniosynostosis. High glucose infusion rate was required for recovery of his vital signs and mentality. Diet rich in high carbohydrate, low fat and inclusion of medium chain triglyceride oil resulted in improvement in cholestatic hepatitis and since then the boy has shown normal growth velocity and developmental milestones to date.

Evaluation of the Underlying Etiology and Long-Term Prognostic Factors in Neonatal Cholestasis (신생아 담즙정체증의 원인질환 및 장기추적 예후인자에 관한 고찰)

  • Kim, Kyung-Mo;Seo, Jeong-Kee
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.2 no.1
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    • pp.46-58
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    • 1999
  • Purpose: The aim of the present study was to evaluate the long-term clinical profile including the underlying etioligy and the prognostic factors of the neonatal cholestasis. Method: We studied the 190 infants presented with neonatal cholestasis for the last 12 years (from 1981 to 1992). The underlying causes, clinical findings and long-term outcomes were evaluated. And the prognostic factors were also analyzed. Result: Underlying disease were neonatal hepatitis in 101 (idiopathic in 77 and infectious in 24), intrahepatic bile duct paucity in 5, biliary atresia in 79, choledochal cyst in 5. Metabolic disease was not observed in this study. The important clinical problems during follow-up were persistent high fever, gastrointestinal bleeding, hepatic encephalopathy and ascites. The main causes of the death were hepatic encephalopathy and gastrointestinal bleeding. While three fourth of infants with idiopathic and infectious neonatal hepatitis recovered usually within a year, five-year survival rate for biliary atresia was just 40%, the mortality observed usually within the first year after Kasai operation and prognostic factor was the time of operation. Underlying disease was the most important prognostic factor of neonatal cholestasis. Conclusion: This study showed that most common causes of neonatal cholestasis were biliary atresia and idiopathic neonatal hepatitis, infectious neonatal hepatitis, choledochal cyst and Alagille syndrome, but few neonatal cholestasis of genetic or metabolic liver disease was observed. The most important long-term prognostic factor of neonatal cholestasis was the underlying disease.

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Hospice and Palliative Care in End Stage Liver Disease (말기 간질환 환자에서의 호스피스 완화의료)

  • Kim, Moon Young
    • Journal of Hospice and Palliative Care
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    • v.20 no.3
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    • pp.167-172
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    • 2017
  • End-stage liver disease (ESLD) is a terminal condition of cirrhosis which cannot be treated without liver transplantation. Thus, it is natural for patients to consider hospice/palliative care (HPC). Since the recent legislation of the Act on Decisions on Life-Sustaining Treatment for Patients in Hospice and Palliative Care or at the End of Life (Act No. 14013) in Korea, the practicality of this law has become an issue. The criteria for HPC should be defined with consideration to how the severity of each ESLD complication may vary by individual patients. Generally, patients qualify if they have an intractable condition despite aggressive treatment such as the hepatorenal syndrome, hepatic encephalopathy or variceal hemorrhage. However, the option of liver transplantation should be sufficiently discussed with patients and their families before making a decision on HPC. The evaluation of which ESLD patients should receive HPC should be based on a long-term doctor-patient relationship and sufficient objective data. Therefore, a multidisciplinary approach and mutual consultation among cirrhosis specialists and doctors with other expertise are essential to offer optimal and balanced treatments between liver-specific treatment and HPC. Discussed in this review are adequate criteria for HPC and special considerations for ESLD at the point of HPC.

Percutaneous Catheter Drainage of Lung Abscess (폐농양의 경피적 카테타 배농법)

  • Kim, Chang-Ho;Cha, Seoung-Ick;Han, Chun-Duk;Kim, Yeon-Jae;Lee, Yeung-Suk;Park, Jae-Yong;Jung, Tae-Hoon
    • Tuberculosis and Respiratory Diseases
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    • v.40 no.2
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    • pp.158-164
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    • 1993
  • Background: Recently, lung abscess tends to be increased in patients with underlying disease, most of whom are unsuitable for surgery when medical treatment fails. The patients with giant lung abscesses do not frequently respond to antibiotics and often have life-threatening complications. Therefore, more intensive cares are required in these patients. We studied the results and effects of percutaneous catheter drainage in these patients. Method: We performed fluoroscopy-guided percutaneous pigtail catheter (8.3 F) drainage by Seldinger technique in 9 cases of lung abscess (in 7 cases, intractable to medical treatment for an average of 8.4 days and in 2 cases, catheter drainage immediately performed due to a large cavity that was initially 10 cm in diameter). We compared 10 cases of lung abscess as control group which had receieved conventional medical treatment alone. Results: Seven of the 9 patients in study group of percutaneous drainage and 7 of the 10 patients in control group of medical treatment alone clinically improved in the average of 1.8 and 8.7 days, respectively. The mean duration of drainage was 13.2 days. There were 3 cases of death from massive hemoptysis, asphyxia of pus, and sepsis in control group, as compared with 2 cases of death from hepatic encephalopathy and sepsis in study group. The malfunctions of catheter occurred in these 2 cases, obstruction and dislodgement. But there were no significant pleuropulmonary complications of percutaneous drainage. Conclusion: Percutaneous drainage is effective and relatively safe in the management of lung abscesses refractory to medical therapy or giant lung abscesses.

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