• 제목/요약/키워드: Biliary

검색결과 586건 처리시간 0.03초

Efficacy of Living Donor Liver Transplantation in Patients with Methylmalonic Acidemia

  • Jang, Jae Guk;Oh, Seak Hee;Kim, Yu Bin;Kim, Seo Hee;Yoo, Han-Wook;Lee, Beom Hee;Namgoong, Jung-Man;Kim, Dae Yeon;Kim, Ki-Hun;Song, Gi-Won;Moon, Deok-Bog;Hwang, Shin;Lee, Sung-Gyu;Kim, Kyung Mo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • 제24권3호
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    • pp.288-294
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    • 2021

  • Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT. Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared. Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed. Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.

  • https://doi.org/10.5223/pghn.2021.24.3.288 인용 PDF KSCI

The Effect of Glycyrrhizae Radix on the Metabolism of Acetaminophen

  • Kim, Seung-Hee;Oh, Jee-Young;Aeree moon, Aeree-Moon;Kim, Hyo-Jung;Lee, Song-Deuck
    • Biomolecules & Therapeutics
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    • 제1권2호
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    • pp.160-165
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    • 1993

  • The effects of Glycyrrhizae Radix (GR) on the metabolism of acetaminophen (AA) were examined in male Sprague-Dawley rats. The methanol extract of GR (500 mg/kg) was administered orally to rats for 6 days. AA and its metabolites excreted in bile, urine and blood within 120 min after dosing of AA (150 mg/kg, i.v.) were assayed by HPLC. Treatment of rats with the methanol extract of GR significantly increased the cumulative biliary excretion of AA-glucuronide (156% of the control) and decreased that of AA-sulfate (63% of the control). The cumulative urinary excretion of AA-glucuronide was also significantly increased to 132% of the control. GR treatment significantly increased total (biliary plus urinary) excretion of AA-glucuronide (172% of the control) without influencing thioether and sulfate conjugates of AA. The results clearly show that GR enhances UDP-glucuronosyl transferase-mediated detoxication of AA, but may not influence sulfotrans-ferase-mediated and cytochrome P-450-mediated metabolites formation.

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소아의 담관낭종 (Choledochal Cyst in Children)

  • 임세웅;이석구;김현학
    • Advances in pediatric surgery
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    • 제5권2호
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    • pp.121-125
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    • 1999

  • To study the role of anomalous pancreatico-biliary ductal union (APBDU) in the development of choledochal cyst, we reviewed 23 cases. APBDU is defined as a long common channel(>0.4 mm). The patients ages ranged from 1 week to 112 months and the mean age was 22.5 months. Right upper quadrant pain was the most prevalent symptom. The diagnosis was made by ultrasonography and operative cholangiography in most patient. The preoperative diagnosis was made in 100 % of the cases. Gallstones were found in 5 cases. Todani type I and type IV were prevalent. A long common channel was found in all cases. The operative treatment consisted of cyst excision and Reux-en-Y hepaticojejunostomy or choledochojejunosotmy. One patient had postoperative pancreatitis. There was no mortality. We conclude that detection of choledochal cyst is occurring at a younger age and APBDU seems to play an important role in the pathogenesis of type I and IV cysts. Cyst excision is the treatment of choice to eliminate repeated cholangitis and malignant transformation.

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