• Title/Summary/Keyword: hepatic encephalopathy

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A Case of Congenital Extra Hepatic Portocaval Shunt (Abernethy Malformation Type 2) with a very Large Liver Mass and an Atrial Septal Defect (거대 간 종괴와 심방 중격 결손을 동반한 Abernethy 기형 2형 1예)

  • Lee, Hae-Jeong;Lee, Jee-Hyun;Huh, June;Kang, I-Seok;Lee, Heung-Jae;Suh, Yeon-Lim;Yoo, So-Young;Choe, Yon-Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.1
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    • pp.56-59
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    • 2008
  • Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.

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An operation for the correction of Membranous obstruction of Inferior Vena Cava using Total Circulatory Arrest (전혈류 정지술을 이용한 하공정맥 폐색증의 교정수술)

  • Kim, Jhin-gook;Na, Myung-Hoon;An, Hyuk
    • Journal of Chest Surgery
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    • v.20 no.4
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    • pp.773-779
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    • 1987
  • MOVC is an uncommon disease which can be corrected by surgical method if early detected. A case of a 34-year-old male with MOVC is reported. Operation was done on cardiopulmonary bypass with circulatory arrest under moderate hypothermia. The incision was done both on the RA extending to level of suprahepatic IVC and on the IVC just proximal to the right renal vein. And then, thrombectomy and membranectomy under the direct visualization was done. Total circulatory arrest was used intermittently in order to get good visual field and for preventing blood loss. The Postop. course was good except one episode of hepatic encephalopathy which was persisted for 12 hours and then controlled by conservative measures.

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A Case of Hyperacute Liver Failure from Mushroom Intoxication in a Child Treated with Penicillin (페니실린으로 완치한 버섯 중독에 의한 소아 급성 간부전 1예)

  • Kwak, Byung-Ok;Bae, Sun-Hwan
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.11 no.2
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    • pp.210-213
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    • 2008
  • Hyperacute liver failure from mushroom intoxication in children is rare and has a low survival rate. We report a case of hyperacute liver failure from mushroom intoxication in a 29-month-old boy. The patient ingested a mushroom about three days prior to presentation. He was admitted to the hospital with vomiting, abdominal pain, seizures, and hematemesis. During the hospitalization the patient developed hepatic encephalopathy (stage IV-a), and a coagulopathy. He recovered fully with specific medication, Penicillin GK and N-acetylcysteine.

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Delayed Continuous Venovenous Hemodiafiltration in Chronic Lithium Intoxication (만성 리튬 중독환자의 지연성 정정맥 혈액 투석여과 사례)

  • Kim, Tae Su;Cha, Yong Sung;Kim, Hyun;Kim, Oh Hyun;Cha, Kyoung Chul;Lee, Kang Hyun;Hwang, Sung Oh
    • Journal of The Korean Society of Clinical Toxicology
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    • v.11 no.1
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    • pp.28-30
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    • 2013
  • A 66-year-old male with chronic alcoholism presented with tremor, gait disturbance, memory impairment, insomnia, decreased appetite, and confusion. The patient had been taking lithium daily for treatment of bipolar disorder. Brain CT showed no specific abnormality, and serum lithium and ammonia levels were 3.63 mEq/L (therapeutic range, 0.6~1.2 mEq/L) and $85{\mu}g/dL$ (reference range: $19{\sim}54{\mu}g/dL$), respectively. Therefore, the initial differential diagnosis included chronic lithium intoxication, hepatic encephalopathy, Wernicke encephalopathy, or alcohol withdrawal syndrome. Even with the provision of adequate hydration, the patient's neurologic status did not show improvement, so that lactulose enema, thiamine replacement, and continuous venovenous hemodiafiltration (CVVHDF) were started on the third admission day. By the fifth admission day he had made a rapid neurologic recovery, and was discharged on the 20th admission day. Therefore, CVVHDF might be a treatment for patients with chronic lithium intoxication, because, even if serum lithium concentration is normal, lithium concentration in the brain may be different from that of the serum.

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Serum Liver Enzyme Pattern in Birth Asphyxia Associated Liver Injury

  • Chhavi, Nanda;Zutshi, Kiran;Singh, Niranjan Kumar;Awasthi, Ashish;Goel, Amit
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.17 no.3
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    • pp.162-169
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    • 2014
  • Purpose: To study temporal pattern of serum liver enzymes levels in newborns with hepatic injury associated with birth asphyxia (BA). Methods: Singleton term newborns with BA and ${\leq}72$ hours of age admitted to neonatal intensive care unit were prospectively enrolled. Term newborns with physiological jaundice and without BA were studied as controls. Serum liver enzymes were measured at <24 hours, 24-72 hours, and at 6-12 days of age for cases and at 1-6 days of age for controls. BA was defined by 1 minute Apgar score <7 or delayed or absent cry with hypoxic ischemic encephalopathy. BA-associated liver injury was defined as serum alanine aminotransferase (ALT) elevation beyond +2 standard deviation (ALT > +2 SD) above the mean of control subjects at any of the three time points. Results: Sixty controls and 62 cases were enrolled. Thirty-five cases (56%) developed BA-associated liver injury (ALT>81 IU/L). They had higher serum levels of ALT, aspartate aminotransferase, lactate dehydrogenase than the control infants, with peak at 24-72 hours. In controls, serum liver enzyme levels were significantly higher in appropriate-for-date (AFD) babies than small-for-date (SFD) babies. Serum enzyme pattern and extent of elevation were comparable between SFD and AFD babies. Degree of serum liver enzyme elevation had no relationship with severity of hypoxic encephalopathy. Conclusion: Serum liver enzyme elevation is common in BA; it peaks at 24-72 hours followed by a sharp decline by 6-12 days of age. Pattern and extent of enzyme elevation are comparable between SFD and AFD babies.

Liver transplantation in a child with acute liver failure resulting from drug rash with eosinophilia and systemic symptoms syndrome

  • Song, Seung Min;Cho, Min Sung;Oh, Seak Hee;Kim, Kyung Mo;Park, Young Seo;Kim, Dae Yeon;Lee, Sung Gyu
    • Clinical and Experimental Pediatrics
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    • v.56 no.5
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    • pp.224-226
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    • 2013
  • Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by a severe idiosyncratic reaction including rash and fever, often with associated hepatitis, arthralgias, lymph node enlargement, or hematologic abnormalities. The mortality rate is approximately 10%, primarily owing to liver failure with massive or multiple disseminated focal necrosis. Here, we report a case of a 14-year-old girl treated with vancomycin because of a wound infection by methicillin-resistant Staphylococcus aureus, who presented with non-specific symptoms, which progressed to acute liver failure, displaying the hallmarks of DRESS syndrome. With the presence of aggravated hepatic encephalopathy and azotemia, the patient was refractory to medical treatments, she received a living-donor liver transplantation, and a cure was achieved without any sign of recurrence. Vancomycin can be a cause of DRESS syndrome. A high index of suspicion and rapid diagnosis are necessary not to miss this potentially lethal disease.

A Case of Reye Syndrome Following Treatment of Kawasaki Disease with Aspirin (가와사키병 치료를 위한 아스피린 사용 후 발생한 라이 증후군 1예)

  • Lee, Joon Kee;Kang, Ji Eun;Choi, Eun Hwa;Choi, Jung Yun
    • Pediatric Infection and Vaccine
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    • v.19 no.2
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    • pp.79-83
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    • 2012
  • Reye syndrome is a rapidly progressive encephalopathy with hepatic dysfunction, which often begins several days after apparent recovery from a viral illness, especially varicella or influenza A or B. Salicylate use was identified as a major precipitating factor for the development of Reye syndrome. With the recommendation to avoid use of salicylates in children, Reye syndrome has virtually disappeared in recent years. We report a case of Reye syndrome in a 5-month-old infant who had been treated with intravenous immunoglobulin and aspirin under the diagnosis of Kawasaki disease, and showed symptoms of sudden onset of irritability, rigidity, decreased activity, vomiting, poor appetite, lethargy, liver dysfunction without jaundice, coagulopathy, and hyperammonemia.

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Chelation of Thallium (III) in Rats Using Combined Deferasirox and Deferiprone Therapy

  • Salehi, Samie;Saljooghi, Amir Sh.;Badiee, Somayeh;Moqadam, Mojtaba Mashmool
    • Toxicological Research
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    • v.33 no.4
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    • pp.299-304
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    • 2017
  • Thallium and its compounds are a class of highly toxic chemicals that cause wide-ranging symptoms such as gastrointestinal disturbances; polyneuritis; encephalopathy; tachycardia; skin eruptions; hepatic, renal, cardiac, and neurological toxicities; and have mutagenic and genotoxic effects. The present research aimed to evaluate the efficacy of the chelating agents deferasirox (DFX) and deferiprone (L1) in reducing serum and tissue thallium levels after the administration of thallium (III), according to two different dosing regimens, to several groups of Wistar rats for 60 days. It was hypothesized that the two chelators might be more efficient as a combined therapy than as monotherapies in removing thallium (III) from the rats' organs. The chelators were administered orally as either single or combined therapies for a period of 14 days. Serum and tissue thallium (III) and iron concentrations were determined by flame atomic absorption spectroscopy. Serum and tissue thallium (III) levels were significantly reduced by combined therapy with DFX and L1. Additionally, iron concentrations returned to normal levels and symptoms of toxicity decreased.

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.

The Characteristics and Outcomes of Abernethy Syndrome in Korean Children: A Single Center Study

  • Kim, Eun Sil;Lee, Ki Wuk;Choe, Yon Ho
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.22 no.1
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    • pp.80-85
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    • 2019
  • Purpose: Abernethy malformation is a rare condition, which was first described in 1793 as a congenital extrahepatic porto-systemic shunt (CEPS) directing splanchnic blood flow into the inferior vena cava. Eighty cases have been published so far that reported CEPS, while in Korea, very few cases have been reported. Through this study, we present 6 cases of patients diagnosed with CEPS at Samsung Medical Center and compare these with other such cases published in France and China. Methods: We reviewed clinical, laboratory, and imaging data of 6 children with CEPS in our pediatric clinic between 2004 and 2017. Results: A total of 6 children with CEPS was included in this study, namely, one with type 1a, two with type 1b, and three with type 2 CEPS. The most common presenting symptom was gastrointestinal bleeding (50.0%). Therapeutic interventions included shunting vessel ligation (16.7%) in type 2 CEPS and liver transplantation (16.7%) in type 2 CEPS patient with suddenly developed hepatic encephalopathy. Conclusion: There is no consensus guideline for the optimal management of patients with CEPS. Large-sample studies regarding CEPS are needed to evaluate the characteristics of patients with CEPS and determine the treatment guideline for CEPS.