Evolution in the management of acute liver failure-associated aplastic anaemia in children: A single centre experience

Hadžić, Nedim; Height, Sue; Ball, Sarah E.; Rela, Mohamed; Heaton, Nigel; Veys, Paul; Mieli‐Vergani, Giorgina

doi:10.1016/j.jhep.2007.08.017

Cited by 43 publications

(19 citation statements)

References 27 publications

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“…Bone marrow failure, characterized by a spectrum of features ranging from mild pancytopenia to aplastic anemia, occurs in a significant minority of children with ALF. 136 It is identified most commonly in the setting of indeterminate ALF and may not be clinically evident until after emergent liver transplantation. 137 Treatment includes immunomodulatory medications that include steroids, cyclosporine A, antilymphocyte or antithymocyte globulin as well as hematopoietic stem cell transplant.…”

Section: Hematologicalmentioning

confidence: 99%

Acute Liver Failure in Children

2008

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Acute liver failure (ALF) in children differs from that observed in adults in both the etiologic spectrum and the clinical picture. Children, particularly very young ones, do not demonstrate classical features of encephalopathy and the definition of ALF has been revised to include patients with advanced coagulopathy, regardless of mental status. A significant number of these children will go on to require transplant or die. Etiologies vary by age with metabolic and infectious diseases prominent in the first year of life and acetaminophen overdose and Wilson's disease occurring in adolescents. In almost 50% of cases, however, the child has an indeterminate cause for ALF. Management requires a multidisciplinary approach and is directed at establishing the etiology where possible and monitoring, anticipating, and managing the multisystem complications that occur in children with ALF. Overall, short-term outcomes are better in children than adults but are dependent upon the degree of encephalopathy and diagnosis.

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Section: Hematologicalmentioning

confidence: 99%

Acute Liver Failure in Children

2008

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“…Acute liver failure can rapidly evolve into MOF and lead to death through multiple mechanisms . ELT is often a life‐saving option for some patients, but it is a high‐risk procedure, both in the immediate perioperative period and in the longer term, mainly due to complications of immunosuppression . The short‐term outcomes have improved over the years from <20% hospital survival in the pre‐transplantation era to up to more than 70% currently .…”

Section: Discussionmentioning

confidence: 99%

Long‐term outcome in patients with acute liver failure

Putignano

Figorilli

Alabsawy

et al. 2018

Liver International

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“…Aplastic anemia typically follows hepatitis within 1-3 months. Aplastic anemia has also been reported after liver transplantation [1]. Viruses such as hepatitis A, B, C, and E, parvovirus B19, echovirus, HIV and adenovirus have been reported to cause AHAA [1,2].…”

Section: Discussionmentioning

confidence: 99%

“…Aplastic anemia has also been reported after liver transplantation [1]. Viruses such as hepatitis A, B, C, and E, parvovirus B19, echovirus, HIV and adenovirus have been reported to cause AHAA [1,2]. However, idiopathic or non-viral hepatitis remains the most common cause [2][3][4][5].…”

Section: Discussionmentioning

confidence: 99%

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Acute Liver Failure and Aplastic Anemia in an 11-Year-Old Girl

2011

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Case Presentation and EvolutionAn 11-year-old previously healthy female presented with a 1-week history of jaundice and fatigue. Her aspartate aminotransferase (AST) level was 1,420 U/L, alanine aminotransferase (ALT) 1,044 U/L, total bilirubin 16.5 mg/dL and international normalized ratio (INR) for prothrombin time 1.2. Complete blood count showed a white blood count of 7,700/mm 3 , hematocrit 40.6 g/dL, and platelet count 509,000/mm 3 . Ultrasound demonstrated mild hepatomegaly but was otherwise normal. Serology was negative for hepatitis A, B, and C and Epstein-Barr virus (EBV) and cytomegalovirus (CMV). After discharge, she was followed by her outpatient pediatric gastroenterologist, who noted that she continued to have jaundice and elevated aminotransferase levels. A liver biopsy was recommended, but her parents refused, and the patient was lost to followup for several months.Four months after her initial presentation, the patient represented with a 2-week history of sleepiness, headaches, increased jaundice, and anorexia. During the time she was lost to follow-up, her parents noted an initial improvement of her jaundice and energy. However, 2 weeks prior to her readmission, she had increasing fatigue, jaundice, and epistaxis. Complete blood count revealed a WBC of 2.3 g/mm 3 , hematocrit 33.2 g/dL 3 , and platelet count 13,000/mm 3 . Total bilirubin was 21.8 mg/dL, direct bilirubin 14.6 mg/dL, AST 3,158 U/L, ALT 3,239 U/L, INR 2.0, erythrocyte sedimentation rate 16, C-reactive protein 23 mg/dL, and ammonia 38 lm/L. She was admitted for lethargy in the setting of liver failure and pancytopenia. On admission to the hospital, her vital signs were normal and physical examination revealed an alert, interactive female with marked jaundice. Lungs were clear to auscultation, and heart tones were regular with no murmurs. Her abdomen was soft, nontender and not distended; her liver was palpated 2 cm below the right costal margin, and the spleen tip was palpated 3 cm below the left costal margin. Ultrasound of the abdomen showed mild hepatomegaly and thickened gallbladder wall with no sludge or stones. She was listed for liver transplantation, and began transfusion therapy with fresh frozen plasma and platelets as needed for procedures.Further blood tests showed negative human immunodeficiency virus (HIV) antibody, CMV polymerase chain reaction (PCR), and EBV PCR. Her alpha-1-antitrypsin level was normal, anti liver-kidney microsome antibody negative, antismooth muscle antibody negative, antinuclear antibody negative, drug screen for urine and serum negative, varicella immunoglobulin (Ig)G negative with an equivocal IgM. Ophthalmology consultation was negative for ocular abnormalities and Kayser-Fleischer rings, and her serum ceruloplasmin was 29 mg/dL. A 24-h urine collection for copper was 260 mcg/specimen (normal, less than 60), but the reliability of this test was questioned given that the patient had 4 L of urine collected while receiving furosemide.A magnetic resonance cholangiopancreatography (MRCP) showed no b...

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Evolution in the management of acute liver failure-associated aplastic anaemia in children: A single centre experience

Cited by 43 publications

References 27 publications

Acute Liver Failure in Children

Acute Liver Failure in Children

Long‐term outcome in patients with acute liver failure

Acute Liver Failure and Aplastic Anemia in an 11-Year-Old Girl

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