CAQ Corner

Pan, Jason J.; Fontana, Robert J.

doi:10.1002/lt.26503

Cited by 9 publications

(6 citation statements)

References 52 publications

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“…The advances in neuromonitoring/protection, hemostasis, and hemodialysis/plasma exchange that favorably impact adult ALF outcomes 37 need further study in pediatric populations. In addition, more accurate prognostic models are needed as transplant decisions remain complex in PALF.…”

Section: Background/epidemiology Of Alfmentioning

confidence: 99%

Future directions in acute liver failure

Stravitz¹,

Fontana²,

Karvellas³

et al. 2023

Hepatology

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Acute liver failure (ALF) describes a clinical syndrome of rapid hepatocyte injury leading to liver failure manifested by coagulopathy and encephalopathy in the absence of pre-existing cirrhosis. The hallmark diagnostic features are a prolonged prothrombin time (ie, an international normalized ratio of prothrombin time of ≥1.5) and any degree of mental status alteration (HE). As a rare, orphan disease, it seemed an obvious target for a multicenter network. The Acute Liver Failure Study Group (ALFSG) began in 1997 to more thoroughly study and understand the causes, natural history, and management of ALF. Over the course of 22 years, 3364 adult patients were enrolled in the study registry (2614 ALF and 857 acute liver injury—international normalized ratio 2.0 but no encephalopathy—ALI) and >150,000 biosamples collected, including serum, plasma, urine, DNA, and liver tissue. Within the Registry study sites, 4 prospective substudies were conducted and published, 2 interventional (N-acetylcysteine and ornithine phenylacetate), 1 prognostic [13C-methacetin breath test (MBT)], and 1 mechanistic (rotational thromboelastometry). To review ALFSG’s accomplishments and consider next steps, a 2-day in-person conference was held at UT Southwestern Medical Center, Dallas, TX, entitled “Acute Liver Failure: Science and Practice,” in May 2022. To summarize the important findings in the field, this review highlights the current state of understanding of ALF and, more importantly, asks what further studies are needed to improve our understanding of the pathogenesis, natural history, and management of this unique and dramatic condition.

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Section: Background/epidemiology Of Alfmentioning

confidence: 99%

Future directions in acute liver failure

Stravitz¹,

Fontana²,

Karvellas³

et al. 2023

Hepatology

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“…Abnormal coagulation tests such as prolonged INR and prothrombin time do not necessarily mean an increased risk of bleeding in patients with ALF, and reports suggest that despite laboratory findings of coagulation abnormalities, most patients have a normal coagulation state [27,28]. Clinically significant bleeding is uncommon in ALF and is seen in approximately 5-10% of patients [29,30]. Prophylactic correction of platelet levels is not necessary, and routine use of fresh frozen plasma (FFP) and other coagulation factors are not supported by evidence, and in fact may lead to increased death or LT due to increased transfusion reactions, thrombosis, and transfusion-related acute lung injury [2,29,31,32].…”

Section: Management Of Acute Liver Failure and The Role Of Prognostic...mentioning

confidence: 99%

“…Living-donor liver transplant (LDLT) is uncommon in the USA and Europe given the availability of deceased donor liver grafts, differing from the majority of countries in Asia where LDLT is more common. In the USA, if the patient is listed, they are at highest priority, status 1A, and LDLT may be considered at centers with experience, and ABO-I (ABO-incompatible) grafts in patients who are rapidly declining [2,30]. LDLT may reduce wait times for ALF and is especially important in regions where the majority of patients undergo LDLT [25,53,54].…”

Section: Role Of Liver Transplantationmentioning

confidence: 99%

A Comprehensive Review of the Diagnosis and Management of Acute Liver Failure

Ozturk,

Herdan,

Saner

et al. 2023

JCM

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Acute liver failure (ALF) is a rare and specific form of severe hepatic dysfunction characterized by coagulopathy and hepatic encephalopathy in a patient with no known liver disease. ALF carries a high morbidity and mortality. Careful attention should be given to hemodynamics and metabolic parameters along with the active surveillance of infections. Timely transfer and supportive management are important in an intensive care unit in a liver transplant center. Identifying patients who will and will not improve with medical management and may need emergent liver transplantation is critical. In this review, we provide a comprehensive update on the etiology, diagnosis, and management of ALF.

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“…4 FWD can quickly progress to hepatic and renal failure and carries 95% mortality if left untreated. 5,6…”

Section: Introductionmentioning

confidence: 99%

“…4 FWD can quickly progress to hepatic and renal failure and carries 95% mortality if left untreated. 5,6 WD should be considered in young patients in ALF or acute-on-chronic liver failure (ACLF). However, ALF/ACLF may cause aberrations in copper metabolism, complicating the diagnosis of FWD.…”

Section: Introductionmentioning

confidence: 99%

Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease

Diamond,

Newman,

Schalet

et al. 2023

ACG Case Rep J

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Wilson disease (WD) is estimated present in 6%–12% of patients younger than 40 years hospitalized with acute liver failure (ALF). Fulminant WD carries a poor prognosis without treatment. A 36-year-old man with HIV, chronic hepatitis B virus, and alcohol use had ceruloplasmin 6.4 mg/dL and 24-hour urine copper 180 μg/L. WD workup was otherwise negative, including ophthalmic examination, hepatic copper quantification, ATP7B sequencing, and brain MRI. ALF commonly features copper dysregulation. Few studies on WD biomarkers have included fulminant WD. Our patient with WD biomarkers and other causes of liver failure highlights the need to study copper dysregulation in ALF.

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CAQ Corner

Cited by 9 publications

References 52 publications

Future directions in acute liver failure

Future directions in acute liver failure

A Comprehensive Review of the Diagnosis and Management of Acute Liver Failure

Dysregulation of Copper Metabolism in a Patient With Acute-on-Chronic Liver Failure Worked up for Fulminant Wilson Disease

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