Hypothermia in Acute Liver Failure

Jalan, Rajiv; Rose, Christopher F.

doi:10.1023/b:mebr.0000043971.72170.a2

Cited by 27 publications

(11 citation statements)

References 37 publications

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“…Neuroprotective measures based on the use of pharmacological agents [241-245] or of hypothermia [246,247] are under investigation to treat hepatic and hyperammonemic encephalopathy. Mild systemic hypothermia (rectal temperature of 34°C for 48 h), together with hemofiltration in a neonatal UCD patient with hyperammonemic coma was reported [248], resulting in a striking fall in plasma ammonia.…”

Section: New Trends and Emerging Therapiesmentioning

confidence: 99%

Suggested guidelines for the diagnosis and management of urea cycle disorders

Häberle

Boddaert

Burlina

et al. 2012

Orphanet J Rare Dis

521

623

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Urea cycle disorders (UCDs) are inborn errors of ammonia detoxification/arginine synthesis due to defects affecting the catalysts of the Krebs-Henseleit cycle (five core enzymes, one activating enzyme and one mitochondrial ornithine/citrulline antiporter) with an estimated incidence of 1:8.000. Patients present with hyperammonemia either shortly after birth (~50%) or, later at any age, leading to death or to severe neurological handicap in many survivors. Despite the existence of effective therapy with alternative pathway therapy and liver transplantation, outcomes remain poor. This may be related to underrecognition and delayed diagnosis due to the nonspecific clinical presentation and insufficient awareness of health care professionals because of disease rarity. These guidelines aim at providing a trans-European consensus to: guide practitioners, set standards of care and help awareness campaigns. To achieve these goals, the guidelines were developed using a Delphi methodology, by having professionals on UCDs across seven European countries to gather all the existing evidence, score it according to the SIGN evidence level system and draw a series of statements supported by an associated level of evidence. The guidelines were revised by external specialist consultants, unrelated authorities in the field of UCDs and practicing pediatricians in training. Although the evidence degree did hardly ever exceed level C (evidence from non-analytical studies like case reports and series), it was sufficient to guide practice on both acute and chronic presentations, address diagnosis, management, monitoring, outcomes, and psychosocial and ethical issues. Also, it identified knowledge voids that must be filled by future research. We believe these guidelines will help to: harmonise practice, set common standards and spread good practices with a positive impact on the outcomes of UCD patients.

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Section: New Trends and Emerging Therapiesmentioning

confidence: 99%

Suggested guidelines for the diagnosis and management of urea cycle disorders

Häberle

Boddaert

Burlina

et al. 2012

Orphanet J Rare Dis

521

623

View full text Add to dashboard Cite

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“…A large body of evidence points to therapeutic hypothermia as a promising therapy for the cerebral complications of ALF [6]. Indeed, hypothermia is unsurpassed by any other interventions reported in experimental ALF in terms of the magnitude of its effects on cerebral and global outcomes as well as of its consistency across independent laboratories, animal models and time (from the 1960s to today) (reviewed in [7,8]). The ability of hypothermia to control ICP and to bridge to liver transplantation (LT), achieving survival rates of up to 70%, has also been reported in patients with ALF presenting refractory intracranial hypertension (a formal contraindication for LT associated with >90% mortality) [9,10].…”

Section: See Article Pages 273-279mentioning

confidence: 99%

Hypothermia in acute liver failure: What got lost in translation?

Porcher

Vaquero

2016

Journal of Hepatology

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“…Hypothermia lowers cerebral metabolic demand and reduces splanchnic ammonia production, thus lowering ICP. Though hypothermia has been shown in multiple studies to reduce ICP in fulminant liver failure patients [12,[45][46][47][48][49][50][51][52][53], it has not been studied in large randomized trials and is associated with serious complications including infections, bleeding diathesis and arrhythmias.…”

Section: Management Of Elevated Intracranial Pressurementioning

confidence: 99%

Neurological Management of Fulminant Hepatic Failure

Frontera

Kalb

2010

Neurocrit Care

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Acute liver failure (ALF) is uncommon in the United States, but presents acutely and catastrophically, often with deadly consequences. Hepatic encephalopathy, cerebral edema, elevated intracranial pressure, and intracranial hemorrhage due to coagulopathy are common occurrences in patients with ALF. Appropriate management of multi-system organ failure and neurological complications are essential in bridging patients to transplant and ensuring satisfactory outcomes.

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Hypothermia in Acute Liver Failure

Cited by 27 publications

References 37 publications

Suggested guidelines for the diagnosis and management of urea cycle disorders

Suggested guidelines for the diagnosis and management of urea cycle disorders

Hypothermia in acute liver failure: What got lost in translation?

Neurological Management of Fulminant Hepatic Failure

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