Paediatric acute liver failure and transplantation: The University of Essen experience

Nadalin, Silvio; Heuer, Matthias; Wallot, M.; Auth, Marcus; Schaffer, Randolph; Sotiropoulos, Georgios C.; Ballauf, A.; Broek, Maartje A J van der; Damink, Steven W. M. Olde; Hoyer, Peter F.; Broelsch, Christoph E.; Malagó, Massimo

doi:10.1111/j.1432-2277.2007.00474.x

Cited by 37 publications

(45 citation statements)

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“…1,4,5 O TRH é o procedimento cirúrgico que, nas últimas décadas, permitiu uma significativa melhoria do seu prognóstico, aumentando a sobrevida para cerca de 60% -85%. 2,4,5,[10][11][12] Por ser rapidamente progressiva, um importante fator no prognóstico destas crianças é a sua referenciação precoce para um centro de transplantação hepática. 11,13,14 A abordagem multidisciplinar e diferenciada permite uma otimização das medidas de suporte de modo a evitar o TRH ou, no caso de ser inevitável, que a criança seja submetida a esse procedimento em melhores condições.…”

Section: Introductionunclassified

“…2,4,5,[10][11][12] Por ser rapidamente progressiva, um importante fator no prognóstico destas crianças é a sua referenciação precoce para um centro de transplantação hepática. 11,13,14 A abordagem multidisciplinar e diferenciada permite uma otimização das medidas de suporte de modo a evitar o TRH ou, no caso de ser inevitável, que a criança seja submetida a esse procedimento em melhores condições. Possibilita ainda identificar situações em que o TRH está contra indicado.…”

Section: Introductionunclassified

“…Possibilita ainda identificar situações em que o TRH está contra indicado. 5,8,11,13,14 A referenciação para um centro de TRH nos primeiros seis dias de evolução de FHA associa-se a menor grau de encefalopatia e valores mais baixos de bilirrubina total, assim como maior sobrevida em relação a casos com referenciação mais tardia. 13,14 Em janeiro de 2008, realizou-se em Portugal uma reunião nacional multidisciplinar monotemática sobre FHA em Pediatria, que envolveu as Sociedades de Gastrenterologia, Hepatologia e Nutrição e de Cuidados Intensivos Pediátricos da Sociedade Portuguesa de Pediatria, da qual resultou um consenso de atuação reforçando a importância da sua abordagem por equipa diferenciada e a referenciação precoce para um centro com TRH.…”

Section: Introductionunclassified

“…4,21 Neste estudo, a sobrevida pós-TRH (78%) foi sobreponível à de outros centros europeus. 4,5,11 A sua diferença relativamente à sobrevida livre de TRH (30%) vem reforçar que o TRH é de fato a terapêutica mais eficaz e que mais influencia a sobrevida destes doentes.…”

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“…4,12 No entanto, consideram-se fatores de mau prognóstico, com base num estudo realizado num pequeno número de doentes na era pré-transplante no King´s College Hospital, os seguintes: INR ≥ 4,0; bilirrubina total ≥ 235 μmol/L; idade inferior a dois anos e contagem de leucócitos ≥ 9x 10 9 /L. 2,11 Existe também um score que parece promissor (LIU score) e que inclui uma fórmula matemática que utiliza os valores laboratoriais da bilirrubina sérica, tempo de protrombina (ou INR) e amónia. No entanto, não foi ainda validado para poder ser amplamente utilizado.…”

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A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica

et al. 2015

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. Inclusion criteria: age < 18 years old and acute liver failure (INR ≥ 2 without vitamin K response and hepatocellular necrosis). Children with previous liver disease were excluded. Results: Fifty children were included, with median age of 24.5 months. The most common etiology under 2 years old was metabolic (34.6%) and above that age was infectious (29.2%). Forty six percent were submitted to liver transplantation and 78% of them survived. Overall mortality was 34%. Median referral time was 7 days in period A (n = 35) and 2 days in period B (n = 15; p = 0.006). Pediatric risk of mortality´s median was 14.7 in period A and 6.5 in B (p = 0.019). Mortality was 37% vs 26% in periods A and B, respectively (p = 0.474). Discussion and Conclusions: Overall mortality was similar to the observed in other European centers. Liver transplantation is in fact the most effective therapeutic option. After 2008, there was a reduction in referral time and cases severity on admission; however, mortality has not reduced so far.

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A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica

et al. 2015

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Experience with molecular adsorbent recirculating system treatment in 20 children listed for high‐urgency liver transplantation

et al. 2015

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For more than 10 years, children at our national center for pediatric liver transplantation (LT) have been treated with Molecular Adsorbent Recirculating System (MARS) liver dialysis as a bridging therapy to high-urgency LT. Treatment was reserved for 20 patients with the highest degrees of hepatic encephalopathy (HE; median grade 5 3.5). Death from neurological sequelae was considered imminent for these patients, and this was further reflected in significantly higher international normalized ratios and ammonia levels and worse prognostic liver indices (Model for End-Stage Liver Disease/Pediatric End-Stage Liver Disease scores and liver injury units) in comparison with 32 wait-listed patients who did not receive MARS dialysis. MARS therapy was generally well tolerated, with a reduction in thrombocytes and hemorrhaging as the most common side effects. HE improvement was documented in 30% of the treated patients, but progression to grade IV encephalopathy occurred in 45% of the patients despite the treatment. Serum ammonia, bilirubin, bile acid, and creatinine levels significantly decreased during treatment. Eighty percent of MARS-treated patients survived to undergo LT, and their survival was equivalent to that of non-MARStreated patients with severe liver failure (69%, P 5 0.52). The heterogeneity between MARS-treated patients and non-MARStreated patients in our cohort precluded a statistical evaluation of a benefit from MARS for patient survival. Our data demonstrate the safety of MARS even in the most severely ill patients awaiting LT, but strategies that promote the more rapid and widespread availability of high-quality donor organs remain of critical importance for improving patient survival in cases of severe acute liver failure. Liver Transpl 21:369-380, 2015. V C 2015 AASLD.

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Artificial Liver Treatment, When and Which One?

Mitzner¹,

Grabow²,

Klammt³

2016

Contemporary Liver Transplantation

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Patients awaiting liver transplantation often are in critical condition with multi-organ failure accompanying the underlying liver disease. Stabilization and safe bridging of the patient to transplantation is a major indication for liver support methods. Various extracorporeal technologies have been tested clinically since the late 1950s. Currently, methods are divided into artificial, or nonbiological, systems based mainly on filtration and adsorption techniques and biological methods employing liver cells, typically of human, sometimes of animal origin. Examples of currently used liver support methods are the molecular adsorbent recirculating system (MARS) as an artificial system and the extracorporeal liver assist device (ELAD) as a biological liver-cell bioreactor. However, a considerable number of other approaches have been studied before or are currently under investigation. As of today, the best clinical experience exists for the MARS system. Liver support treatment should be considered early in the course of liver failure. Stabilization of various organ functions including circulatory, hepatic, and renal function, improvement of hepatic encephalopathy, and reduction of elevated intracranial pressure were observed in clinical studies. Modern extracorporeal liver support methods are safe to perform and a valuable addition to the therapeutic armamentarium of liver failure care.

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Paediatric acute liver failure and transplantation: The University of Essen experience

Cited by 37 publications

References 38 publications

A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica

A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica

Experience with molecular adsorbent recirculating system treatment in 20 children listed for high‐urgency liver transplantation

Artificial Liver Treatment, When and Which One?

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