A Importância da Referenciação Precoce na Falência Hepática Aguda Pediátrica

Jerónimo, Mónica; Moinho, Rita; Pinto, Carla; Carvalho, Leonor; Gonçalves, Isabel; Furtado, Emanuel; Neves, José Farela

doi:10.20344/amp.6195

Cited by 4 publications

(6 citation statements)

References 21 publications

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“…At this age group the liver transplantation is not possible to be performed in our country. In other studies, the mortality due to ALF in infants varies from 20% in the PALF study, to 34% in Portugal, and 42% in young infants (0–4 months) in the UK [ 15 , 16 , 22 ].…”

Section: Discussionmentioning

confidence: 99%

Etiology and Outcome of Acute Liver Failure in Children—The Experience of a Single Tertiary Care Hospital from Romania

et al. 2020

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Background: Acute liver failure (ALF) is a rare disease, associated with high mortality, despite optimal medical therapy without emergency liver transplantation. Knowing the possible cause of ALF plays a vital role in the management, as the child could benefit from effective specific therapies in emergencies. Methods: We have analyzed the etiology and outcome of ALF in children followed-up in a tertiary care hospital between January 2012–December 2018. The patients were grouped into different age categories: neonates (0–1 month), infants (1–12 months), children (1–14 years), and teenagers (14–18 years). Results: 97 children (46 males, 47.42%, the mean age of 7.66 ± 8.18 years) were admitted with ALF. The most important causes of ALF were in neonates and infants, infections (72.72%), and metabolic disorders (43.47%), in children and adolescents were the toxic causes (60% and 79.41%). The mortality rate was 31.95% (31 patients), mainly in ALF due to infections or metabolic disorders. Conclusions: In neonates and infants, the main causes of ALF were infections and metabolic diseases, while in older children and teenagers, were toxin-induced liver injuries. The mortality among neonates and infants was significantly higher than in other ages. Early recognition and immediate therapeutic intervention could improve the outcome of these patients.

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Section: Discussionmentioning

confidence: 99%

Etiology and Outcome of Acute Liver Failure in Children—The Experience of a Single Tertiary Care Hospital from Romania

et al. 2020

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“…PALF treatment involves the challenging decision whether to maintain only supportive medical therapy or to advance to an urgent LT. LT is the only proven treatment for PALF and has allowed, in recent decades, a significant improvement in its prognosis, increasing survival to about 60–85% [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Another critical factor in the prognosis of PALF is the early referral to a LT center [ 8 ]. The multidisciplinary and differentiated approach allows optimization of support measures, undergoing LT in better conditions, identifying situations in which LT is contraindicated and eventually enhancing the probability of SR. An early referral to the LT center is associated with higher survival than a late referral [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Regarding the etiology of PALF, metabolic disorders were the most frequent (25.4%), possibly because our hospital is a reference for this type of disease. Metabolic conditions account for approximately 10% of all cases of PALF and 18% of PALF cases among children younger than 3 years [ 8 ].…”

Section: Discussionmentioning

confidence: 99%

“…Despite the advances in supportive measures and target treatment for specific etiologies in intensive care units [ 6 ], ALF carries a high mortality risk or requires LT in 70% of cases [ 2 , 7 ]. Since that a critical factor in the prognosis of PALF is the early referral to a LT center [ 8 ], in January 2008, a multidisciplinary national meeting was held in Portugal on PALF, which involved the Societies of Gastroenterology, Hepatology and Nutrition and Pediatric Intensive Care of the Portuguese Society of Pediatrics, resulting in a consensus on action reinforcing the importance of its differentiated approach and early referral to the only one pediatric LT national center. Therefore, when patients have a PALF diagnosis in any hospital in our country, contact should be made with the LT center, in order to optimize its management and, eventually, provide an early hospital transfer [ 8 ].…”

Section: Introductionmentioning

confidence: 99%

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Prognostic Markers in Pediatric Acute Liver Failure

Nogueira¹,

Teixeira²,

Rodrigues-Fernandes³

et al. 2023

GE Port J Gastroenterol

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Introduction: Acute liver failure (ALF), although rare in children, is a complex progressive pathology, with multisystem involvement and high mortality. Isolated variables or those included in prognostic scores have been studied, to optimize organ allocation. However, its validation is challenging. This study aimed to assess the accuracy of several biomarkers and scores as predictors of prognosis in pediatric ALF (PALF). Methods: An observational study with retrospective data collection, including all cases of ALF, was defined according to the criteria of the Pediatric Acute Liver Failure Study Group, admitted to a pediatric intensive care unit (PICU) for 28 years. Two groups were defined: spontaneous recovery (SR) and non-SR (NSR) – submitted to liver transplantation (LT) or death at PICU discharge. Results: Fifty-nine patients were included, with a median age of 24 months, and 54% were female. The most frequent etiologies were metabolic (25.4%) and infectious (18.6%); 32.2% were undetermined. SR occurred in 21 patients (35.6%). In NSR group (N = 38, 64.4%), 25 required LT (42.4%) and 19 died (32.2%), 6 (15.7%) of whom after LT. The accuracy to predict NSR was acceptable for lactate at admission (AUC 0.72; 95% CI: 0.57–0.86; p = 0.006), ammonia peak (AUC 0.72; 95% CI: 0.58–0.86; p = 0.006), and INR peak (AUC 0.70; 95% CI: 0.56–0.85; p = 0.01). The cut-off value for lactate at admission was 1.95 mmol/L (sensitivity 78.4% and specificity 61.9%), ammonia peak was 64 μmol/L (sensitivity 100% and specificity 38.1%), and INR peak was 4.8 (sensitivity 61.1% and specificity 76.2%). Lactate on admission was shown to be an independent predictor of NSR on logistic regression model. Two prognostic scores had acceptable discrimination for NSR, LIU (AUC 0.73; 95% CI: 0.59–0.87; p = 0.004) and PRISM (AUC 0.71; 95% CI: 0.56–0.86; p = 0.03). In our study, the PALF delta score (PALF-ds) had lower discrimination capacity (AUC 0.63; 95% CI: 0.47–0.78; p = 0.11). Conclusions: The lactate at admission, an easily obtained parameter, had a similar capacity than the more complex scores, LIU and PRISM, to predict NSR. The prognostic value in our population of the promising dynamic score, PALF-ds, was lower than expected.

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