Diagnostic Approach to Acute Liver Failure in Children: A Position Paper by the SIGENP Liver Disease Working Group

Giorgio, Angelo Di; Bartolini, Elisa; Calvo, Pier Luigi; Cananzi, Mara; Cirillo, Francesco; Corte, Claudia Della; Dionisi‐Vici, Carlo; Indolfi, Giuseppe; Iorio, Raffaele; Maggiore, Giuseppe; Mandato, Claudia; Nebbia, Gabriella; Nicastro, Emanuele; Pinon, Michele; Ranucci, Giusy; Sciveres, Marco; Vajro, Pietro; D’Antiga, Lorenzo

doi:10.1016/j.dld.2021.03.004

Cited by 23 publications

(42 citation statements)

References 104 publications

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“…Di Giorgio et al’s position paper on the evaluation of pediatric ALF suggested several factors to establish the diagnosis of autoimmune ALF. These included a combination of biochemical features such as positive autoantibodies, histological features such as interface hepatitis, and the presence of autoimmunity in the patient or family 12 . In our patient, the autoimmune nature of the ALF was strongly suggested by the presence of positive anti‐SMA, elevated IgG, and the finding of the AIRE mutation on genetic testing.…”

Section: Discussionmentioning

confidence: 63%

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Rare genetic mutation triggering acute liver failure in a toddler requiring a liver transplant

Chen

Ambani

Zeft

et al. 2021

Pediatric Transplantation

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APS‐1 is an extremely rare, autosomal recessive condition that often presents with candidiasis, adrenal insufficiency, and hypoparathyroidism. This condition is associated with autoimmune hepatitis in less than 20% of cases, and there have only been a few reports of children with the condition who developed ALF. We present a unique case of an infant with APS‐1 who developed ALF and subsequently required liver transplantation.

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Section: Discussionmentioning

confidence: 63%

“…Autoimmune hepatitis only represents 6–22% of total cases of pediatric ALF and the diagnosis may be difficult to establish 11‐13 . Di Giorgio et al’s position paper on the evaluation of pediatric ALF suggested several factors to establish the diagnosis of autoimmune ALF.…”

Section: Discussionmentioning

confidence: 99%

Rare genetic mutation triggering acute liver failure in a toddler requiring a liver transplant

Chen

Ambani

Zeft

et al. 2021

Pediatric Transplantation

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“…1 Diagnostic evaluation should be tailored according to clinical information, age of presentation, and geographical location. 4,7…”

Section: Clinical Evaluationmentioning

confidence: 99%

“…8,9 DNA sequencing technology (e.g., clinical exome sequencing and next generation sequencing) is increasingly effective in detecting inherited metabolic disorders and monogenic diseases. 4 In their cohort of 45 children with indeterminate PALF, Hegarty et al 9 reported that next generation sequencing helped identifying specific etiologies in 20% of the cases. The most common findings were variants involving neuroblastoma amplified sequence ( NBAS ) and mitochondrial DNA maintenance genes.…”

Section: Clinical Evaluationmentioning

confidence: 99%

“…Biopsy of the oral mucosa is also helpful as it would show iron staining in the minor salivary glands. 4 Histologically, the liver in GALD is characterized by panlobular hepatocellular damage with reticulin collapse and variable degree of pericellular fibrosis. Hepatocytes may show siderosis, pseudoacinar, and occasional giant cell transformation with canalicular cholestasis (Figure 2).…”

Section: Specific Etiologies Of Pediatric Acute Liver Failurementioning

confidence: 99%

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Pediatric Acute Liver Failure: A Clinicopathological Perspective

Putra

Perez‐Atayde

2022

Pediatr Dev Pathol

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Pediatric acute liver failure (PALF) is a life-threatening disorder characterized by acute hepatocellular injury occurring in children without recognized underlying liver disease. The clinicopathologic evaluation of PALF requires a different approach from that in adults. The diagnostic considerations differ depending on the age, personal and family history, geographical region, and clinical presentation. Distinct entities such as gestational alloimmune liver disease, herpes simplex virus infection, and metabolic disorders should be considered in neonates with acute liver failure, while acetaminophen toxicity and autoimmune hepatitis are more frequently seen in older children and adolescents. An identified cause for PALF despite a negative complete evaluation (indeterminate) is lacking in 30 to 50% of cases. Although not routinely performed in the setting of PALF, liver biopsy may be helpful in assessing the etiology, potential mechanisms of injury, determining the appropriateness of liver transplantation, and prognostication of the patients. In this article, we review the clinicopathologic characteristics of PALF with an emphasis on general approach of pathologic evaluation and histopathologic characteristic of selected entities.

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Pediatric liver transplant for acute liver failure: Defining the standard nutrition and clinical evolution: An observational study

Zamberlan,

Bonfim,

Tannuri

et al. 2024

Nut in Clin Prac

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BackgroundPediatric acute liver failure (PALF) is a clinical diagnosis in which previously asymptomatic children exhibit a rapid loss of liver function.ObjectivesTo describe the clinical and nutrition conditions at admission and during hospitalization in patients with PALF who underwent liver transplantation (LT) at a referral center and establish a possible association between nutrition status and prognosis in this population.MethodsA prospective study was conducted on children underwent LT because of PALF. Nutrition assessment was performed at admission and repeated weekly until hospital discharge, and their relationships with clinical outcomes were tested.ResultsThe study population consisted of 23 patients with a median age of 60 months and, in most cases, the etiology of acute liver failure (ALF) was indeterminate (69.6%). The majority of the patients were well nourished according to the z score body mass index‐for‐age (82.6%) and z arm circumference‐for‐age (zAC/A) (82.6%). A decline in nutrition status was observed in 47.6% of the patients, as indicated by a decrease in zAC. Clinical and nutrition improvement was demonstrated by the progressive increase in zAC, serum albumin levels, and phase angle (PA) values and by the reduction in C‐reactive protein levels. There was no statistically significant relationship between nutrition status and clinical outcomes.ConclusionMost children and adolescents with ALF presented adequate nutrition status at the time of LT, and some patients worsened their nutrition status during hospitalization, suggesting that post‐LT is a period of high nutrition risk, and early nutrition therapy (NT) must be a priority in these patients.

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Diagnostic Approach to Acute Liver Failure in Children: A Position Paper by the SIGENP Liver Disease Working Group

Cited by 23 publications

References 104 publications

Rare genetic mutation triggering acute liver failure in a toddler requiring a liver transplant

Rare genetic mutation triggering acute liver failure in a toddler requiring a liver transplant

Pediatric Acute Liver Failure: A Clinicopathological Perspective

Pediatric liver transplant for acute liver failure: Defining the standard nutrition and clinical evolution: An observational study

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