Journal: | Annals of hepatology |
Database: | PERIÓDICA |
System number: | 000415667 |
ISSN: | 1665-2681 |
Authors: | Brett, Ana1 Pinto, Carla1 Carvalho, Leonor1 Garcia, Paula1 Diogo, Luísa1 Goncalves, Isabel1 |
Institutions: | 1Centro Hospitalar e Universitario de Coimbra, Hospital Pediatrico de Coimbra, Coimbra. Portugal |
Year: | 2013 |
Season: | Sep-Oct |
Volumen: | 12 |
Number: | 5 |
Pages: | 791-796 |
Country: | México |
Language: | Inglés |
Document type: | Artículo |
Approach: | Analítico, descriptivo |
English abstract | The early establishment of an etiology for acute liver failure (ALF) in infants is essential for the start of adequate treatment in the shortest timeframe possible. Aim. To identify markers of inherited metabolic disease on admission in children under two years of age with ALF. Material and methods. A retrospective review of the medical records of all children (< 2 years old) with ALF admitted to the pediatric hepatology or intensive care units of a tertiary center over a twenty-three year period (January 1989 to December 2011) was done. Patients were divided into two groups: with (group A) or without (group B) a metabolic etiology. Clinical and laboratory parameters on admission were compared. Results. Twenty-three children met inclusion criteria. Twelve had ALF of metabolic origin (group A). The median age in this group was 2.25 (Q1-Q3: 0.63-4.65) months and in group B 8.0 (Q1-Q3: 1.5-15) months. History of failure to thrive and/or vomiting was more frequent in group A (p = 0.022). Age, gender, encephalopathy and left ventricular hypertrophy were similar in both groups (p = 0.147, p = 1.000, p = 0.637, p = 1.000, respectively). Laboratory tests on admission (plasma lactate, ammonia, cholesterol, phosphate, INR, glucose, bilirubin, ALT, base excess and the presence of reducing substances in urine) showed no statistically significant differences between groups. Conclusion. This study showed that although infants with inborn errors of metabolism showed a trend towards lower age at presentation, the only marker of inherited metabolic disease found on admission was history of vomiting and/or failure to thrive |
Disciplines: | Medicina |
Keyword: | Gastroenterología, Pediatría, Falla hepática aguda, Niños, Errores innatos del metabolismo, Marcadores diagnósticos |
Keyword: | Medicine, Gastroenterology, Pediatrics, Acute liver failure, Children, Inborn metabolic errors, Diagnostic markers |
Full text: | Texto completo (Ver PDF) |