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- [The Importance of Early Referral in Pediatric Acute Liver Failure]Publication . Jerónimo, M; Moinho, R; Pinto, C; Carvalho, L; Gonçalves, I; Furtado, E; Farela Neves, JINTRODUCTION: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus. OBJECTIVES: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008. MATERIAL AND METHODS: Observational, retrospective study during a 20 year period (1994-2014). 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.
- Liver transplantation for colorectal liver metastasis: Survival without recurrence can be achievedPublication . Toso, C; Pinto Marques, H; Andres, A; Castro e Sousa, F; Adam, R; Kalil, A; Clavien, PA; Furtado, E; Barroso, E; Bismuth, H
- Acute liver failure related to inherited metabolic diseases in young childrenPublication . Dias-Costa, F; Moinho, R; Ferreira, S; Garcia, P; Diogo, L; Gonçalves, I; Pinto, CINTRODUCTION: Pediatric acute liver failure (ALF) due to inherited metabolic diseases (IMD) is a rare life-threatening condition with a poor prognosis. Early intervention may be lifesaving. OBJECTIVE: To describe clinical presentation, investigation and outcomes of ALF related to IMD in young children. MATERIAL AND METHODS: Retrospective review of the medical records of children aged up to 24 months, admitted to a tertiary pediatric and neonatal Intensive Care Unit during a 27-year period, fulfilling the ALF criteria, with documented metabolic etiology. RESULTS: From 34 ALF cases, 18 were related to IMD: galactosemia (4), mitochondrial DNA depletion syndrome (MDS) (3), ornithine transcarbamilase deficiency (3), congenital defects of glycosylation (2), tyrosinemia type 1 (2), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (1), hereditary fructose intolerance (1), classic methylmalonic aciduria (1) and citrulinemia type 1 (1). The median age was 1.3 months. At least one previous suggestive sign/symptom of IMD (vomiting, failure to thrive, hypotonia or developmental delay) was observed in 67% of the cases. The most common physical signs at admission included: hepatomegaly (72%), jaundice (67%) and encephalopathy (44%). The peak laboratorial findings were: mean international normalizad ratio 4.5, median lactate 5mmol/L, mean bilirubin 201μmol/L, median alanine aminotransferase (ALT) 137 UI/L and median ammonia 177μmol/L. One patient was submitted to liver transplant in ALF context (MSD). The mortality rate was 44%. DISCUSSION: The identification of IMD as a frequent cause of ALF allowed specific therapeutic measures and adequate family counselling. Particular clinical features and moderated ALT and bilirubin levels can lead to its suspicion.
- A Importância da Referenciação Precoce na Falência Hepática Aguda PediátricaPublication . Jerónimo, M; Moinho, R; Carvalho, L; Gonçalves, I; Furtado, E; Farela-Neves, JINTRODUCTION: Acute liver failure is a rare disorder associated to high morbidity and mortality despite survival improvement through liver transplantation. The importance of a multidisciplinary approach and early referral to a pediatric liver transplantation center were important conclusions of a national meeting in 2008, from which resulted an actuation consensus. OBJECTIVES: To characterize acute liver failure admissions in a Pediatric Intensive Care Unit of the portuguese pediatric livertransplantation center. To compare results before (A) and after (B) 2008. MATERIAL AND METHODS: Observational, retrospective study during a 20 year period (1994-2014). 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.
- Diastolic Dysfunction in Liver Cirrhosis: Prognostic Predictor in Liver Transplantation?Publication . Carvalheiro, F; Rodrigues, C; Adrego, T; Viana, JS; Vieira, H; Seco, C; Pereira, L; Pinto, F; Eufrásio, A; Bento, C; Furtado, EBACKGROUND: Patients with liver cirrhosis may develop cirrhotic cardiomyopathy (CC), characterized by blunted contractile responsiveness to stress, diastolic dysfunction (DD), and electrophysiological abnormalities. It may adversely affect the long-term prognosis of these patients. METHODS: We conducted a retrospective analysis of patients undergoing liver transplantation (LT) for cirrhosis from January 2012 to June 2015. We analyzed demographic characteristics, the etiology of cirrhosis, Child-Pugh and Model for End-Stage Liver Disease (MELD) scores, the corrected QT (QTc) interval in the preoperative period, diastolic and systolic dysfunction, mortality and survival, and duration of mechanical ventilation and vasopressor support in the post-LT period. These variables were compared with diastolic dysfunction and prolongation of QTc, with the use of chi-square, Fisher, and Mann-Whitney U tests. RESULTS: The study included 106 patients, 80.2% male and overall average age 54.83 years. The median MELD score was 16, and Child-Pugh class C in 55.4%. Prolonged QTc interval before LT was present in 19% and DD in 35.8% of patients. QTc before LT or DD did not vary significantly with MELD or Child-Pugh score. CONCLUSIONS: The patients in the pre-LT period presented with a significant incidence of DD, which can predispose them to adverse cardiac events. The presence of DD correlates with mortality after LT in patients with hepatic cirrhosis.
- Manifestações Oculares de Polineuropatia Amiloidótica Familiar Tipo I em Doentes Submetidos a Transplante HepáticoPublication . Rosa, AM; Quadrado, MJ; Ferrão, J; Marques, I; Pereira, H; Costa, E; Murta, JN
- Peripheral blood lymphocyte phenotype can predict rejection episodes after orthotopic liver transplantationPublication . Perdigoto, R; Paiva, A; Freitas, A; Coimbra, HB; Mota, O; Ferrão, J; Tomé, L; Furtado, E; Cipriano, MA; Oliveira, FJ; Furtado, AL
- Evolution of organ donation in central Portugal 1994-2009: a change in donor profilePublication . Calvão da Silva, AM; Saavedra, T; Alves, A; Henriques, MJ
- Recombinant factor VIIa in major abdominal surgery and liver transplantationPublication . Viana, JSThe author reviewed the literature regarding recombinant activated Factor VII (rFVIIa) in major abdominal surgery and liver transplantation and concluded that, on the basis of evidence-based medicine, there is no evidence to support an extensive use of rFVIIa. Nevertheless, various case reports suggest the usefulness of rFVIIa to treat life-threatening bleeding after failure of conventional therapies. It appears that there is a consensus that rFVIIa can be used with good results as a rescue therapy in extremely severe situations. Economic cost and potential thrombosis risk remain arguments against more widespread use of rFVIIa. Doses from 5 to 120 kg/kg in each administration have been reported without clear evidence to support a specific protocol. Efficacy of 15 to 20 kg/kg in surgical settings has been reported, but higher doses are more frequently used. The majority of the reviewed investigators accepted the use of rFVIIa after or simultaneously with the use of aprotinin; no data refute the safety of this association.
- Sequential liver transplantation: 27 cases in 25 patientsPublication . Tomé, L; Ferrão, J; Furtado, E; Geraldes, J; Mota, O; Oliveira, F; Perdigoto, R; Viana, JS; Cipriano, MA; Furtado, AL
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