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- 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.
- Air embolism as a complication of venovenous bypass during liver transplant for diffuse hemangiomatosisPublication . Viana, JS; Furtado, E; Romero, A; Furtado, AL
- The Coimbra University Hospital experience in liver transplantation in patients with familial amyloidotic polyneuropathyPublication . Perdigoto, R; Furtado, AL; Furtado, E; Oliveira, FJ; Geraldes, B; Mota, O; Ferrão, J; Tomé, L; Viana, JS
- Cytokine-producing T lymphocytes as a marker of prognosis and rejection episodes in orthotopic liver transplantationPublication . Paiva, A; Pereira, SV; Ballesteros, R; Freitas, A; Perdigoto, R; Mota, O; Ferrão, J; Tomé, L; Furtado, E; Cipriano, MA; Geraldes, B; Oliveira, FJ; Furtado, AL; Regateiro, FJ
- Deposition and passage of transthyretin through the blood-nerve barrier in recipients of familial amyloid polyneuropathy liversPublication . Sousa, MM; Ferrão, J; Fernandes, R; Guimarães, A; Geraldes, B; Perdigoto, R; Tomé, L; Mota, O; Negrão, L; Furtado, AL; Saraiva, MJFamilial amyloid polyneuropathy (FAP) is characterized by deposition of mutated transthyretin (TTR) in the peripheral nervous system. Prior to amyloid fibrils, nonfibrillar TTR aggregates are deposited inducing oxidative stress with increased nitration (3-NT). As the major source of TTR is the liver, liver transplantation (LT) is used to halt FAP. Given the shortage of liver donors, domino LT (DLT) using FAP livers is performed. The correlation between TTR deposition in the skin and nerve was tested in biopsies from normal individuals, asymptomatic carriers (FAP 0) and FAP patients; in FAP 0, nonfibrillar TTR was observed both in the skin and nerve in the same individuals; in patients, amyloid was detected in both tissues. The occurrence of amyloidosis in recipients of FAP livers was evaluated 1-7 years after DLT: TTR deposition occurred in the skin 3 years after transplantation either as amyloid or aggregates; in one of the recipients, fibrillar TTR was present in the epineurium 6 years after DLT. Deposits were scarce and 3-NT immunostaining was irrelevant. Nerve biopsies from DLT recipients had no FAP-related neuropathy. Our findings suggest that TTR amyloid formation occurs faster than predicted and that TTR of liver origin can cross the blood-nerve barrier. Recipients of FAP livers should be under surveillance for TTR deposition and tissue damage
- 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.
- Domino liver transplantation using FAP grafts. HUC experience: hopes and realitiesPublication . Furtado, ALDomino liver transplantation using FAP livers (DLTx/FAP) has become a worldwide strategy that is contributing significantly to the increase in liver grafts offered in Portugal. The safety of the procedure both in the FAP donors and in the recipients is emphasized in the series of 64 DLTx/FAP that has already been performed in the Hospital of the University of Coimbra (HUC). The occurrence of amyloidosis in the long-term survivors after DLTx/FAP is a serious possibility, although after 6 and 7 years post-transplant no symptoms have been reported by any of the patients. Indeed, amyloid deposits, although small, have been found in the skin and nerve biopsies of the first domino recipients (HUC unpublished data). Hopes that the disease will have a slow course in non-genetically affected patients still awaits the proof of time. Thus, DLTx/FAP should be considered an experimental procedure, although there is good reason to expect that the amyloidosis in the DLTx/FAP recipients will have a longer time course than in the genetically affected donors. The ethical implications of all these facts and expectations are discussed. A case by case approach is recommended. In such an approach, individual prognosis, urgency, graft shortage and the will of very well-informed patients or their legal representatives are determinants for judicious decisions when considering DLTx/FAP in patients under 50 years of age.
- Evolution of organ donation in central Portugal 1994-2009: a change in donor profilePublication . Calvão da Silva, AM; Saavedra, T; Alves, A; Henriques, MJ
- Haemodynamics during liver transplantation in familial amyloidotic polyneuropathy: study of the intraoperative cardiocirculatory data of 50 patientsPublication . Viana, JS; Bento, C; Vieira, H; Neves, S; Seco, C; Elvas, L; Coelho, L; Ferrão, J; Tomé, L; Perdigoto, R; Craveiro, AL; Providência, LA; Furtado, ALBACKGROUND: Circulatory instability frequently complicates liver transplantation for familial amyloidotic polyneuropathy (FAP) and may be a source of surgical morbidity and mortality. OBJECTIVE: To evaluate FAP intraoperative haemodynamic data and their relation to the duration of surgery, and need for anaesthetic drugs. RBC and sympathomimetic amines. SETTING: Clinical study during a four year period. PATIENTS (mean +/- SD): Group I included 50 consecutive FAP ATTR Met 30 recipients of first transplantation. Age was 35.3 +/- 7.1 years, neurological score 34.3 +/- 13 in 100 and time elapsed from first symptom 5.0 +/- 2.7 years. Group II (control), not different concerning age and sex, included 51 patients transplanted during the same period with other pathologies. METHOD: Anaesthetic protocol, monitoring and surgical techniques were similar in both groups. Data of the two groups were compared either by the Student's t-test or Fisher's exact test. RESULTS: Low values of systemic vascular resistance index were observed in both groups, with no differences between them. Systemic arterial pressures were usually lower in group I, because cardiac index and heart rate were also significantly lower, although within normal values. However, in group I, isoflurane (a vasodilator anaesthetic) was used during less time (p < 0.05) and in lower concentrations (p < 0.01) and phenylephrine was necessary in 26% of patients vs 0 patients in group II (p < 0.001). CONCLUSION: FAP patients presented a different intraoperative behaviour when compared to other patients submitted to liver transplantation. From a clinical point of view, the authors stress: 1--As a result of autonomic dysfunction, the administration of anaesthetic drugs to FAP patients always presents the risk of producing significant hypotension; even the use of ketamine does not prevent hypotension; 2--Safety is ensured by beat-to-beat surveillance of arterial pressures and the capacity to act immediately to support circulation; 3--These patients seem to be very sensitive to decreases in the pre-load; 4--Hypotension is also frequent with an adequate pre-load, usually as the result of low SVR; an infusion of a vasoconstrictor drug emerges as the most frequent treatment requested and our experience supports it as an effective one.
- 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.
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