Primary Disorders of Neurotransmitter Metabolism: Experience of a Tertiary Center

Neurotransmitter diseases are a group of inherited disorders attributable to a disturbance of neurotransmitter metabolism. Biogenic amines are neurotransmitters with multiple roles including psychomotor function, hormone secretion, cardiovascular, respiratory and gastrointestinal control, sleep mechanisms, body temperature and pain. Given the multiple functions of monoamines, disorders of their metabolism comprise a wide spectrum of manifestations, with motor dysfunction being the most prominent clinical feature. Methods: Case review of 12 patients from 4 families, with primary disorders of biogenic amine metabolism. Results: Aromatic L-amino acid decarboxylase deficiency (4 patients from 2 families), and GTP-cyclohydrolase (8 patients from 2 families) were the two diseases identified. Age at first symptoms varied between 2 months and 6 years. Developmental delay was present in all cases except 2 patients with GTP cyclohydrolase deficiency. The combination of axial hypotonia and limb dystonia was also frequent. Children with aromatic L-amino acid decarboxylase deficiency exhibited temperature instability, oculogyric crisis and disturbances of sleep. The index case of one family with GTP cyclohydrolase deficiency presented with Parkinsonism (bradykinesia, rigidity and hypomimia). Analysis of neurotransmitters and their metabolites in CSF was crucial for the identification of index cases. Response to therapy was variable but in general unsatisfactory except in a family with GTP cyclohydrolase deficiency. Conclusions: These disorders should be considered in the differential diagnosis of paediatric neurodegenerative diseases, in order to allow an adequate therapeutic trial that can favor prognosis.

Early Infantil Krabbe Disease with Unusual Survival

Introduction: Globoid cell leukodystrophy (Krabbe disease) is caused by a deficiency of the lysosomal galactocerebrosidase that results in progressive demyelination. The sole treatment is hematopoietic cell transplantation, which is only effective if performed before the onset of signs. In the absence of treatment, most children with early infantile Krabbe disease die within 2 years. Case Report: Female patient, first child of non-consanguineous parents, apparently normal till the fifth month of age when she presented with irritability, stiffness with clenched fists, developmental delay and feeding difficulties that progressed rapidly to failure to thrive, apathy, psychomotor regression, few spontaneous movements and spastic tetraparesis. Cerebral MRI showed extensive cerebral white matter abnormalities, relatively sparing the U-fibers, with a pattern of radiating stripes. Galactocerebrosidase activity in leukocytes and fibroblasts and molecular studies confirmed the diagnosis of Krabbe disease. After the rapid and regressive initial phase, she showed no further clinical progression of the disorder and although she did not grow she even showed regression of irritability and had a stable evolution and good visual contact until death over the age of 5 years. Comments: Our case shows that patients may have a stabilized form of disease and that a longer survival than described in the literature without transplant is possible in some patients.

Focal Malignant Liver Lesions: Diagnosis by Dynamic Incremental CT, Early Phase

The purpose of our study was to evaluate the accuracy of dynamic incremental bolus-enhanced conventional CT (DICT) with intravenous contrast administration, early phase, in the diagnosis of malignancy of focal liver lesions. A total of 122 lesions were selected in 74 patients considering the following criteria: lesion diameter 10 mm or more, number of lesions less than six per study, except in multiple angiomatosis and the existence of a valid criteria of definitive diagnosis. Lesions were categorized into seven levels of diagnostic confidence of malignancy compared with the definitive diagnosis for acquisition of a receiver-operator-characteristic (ROC) curve analysis and to determine the sensitivity and specificity of the technique. Forty-six and 70 lesions were correctly diagnosed as malignant and benign, respectively; there were 2 false-positive and 4 false-negative diagnoses of malignancy and the sensitivity and specificity obtained were 92 and 97%. The DICT early phase was confirmed as a highly accurate method in the characterization and diagnosis of malignancy of focal liver lesions, requiring an optimal technical performance and judicious analysis of existing semiological data.

Segmentectomia Pulmonar Anatómica por Videotoracoscopia: Experiência Inicial de um Centro

The use of minimally invasive surgery for the treatment of lung cancer has been growing worldwide. Between May 2008 and November 2012, we performed 24 videothoracoscopic anatomical lung resections in our department. This includes 22 lobectomies and 2 anatomic segmentectomies, which is known to be a more complex surgery, since it demands a finer dissection of sub-lobar structures. We report the clinical cases of two patients who underwent anatomic segmentectomies. The first one was a 63 year old woman, smoker and with a history of breast cancer 20 years earlier. An incidental 9 mm node was found in the lingula. The patient underwent an anatomic lingulectomy and the frozen section was suggestive of a primary lung cancer. Therefore, we proceeded to a full lymphadenectomy. The final pathology evaluation showed a typical carcinoid tumour (pT1aN0). The second patient was a 50 year old woman, a smoker and with a heavy family history of lung cancer. In a screening CT scan a 8 mm ground glass opacity was identified in the left lower lobe (segment VI). After a VATS wedge resection of the node the frozen section evaluation was compatible with adenocarcinoma. We then proceeded to an anatomic segmentectomy with lymphadenectomy. The definitive pathology evaluation confirmed that it was a pT1a N0 bronchioloalveolar adenocarcinoma. The patients now have 5 and 2 months of follow up respectivelly and neither of them has signs of recurrence and the surgical incision showed a good aesthetic result. Anatomic segmentectomy is the indicated surgery especially in patients with low grade tumours, in early stage lung cancers or in patients without pulmonary function for a lobar resection, and it can be done safely using VATS.

Safety and Effectiveness of the Genous™ Endothelial Progenitor Cell-Capture Stent in the First Year Following ST-Elevation Acute Myocardial Infarction: A Single Center Experience and Review of the Literature

PURPOSE: The Genous™ stent (GS) is designed to accelerate endothelization, which is potentially useful in the pro-thrombotic environment of ST-elevation acute myocardial infarction (STEMI). We aimed to evaluate the safety and effectiveness of the GS in the first year following primary percutaneous coronary intervention (PCI) and to compare our results with the few previously published studies. METHODS AND MATERIALS: All patients admitted to a single center due to STEMI that underwent primary PCI using exclusively GS, between May 2006 and January 2012, were enrolled. The primary study endpoints were major adverse cardiac events (MACEs), defined as the composite of cardiac death, acute myocardial infarction and target vessel revascularization, at one and 12months. RESULTS: In the cohort of 109 patients (73.4% male, 59 ±12years), 24.8% were diabetic. PCI was performed in 116 lesions with angiographic success in 99.1%, using 148 GS with median diameter of 3.00mm (2.50-4.00) and median length of 15mm (9-33). Cumulative MACEs were 2.8% at one month and 6.4% at 12months. Three stent thromboses (2.8%), all subacute, and one stent restenosis (0.9%) occurred. These accounted for the four target vessel revascularizations (3.7%). At 12months, 33.9% of patients were not on dual antiplatelet therapy. CONCLUSIONS: GS was safe and effective in the first year following primary PCI in STEMI, with an apparently safer profile comparing with the previously published data. SUMMARY: We report the safety and effectiveness of the Genous™ stent (GS) in the first year following primary percutaneous coronary intervention in ST-elevation acute myocardial infarction. A comprehensive review of the few studies that have been published on this subject was included and some suggest a less safe profile of the GS. Our results and the critical review included may add information and reinforce the safety and effectiveness of the GS in ST-elevation in acute myocardial infarction.

Alcohol Septal Ablation in Obstructive Hypertrophic Cardiomyopathy: Four Years of Experience at a Reference Center

INTRODUCTION: We describe our center's initial experience with alcohol septal ablation (ASA) for the treatment of obstructive hypertrophic cardiomyopathy. The procedure, its indications, results and clinical outcomes will be addressed, as will its current position compared to surgical myectomy. OBJECTIVE: To assess the results of ASA in all patients treated in the first four years of activity at our center. METHODS: We retrospectively studied all consecutive and unselected patients treated by ASA between January 2009 and February 2013. RESULTS: In the first four years of experience 40 patients were treated in our center. In three patients (7.5%) the intervention was repeated. Procedural success was 84%. Minor complications occurred in 7.5%. Two patients received a permanent pacemaker for atrioventricular block (6% of those without previous pacemaker). The major complication rate was 5%. There were no in-hospital deaths; during clinical follow-up (22 ± 14 months) cardiovascular mortality was 2.5% and overall mortality was 5%. DISCUSSION AND CONCLUSION: The results presented reflect the initial experience of our center with ASA. The success rate was high and in line with published results, but with room to improve with better patient selection. ASA was shown to be safe, with a low complication rate and no procedure-related mortality. Our experience confirms ASA as a percutaneous alternative to myectomy for the treatment of symptomatic patients with obstructive hypertrophic cardiomyopathy refractory to medical treatment.

The Role of Sensory Modulation Deficits and Behavioral Symptoms in a Diagnosis for Early Childhood

To contribute to the validation of the sensory and behavioral criteria for Regulation Disorders of Sensory Processing (RDSP) (DC:0-3R, 2005), this study examined a sample of toddlers in a clinical setting to analyze: (1) the severity of sensory modulation deficits and the behavioral symptoms of RDSP; (2) the associations between sensory and behavioral symptoms; and (3) the specific role of sensory modulation deficits in an RDSP diagnosis. Based on clinical observations, 78 toddlers were classified into two groups: toddlers with RDSP (N = 18) and those with‘‘other diagnoses in Axis I/II of the DC:0-3R’’ (OD3R; N = 60). The parents completed the Infant Toddler Sensory Profile and the Achenbach Checklist. The results revealed that the RDSP group had more severe sensory modulation deficits and specific behavioral symptoms; stronger, although not significant, associations between most sensory and behavioral symptoms; and a significant sensory modulation deficit effect. These findings support the validity of RDSP.

Analysis of the Generators of Epileptic Activity in Early-Onset Childhood Benign Occipital Lobe Epilepsy

Objective: The Panayiotopoulos type of idiopathic occipital epilepsy has peculiar and easily recognizable ictal symptoms, which are associated with complex and variable spike activity over the posterior scalp areas. These characteristics of spikes have prevented localization of the particular brain regions originating clinical manifestations. We studied spike activity in this epilepsy to determine their brain generators. Methods: The EEG of 5 patients (ages 7–9) was recorded, spikes were submitted to blind decomposition in independent components (ICs) and those to source analysis (sLORETA), revealing the spike generators. Coherence analysis evaluated the dynamics of the components. Results: Several ICs were recovered for posterior spikes in contrast to central spikes which originated a single one. Coherence analysis supports a model with epileptic activity originating near lateral occipital area and spreading to cortical temporal or parietal areas. Conclusions: Posterior spikes demonstrate rapid spread of epileptic activity to nearby lobes, starting in the lateral occipital area. In contrast, central spikes remain localized in the rolandic fissure. Significance: Rapid spread of posterior epileptic activity in the Panayitopoulos type of occipital lobe epilepsy is responsible for the variable and poorly localized spike EEG. The lateral occipital cortex is the primary generator of the epileptic activity.

Origin of Frontal Lobe Spikes in the Early Onset Benign Occipital Lobe Epilepsy (Panayiotopoulos Syndrome)

Objective: Early onset benign occipital lobe epilepsy (Panayiotopoulos syndrome [PS]) is a common and easily recognizable epilepsy. Interictal EEG spike activity is often multifocal but most frequently localized in the occipital lobes. The origin and clinical significance of the extra-occipital spikes remain poorly understood. Methods: Three patients with the PS and interictal EEG spikes with frontal lobe topography were studied using high-resolution EEG. Independent component analysis (ICA) was used to decompose the spikes in components with distinct temporal dynamics. The components were mapped in the scalp with a spline-laplacian algorithm. Results: The change in scalp potential topography from spike onset to peak, suggests the contribution of several intracranial generators, with different kinetics of activation and significant overlap. ICA was able to separate the major contributors to frontal spikes and consistently revealed an early activating group of components over the occipital areas in all the patients. The local origin of these early potentials was established by the spline-laplacian montage. Conclusions: Frontal spikes in PS are consistently associated with early and unilateral occipital lobe activation, suggesting a posteroanterior spike propagation. Significance: Frontal spikes in the PS represent a secondary activation triggered by occipital interictal discharges and do not represent an independent focus.

Myomectomy in Early Pregnancy - A Case Report

Uterine leiomyomas are by far the most common benign tumours of the female genital tract. The true incidence of leiomyomas during pregnancy is, however, unknown. Although leiomyomas usually remain asymptomatic during pregnancy, they may complicate its course. The management of leiomyoma during pregnancy is medical, but, in rare circumstances, surgical intervention and myomectomy may be required. A case of myomectomy in early pregnancy is described.

Métodos Preditivos de Metastização Ganglionar Adicional em Cancro da Mama com Gânglio Sentinela Positivo

Objectivos As recomendações para a realização de linfadenectomia axilar nos doentes com cancro da mama em estádio precoce e sujeitas a biópsia de gânglio sentinela com presença de macrometástase foram recentemente actualizadas, baseadas nos últimos estudos publicados (Z0011 e IBCSG 23-01). Mantém-se, no entanto, alguma controvérsia na decisão de não realização de cirurgia radical axilar nos doentes com gânglio sentinela positivo. Têm sido desenvolvidos métodos preditivos de metastização ganglionar adicional. Um dos mais conhecidos, desenvolvido no Memorial Sloan-Kettering Cancer Center (MSKCC), recorre a variáveis do tumor e características do gânglio sentinela. Mais recentemente com a utilização de métodos moleculares (como o One Step Nucleic Acid - OSNA) para estudo do gânglio sentinela tem sido avaliada a capacidade preditiva da quantidade total de cópias mRNA da citoqueratina 19. Pretende-se estudar na nossa amostra a capacidade preditiva do nomograma do MSKCC e da carga tumoral total. Propõe-se ainda avaliar o número de macrometástases encontradas na biópsia de gânglio sentinela e sua relação na metastização ganglionar adicional. Material e métodos Avaliação retrospectiva de 819 doentes com cancro da mama (Tis – T2) submetidos a biópsia de gânglio sentinela no Centro Hospitalar de Lisboa Central durante o período de 1 de Janeiro de 2005 e 31 de Dezembro de 2013. Foram identificados 123 doentes com gânglio sentinela positivo que realizaram linfadenectomia axilar imediata. A análise do gânglio sentinela foi executada por métodos histológicos em 78 doentes e por método molecular (OSNA) em 45 doentes. Os dois grupos foram estudados separadamente, tendo sido no primeiro aplicado o nomograma do MSKCC e no segundo obtida a carga tumoral total. Utilizou-se o modelo de regressão logística para analisar o poder preditivo e discriminativo destes dois métodos. Adicionalmente, para avaliar a potencial importância do número de macrometástases na metastização ganglionar adicional, foi ajustado um novo modelo de regressão logística considerando esta variável e a carga tumoral total. Ambos os métodos foram também avaliados através da área sob a curva ROC (AUC) e do teste de Hosmer-Lemeshow, respectivamente. O nível de significância adoptado foi α = 0.05. O estudo estatístico foi realizado com recurso ao SPSS. Resultados No grupo em que foi aplicado o nomograma do MSKCC obteve-se uma AUC=0.67 (I.C. 95% = 0.55 – 0.79), e no grupo em que foi avaliada a carga tumoral total uma AUC=0.78 (I.C. 95% = 0.64 – 0.91). Os poderes preditivos de ambos foram, respectivamente, p=0.15 e p=0.46. Constatou-se que o desempenho do modelo resultante da junção da carga tumoral total com o número de macrometástases encontradas no estudo do gânglio sentinela foi bastante satisfatório (AUC=0.87, I.C 95% = 0.76 – 0.98, poder preditivo p=0.33). Conclusão Foi validado externamente o modelo do MSKCC para a amostra em estudo, apresentando uma menor acuidade discriminativa em relação ao estudo original (AUC=0.67 versus AUC=0.75). Por outro lado, após verificação da homogeneidade de ambos os grupos no que diz respeito a todas as variáveis de interesse, conclui-se que a carga tumoral total aparenta uma maior acuidade preditiva e discriminativa na metastização ganglionar adicional que o nomograma do MSKCC. Quando desenvolvido um modelo agregando a carga tumoral total avaliada por OSNA e o número de macrometástases no gânglio sentinela, obteve-se uma capacidade discriminativa ainda superior. A carga tumoral total avaliada por OSNA, ou esta incluída num modelo conjunto com o número de macrometástases obtidas no estudo do gânglio sentinela, poderão representar importantes ferramentas preditivas. Serão no entanto necessários estudos adicionais com amostras superiores para consolidar estes resultados. Bibliografia 1. Giuliano AE, McCall L, Beitsch P, Whitworth PW, Blumencranz P, Leitch AM, et al. Locoregional recurrence after sentinel lymph node dissection with or without axillary dissection in patients with sentinel lymph node metastases: the American College of Surgeons Oncology Group Z0011 randomized trial. Ann Surg. Setembro de 2010;252(3):426–32; discussion 432–3. 2. Galimberti V, Cole BF, Zurrida S, Viale G, Luini A, Veronesi P, et al. Axillary dissection versus no axillary dissection in patients with sentinel-node micrometastases (IBCSG 23-01): a phase 3 randomised controlled trial. Lancet Oncol. Abril de 2013;14(4):297–305. 3. Bevilacqua JLB, Kattan MW, Fey JV, Cody HS, Borgen PI, Van Zee KJ. Doctor, what are my chances of having a positive sentinel node? A validated nomogram for risk estimation. J Clin Oncol. 20 de Agosto de 2007;25(24):3670–9. 4. Piñero A, Canteras M, Moreno A, Vicente F, Giménez J, Tocino A, et al. Multicenter validation of two nomograms to predict non-sentinel node involvement in breast cancer. Clin Transl Oncol. Fevereiro de 2013;15(2):117–23. 5. Banerjee SM, Michalopoulos NV, Williams NR, Davidson T, El Sheikh S, McDermott N, et al. Detailed evaluation of one step nucleic acid (OSNA) molecular assay for intra-operative diagnosis of sentinel lymph node metastasis and prediction of non-sentinel nodal involvement: experience from a London teaching hospital. Breast. Agosto de 2014;23(4):378–84. 6. Peg V, Espinosa-Bravo M, Vieites B, Vilardell F, Antúnez JR, de Salas MS, et al. Intraoperative molecular analysis of total tumor load in sentinel lymph node: a new predictor of axillary status in early breast cancer patients. Breast Cancer Res Treat. Maio de 2013;139(1):87–93. 7. Tiernan JP, Verghese ET, Nair A, Pathak S, Kim B, White J, et al. Systematic review and meta-analysis of cytokeratin 19-based one-step nucleic acid amplification versus histopathology for sentinel lymph node assessment in breast cancer. Br J Surg. Março de 2014;101(4):298–306. 8. Heilmann T, Mathiak M, Hofmann J, Mundhenke C, van Mackelenbergh M, Alkatout I, et al. Intra-operative use of one-step nucleic acid amplification (OSNA) for detection of the tumor load of sentinel lymph nodes in breast cancer patients. J Cancer Res Clin Oncol. Outubro de 2013;139(10):1649–55. 9. Chaudhry A, Williams S, Cook J, Jenkins M, Sohail M, Calder C, et al. The real-time intra-operative evaluation of sentinel lymph nodes in breast cancer patients using One Step Nucleic Acid Amplification (OSNA) and implications for clinical decision-making. Eur J Surg Oncol. Fevereiro de 2014;40(2):150–7.

Prosthetic Mitral Valve Thrombosis in Pregnancy: From Thrombolysis to Anticoagulation

INTRODUCTION: Pregnant women with mechanical prosthetic heart valves are at increased risk for valve thrombosis. Management decisions for this life-threatening complication are complex. Open-heart surgery has a very high risk of maternal mortality and fetal loss. Bleeding and embolic risks associated with thrombolytic agents, the limited efficacy of thrombolysis in certain subgroups, and a lack of experience in the setting of pregnancy raise important concerns. CASE REPORT: We report a case of mitral prosthetic valve thrombosis in early pregnancy, which was successfully treated with streptokinase. Ten years later, the same patient had an uneventful pregnancy, throughout which acenocoumarol was maintained. CONCLUSION: With this case we review the prevention (with oral anticoagulant therapy) and treatment of prosthetic valve thrombosis during pregnancy, which is important for both obstetrician and cardiologist.

Renal Disease in Madeira Islands: Twenty-Seven Years' Experience of Native Kidney Biopsies

Introduction: Renal biopsy plays an essential role either in the diagnosis or in the prognosis of patients with renal disease. In order to assess its epidemiology and evolution in Madeira Islands, we analysed twenty-seven years of native kidney biopsies. Methods: We performed a retrospective analysis of clinical records, including histological revision from 1986 to 2012, totalling 315 native kidney biopsies. They were assessed regarding the temporal evolution both for the quality/indications for renal biopsy and for the patterns of kidney disease. Results: A total of 315 native kidney biopsies were analysed. The patients’ mean age was of 40.8 ± 18.4 years and 50.5%(n = 159) were males. The most common indications for renal biopsy were nephrotic syndrome (36.2%, n = 114) and acute kidney injury (20.0%, n = 63). Among primary glomerular diseases (41.5%, n = 115) the most common were IgA nephropathy (26.1%, n = 30) and focal-segmental glomerulosclerosis (17.4%, n = 20) and among secondary glomerular diseases (31.4%, n = 87), lupus nephritis (51.7%, n = 45) and amyloidosis (20.7%, n = 18). Statistical analysis revealed significant correlation between gender and major pathological diagnosis (Fisher’s exact test, p <.01) and between indications for renal biopsy and major pathological diagnosis (χ2, p <.01). Regarding the temporal evolution, no statistically significant differences were found in the number of renal biopsies (χ2, p =.193), number of glomeruli per sample (Fisher’s exact test, p =.669), age (Kruskal-Wallis, p =.216), indications for renal biopsy (χ2, p =.106) or major pathological diagnosis groups (χ2,p =.649). However, considering the specific clinico-pathological diagnoses and their temporal variation, a statistically significant difference (Fisher’s exact test, p <.05) was found for lupus nephritis and membranous nephropathy with an increasing incidence and for amyloidosis with an opposite tendency. Discussion: The review of the native kidney biopsies from a population with particular characteristics, geographically isolated, such as those from Madeira Islands, showed parallel between epidemiological numbers referring to other European subpopulations, allowing simultaneously a comprehensive approach to our renal biopsy policies.

Prophylactic Use of Liposomal Amphotericin B in Preventing Fungal Infections Early After Liver Transplantation: a Retrospective, Single-Center Study

In this study the authors evaluated the efficacy of prophylaxis with liposomal amphotericin B (L-AmB) in the incidence of fungal infections (FI) during the first 3 months after liver transplant (LT). The study was retrospective and accessed a 4-year period from 2008 to 2011. All patients who died in the first 48 hours after LT were excluded. Patients were divided by the risk groups for FI: Group 1, high-risk (at least 1 of the following conditions: urgent LT; serum creatinine >2 mg/dL; early acute kidney injury [AKI] after LT; retransplantation; surgical exploration early post-LT; transfused cellular blood components [>40 U]); and Group 2, low-risk patients. Group 1 patients were further separated into those who received antifungal prophylaxis with L-AmB and those who did not. Prophylaxis with L-AmB consisted of intravenous administration of L-AmB, 100 mg daily for 14 days. Four hundred ninety-two patients underwent LT; 31 died in the first 48 hours after LT. From the remaining 461 patients, 104 presented with high-risk factors for FI (Group 1); of these, 66 patients received antifungal prophylaxis and 38 did not. In this group 8 FI were observed, 5 in patients without antifungal prophylaxis (P = .011). Three more FI were identified in Group 2. By logistic regression analysis, the categorical variable high-risk group was independently related to the occurrence of invasive FI (P = .006). We conclude that prophylaxis with L-AmB after LT was effective in reducing the incidence of FI. No influence on mortality was detected.

Liver Depurative Techniques: A Single Liver Transplantation Center Experience

In a liver transplant (LT) center, treatments with Prometheus were evaluated. The main outcome considered was 1 and 6 months survival. Methods. During the study period, 74 patients underwent treatment with Prometheus; 64 were enrolled,with a mean age of 51 13 years; 47men underwent 212 treatments (mean, 3.02 per patient). The parameters evaluated were age, sex, laboratorial (liver enzymes, ammonia) and clinical (model for end-stage liver disease and Child-Turcotte-Pugh score) data. Results. Death was verified in 23 patients (35.9%) during the hospitalization period, 20 patients (31.3%) were submitted to liver transplantation, and 21 were discharged. LT was performed in 4 patients with acute liver failure (ALF, 23.7%), in 7 patients with acute on chronic liver failure (AoCLF, 43.7%), and in 6 patients with liver disease after LT (30%). Seven patients who underwent LT died (35%). In the multivariate analysis, older age (P ¼ .015), higher international normalized ratio (INR) (P ¼ .019), and acute liver failure (P ¼ .039) were independently associated with an adverse 1-month clinical outcome. On the other hand, older age (P ¼ .011) and acute kidney injury (P ¼ .031) at presentation were both related to worse 6-month outcome. For patients with ALF and AoCLF we did not observe the same differences. Conclusions. In this cohort, older age was the most important parameter defining 1- and 6-month survival, although higher INR and presence of ALF were important for 1-month survival and AKI for 6-month survival. No difference was observed between patients who underwent LT or did not have LT.