Epidemiological and Clinical Aspects of Cutaneous and Mucosal Leishmaniases in Portugal: Retrospective Analysis of Cases Diagnosed in Public Hospitals and Reported in the Literature between 2010 and 2020.

Leishmania infantum, a zoonotic vector-born parasite, is endemic in the Mediterranean region, presenting mostly as visceral (VL), but also as cutaneous (CL) and mucosal leishmaniasis (ML). This study aimed to describe the epidemiological and clinical aspects of the CL and ML cases diagnosed in mainland Portugal between 2010 and 2020. Collaboration was requested from every hospital of the Portuguese National Health System. Cases were screened through a search of diagnostic discharge codes or positive laboratory results for Leishmania infection. Simultaneously, a comprehensive literature search was performed. Descriptive statistics and hypothesis testing were performed using IBM® SPSS® Statistics. A total of 43 CL and 7 ML cases were identified, with a predominance of autochthonous cases (86%). In CL, immunosuppressed individuals constituted a significant proportion of patients (48%), and in this group, disseminated CL (22%) and simultaneous VL (54%) were common. In autochthonous cases, lesions, mostly papules/nodules (62%), were frequently observed on the head (48%). The approach to treatment was very heterogeneous. ML cases were all autochthonous, were diagnosed primarily in older immunosuppressed individuals, and were generally treated with liposomal amphotericin B. The findings suggest a need for enhanced surveillance and reporting, clinical awareness, and diagnostic capacity of these forms of leishmaniasis to mitigate underdiagnosis and improve patient outcomes. A holistic One Health approach is advocated to address the multifaceted challenges posed by leishmaniases in Portugal and beyond.

Innovative microcosm experiments for the evaluation of the regeneration rates of nutrients in sediments of a hypersaline lagoon.

The aim of this study was to evaluate the regeneration of nutrients from the sediments to the water column in varying salinities, with an innovative experiment that closely simulates real environment. In vitro experiments were carried out simulating six scenarios with two sediment types (low carbonate and high carbonate). Local water and sediments were added to microcosms where circulation was forced. Results showed nitrogen release from low carbonate sediment in the lagoon, mixed and seawater (1.69, 4.68 and 7.36 µmol m-2 day-1, respectively). Phosphate diffusive fluxes were positive in lagoon water and low carbonate sediment (2.24 µmol m-2 day-1), negative with mixed water (-0.30 µmol m-2 day-1) and seawater (-0.51 µmol m-2 day-1). A phosphate release surge was observed in the low-carbonate sediment with overlying mixed water and seawater that, in the natural environment, may boost primary production.

Chronic hepatitis E virus infection in a pediatric female liver transplant recipient.

We describe a case of chronic hepatitis E virus (HEV) infection in a 13-year-old female liver transplant recipient with recurrent increased aminotransferase levels and acute cellular rejection. This finding demonstrates that chronic HEV infection can occur and should be further investigated in immunocompromised patients in Latin America.

Analysis of factors associated with portal vein thrombosis in pediatric living donor liver transplant recipients.

The technique of vascular reconstruction plays a major role in the outcome of living donor liver transplantation (LDLT). An increased use of vascular grafts (VGs) as replacements for sclerotic portal veins has become a standard technique for our group. The aim of this study was to analyze the factors associated with portal vein thrombosis (PVT) in pediatric LDLT. We performed a retrospective analysis of 486 primary pediatric LDLT procedures performed between October 1995 and May 2013. VGs used for portal reconstruction included living donor inferior mesenteric veins, living donor ovarian veins, recipient internal jugular veins, deceased donor iliac arteries, and deceased donor iliac veins. Thirty-four patients (7.0%) developed PVT. The incidence of PVT dropped from 10.1% to 2%; the overall utilization of VGs increased from 3.5% to 37.1%. In a multivariate analysis, only the use of VGs remained an independent risk factor for the occurrence of PVT (hazard ratio = 7.2, 95% confidence interval = 2.8-18.7, P < 0.001). There was no difference in survival rates between patients with PVT and patients without PVT. No patient with PVT underwent retransplantation. In conclusion, the use of VGs was independently associated with the development of PVT. Over time, there was a reduction in the incidence of early PVT in this cohort, and there was a trend toward a reduction in total PVT. The occurrence of isolated PVT in this study was not associated with decreased patient or graft survival.

Diagnosis and management of biliary complications in pediatric living donor liver transplant recipients.

The incidence of biliary complications (BCs) after living donor liver transplantation (LDLT) can reach 40%. Published data on the pediatric population are limited, and treatment protocols vary. Our aim was to describe the clinical scenario for BCs and treatment approaches after LDLT. Between October 1995 and December 2012, 489 pediatric LDLT procedures were performed. BCs developed in 71 patients (14.5%). Biliary strictures (BSs) developed in 45 (9.2%) patients, and bile leaks (BLs) developed in 33 patients (6.7%). The BL diagnosis was clinical in all cases, and 69.7% of the patients underwent surgery. Nearly half of the BS cases had clinical features or suggestive ultrasound findings. Liver biopsy findings suggested BSs in 51.7%. Percutaneous transhepatic cholangiography was performed in 95.6% of the BS patients. The success rate was 77% [mean number of percutaneous biliary interventions (PBIs) = 3.9 ± 1.98, median drainage time = 8 months]. In conclusion, BL patients can be managed with conservative therapy, even though most of these patients will ultimately be treated with surgery. Diagnosing a BS requires a high degree of clinical suspicion because the available resources for its identification can fail in up to 50% of cases. A higher number of PBIs and the use of a drainage catheter for a longer time may be required to achieve better results with this technique.

Ascites and serum sodium are markers of increased waiting list mortality in children with chronic liver failure.

UNLABELLED: Ascites is the most common complication of cirrhosis and in adults it is associated with 50% mortality at 5 years if patients do not receive a liver transplant. The occurrence of hyponatremia in these patients has been associated with increased mortality on the waiting list. The importance of serum sodium levels and the presence of ascites in the pediatric setting remain to be clarified. A retrospective analysis of pediatric patients with cirrhosis on the transplant list was carried out between October 2000 and February 2012. The primary objective of this study was to evaluate the association of pretransplant variables with mortality within 90 days following the inclusion of patients on the waiting list. In all, 522 patients were included in the study; 345 (66%) patients were under 1 year of age; 208 (40%) of the children presented ascites. A multivariate Cox proportional hazards analysis was conducted and total bilirubin (P < 0.001, hazard ratio [HR] = 2.09, 95% confidence interval [CI] = 1.35-3.21), international normalized ratio (INR) (P < 0.001, HR = 9.83, 95% CI = 4.51-21.45), serum sodium levels (P = 0.03, HR = 0.96, 95% CI = 0.92-0.99), ascites (P = 0.001, HR = 2.59, 95% CI = 1.44-4.64), and categorized age (0-1 versus ≥ 1 year old) (P = 0.025, HR = 2.33, 95% CI = 1.11-4.86) were independently associated with risk of death in 90 days. Malnutrition (Z score height/age, weight/age) and serum albumin (pediatric endstage liver disease [PELD] formula) were not included in the final model. CONCLUSION: The presence of ascites and serum sodium levels are important variables associated with decreased patient survival while candidates wait for a liver graft. Multicenter studies are necessary to validate these findings in order to improve current allocation policies based on the PELD score.

Technical aspects and outcomes of living donor liver transplantation for pediatric patients with situs inversus.

The vascular anomalies encountered in patients with biliary atresia associated with polysplenia syndrome and situs inversus (SI) demand technical refinements when liver transplantation is being performed. The available data describing the technique used in living donor liver transplantation (LDLT) in this population are limited; the short vascular stumps of the donor's liver can impart additional technical difficulties during vascular reconstruction. Here we describe our experience with 9 children with biliary atresia and SI who underwent LDLT. In our series, the retrohepatic vena cava was absent for 7 patients, 7 had a preduodenal portal vein (PV), and 4 had a variant arterial anatomy. The donor's left hepatic vein was anastomosed to the confluence of the recipient's 3 hepatic veins in 7 patients. Vascular grafts were used for PV reconstruction in 3 cases. A left lateral segment graft was used in all but 1 patient who needed a graft reduction. All grafts were placed in the upper left abdomen. There were no vascular complications after transplantation. All patients were alive and well at a median follow-up of 55 months. In conclusion, LDLT can be successfully performed in pediatric patients with SI. Complex vascular anomalies associated with the use of partial liver grafts obtained from living donors are not associated with an increased occurrence of vascular complications.

Liver transplantation after stage II palliation for hypoplastic left heart syndrome.

The association of biliary atresia (BA) with congenital heart diseases has been extensively described, and there are a number of reports on the outcomes of patients in this group who undergo liver transplantation (LT). The intraoperative management and the timing of LT for patients with end-stage liver disease are matters of debate, especially when complex heart diseases are involved. This report describes the outcome after LT for a pediatric recipient with BA and hypoplastic left heart syndrome. The patient underwent Norwood-Sano and Glenn procedures for heart palliation before LT. He was cyanotic, was severely malnourished, and had complications secondary to chronic liver failure. At the time of transplantation, the child was 16 months old and weighed 5175 g. Despite the critical clinical scenario and the long hospitalization period, there were no cardiac, vascular, or biliary complications after LT. At the age of 48 months, the patient was awaiting the final cardiac repair. In conclusion, the presence of complex cardiac malformations may not be a contraindication to LT. An experienced surgical team and a multidisciplinary approach are key to a successful outcome.

Relacionamento professor-aluno e auto-regulação da aprendizagem no 3º ciclo do ensino médio português / Teacher-student relationship and self-regulated learning in portuguese compulsory education / Relación maestro-alumno y el aprendizaje autorregulado en eso portugués

Este artigo teve como objetivo explorar a relação entre a percepção do comportamento do professor e auto-regulação da aprendizagem na Matemática. As respostas de 625 alunos do 3ºciclo do Ensino Médio Português (7º ao 9º anos) aos questionários QIPBásico e IPAAr permitiram perceber diferenças nas variáveis supracitadas em função do sexo, da idade, do ano de escolaridade e do número de retenções, do nível educacional dos pais, do sexo do professor de Matemática e do número de anos com o mesmo professor. As conclusões mais relevantes para a prática pedagógica prendem-se à relação positiva entre a auto-regulação da aprendizagem dos alunos e a percepção de liderança, apoio e compreensão por parte dos professores, e relação negativa entre a auto-regulação e a percepção de insatisfação, insegurança e repreensão dos seus professores.

The aim of this paper was to explore the relationship between the students´ perceptions of teachers' behavior and self-regulated learning in Mathematics. The study, conducted among a group of 625 students of Portuguese Compulsory Education (7th to 9th grades) using the QIPBásic and the IPAAr allowed us to understand differences in the aforementioned variables related with students´ gender, age, grades and number of retentions, parents education, professor of Mathematics gender and number of years with the same teacher. The most relevant findings to teaching practice are relate to the positive relation between self-regulated learning and students' perception of their teachers with regard to leadership, helping/friendly, and understanding, as well as the negative relation between self -regulated learning and students' perception of uncertain, dissatisfied and admonishing of their teachers.

Este artículo explora la relación entre la percepción de la conducta del maestro y el aprendizaje autorregulado en Matemáticas. Las respuestas del grupo de 625 estudiantes de ESO Portugués (7º a 9º grados) a los instrumentos QIPBásico y IPAAr nos permiten percibir diferencias en las variables antes mencionadas en materia de género, edad, años de escolaridad y número de cursos suspensos, estudios de los padres, género del maestro en Matemáticas y el número de años con el mismo maestro. Las implicaciones educativas más relevantes se refieren a la relación positiva entre el aprendizaje autorregulado y la percepción de los estudiantes en lo que respecta al liderazgo, apoyo y comprensión, así como la relación negativa entre la autorregulación del aprendizaje con la percepción de insatisfacción, inseguridad y reprensión de sus profesores.

Relacionamento professor-aluno e auto-regulação da aprendizagem no 3º ciclo do ensino médio português / Teacher-student relationship and self-regulated learning in portuguese compulsory education / Relación maestro-alumno y el aprendizaje autorregulado en eso portugués

Este artigo teve como objetivo explorar a relação entre a percepção do comportamento do professor e auto-regulação da aprendizagem na Matemática. As respostas de 625 alunos do 3ºciclo do Ensino Médio Português (7º ao 9º anos) aos questionários QIPBásico e IPAAr permitiram perceber diferenças nas variáveis supracitadas em função do sexo, da idade, do ano de escolaridade e do número de retenções, do nível educacional dos pais, do sexo do professor de Matemática e do número de anos com o mesmo professor. As conclusões mais relevantes para a prática pedagógica prendem-se à relação positiva entre a auto-regulação da aprendizagem dos alunos e a percepção de liderança, apoio e compreensão por parte dos professores, e relação negativa entre a auto-regulação e a percepção de insatisfação, insegurança e repreensão dos seus professores.(AU)

The aim of this paper was to explore the relationship between the students´ perceptions of teachers' behavior and self-regulated learning in Mathematics. The study, conducted among a group of 625 students of Portuguese Compulsory Education (7th to 9th grades) using the QIPBásic and the IPAAr allowed us to understand differences in the aforementioned variables related with students´ gender, age, grades and number of retentions, parents education, professor of Mathematics gender and number of years with the same teacher. The most relevant findings to teaching practice are relate to the positive relation between self-regulated learning and students' perception of their teachers with regard to leadership, helping/friendly, and understanding, as well as the negative relation between self -regulated learning and students' perception of uncertain, dissatisfied and admonishing of their teachers.(AU)

Este artículo explora la relación entre la percepción de la conducta del maestro y el aprendizaje autorregulado en Matemáticas. Las respuestas del grupo de 625 estudiantes de ESO Portugués (7º a 9º grados) a los instrumentos QIPBásico y IPAAr nos permiten percibir diferencias en las variables antes mencionadas en materia de género, edad, años de escolaridad y número de cursos suspensos, estudios de los padres, género del maestro en Matemáticas y el número de años con el mismo maestro. Las implicaciones educativas más relevantes se refieren a la relación positiva entre el aprendizaje autorregulado y la percepción de los estudiantes en lo que respecta al liderazgo, apoyo y comprensión, así como la relación negativa entre la autorregulación del aprendizaje con la percepción de insatisfacción, inseguridad y reprensión de sus profesores.(AU)

Four hundred thirty consecutive pediatric living donor liver transplants: variables associated with posttransplant patient and graft survival.

The availability of living donors allows transplant teams to indicate living donor liver transplantation (LDLT) early in the course of liver disease before the occurrence of life-threatening complications. Late referral to transplant centers is still a problem and can compromise the success of the procedure. The aim of this study was to examine the perioperative factors associated with patient and graft survival for 430 consecutive pediatric LDLT procedures at Sirio-Libanes Hospital/A. C. Camargo Hospital (São Paulo, Brazil) between October 1995 and April 2011. The studied pretransplant variables included the following: recipient age and body weight, Pediatric End-Stage Liver Disease score, z score for height/age, bilirubin, albumin, international normalized ratio, hemoglobin, sodium, presence of ascites, and previous surgery. The analyzed technical aspects included the graft-to-recipient weight ratio and the use of vascular grafts for portal vein reconstruction. In addition, the occurrence of hepatic artery thrombosis (HAT), portal vein thrombosis (PVT), and biliary complications was also analyzed. The liver grafts included 348 left lateral segments, 5 monosegments, 51 left lobes, and 9 right lobes. In a univariate analysis, an age < 12 months, a low body weight (≤10 kg), malnutrition, hyperbilirubinemia, and HAT were associated with decreased patient and graft survival after LDLT. In a multivariate analysis, a body weight ≤ 10 kg and HAT were significantly associated with decreased patient and graft survival. The use of vascular grafts significantly increased the occurrence of PVT. In conclusion, a low body weight (≤10 kg) and the occurrence of HAT independently determined worse patient and graft survival in this large cohort of pediatric LDLT patients.

Schistosoma mansoni infection in the liver graft: The impact on donor and recipient outcomes after transplantation.

The increasing number of transplants performed each year has led to the identification of unusual diseases in liver grafts from asymptomatic donors that were unrecognized before liver transplantation. Here we report our experience with patients who received liver grafts infected with schistosomiasis. From September 1991 to August 2010, 482 pediatric liver transplants were performed at A. C. Camargo Hospital/Sírio-Libanês Hospital (São Paulo, Brazil). For the identification of Schistosoma mansoni infections, pathology slides for the recipients were reviewed; these included postreperfusion and follow-up liver biopsy samples. We were able to identify 6 cases of schistosomiasis transmitted through infected grafts (5 of these grafts were from living donors). All living donors were confirmed to have normal liver chemistries, negative fecal tests for parasitic diseases, and normal abdominal ultrasound findings. Liver biopsy was not performed before transplantation. In all cases, features of schistosomiasis were absent in the liver explants. The living donors were treated with praziquantel and were taught to avoid risk factors for reinfection. No specific treatment for schistosomiasis was given to the recipients. There were no perioperative deaths, but 2 recipients died after living donor liver transplantation (LDLT) because of Kaposi's sarcoma and non-Hodgkin's lymphoma. In conclusion, using liver grafts infected with S. mansoni eggs did not compromise the results of LDLT in this pediatric cohort. Because of the parasite's life cycle and the therapeutic target of praziquantel, only donors should be treated for the infection. Three years of follow-up showed an uneventful recovery for the living donors.

Portal vein obstruction after liver transplantation in children treated by simultaneous minilaparotomy and transhepatic approaches: initial experience.

Portal vein thrombosis is a complication that occurs anytime after liver transplantation and can compromise the patient and graft survival. We describe a combined technique for PV recanalization in cases of PV obstruction after liver transplantation. Four children (1%), of 367 subjected to liver transplantation from June 1991 to December 2008, underwent PV recanalization through a combined approach (transhepatic and minilaparotomy). All children received left lateral hepatic segments, developed Portal vein thrombosis (n=3) and stenosis (n=1), and presented with symptoms of portal hypertension after transplantation. PV recanalization was tried by transhepatic retrograde access, and a minilaparotomy was performed when percutaneous recanalization was unsuccessful. Three patients underwent a successful portal recanalization and stent placement with the combined technique. In one patient, the recanalization was unsuccessful because of an extensive portomesenteric thrombosis. The other three children had the portal flow reestablished and followed with Doppler US studies. They received oral anticoagulation for three consecutive months after the procedure and the clinical symptoms subsided. In case of PV obstruction, the combined approach is technically feasible with good clinical and hemodynamic results. It' is a minimally invasive procedure and can be tried to avoid or delay surgical treatment or retransplantation.

Modified pediatric end-stage liver disease scoring system and pediatric liver transplantation in Brazil.

The Pediatric End-Stage Liver Disease (PELD) scoring system is a formula developed to provide a continuous numerical assessment of the risk of death in order to allocate livers to children in need of transplantation. The PELD scoring system was introduced in Brazil in July 2006. An important change was made in the system: the final number for listing patients less than 12 years old for transplantation was the calculated PELD score multiplied by 3. The consequences of this allocation policy were analyzed in 2 ways in this research: nationally and in the state of São Paulo (SP State). In the analysis of the national data, a comparison of the pre-PELD era (July 2003 to July 2006) and the post-PELD era (August 2006 to April 2009) showed that the total number of pediatric transplants for children under 12 years of age decreased 7%. Regionally, in SP State, there was a 62% increase in the number of deceased donor liver transplantation procedures for the pediatric population after the introduction of the modified PELD system. There was also a 6.1-fold increase in split liver transplantation as well as a statistically significant decrease in the time on the waiting list (P < 0.001). In conclusion, changing the allocation policy in Brazil in order to benefit pediatric patients on the waiting list had different results according to analyses of national and regional data. A significant increase in deceased donor liver transplantation/split liver transplantation and a shorter time on the waiting list were observed in SP State. The modified PELD scoring system is simple and optimizes the utilization of deceased donor liver grafts in centers performing pediatric transplants.

Long-lasting control with erlotinib in advanced non-small cell lung cancer (NSCLC). / Erlotinib no controlo tumoral prolongado do carcinoma do pulmão de não pequenas células avançado (CPNPC).

The authors present a clinical case of a caucasian male patient, 59 years-old, non-smoker, with an advanced non-small cell lung carcinoma (NSCLC), with 3 years of follow-up, received erlotinib for 18 months, after failure of more than one chemotherapy schedule, without evidence of oncologic progression. The patient evidences excellent quality of life, controlled sintomatology, recovery of the capacity of tolerance to the effort and it maintains his professional activities. The treatment with erlotinib has been well tolerated, although exhibiting grade 1 cutaneous toxicity. Rev Port Pneumol 2008; XIV (Supl 3): S9-S15.

Living donor liver transplantation for children in Brazil weighing less than 10 kilograms.

Infants with end-stage liver disease represent a treatment challenge. Living donor liver transplantation (LDLT) is the only option for timely liver transplantation in many areas of the world, adding to the technical difficulties of the procedure. Factors that affect morbidity and mortality can now be determined, which opens a new era for improvement. We have accumulated an 11-year experience with LDLT for children weighing<10 kg. From October 1995 to October 2006, a total of 222 LDLT in patients<18 years of age were performed; 129 primary LDLT and 7 retransplants (4 LDLT and 3 deceased donor grafts) were performed in 129 infants weighing<10 kg. Forty-seven patients received grafts with graft-to-recipient weight ratio (GRWR) of >4%. Two patients received monosegmental grafts, and 2 patients underwent delayed abdominal wall closure. Portal vein thrombosis occurred in 5.4% of the patients, hepatic artery thrombosis in 3.1%, and both in 1.5%. Among several variables studied, only the bilirubin level at the time of transplantation was associated with increased risk of death (P=0.009). Grafts with GRWR>4% had no negative effect on patient survival. There were 7 retransplants, and 4 patients received a second parental LDLT. Patient survival rates at 1, 3, and 10 years after transplantation were 88.8%, 84.7%, and 82% for all children, and 87.5%, 84.9%, and 84.9% for infants weighing<10 kg. LDLT has results comparable to other modalities of liver transplantation in infants. Monosegment grafts were rarely required in this series, although they may be necessary in patients with lower body weight.