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1.
Pancreatology ; 22(7): 1028-1034, 2022 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-35948507

RESUMO

BACKGROUND AND AIMS: Pancreatic resection is associated with pancreatic exocrine insufficiency (PEI) leading to nutritional consequences. The Pancreatic Nutrition Clinic was established to diagnose and manage PEI through standardised nutritional assessment. In this prospective observational study, we aimed to define the rate of PEI, diabetes mellitus and nutritional abnormalities in patients who underwent pancreatic resection. METHODS: All Pancreatic Nutrition Clinic patients were included for analysis. Clinical data were prospectively obtained at initial assessment. Biochemical data included micronutrient levels, faecal elastase-1 and haemoglobin A1c. Bone mineral density and nutritional assessment were undertaken. RESULTS: Ninety-eight patients were included. Fifty-nine per cent (58/98) had undergone a pancreatoduodenectomy. Ninety-three patients had a faecal elastase-1 result, 65% (60/93) of which had a faecal elastase-1 less than 200 µg/g of faeces. Seventy-five patients (76%) of the total population required PERT, and thirty-nine (40%) were classified as malnourished using the patient-generated subjective global assessment tool. Seventy-two per cent (70/97) had a biochemical deficiency of one or more micronutrients. Thirty-eight people (39%) had diabetes mellitus. Of the seventy-eight patients with a bone mineral density scan available for analysis, 29% (23/78) had osteoporosis and 49% (38/78) osteopenia. CONCLUSIONS: Pancreatic exocrine insufficiency, micronutrient deficiency, bone disease, diabetes mellitus and malnutrition are highly prevalent in patients who have undergone pancreatic resection.


Assuntos
Diabetes Mellitus , Insuficiência Pancreática Exócrina , Desnutrição , Doenças Metabólicas , Humanos , Insuficiência Pancreática Exócrina/diagnóstico , Elastase Pancreática/análise , Micronutrientes
2.
Subst Abus ; 42(3): 284-293, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-31657675

RESUMO

INTRODUCTION: Rising overdose deaths are a devastating problem across the United States and Canada. Despite the availability of observed consumption sites in British Columbia (BC), data suggest people who use drugs (PWUD) alone in private residences are most at risk of overdose death. These individuals may not use consumption sites due to fear of stigmatization, lack of privacy, or personal preference. It is this population that would benefit from overdose monitoring alternatives. METHODS: This 2018 study employed two sources of data. (1) A provincial harm reduction distribution site client survey administered at 27 sites across BC asked about cell phone possession and functionality. (2) Structured interviews with PWUD in Vancouver gathered perspectives regarding monitoring devices and alerting systems. Results: The client survey was administered to 486 individuals. Among 443 respondents, 48% (n = 212) owned a cellphone and 68% (n = 115) of individuals with a cellphone with access to internet (n = 168) would use an application to mitigate opioid-related overdose deaths. Thirty qualitative interviews were performed; thematic analysis of the responses identified three major themes - safety, public versus private drug use, and technological monitoring and function. The relevance of technological devices was affected by the inconsistent availability of cellphones, concerns about anonymity, as well as personal comfort while using drugs. Conclusions: Technological applications may not be suitable for clients with transient lifestyles, no permanent home, and lack of consistent access to a mobile device. Thorough needs assessments, considerations of lifestyle limitations, and understanding perceptions around cellphone and overdose monitoring can increase the potential that devices will be used.Highlights45% (n = 219) of client survey respondents owned a cellphoneOut of cellphone owners, 59% (n = 102) of client survey respondents had access to internetOf 168 with internet access, 68% willing to use any applications for overdose responseApplications not suitable for transient lifestyles, no permanent home, and lack of consistent access to cellphone.


Assuntos
Overdose de Drogas , Transtornos Relacionados ao Uso de Opioides , Colúmbia Britânica/epidemiologia , Overdose de Drogas/tratamento farmacológico , Redução do Dano , Humanos , Transtornos Relacionados ao Uso de Opioides/epidemiologia , Inquéritos e Questionários
3.
Int J Pediatr Adolesc Med ; 7(4): 166-173, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33319014

RESUMO

INTRODUCTION: Increasing demands from public and private healthcare coupled with national initiatives in patient-oriented research has led to an increase in avenues to allow patients to be directly involved in research. In particular, the push towards participation of children and youth has resulted in the formation of pediatric patient advisory groups with broad partnerships and consultation requests across the globe. However, there is a lack of evidence to examine the challenges in formation and training of young persons' advisory groups (YPAGs) and management processes required thereafter. PURPOSE AND OBJECTIVES: This study's purpose is to document YPAG formation and training protocols around the world, highlight common strengths, and evaluate pitfalls and challenges. The results from this study will subsequently inform the development of standardized training protocols for children and youth to be piloted globally. METHODS: In this study, 17 select YPAG team leaders from 7 countries were surveyed to determine current training techniques used within existing groups. 17 youth representatives and 16 team leaders were then interviewed to gather further qualitative data on facilitators and barriers that aid or prevent successful initiation and maintenance of these groups. Qualitative interview data was coded and analyzed using NVivo by two independent reviewers (SYC, VWLT). Any inconsistencies in thematic analysis was confirmed by a third reviewer (JB). RESULTS: The most common training topics include consent and assent (64.71%), clinical trials (64.71%), and patient safety (70.59%). There are significant discrepancies to the amount of training received by each team. Most YPAGs out of the 17 groups receive no formal training (58.82%) while training sessions in the remaining 7 groups vary in both duration and frequency. Collectively, meetings ranged from 15  minutes to 6  hours long, with the majority of team meetings being 2-3  hours long (58.82%). The most common training facilitators are a positive relationship with a local hospital (82.35%) and access to a dedicated team coordinator (64.71%). 70.59% of team leaders identified a lack of access to appropriate educational materials available as a drawback to the impact of their YPAG, making this the greatest common barrier. CONCLUSION: Bringing children and youth to the forefront of paediatric trials and clinical research facilitates appropriate patient representation in subsequent research decision-making. There is an urgency to create and implement standardized protocols for the training of children and youth, especially in preparation for national and international research consultations. This low barrier framework may be of special interest to lower-middle-income countries who wish to encourage community participation in healthcare.

4.
Ir Med J ; 111(7): 789, 2018 08 13.
Artigo em Inglês | MEDLINE | ID: mdl-30520616

RESUMO

Introduction Quick, painless, cheap and reliable, the sweat test remains the gold standard diagnostic test for cystic fibrosis. We aimed to describe the pattern of testing in Ireland over a calendar year. Methods Information on sweat test practices was requested from each centre between 1st January 2011 and 31st December 2011, and the number of positive, negative, equivocal, and insufficient samples was recorded. Results In 2011 there were 2555 sweat tests performed in 15 centres, ranging from 35 to over 450 tests per centre. 35 (1.4%) were in the diagnostic range. The overall quantity not sufficient (QNS) rate was 10.3% (range 0-28.3%). Testing was performed across a wide age range (2.5 weeks to 75 years). The mean sweat chloride value was 16.5 mmol/L (SD 16.1 mmol/L). Discussion Our study demonstrates a high number of sweat tests performed in Ireland with significant variation in sweat testing practices across 15 different sites.

5.
Eur Heart J Cardiovasc Imaging ; 19(7): 808-815, 2018 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-28950308

RESUMO

Aims: The Cone reconstruction in Ebstein's anomaly (EA) aims to reduce tricuspid valve regurgitation (TR) and reposition the valve to the anatomic annulus, but post-operative progress of ventricular function is poorly understood. This study evaluated biventricular function after Cone reconstruction using echocardiographic techniques. Methods and results: A retrospective study assessing longitudinal change was conducted from 2009 to 2014. All symptomatic patients with EA and severe TR undergoing surgery were included. Transthoracic advanced echocardiography was performed pre- and post-operatively (at short-term (<30 days) and mid-term). Conventional and longitudinal 2D strain parameters were measured for left ventricle (LV) and right ventricle (RV). Paired analyses were compared using Wilcoxon Matched-pairs signed rank test. From the 38 patients operated for EA, the echocardiographic data of 17 patients, aged 15 (1-57 years) at operation could be analysed. Median follow up was 6 months (8 days-54 months). The tricuspid annular plane systolic excursion (26.42 ± 5.79 mm vs. 8.75 ± 3.18 mm, P < 0.001), RV fractional area change (FAC) (45.00 ± 8.13% vs. 35.46 ± 5.76%, P = 0.038) and LV 2D peak systolic strain were significantly reduced post-operatively (-20.49 ± 2.79 vs. -17.73 ± 2.76, P = 0.041), with a trend to later recovery for LV 2D strain. There was no evidence of systolic mechanical dys-synchrony before or after operation. Conclusion: Although clinical outcome of Cone reconstruction for EA remains excellent, acute post-operative changes leads to reduction of myocardial function of both ventricles, with a trend to later recovery for LV. Continuing impairment of RV function is multifactorial but may reflect intrinsic myocardial deficiency.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Anomalia de Ebstein/diagnóstico por imagem , Interpretação de Imagem Assistida por Computador , Insuficiência da Valva Tricúspide/prevenção & controle , Disfunção Ventricular Direita/etiologia , Adolescente , Adulto , Procedimentos Cirúrgicos Cardíacos/métodos , Criança , Pré-Escolar , Estudos de Coortes , Anomalia de Ebstein/cirurgia , Ecocardiografia/métodos , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Variações Dependentes do Observador , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/fisiopatologia , Prognóstico , Valores de Referência , Estudos Retrospectivos , Medição de Risco , Resultado do Tratamento , Insuficiência da Valva Tricúspide/diagnóstico por imagem , Disfunção Ventricular Direita/diagnóstico por imagem , Adulto Jovem
6.
Open Biol ; 7(6)2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28615472

RESUMO

Internal clocks driving rhythms of about a day (circadian) are ubiquitous in animals, allowing them to anticipate environmental changes. Genetic or environmental disturbances to circadian clocks or the rhythms they produce are commonly associated with illness, compromised performance or reduced survival. Nevertheless, some animals including Arctic mammals, open sea fish and social insects such as honeybees are active around-the-clock with no apparent ill effects. The mechanisms allowing this remarkable natural plasticity are unknown. We generated and validated a new and specific antibody against the clock protein PERIOD of the honeybee Apis mellifera (amPER) and used it to characterize the circadian network in the honeybee brain. We found many similarities to Drosophila melanogaster and other insects, suggesting common anatomical organization principles in the insect clock that have not been appreciated before. Time course analyses revealed strong daily oscillations in amPER levels in foragers, which show circadian rhythms, and also in nurses that do not, although the latter have attenuated oscillations in brain mRNA clock gene levels. The oscillations in nurses show that activity can be uncoupled from the circadian network and support the hypothesis that a ticking circadian clock is essential even in around-the-clock active animals in a constant physical environment.


Assuntos
Abelhas/fisiologia , Relógios Circadianos/fisiologia , Animais , Western Blotting , Encéfalo/metabolismo , Química Encefálica/fisiologia , Imuno-Histoquímica , Neurônios/metabolismo
7.
Pediatr Transplant ; 20(6): 859-65, 2016 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-27384867

RESUMO

With the imbalance between donation rates and potential recipients growing, transplant programs are increasingly using non-ideal organs from so-called marginal donors. This is the first reported case of the intentional use of a donor heart with ALCAPA. The recipient was aged one yr with restrictive cardiomyopathy who had been supported with BiVAD for over six months. Function of the donor left ventricle was shown to be well preserved, with no obvious signs of ischemia, except for a fibrotic layer on the anterolateral papillary muscle of the mitral valve. To prevent coronary steal, the anomalous left coronary artery ostium from the MPA was oversewn prior to implantation. The transplanted heart spontaneously regained sinus rhythm immediately following cross-clamp release and showed good contractility from the first postoperative echocardiogram. The patient continues to do well 18 months post-transplant, with excellent function on echocardiography, and good flow on coronary angiography.


Assuntos
Síndrome de Bland-White-Garland , Cardiomiopatia Restritiva/cirurgia , Transplante de Coração/métodos , Doadores de Tecidos , Humanos , Lactente , Masculino , Transplante Homólogo/métodos
8.
World J Surg ; 40(7): 1618-24, 2016 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-27138882

RESUMO

BACKGROUND: The BRAF (V600E) mutation is a recognised molecular marker in papillary thyroid cancer (PTC), reported incidence from 30 to 80 %. BRAF(V600E) aberrantly activates the MAPK pathway, a central regulator of cell growth and proliferation. Previous studies have reported conflicting data regarding the impact of BRAF(V600E) on clinicopathological features of PTC. The study aims to determine whether BRAF(V600E) is useful as a prognostic biomarker in PTC. METHODS: A cohort study of patients undergoing surgery for PTC was undertaken. The primary outcome measure was disease-free survival. Secondary outcome measures were tumour size, nodal positivity and radioactive iodine ablation rate. All cases were re-examined to confirm PTC. Immunohistochemistry for BRAF(V600E) was performed on tissue microarrays. A single endocrine pathologist, blinded to clinicopathological data, interpreted staining. RESULTS: 496 patients with PTC were included, and 309 (62 %) were BRAF(V600E) positive. Tumour size was similar for BRAF(V600E)-positive and -negative tumours (21.3 vs. 23.2 mm, p = 0.23). BRAF(V600E)-positive patients were significantly older at first operation (mean age 45 versus 49 years, p = 0.003). BRAF(V600E)-positive PTCs had a higher rate of disease recurrence (12.9 vs. 5.6 %, p = 0.004), lymph node metastasis (44 vs. 29.4 %, p = 0.004) and extra-thyroidal extension (44 vs. 22 %, p < 0.001). Five-year disease-free survival was 89.6 % for BRAF(V600E) positive and 96.3 % for negative tumours, p < 0.001. There was no difference between groups for vascular invasion or multifocality. The mean follow-up was 57 months for both groups. CONCLUSION: BRAF(V600E) in PTC predicts an increased risk of lymph node metastasis, extra-thyroidal extension and reduced disease-free survival. It is an additional useful prognostic biomarker.


Assuntos
Carcinoma/genética , Carcinoma/secundário , Recidiva Local de Neoplasia/genética , Proteínas Proto-Oncogênicas B-raf/genética , Neoplasias da Glândula Tireoide/genética , Neoplasias da Glândula Tireoide/patologia , Técnicas de Ablação , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Carcinoma/cirurgia , Carcinoma Papilar , Criança , Estudos de Coortes , Intervalo Livre de Doença , Feminino , Humanos , Radioisótopos do Iodo/uso terapêutico , Metástase Linfática , Masculino , Pessoa de Meia-Idade , Mutação , Proteínas Proto-Oncogênicas B-raf/análise , Câncer Papilífero da Tireoide , Neoplasias da Glândula Tireoide/cirurgia , Carga Tumoral/genética , Adulto Jovem
10.
Endocr Relat Cancer ; 22(3): 387-97, 2015 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-25972245

RESUMO

Mitochondrial dysfunction, due to mutations of the gene encoding succinate dehydrogenase (SDH), has been implicated in the development of adrenal phaeochromocytomas, sympathetic and parasympathetic paragangliomas, renal cell carcinomas, gastrointestinal stromal tumours and more recently pituitary tumours. Underlying mechanisms behind germline SDH subunit B (SDHB) mutations and their associated risk of disease are not clear. To investigate genotype-phenotype correlation of SDH subunit B (SDHB) variants, a homology model for human SDH was developed from a crystallographic structure. SDHB mutations were mapped, and biochemical effects of these mutations were predicted in silico. Results of structural modelling indicated that many mutations within SDHB are predicted to cause either failure of functional SDHB expression (p.Arg27*, p.Arg90*, c.88delC and c.311delAinsGG), or disruption of the electron path (p.Cys101Tyr, p.Pro197Arg and p.Arg242His). GFP-tagged WT SDHB and mutant SDHB constructs were transfected (HEK293) to determine biological outcomes of these mutants in vitro. According to in silico predictions, specific SDHB mutations resulted in impaired mitochondrial localisation and/or SDH enzymatic activity. These results indicated strong genotype-functional correlation for SDHB variants. This study reveals new insights into the effects of SDHB mutations and the power of structural modelling in predicting biological consequences. We predict that our functional assessment of SDHB mutations will serve to better define specific consequences for SDH activity as well as to provide a much needed assay to distinguish pathogenic mutations from benign variants.


Assuntos
Neoplasias das Glândulas Suprarrenais/enzimologia , Paraganglioma/enzimologia , Feocromocitoma/enzimologia , Succinato Desidrogenase/química , Succinato Desidrogenase/metabolismo , Neoplasias das Glândulas Suprarrenais/genética , Técnicas de Cultura de Células , Predisposição Genética para Doença , Células HEK293 , Humanos , Mitocôndrias/enzimologia , Mitocôndrias/patologia , Modelos Moleculares , Mutação , Paraganglioma/genética , Feocromocitoma/genética , Estrutura Secundária de Proteína , Succinato Desidrogenase/genética , Transfecção
12.
Perfusion ; 29(6): 567-70, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-24947458

RESUMO

Major aortopulmonary collateral arteries (MAPCAs) provide significant issues during cardiopulmonary bypass, including flooding of the surgical field which requires significant blood volumes to be returned to the extracorporeal circuit via handheld suckers. This has been shown to be the major source of gaseous microemboli and is associated with adverse neurological outcome. Use of pH-stat has been previously shown to decrease the shunt through MAPCAs via an unknown mechanism. Here, we report the associated benefits of pH-stat in decreasing sucker usage and gaseous microemboli in a patient with known MAPCAs presenting for repair of tetralogy of Fallot and pulmonary atresia.


Assuntos
Ponte Cardiopulmonar , Atresia Pulmonar , Tetralogia de Fallot , Feminino , Humanos , Lactente , Atresia Pulmonar/complicações , Atresia Pulmonar/patologia , Atresia Pulmonar/cirurgia , Tetralogia de Fallot/complicações , Tetralogia de Fallot/patologia , Tetralogia de Fallot/cirurgia
13.
Endocr Relat Cancer ; 21(3): 415-26, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24623741

RESUMO

miR-210 is a key regulator of response to hypoxia. Pheochromocytomas (PCs) and paragangliomas (PGLs) with germline SDHx or VHL mutations have pseudohypoxic gene expression signatures. We hypothesised that PC/PGLs containing SDHx or VHL mutations, and succinate dehydrogenase (SDH)-deficient gastrointestinal stromal tumours (GISTs), would overexpress miR-210 relative to non-SDH or -VHL-mutated counterparts. miR-210 was analysed by quantitative PCR in i) 39 PC/PGLs, according to genotype (one SDHA, five SDHB, seven VHL, three NF1, seven RET, 15 sporadic, one unknown) and pathology (18 benign, eight atypical, 11 malignant, two unknown); ii) 18 GISTs, according to SDHB immunoreactivity (nine SDH-deficient and nine SDH-proficient) and iii) two novel SDHB-mutant neurosphere cell lines. miR-210 was higher in SDHx- or VHL-mutated PC/PGLs (7.6-fold) compared with tumours without SDHx or VHL mutations (P=0.0016). miR-210 was higher in malignant than in unequivocally benign PC/PGLs (P=0.05), but significance was lost when benign and atypical tumours were combined (P=0.08). In multivariate analysis, elevated miR-210 was significantly associated with SDHx or VHL mutation, but not with malignancy. In GISTs, miR-210 was higher in SDH-deficient (median 2.58) compared with SDH-proficient tumours (median 0.60; P=0.0078). miR-210 was higher in patient-derived neurosphere cell lines containing SDHB mutations (6.5-fold increase) compared with normal controls, in normoxic conditions (P<0.01). Furthermore, siRNA-knockdown of SDHB in HEK293 cells increased miR-210 by 2.7-fold (P=0.001) under normoxia. Overall, our results suggest that SDH deficiency in PC, PGL and GISTs induces miR-210 expression and substantiates the role of aberrant hypoxic-type cellular responses in the development of these tumours.


Assuntos
Neoplasias das Glândulas Suprarrenais/genética , Tumores do Estroma Gastrointestinal/genética , MicroRNAs/genética , Mutação/genética , Paraganglioma/genética , Feocromocitoma/genética , Succinato Desidrogenase/genética , Neoplasias das Glândulas Suprarrenais/patologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/genética , Criança , Feminino , Seguimentos , Tumores do Estroma Gastrointestinal/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Gradação de Tumores , Paraganglioma/patologia , Feocromocitoma/patologia , Prognóstico , RNA Mensageiro/genética , Reação em Cadeia da Polimerase em Tempo Real , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Proteína Supressora de Tumor Von Hippel-Lindau/genética , Adulto Jovem
14.
Perfusion ; 29(3): 194-8, 2014 May.
Artigo em Inglês | MEDLINE | ID: mdl-24447948

RESUMO

INTRODUCTION: Great Ormond Street Children's Hospital undertakes over 500 open heart cardiothoracic procedures requiring cardiopulmonary bypass per year. Data from our centre show that many of our neonatal/paediatric patients require higher cardiac indexes than previously thought. We evaluated the new Pixie oxygenation system, rated from 0.1 L/min to 2 L/min, to determine if it could be used for these patients. METHODS: Between 2010 and 2012, 250 Pixie oxygenators were used on consecutive patients requiring correction of congenital cardiac defects. Data were collected on FiO2 requirements, oxygenator pressure drop and gaseous microemboli handling. Retrospective analysis was also undertaken on the procedures and demographics of all patients during 2011-2012 to determine the percentage of patients on whom the Pixie could be used. RESULTS: Analysis of the procedures undertaken at Great Ormond Street Hospital (GOSH) showed that 89% were in patients under 20 kg, requiring a flow rate of <2 L/min (at a base cardiac index of 2.8 L/min/m2). The maximum FiO2 required at 2.5 L/min was 85%. Gaseous microemboli were reduced by 82.5±9.9% and bubble volume was decreased by 94.3±8.4% from the 'venous' pre-oxygenator to the 'arterial' post-oxygenator. DISCUSSION: The Pixie oxygenator proved effective at flows up to 2.5 L/min, with air-handling capabilities comparable with other oxygenators. This represents a single oxygenator that could potentially be used to cover 89% of our surgical procedures. However, we believe that, for the smallest patients (i.e., < 2 kg), a smaller priming oxygenator should be used in order to limit unnecessary haemodilution in this vulnerable group.


Assuntos
Ponte Cardiopulmonar/instrumentação , Oxigenação por Membrana Extracorpórea/instrumentação , Cardiopatias Congênitas/cirurgia , Ponte Cardiopulmonar/métodos , Pré-Escolar , Oxigenação por Membrana Extracorpórea/métodos , Feminino , Humanos , Lactente , Masculino , Estudos Prospectivos
15.
Perfusion ; 27(6): 480-5, 2012 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-22773392

RESUMO

OBJECTIVES: A decade ago, the first series of ABO-incompatible heart transplants was published, with surprising and extremely promising results; drastically reduced waiting list mortalities of infants listed for heart transplantation. Essential to the procedure was the process of plasma exchange transfusion, required to reduce isohaemagglutinin titres and facilitate the crossing of ABO blood group boundaries. Since then, Great Ormond Street Hospital, London has offered ABO-incompatible heart transplants to infants who potentially would die waiting for a suitable organ. We report the results of a decade of evolving plasma exchange experience and its impact upon patient selection. METHODS: A retrospective analysis was undertaken of all elective ABO-incompatible heart transplants at Great Ormond Street Children's Hospital from January 2001 until January 2011. Data were sought on underlying conditions and demographics of the patients, the isohaemagglutinin titre before and after plasma exchange and survival figures to date. RESULTS: Twenty-one patients underwent ABO-incompatible heart transplantation, ranging from 3 to 44 months, with preoperative isohaemagglutinin titres ranging from 0 to 1:32. All patients underwent a "3 times" plasma exchange before transplantation, requiring exchange volumes of up to 3209 mL. Postoperative isohaemagglutinin titres ranged from 0 to 1:16. One patient died of causes unrelated to organ rejection. CONCLUSIONS: Our data showed that eight patients (38.1%) were older than the previously suggested 12-month cut-off age. Using a combination of adult reservoir/paediatric oxygenator and extracorporeal circuit, ABO-incompatible plasma exchange transfusions can be undertaken safely using a simplified '3 times' method, reducing the circulating levels of isohaemagglutinins whilst providing minimal circuit size. This allows ABO-incompatible heart transplantation in a broader patient population than previously reported.


Assuntos
Sistema ABO de Grupos Sanguíneos/imunologia , Incompatibilidade de Grupos Sanguíneos/imunologia , Transplante de Coração/imunologia , Troca Plasmática/métodos , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Transplante de Coração/efeitos adversos , Transplante de Coração/métodos , Histocompatibilidade , Humanos , Masculino , Troca Plasmática/efeitos adversos , Estudos Retrospectivos , Taxa de Sobrevida , Adulto Jovem
16.
Neurology ; 75(7): 654-8, 2010 Aug 17.
Artigo em Inglês | MEDLINE | ID: mdl-20713953

RESUMO

A single enhancing lesion in the brain parenchyma, also called an inflammatory granuloma, is a frequent neurologic diagnosis. One of the commonest causes of this lesion is human neurocysticercosis, the infection by the larvae of the pork tapeworm, Taenia solium. Following the demonstration that viable cysticercosis cysts survive in good conditions for several years in the human brain, single cysticercal granulomas have been consistently interpreted as representing late degeneration of a long-established parasite. On the basis of epidemiologic, clinical, and laboratory evidence detailed in this article, we hypothesize that in most cases these inflammatory lesions correspond to parasites that die in the early steps of infection, likely as the natural result of the host immunity overcoming mild infections.


Assuntos
Granuloma/etiologia , Neurocisticercose/complicações , Animais , Encéfalo/microbiologia , Encéfalo/patologia , Granuloma/epidemiologia , Granuloma/imunologia , Humanos , Imageamento por Ressonância Magnética/métodos , Neurocisticercose/epidemiologia , Neurocisticercose/imunologia , Taenia solium/patogenicidade , Tomografia Computadorizada por Raios X/métodos
17.
Lancet ; 374(9687): 387-92, 2009 Aug 01.
Artigo em Inglês | MEDLINE | ID: mdl-19604574

RESUMO

BACKGROUND: Cardiac transplantation is a life-saving procedure in infants and children with advanced cardiomyopathy. However, it is greatly limited by shortage of paediatric donors and the complications of long-term immunosuppression, including post-transplant lymphoproliferative disorder (PTLD). We report the management of an infant who had heterotopic cardiac transplantation for advanced cardiomyopathy with secondary pulmonary hypertension who developed seemingly incurable PTLD. METHODS: An 8-month-old girl presented in 1994 with signs of severe heart failure, secondary to dilated cardiomyopathy. At age 11 months, the patient underwent a heterotopic cardiac transplantation. FINDINGS: The patient developed many episodes of PTLD associated with Epstein-Barr virus infection that were resistant to several therapies, including reduction of immunosuppression. Native heart recovery enabled removal of the donor heart 10.5 years after the original operation to allow complete cessation of immunosuppression. Her postoperative course was uncomplicated and the outcome was excellent. 3.5 years after surgery, the patient remains well, in complete remission from her PTLD, and has normal cardiac function. INTERPRETATION: This case shows several issues relating to the use of heterotopic cardiac transplantation in infants and the capacity of the heart to recover. It also provides new insights into the interaction between the immune system with several aspects of modern management of post-transplantation PTLD. FUNDING: None.


Assuntos
Cardiomiopatia Dilatada/cirurgia , Infecções por Vírus Epstein-Barr/complicações , Transplante de Coração/métodos , Imunossupressores/efeitos adversos , Transtornos Linfoproliferativos/virologia , Farmacorresistência Viral , Infecções por Vírus Epstein-Barr/imunologia , Feminino , Transplante de Coração/efeitos adversos , Transplante de Coração/imunologia , Humanos , Terapia de Imunossupressão/efeitos adversos , Lactente , Transtornos Linfoproliferativos/imunologia , Imageamento por Ressonância Magnética , Tomografia Computadorizada por Raios X , Transplante Heterotópico , Carga Viral
18.
Thorac Cardiovasc Surg ; 57(5): 257-69, 2009 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-19629887

RESUMO

OBJECTIVE: The valved bovine conduit "Contegra" for RVOT reconstruction became available for clinical use within a 100 % source data monitored and echo core lab controlled prospective European Multicentre Study, carried out from 1999 to 2006. We present the results of this study. METHODS: A total of 165 Contegras were implanted in 8 centres. The mean patient age was 3.9 years (2 days - 18 years, median 2.0). Total follow-up was 687 patient years. Diagnoses included: tetralogy of Fallot (64 patients, 39 %), truncus arteriosus (50, 30 %), double outlet right ventricle (16, 10 %), aortic valve disease/Ross procedure (11, 7 %), pulmonary valve atresia (10, 6 %), transposition of the great arteries (10, 6 %), 4 other malformations (2 %). Previous procedures were: 82 patients (50 %) - none; 37 (22 %) - valved conduit implantation; 14 (8 %) aortopulmonary shunt; 6 (4 %) catheter intervention. Follow-up appointments which included standardised echocardiography investigations were scheduled at 1, 3, 6, and 12 months, then annually. We evaluated freedom from death, explantation, intervention, stenosis, insufficiency, and degeneration. Results were stratified by age, diagnosis group and conduit size. RESULTS: The 5-year freedom-from rates were: explantation - 90 % (for patients aged 1 to 10 years) and 68 % (for younger patients); endocarditis - over 92 %; catheter intervention - 74 % (patients with congenital malformations); stenosis - 75 % and more (any group); insufficiency - 50 % (12 and 14 mm diameter conduits); any event - 13 % (patients under 1 year), 58 % (1 to 10 years), 82 % (> 10 years). Trace or mild insufficiency was a frequent, but not progressive finding. Mild calcification was detected in only 8 examinations. CONCLUSIONS: The performance of the Contegra conduit compares well with that of homografts when used to reconstruct paediatric right ventricular outflow tracts.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Cardiopatias Congênitas/cirurgia , Veias Jugulares/transplante , Obstrução do Fluxo Ventricular Externo/cirurgia , Adolescente , Animais , Calcinose/etiologia , Calcinose/terapia , Cateterismo Cardíaco , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Procedimentos Cirúrgicos Cardíacos/mortalidade , Bovinos , Criança , Pré-Escolar , Endocardite/etiologia , Endocardite/terapia , Europa (Continente) , Feminino , Oclusão de Enxerto Vascular/etiologia , Oclusão de Enxerto Vascular/terapia , Cardiopatias Congênitas/mortalidade , Humanos , Lactente , Recém-Nascido , Veias Jugulares/diagnóstico por imagem , Estimativa de Kaplan-Meier , Masculino , Modelos de Riscos Proporcionais , Estudos Prospectivos , Reoperação , Medição de Risco , Fatores de Risco , Fatores de Tempo , Transplante Heterólogo , Resultado do Tratamento , Ultrassonografia , Obstrução do Fluxo Ventricular Externo/diagnóstico por imagem
19.
Am J Trop Med Hyg ; 74(5): 850-5, 2006 May.
Artigo em Inglês | MEDLINE | ID: mdl-16687692

RESUMO

A combined (human and porcine) mass chemotherapy program was tested in a controlled design in 12 village hamlets in the Peruvian highlands. A single dose of 5 mg of praziquantel was given to eliminate intestinal taeniasis in humans, and two rounds of oxfendazole (30 mg/kg) were administered to all pigs. The total population in the study villages was 5,658 resident individuals, and the porcine population at the beginning of the study was 716 pigs. Human treatment coverage was 75%, ranging from 69% to 80%. There were only a few refusals of owners for porcine treatment of their animals. The effect of the intervention was measured by comparing incidence rates (seroconversion in pigs who were seronegative 4 months before) in treatment versus control villages, before and up to 18 months after treatment. There was a clear effect in decreasing prevalence (odds ratio, 0.51; P < 0.001) and incidence (odds ratio, 0.39; P < 0.013) in the treatment area after the intervention, which did not leave to extinction of the parasite but stabilized in slightly decreased rates persisting along the follow-up period. Mass chemotherapy was effective in decreasing infection pressure in this hyperendemic area. However, the magnitude of the effect was small and did not attain the goal of eliminating transmission.


Assuntos
Anticestoides/administração & dosagem , Benzimidazóis/administração & dosagem , Praziquantel/administração & dosagem , Teníase/epidemiologia , Teníase/prevenção & controle , Adolescente , Adulto , Animais , Criança , Feminino , Humanos , Incidência , Masculino , Peru/epidemiologia , Estudos Soroepidemiológicos , Suínos , Doenças dos Suínos/sangue , Doenças dos Suínos/tratamento farmacológico , Doenças dos Suínos/epidemiologia , Doenças dos Suínos/prevenção & controle , Taenia solium , Teníase/sangue , Teníase/tratamento farmacológico
20.
Neurology ; 65(2): 229-33, 2005 Jul 26.
Artigo em Inglês | MEDLINE | ID: mdl-16043791

RESUMO

BACKGROUND: Neurocysticercosis (NCC) is the commonest helminthic CNS infection and the main cause of adult-onset seizures in developing countries, also frequent in industrialized countries because of immigration from endemic zones. Although NCC is commonly seen in individuals with seizures in endemic areas, its role as a cause of epilepsy has been questioned on the basis of the poor methodology of published studies. OBJECTIVE: To determine, in a cysticercosis-endemic area of the northern Peruvian coast, the frequency of 1) epileptic seizures, 2) serum antibodies to Taenia solium, 3) NCC-compatible findings on brain CT, and 4) the associations between these variables. METHODS: A community-wide screening survey for possible seizure cases was performed using a validated questionnaire. Positive respondents were later examined in the field by neurologists. Seizure cases were categorized as single seizure, active epilepsy, or inactive epilepsy. Serology was performed for all consenting individuals using immunoblot. Noncontrast brain CT scans were performed in all individuals with seizures and two groups of control subjects without seizures (seropositive and seronegative). RESULTS: The screening survey was applied to 903 permanent residents. Most positive respondents (114/137 [83.2%]) were examined by neurologists. The overall prevalence of epilepsy was 32.1 per 1,000 and that of active epilepsy was 16.6 per 1,000. Seroprevalence was 24.2% (200/825). Seroprevalence was associated with seizures (odds ratio 2.14; p = 0.026). Brain CT abnormalities compatible with NCC were more frequent in individuals with seizures and in those seropositive. CONCLUSION: In this hyperendemic area, an important proportion of seizure cases are associated with neurocysticercosis as demonstrated by serology or brain CT.


Assuntos
Encéfalo/parasitologia , Epilepsia/epidemiologia , Epilepsia/parasitologia , Neurocisticercose/diagnóstico por imagem , Neurocisticercose/epidemiologia , Adolescente , Adulto , Anticorpos/sangue , Encéfalo/diagnóstico por imagem , Encéfalo/patologia , Causalidade , Criança , Pré-Escolar , Comorbidade , Estudos Transversais , Epilepsia/sangue , Feminino , Inquéritos Epidemiológicos , Humanos , Lactente , Recém-Nascido , Masculino , Programas de Rastreamento , Pessoa de Meia-Idade , Neurocisticercose/patologia , Peru/epidemiologia , Estudos Soroepidemiológicos , Inquéritos e Questionários , Taenia solium/imunologia , Tomografia Computadorizada por Raios X
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