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1.
Curr Opin Pulm Med ; 2024 Jul 31.
Artigo em Inglês | MEDLINE | ID: mdl-39082306

RESUMO

PURPOSE OF REVIEW: Lung transplantation (LTX) has transformed care for people with cystic fibrosis (pwCF) suffering from advanced cystic fibrosis lung disease (ACFLD), and it has evolved into an accepted therapy for patients with ACFLD across all ages. We review cystic fibrosis as a major indication for LTX, particularly highlighting outcomes including survival, a changing landscape over time, and factors affecting sequelae following LTX in cystic fibrosis. RECENT FINDINGS: Although some populations such as those undergoing lung retransplantation exhibit inferior posttransplant outcomes, LTX for pwCF provides an excellent long-term survival that has significantly improved over time, likely due to specialized cystic fibrosis center care and recognition of common comorbidities in pwCF post-LTX. There are gaps in post-LTX outcomes for pwCF, including that identified between Canada and the United States, and that seen in adolescents - both of which are likely multifactorial. In particular, the revolution in cystic fibrosis medical therapy with CFTR modulator therapy has resulted in a dramatic decline in programs performing LTX for cystic fibrosis. How durable this effect will remains to be seen. SUMMARY: Overall, LTX remains a well accepted ultimate therapy option in patients with ACFLD if compatible with the individual's goals of care, offering an improved quality of life and maximization of overall survival.

3.
Clin Transplant ; 37(6): e15018, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37218644

RESUMO

Pediatric lung transplantation (LTX) remains limited by the scarcity of small donor lungs, particularly in less populated parts of the world. Optimal organ allocation, including the prioritization and ranking of pediatric LTX candidates, and the appropriate matching of pediatric donors to recipients have been crucial elements in improving pediatric LTX outcomes. We aimed to elucidate the various pediatric lung allocation practices worldwide. A global survey of current pediatric solid organ transplantation deceased donation allocation practices was conducted by the International Pediatric Transplant Association (IPTA), and these policies were subsequently analyzed if publicly available, with focus on pediatric lung transplantation. Significant variation was found in lung allocation systems worldwide both in terms of prioritization and distribution for children. Definition of pediatrics varied from <12 years of age to <18 years of age. While several countries performing LTX for young children do not have a formal system to prioritize pediatric candidates, many countries that perform LTX at higher rates do offer prioritization methods for children: including the United States, United Kingdom, France, Italy, Australia, and countries serviced by Eurotransplant. Certain lung allocation practices for pediatrics are highlighted herein, including the newly instituted Composite Allocation Score (CAS) system in the United States, pediatric matching with Eurotransplant, and pediatric prioritization in Spain. The systems highlighted here explicitly aim to provide judicious and high quality LTX care for children.


Assuntos
Transplante de Pulmão , Obtenção de Tecidos e Órgãos , Humanos , Criança , Estados Unidos , Pré-Escolar , Estudos Retrospectivos , Transplante de Pulmão/métodos , Doadores de Tecidos , Reino Unido , Listas de Espera
4.
Pediatr Pulmonol ; 58(1): 213-221, 2023 01.
Artigo em Inglês | MEDLINE | ID: mdl-36200536

RESUMO

INTRODUCTION: Chronic lung allograft dysfunction (CLAD) continues to negatively impact the survival of pediatric lung transplant (LTx) recipients. Current consensus guidelines are adult-focused. We sought to examine CLAD detection and monitoring practices at pediatric LTx programs. METHODS: We conducted a survey among the International Pediatric Lung Transplant Collaborative. Questions consisted of practitioner's experience, LTx program demographics, and querying tests used for CLAD surveillance and detection. Investigations queried included: chest x-ray (CXR), chest computed tomography (CT), lung magnetic resonance imaging (MRI), ventilation/perfusion scanning, conventional pulmonary function testing (PFT), multiple breath washout (MBW), infant/preschool PFT, bronchoalveolar lavage, transbronchial biopsies (TBBx), or other tissue sampling techniques. Preferences for certain modalities over others were questioned based on a five-point Likert scale. RESULTS: Twenty-four of 25 programs responded. Chest CT and CXR are used generally for both CLAD surveillance and detection. No programs use lung MRI clinically, it may have some utility in the future. While all centers use conventional PFT, MBW, and infant/preschool PFT are used in one-fifth and one-third of centers, respectively. While the majority of programs use TBBx, only 41.7% would obtain a diagnosis based on tissue histopathology over noninvasive techniques if CLAD is suspected. Utilization of biomarkers is still limited. CONCLUSIONS: Our results indicate continued use of conventional PFT along with chest CT and less so CXR for CLAD detection and monitoring in the large majority of centers. Infant/preschool PFT and novel methods such as MBW are used in a few centers only. Respondents agreed there is a timely need for pediatric consensus guidelines on CLAD detection and monitoring.


Assuntos
Transplante de Pulmão , Adulto , Humanos , Criança , Pré-Escolar , Estudos Retrospectivos , Pulmão/diagnóstico por imagem , Transplante Homólogo , Aloenxertos/diagnóstico por imagem
5.
Clin Transplant ; 36(4): e14634, 2022 04.
Artigo em Inglês | MEDLINE | ID: mdl-35244236

RESUMO

There has been a shift over decades in the diagnostic indications for lung transplantation in children; in particular, there has been a reduction in the proportion of pediatric cystic fibrosis (CF) patients undergoing lung transplantation early in life, and more transplants occurring in other diagnostic groups. Here, we examine trends in pediatric lung transplantation with regards to indications by analyzing data from the United Network of Organ Sharing, the International Society for Heart and Lung Transplantation Thoracic Transplant Registry, and other sources. Over the past two years, there has been a precipitous decline in both the number of transplants due to CF and the proportion of CF cases relative to the total number of transplants, likely not solely due to the COVID-19 pandemic. In 2020, primary pulmonary arterial hypertension for the first-time surpassed CF as main indication for pediatric lung transplantation in the United States, a finding that is also reflected in international data. We discuss the effect of novel CFTR modulator therapies as a major factor leading to this shifting landscape. Based on our trending, pulmonary hypertension-related diagnoses and pediatric interstitial lung diseases are rising indications, for which we suggest adjustments of consensus guidelines around candidate selection criteria.


Assuntos
COVID-19 , Fibrose Cística , Transplante de Coração , Transplante de Coração-Pulmão , Transplante de Pulmão , COVID-19/epidemiologia , Criança , Fibrose Cística/cirurgia , Humanos , Transplante de Pulmão/efeitos adversos , Pandemias , Taxa de Sobrevida , Doadores de Tecidos , Estados Unidos
6.
Pediatr Transplant ; 26(4): e14252, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-35187796

RESUMO

BACKGROUND: Atopic disorders are more common in children after heart transplant (HTx). We hypothesized that HTx at an early age and thymus excision (TE) affect development of T and B cells, especially regulatory T cells (Tregs), which help maintain tolerance. METHODS: In this single-center study including 24 patients transplanted between 2013 and 2018, we investigated lymphocyte patterns in relation to these factors using flow cytometry. Clinical data were collected from standardized questionnaires and medical charts. Patients were stratified into TE and non-TE groups as well as patients with and without post-transplant atopy development/worsening. RESULTS: 64% of TE patients experienced new or worsening asthma/eczema post-transplant compared to 20% of non-TE patients. TE patients had higher total Treg proportions (CD4+CD25+CD127lo) than non-TE patients (p = .043), but borderline significantly lower naïve Tregs (CD45RA+CD27-) (p = .057). Memory CD4+ T cells were higher in TE patients in trend (p = .084). Total Tregs did not differ between atopic/nonatopic groups, although naïve Tregs were significantly lower in atopic patients (p = .028). Memory CD4+ T cells were higher in atopic patients in trend (p = .082). IgM+IgD+ B cells were higher in nonatopic patients in trend (p = .064). CONCLUSIONS: New/worsening atopy is more common in thymectomized HTx children and is associated with alterations in T-cell profiles. Avoiding TE may prevent these alterations and reduce incidence of atopy post-HTx.


Assuntos
Transplante de Coração , Humanos , Imunofenotipagem , Fenótipo , Linfócitos T Reguladores , Timectomia
7.
BMJ Case Rep ; 14(3)2021 Mar 04.
Artigo em Inglês | MEDLINE | ID: mdl-33664030

RESUMO

A 17-year-old previously healthy man presented with a 4-week history of progressive bilateral leg swelling with discomfort and erythema, but no signs of arthritis or erythema nodosum. An incidental finding of a query pulmonary nodule on chest X-ray prompted chest CT for further evaluation, revealing bilateral hilar and mediastinal lymphadenopathy. The patient then underwent endobronchial ultrasound and transbronchial needle aspiration biopsies of mediastinal lymph nodes. Biopsies and bronchoalveolar lavage samples were negative for microbiology, including mycobacterial culture. Pathology demonstrated non-caseating granulomas consistent with a diagnosis of sarcoidosis. Weeks later, he developed arthralgias of the left metacarpophalangeal joints and erythema nodosum and was diagnosed with Löfgren syndrome, a phenomenon rarely described in the paediatric population. This case highlights an approach to lower extremity swelling as well as hilar lymphadenopathy in the paediatric population. In addition, it emphasises the importance of multidisciplinary teamwork for accurate and timely diagnoses.


Assuntos
Eritema Nodoso , Sarcoidose , Adolescente , Criança , Eritema Nodoso/diagnóstico , Humanos , Perna (Membro) , Linfonodos , Masculino , Mediastino
8.
BMJ Case Rep ; 13(10)2020 Oct 01.
Artigo em Inglês | MEDLINE | ID: mdl-33004353

RESUMO

A 9-year-old previously well girl presented with multiple episodes of large volume haemoptysis and right sided consolidation. She continued to have haemoptysis despite intravenous antibiotics. CT chest suggested a right mainstem endobronchial lesion; this was not seen on bronchoscopy where an extensive blood clot was removed. Distal flexible bronchoscopy could not identify the source of bleeding. CT angiogram revealed a broncho-pulmonary arterial fistula, a rare cause of haemoptysis in children. Endovascular embolisation resulted in short-term symptom resolution; however, haemoptysis recurred months later, leading to re-embolisation. This case highlights a stepwise approach to the workup of large volume haemoptysis.


Assuntos
Fístula Brônquica , Broncoscopia/métodos , Angiografia por Tomografia Computadorizada/métodos , Embolização Terapêutica/métodos , Procedimentos Endovasculares/métodos , Hemoptise , Artéria Pulmonar , Fístula Vascular , Fístula Brônquica/diagnóstico , Fístula Brônquica/fisiopatologia , Fístula Brônquica/cirurgia , Criança , Diagnóstico Diferencial , Feminino , Hemoptise/diagnóstico , Hemoptise/etiologia , Hemoptise/cirurgia , Humanos , Pulmão/diagnóstico por imagem , Artéria Pulmonar/diagnóstico por imagem , Artéria Pulmonar/patologia , Recidiva , Reoperação , Resultado do Tratamento , Fístula Vascular/diagnóstico , Fístula Vascular/fisiopatologia , Fístula Vascular/cirurgia
9.
Pediatr Pulmonol ; 55(6): 1394-1399, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32203645

RESUMO

BACKGROUND: In pediatric cystic fibrosis (CF) ambulatory care, handheld spirometry in individual clinic rooms would improve patient flow and potentially reduce patient-to-patient contact. A validation study was conducted to examine the accuracy of an entirely handheld turbine spirometer vs a standard laboratory device in pediatric CF patients. METHODS: Spirometric data were obtained from 76 CF patients aged less than 18 years in the ambulatory setting using the Micro Loop Spirometer (CareFusion) and compared to same-day data from conventional laboratory spirometry. RESULTS: Linear relationships were obtained between devices, demonstrating good correlation: r = .99, .99, .97, and .82 for forced expiratory volume in 1 second (FEV1) , forced vital capacity (FVC), FEF25%-75% , and peak expiratory flow, respectively (P < .001 for all). Biases (mean differences between devices) were -65 mL for FEV1 (P < .001) and -115 mL for FVC (P < .001) on the handheld. Bland-Altman plots demonstrated scatter in bias across all volumes. Limits of agreement (defined as mean ± 2 standard deviations [SD]) were large: +189 to -319 mL for FEV1 , equating to large limits of agreement for FEV1 percent predicted of +9.0% to -13.9%. For repeated measurements on the same device on different days, a larger percent SD was obtained with the handheld compared to the conventional spirometer (6.7% vs 5.1%, respectively). Importantly, a relatively large number (15%) demonstrated a decrease in FEV1 percent predicted of ≥10% on the handheld compared to conventional. CONCLUSIONS: This suggests that while both devices have passed the recommendations for spirometry testing per American Thoracic Society/European Respiratory Society, handheld turbine vs conventional spirometers may not be used interchangeably in the pediatric CF population.


Assuntos
Fibrose Cística/fisiopatologia , Espirometria/instrumentação , Adolescente , Criança , Feminino , Volume Expiratório Forçado , Humanos , Masculino , Pico do Fluxo Expiratório , Capacidade Vital
10.
Clin Transplant ; 32(10): e13400, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30176068

RESUMO

Pediatric heart transplantation requires lifelong immune suppression and may require thymectomy, both of which alter T-cell repertoires. We hypothesized that atopic and autoimmune diseases are more common in pediatric heart transplant patients than the general population, and that transplantation in early childhood increases the risk of development or worsening of atopic or autoimmune disease. A cross-sectional single-center study including 21 heart transplant patients aged ≤18 years was conducted. Data collected included age at transplant, induction, thymectomy, and development and severity of atopic or autoimmune disease. A majority (67%) reported having any atopic disease post-transplant, all of whom reported onset or worsening post-transplantation. Thymectomized patients were significantly more likely to have asthma (P = 0.018) and report asthma worsening post-transplant (P = 0.045). Patients with worsening of asthma post-transplant were transplanted at a significantly younger age (P = 0.040). ABO incompatible and ABO compatible recipients presented similarly. Anemia was common (38%) but not always clearly of autoimmune origin. Atopic diseases are common in children following heart transplantation: Compared to the general population, there is a higher prevalence of eczema (43% vs 11%) and asthma (33% vs 9%). Both thymectomy and younger age at transplant are associated with atopic disorders, possibly due to altered T-cell repertoires.


Assuntos
Doenças Autoimunes/etiologia , Transplante de Coração/efeitos adversos , Hipersensibilidade/etiologia , Timectomia/efeitos adversos , Adolescente , Doenças Autoimunes/patologia , Criança , Pré-Escolar , Estudos Transversais , Feminino , Seguimentos , Humanos , Hipersensibilidade/patologia , Lactente , Recém-Nascido , Masculino , Prognóstico , Fatores de Risco
11.
J Trauma Acute Care Surg ; 72(4): 1031-4, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-22491622

RESUMO

BACKGROUND: Based on our previous study, pediatric intentional trauma injuries with Injury Severity Scores (ISS) ≥ 12 were more commonly observed in the urban than the rural setting (15.2% vs. 5.5%) in Alberta from 1996 to 2006. We wish to understand differences between urban and rural pediatric intentional trauma to plan for prevention and supportive strategies. METHODS: Data were extracted from the Alberta Trauma Registry on pediatric patients (0-17 years) with ISS ≥ 12, treated from 1996 to 2010 at the Stollery Children's Hospital. Statistical analysis was made comparing urban versus rural groups using t test and χ2 with p < 0.05 considered significant. RESULTS: There were 170 pediatric patients who suffered intentional injury (urban = 58.3%; rural = 41.8%; not significant), with a majority of males (72.4%). Two groups were predominant: the very young (<1 year) at 17.1% of all injuries and the teens (≥ 15 years) at 54.1%. The cause of intent injury was child abuse (31.2%), assault with blunt object (24.6%), assault with a sharp object (22.9%), and suicide (18.2%). The mean ISS was 22.9 ± 7.8 standard deviation. Tragically, 29 patients (17.1%) died. There were no differences between urban and rural pediatric trauma in terms of age, gender, cause of injury, ISS, survival, length of stay, pediatric intensive care unit length of stay, number of operations needed, or alcohol. CONCLUSION: An important pattern of intentional injuries can be seen where preventative efforts can be strengthened regardless of urban or rural area: the very young as shaken baby cases and the teens, who unfortunately, accounted for the majority of suicidal attempts.


Assuntos
Maus-Tratos Infantis/estatística & dados numéricos , Ferimentos e Lesões/epidemiologia , Adolescente , Fatores Etários , Alberta/epidemiologia , Distribuição de Qui-Quadrado , Criança , Maus-Tratos Infantis/prevenção & controle , Pré-Escolar , Feminino , Humanos , Lactente , Escala de Gravidade do Ferimento , Tempo de Internação , Masculino , Sistema de Registros , População Rural/estatística & dados numéricos , Fatores Sexuais , População Urbana/estatística & dados numéricos , Ferimentos e Lesões/etiologia
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