Your browser doesn't support javascript.
Mostrar: 20 | 50 | 100
Resultados 1 - 11 de 11
Filtrar
Mais filtros










Base de dados
Tipo de estudo
Intervalo de ano de publicação
3.
BMC Pulm Med ; 17(1): 17, 2017 01 13.
Artigo em Inglês | MEDLINE | ID: mdl-28086849

RESUMO

BACKGROUND: Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as "destroyed lung". Lung resection surgery is seldom indicated in cystic fibrosis and the associated medical literature is relatively sparse. CASE PRESENTATION: A 14 year old boy was referred to our centre for lung transplantation assessment. He had a chronic history of complete collapse and consolidation of his entire right lung. This was causing severe morbidity in terms of a continuous requirement for intravenous antibiotics over the last year, poor exercise tolerance with forced expiratory volume in 1 s of 35-40% predicted and need for home tuition. He also had significant nutritional problems and gastrointestinal symptoms following a Nissen's fundoplication operation a year earlier. His nutritional status was firstly improved by the institution of jejunal feeding, which also greatly improved his distressing symptoms of nausea and wretching. After thorough multidisciplinary assessment the therapeutic option of performing a right pneumonectomy was considered due to relative sparing of the left lung, which demonstrated only mild bronchiectasis on computed tomography scan. This was performed uneventfully with a smooth peri-operative course. Targeted antimicrobials were used to treat the multiresistant organisms colonising his airways. Subsequently his quality of life, nutritional status and lung function all improved significantly and requirement for lung transplantation has been delayed. CONCLUSIONS: We report a successful outcome following pneumonectomy in a teenage boy with cystic fibrosis referred to our centre for lung transplantation assessment with chronic unilateral collapse and consolidation of his right lung. We believe that improvement of nutritional status pre-operatively and targeted antimicrobial therapy, all contributed to the smooth peri-operative course. Pneumonectomy can be a feasible option in this clinical situation in cystic fibrosis but the associated risks must be considered carefully on a case-by-case basis.


Assuntos
Fibrose Cística/cirurgia , Pulmão/cirurgia , Pneumonectomia , Adolescente , Bronquiectasia/diagnóstico por imagem , Fibrose Cística/diagnóstico por imagem , Humanos , Pulmão/diagnóstico por imagem , Transplante de Pulmão , Masculino , Qualidade de Vida , Radiografia Torácica , Tomografia Computadorizada por Raios X
5.
Hematology ; 20(1): 50-2, 2015 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-24716903

RESUMO

OBJECTIVE AND IMPORTANCE: To describe two novel hemoglobin mutations that resulted in an unstable hemoglobin with a severe hemolytic phenotype. CLINICAL PRESENTATION: A patient with an unstable hemoglobin and chronic hemolysis underwent splenectomy at age 15, subsequently developing chronic thrombo-embolic pulmonary hypertension at age 27 that was ultimately fatal. INTERVENTION: DNA sequencing of the alpha globin gene revealed heterozygous inheritance of Hb Taybe, arising from a novel mutation in the HBA2 gene and Hb Bridlington, a novel HBA1 mutation. Greater disease severity is predicted by the position of the Hb Taybe mutation on the HBA2 gene (which transcribes more globin than the HBA1 gene). CONCLUSION: Splenectomy was not clearly beneficial and may have contributed to the development of pulmonary hypertension. The case favors a cautious approach when considering splenectomy for patients with Hb Taybe.


Assuntos
Hemoglobinas Anormais/genética , Hipertensão Pulmonar/genética , alfa-Globinas/genética , Adulto , Anemia Hemolítica/sangue , Anemia Hemolítica/genética , Feminino , Hemólise , Humanos , Hipertensão Pulmonar/sangue
6.
Transpl Int ; 27(8): 857-67, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-24750386

RESUMO

Distinct phenotypes of chronic lung allograft dysfunction (CLAD) after lung transplantation are emerging with lymphocytic bronchiolitis (LB)/azithromycin reversible allograft dysfunction (ARAD), classical or fibrotic bronchiolitis obliterans syndrome (BOS), and restrictive allograft syndrome (RAS) proposed as separate entities. We have additionally identified lung transplant recipients with prior LB, demonstrating persistent airway neutrophilia (PAN) despite azithromycin treatment. The aim of this study was to evaluate differences in the airway microenvironment in different phenotypes of CLAD. Bronchoalveolar lavage (BAL) from recipients identified as stable (control), LB/ARAD, PAN, BOS, and RAS were evaluated for differential cell counts and concentrations of IL-1α, IL-1ß, IL-6, IL-8, and TNF-α. Primary human bronchial epithelial cells were exposed to BAL supernatants from different phenotypes and their viability measured. BOS and RAS showed increased BAL neutrophilia but no change in cytokine concentrations compared with prediagnosis. In both LB/ARAD and PAN, significant increases in IL-1α, IL-1ß, and IL-8 were present. BAL IL-6 and TNF-α concentrations were increased in PAN and only this phenotype demonstrated decreased epithelial cell viability after exposure to BAL fluid. This study demonstrates clear differences in the airway microenvironment between different CLAD phenotypes. Systematic phenotyping of CLAD may help the development of more personalized approaches to treatment.


Assuntos
Bronquiolite Obliterante/etiologia , Rejeição de Enxerto/etiologia , Transplante de Pulmão/efeitos adversos , Adulto , Aloenxertos , Células Cultivadas , Doença Crônica , Citocinas/análise , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Fenótipo
7.
Interact Cardiovasc Thorac Surg ; 15(3): 432-6; discussion 436, 2012 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-22714587

RESUMO

OBJECTIVES: Many centres avoid using cardiopulmonary bypass (CPB) for lung transplant due to concerns over aggravated lung reperfusion injury and excessive blood loss. We reviewed our 23-years' experience of single lung transplantation. METHODS: A retrospective review of single lung transplants at our institution (1987-2010), examining differences in allograft function and postoperative complications between CPB and non-bypass (non-CPB) cases. RESULTS: Two hundred and fifty-nine single lung transplants were undertaken. Fifty-three (20.5%) with CPB. There was no difference demographically between the two groups. No difference existed in preoperative PO(2)/FiO(2). At 1 and 24 h, the postoperative PO(2)/FiO(2) ratio was no different (mean 2.95 and 3.24 in non-CPB cases; 3.53 and 3.75 in CPB patients, P = 0.18 and P = 0.34, respectively). Extubation time was not influenced by the use of CPB. Postoperative blood loss was greater in the CPB group. The usage of fresh frozen plasma and platelets was similar (P = 0.64 and 0.41, respectively). More blood was transfused during postoperative care of CPB patients (P = 0.02). CONCLUSIONS: Fears of poor postoperative lung function after CPB appear unfounded. We could detect no difference in function or extubation time. Although the use of CPB increases postoperative bleeding and the need for transfusion, it may be used safely to facilitate lung transplantation.


Assuntos
Ponte Cardiopulmonar/estatística & dados numéricos , Hipertensão Pulmonar/cirurgia , Transplante de Pulmão/métodos , Adolescente , Adulto , Idoso , Feminino , Seguimentos , Humanos , Incidência , Transplante de Pulmão/fisiologia , Masculino , Pessoa de Meia-Idade , Hemorragia Pós-Operatória/epidemiologia , Período Pós-Operatório , Traumatismo por Reperfusão/epidemiologia , Estudos Retrospectivos , Transplante Homólogo , Resultado do Tratamento , Reino Unido/epidemiologia , Adulto Jovem
9.
J Exp Med ; 208(2): 227-34, 2011 Feb 14.
Artigo em Inglês | MEDLINE | ID: mdl-21242295

RESUMO

Congenital or acquired cellular deficiencies in humans have the potential to reveal much about normal hematopoiesis and immune function. We show that a recently described syndrome of monocytopenia, B and NK lymphoid deficiency additionally includes the near absence of dendritic cells. Four subjects showed severe depletion of the peripheral blood HLA-DR(+) lineage(-) compartment, with virtually no CD123(+) or CD11c(+) dendritic cells (DCs) and very few CD14(+) or CD16(+) monocytes. The only remaining HLA-DR(+) lineage(-) cells were circulating CD34(+) progenitor cells. Dermal CD14(+) and CD1a(+) DC were also absent, consistent with their dependence on blood-derived precursors. In contrast, epidermal Langerhans cells and tissue macrophages were largely preserved. Combined loss of peripheral DCs, monocytes, and B and NK lymphocytes was mirrored in the bone marrow by complete absence of multilymphoid progenitors and depletion of granulocyte-macrophage progenitors. Depletion of the HLA-DR(+) peripheral blood compartment was associated with elevated serum fms-like tyrosine kinase ligand and reduced circulating CD4(+)CD25(hi)FoxP3(+) T cells, supporting a role for DC in T reg cell homeostasis.


Assuntos
Células Dendríticas/citologia , Suscetibilidade a Doenças/etiologia , Antígenos HLA-DR/sangue , Leucopenia/genética , Monócitos/citologia , Adulto , Antígenos CD/metabolismo , Células da Medula Óssea/citologia , Criança , Células Dendríticas/patologia , Suscetibilidade a Doenças/microbiologia , Suscetibilidade a Doenças/virologia , Ensaio de Imunoadsorção Enzimática , Citometria de Fluxo , Humanos , Interferon gama/sangue , Leucopenia/sangue , Leucopenia/complicações , Microscopia de Fluorescência , Monócitos/patologia , Infecções por Mycobacterium/imunologia , Síndrome
10.
Transplantation ; 85(5): 771-4, 2008 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-18337673

RESUMO

Long-term survival after lung transplantation remains limited by the development of bronchiolitis obliterans syndrome (BOS). Allograft colonization with Pseudomonas aeruginosa is common particularly in recipients with BOS, but a possible etiological relationship remains unexplored. In 155 consecutive lung transplants, the development of allograft colonization with Pseudomonas was strongly associated with the development of BOS within 2 years of transplant (23.4% vs. 7.7% in those colonized and not colonized, respectively, P=0.006). Freedom from BOS was significantly shorter in those patients without any pretransplant bacterial reservoir developing de novo allograft pseudomonal colonization as compared with those remaining free of colonization (Kaplan-Meier log-rank P=0.014). The isolation of Pseudomonas preceded the diagnosis of BOS in 14 of 18 (78%) and by a median of 204 days (95% confidence interval 115-492) in patients developing both these complications. We conclude that de novo colonization of the lung allograft by Pseudomonas is strongly associated with the subsequent development of BOS.


Assuntos
Bronquiolite Obliterante/epidemiologia , Transplante de Pulmão/efeitos adversos , Pseudomonas aeruginosa/isolamento & purificação , Adulto , Bronquiolite Obliterante/microbiologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infecções por Pseudomonas/epidemiologia , Doença Pulmonar Obstrutiva Crônica/epidemiologia , Estudos Retrospectivos , Fatores de Risco , Fatores de Tempo , Transplante Homólogo
11.
J Heart Lung Transplant ; 25(12): 1436-40, 2006 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17178338

RESUMO

BACKGROUND: Bronchiolitis obliterans syndrome (BOS) remains the major cause of long-term morbidity and mortality after lung transplantation, and new therapeutic measures are needed. We speculated that cilomilast might reduce mediators of airway inflammation and angiogenesis from the airway epithelium, supporting a potential value in the treatment of BOS. We used an ex vivo primary bronchial epithelial cell culture (PBEC) model to investigate this hypothesis. Increasing evidence suggests the epithelium is central in stimulating both inflammatory and proliferative responses in the airway. METHODS: Bronchial brushings were taken from 7 stable lung allograft recipients and were used to establish sub-confluent PBECs. The effect of incubation for 48 hours with 0.1 to 10 micromol/liter cilomilast on basal production of interleukin (IL)-8, IL-6, granulocyte macrophage colony-stimulating factor (GMCSF), and vascular endothelial growth factor (VEGF) were assayed by multiplex analyser. RESULTS: There was a dose dependent fall in basal IL-8 and GMCSF levels with cilomilast. Median change for IL-8 was -25% (range, -66% to 5%; p = 0.035) at 1 micromol/liter , and -40% (range, -72% to -20; p = 0.022) at 10 micromol/liter. Median GMSCF change was -34% (range, -70% to 16%; p = 0.05) at 1 micromol/liter, and 37% (range, -80% to -8%; p = 0.04) at 10 micromol/liter. There were no effects on VEGF. CONCLUSION: The phosphodiesterase type IV inhibitor cilomilast reduced IL-8 and GMCSF release from PBECs. These cytokines are associated with the persistence of airway neutrophilic inflammation and airway remodelling seen in obliterative bronchiolitis. These ex vivo results suggest a potential for cilomilast in the treatment of BOS, which would need to be evaluated in appropriate clinical studies.


Assuntos
3',5'-AMP Cíclico Fosfodiesterases/antagonistas & inibidores , Brônquios/metabolismo , Mediadores da Inflamação/antagonistas & inibidores , Transplante de Pulmão , Nitrilos/uso terapêutico , Inibidores de Fosfodiesterase/uso terapêutico , Ácidos Carboxílicos/administração & dosagem , Ácidos Carboxílicos/uso terapêutico , Células Cultivadas , Nucleotídeo Cíclico Fosfodiesterase do Tipo 4 , Ácidos Cicloexanocarboxílicos , Relação Dose-Resposta a Droga , Células Epiteliais/metabolismo , Fator Estimulador de Colônias de Granulócitos e Macrófagos/antagonistas & inibidores , Humanos , Interleucina-6/antagonistas & inibidores , Interleucina-8/antagonistas & inibidores , Nitrilos/administração & dosagem , Inibidores de Fosfodiesterase/administração & dosagem , Fator A de Crescimento do Endotélio Vascular/metabolismo
SELEÇÃO DE REFERÊNCIAS
DETALHE DA PESQUISA