The withdrawal of replacement therapy and outcomes in alpha-1 antitrypsin deficiency lung transplant recipients

Alpha-1 antitrypsin deficiency (AATD) accounts for 5% of lung transplants performed worldwide. Ireland has a high frequency of 1 in 25 for the Z allele and 1 in 10 for the S allele [1]. These variants are associated with decreased production of alpha-1 antitrypsin (AAT), which predisposes to unprotected proteolytic activity of neutrophil elastase (NE) and proteinase 3 (PR3), linked to an increased risk of emphysema and hepatic disease. Associated inflammatory conditions such as vasculitis, panniculitis and inflammatory bowel disease are also suspected to be caused by uncontrolled neutrophil proteinase activity [2–4]. Vascular problems, including aortic aneurysm, in AATD patients are more severe and may reflect abnormal elastic tissue properties potentially impacting on wound healing [5].
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Research letters Source Type: research

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Conclusion: The presentation of PPCRA has been sporadic in the majority of cases. Inflammatory and hereditary origins have been anecdotally cited. Our young patient showed concurrent presentation of inflammatory and hereditary origin of PPCRA. We suggest that a careful investigation of systemic inflammation should be done in children with suggestive extraocular symptoms in the setting of PPCRA. PMID: 31631731 [PubMed - as supplied by publisher]
Source: Ophthalmic Genetics - Category: Opthalmology Tags: Ophthalmic Genet Source Type: research
Authors: special issue: “Focus on pediatric nephrology”, Bruni V, Petrisano M, Tarsitano F, Falvo F, Parisi F, Cucinotta U, Betta P, Di Benedetto V, Scuderi MG, Pensabene L, Sestito S, Cuppari C, Fede C, Chimenz R, Concolino D Abstract Alport's syndrome (AS, OMIM 301050) is a hereditary disorder characterized by progressive renal failure, hearing impairment and ocular changes. It is clinically and genetically heterogeneous and in its natural history, renal disease progresses from microscopic haematuria to proteinuria, and finally to progressive renal insufficiency. AS is caused by an inherited defect in...
Source: Journal of Biological Regulators and Homeostatic Agents - Category: Biomedical Science Tags: J Biol Regul Homeost Agents Source Type: research
Authors: special issue: “Focus on pediatric nephrology”, Chimenz R, Sallemi A, Fusco M, Cannavò L, Salvo V, Marseglia L, Cucinotta U, Gitto E, Concolino D, Arrigo T, Salpietro C Abstract The natural history of children with end stage renal disease is dialysis until a transplant can be done. There are two types of dialysis: hemodialysis and peritoneal dialysis (1). Peritoneal dialysis is preferred in young children because getting the vascular access for hemodialysis is challenging (2). Catheters should be surgically placed in a paramedian or lateral abdominal region with an extremity located in D...
Source: Journal of Biological Regulators and Homeostatic Agents - Category: Biomedical Science Tags: J Biol Regul Homeost Agents Source Type: research
The transcription factor B cell CLL/lymphoma 11B (BCL11B) is indispensable for T lineage development of lymphoid progenitors. Here, we show that chimeric antigen receptor (CAR) expression during early phases of ex vivo generation of lymphoid progenitors suppressed BCL11B, leading to suppression of T cell–associated gene expression and acquisition of NK cell–like properties. Upon adoptive transfer into hematopoietic stem cell transplant recipients, CAR-expressing lymphoid progenitors differentiated into CAR-induced killer (CARiK) cells that mediated potent antigen-directed antileukemic activity even across MHC b...
Source: Journal of Clinical Investigation - Category: Biomedical Science Authors: Source Type: research
Authors: Cui L, Cheng Z, Hu K, Pang Y, Liu Y, Qian T, Quan L, Dai Y, Pang Y, Ye X, Shi J, Fu L Abstract Acute myeloid leukemia (AML) is a genetically complex, highly aggressive hematological malignancy. Prognosis is usually with grim. PDZ and LIM domain proteins (PDLIM) are involved in the regulation of a variety of biological processes, including cytoskeletal organization, cell differentiation, organ development, neural signaling or tumorigenesis. The clinical and prognostic value of the PDLIM family in AML is unclear. To understand the role of PDLIM expression in AML, The Cancer Genome Atlas (TCGA) database was s...
Source: American Journal of Translational Research - Category: Research Tags: Am J Transl Res Source Type: research
Authors: Chan EG, Chan PG, Sanchez PG PMID: 31632776 [PubMed]
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
Authors: Yun JJ, Unai S, Pettersson G Abstract Lung transplantation (LTx) is routinely performed with sacrifice of the bronchial arterial circulation. We review bronchial artery anatomy and surgical technique for LTx with direct bronchial arterial revascularization (BAR). We also update the published clinical experience, including from our center, of LTx with BAR. Published series of LTx with BAR are from single centers, and all cite high technical success rates and good short-term outcomes. Technically, double LTx (DLTx) with BAR is almost always possible if a deliberate review of bronchial artery anatomy is perfo...
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
Authors: Taimeh Z Abstract Long considered an insignificant chamber, the right ventricle has been recently under spotlight, especially with emerging data supporting its critical role in disease progression. The right ventricle is a triangular heart chamber with complex physiology and pathophysiology. In this review, we discuss the normal physiology of the right ventricle, right ventricular failure, and recent advances in treatment with emphasis on optimal timing for transplantation referral. PMID: 31632759 [PubMed]
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
Authors: Kerdidani D, Magkouta S, Tsoumakidou M PMID: 31632820 [PubMed]
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
Authors: Alvarez A, Moreno P PMID: 31632807 [PubMed]
Source: Journal of Thoracic Disease - Category: Respiratory Medicine Tags: J Thorac Dis Source Type: research
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