Kamada starts Alpha-1 Antitrypsin phase 2 clinical trial to prevent lung transplant rejection
Kamada has started a phase 2 clinical trial with its proprietary drug Alpha-1 Antitrypsin (AAT) for preventing rejection in lung transplants, as part of a collaboration with Baxalta.
The objective of this survey was to assess the practices of international lung transplant (LTx) centers, pertaining to the testing for, and replacement of, AAT in patients with AATD.
Conclusion: CLO in adults is rare and is associated with a broad spectrum of symptoms. Precise anamnesis and radiological findings confirm the diagnosis. Surgical resection is the treatment of choice in symptomatic patients.
Conclusions: Men and smoking habits history were more prevalent. There was a significant diagnosis delay since symptoms onset. Most of them started follow-up due to respiratory symptoms. The commonest phenotypes were SZ and ZZ; ZZ was associated to lower AAT and DLCO levels and more pneumonia hospitalizations. Obstruction on PFT was prevalent. Few pts had criteria to prolastin treatment and transplant.
Conclusions: Despite the small sample, both groups had similar characteristics before and after LT, with a tendency for a lower survival rate in AATD-g.
ConclusionsLung and liver transplantation in AATD patients are associated with very good long-term survival rates that are comparable to, and sometimes superior to, other transplant indications. Although not currently recommended in AATD, LVRS may have a role in a minority of patients. The value of Alpha 1 Antitrypsin (AAT) augmentation therapy following lung transplantation requires further study. Wherever possible, AAT therapy should be continued in the period around elective surgeries.SummaryAlthough AATD is a widely discussed and researched topic in the literature, surgical intervention for AATD, in addition to the man...
A 62-year old non-smoker, Caucasian male was evaluated for lung transplantation based on severe upper-lobe predominant emphysema with superimposed fibrosis in the lower lung zones (Figure 1). The patient had no significant exposure history, and prior work-up for alpha-1-antitrypsin deficiency, autoimmune disease, and hypersensitivity pneumonitis was unremarkable. The diagnosis of Niemann Pick disease, type B was eventually established based on a compatible family history with an almost identical disease presentation in a sibling.
Authors: Hsin MKY, Wong CF, Yan SW, Fan KY, Ho CKL, Bhatia I, Au TWK Abstract Clinical lung transplant was first performed in Hong Kong in 1995. In the early years, the volume of activity was very low. There has been a clear trend of increasing volume in the past few years. The recipient pathology is very different from the International Society for Heart and Lung Transplantation (ISHLT) database, with complete absence of cystic fibrosis and alpha-1-antitrypsin deficiency, and a predominance of diseases of the pulmonary circulation. Lymphangioleiomyomatosis (LAM) has a much higher representation on the waiting list...
The risk of primary graft dysfunction (PGD) of donor lungs and early postoperative mortality after lung transplantation (LTx) increases with prolonged preservation times. PGD is triggered by early infiltrating neutrophils which release large amounts of elastase-related serine proteases and inflict irreversible damage on lung graft after cold ischemic storage. Cold ischemic storage for only 6 hours is generally accepted, when lungs are cooled down and preserved in Perfadex immediately after their removal from donors.
Primary graft dysfunction (PGD) is the most serious early complication and it occurs within the first 72 hours post-transplantation. There is currently no specific treatment to prevent or treat PGD. Ischemia reperfusion injury (IRI) is an important component of PGD. Alpha 1 antitrypsin (A1AT) is a serine-protease inhibitor that inhibits excessive proteolytic enzymes so they don't accumulate and break down normal tissue. A1AT primarily inhibits neutrophil elastase, an important contributor to IRI.
Vascular damage and primary graft dysfunction increases with prolonged preservation times of transplanted donor lungs. Hence, storage and conservation of donated lungs in protein-free, dextran-containing electrolyte solutions like Perfadex ® is limited to about six hours.