Cystic fibrosis transplantation

Sepsis syndrome Anorexia nervosa Complications during pregnancy Geriatric patients with multiple chronic disease Organ transplantation Inborn errors of metabolism Cystic fibrosis Extreme prematurity... 

Patients suffering from cystic fibrosis often use various aerosolized drugs. To reduce the viscosity of the mucus in the airways, recombinant human deoxyribonuclease is used. This enzyme is the first recombinant protein that has been developed for specific delivery to the lungs via the airways. It has a local action on the mucus in the airways and its absorption is minimal. Another drug that decreases the viscosity of the mucus is acetylcysteine. Aerosolized antibiotics are a further group of therapeutics that is widely used by cystic fibrosis patients. Solutions of antibiotics like tobramycin or colistin are used in nebulizers to prevent exacerbation of the disease. Pentamidine has been used for the prophylaxis of Pneumocystis pneumonia in patients infected with HIV virus, while chronic rejection of lung transplants provided a reason to develop an aerosol formulation of cyclosporine A. 

The first combined heart-lung transplant was performed in 1981 at Stanford University. Combined heart-lung donors need to satisfy both the requirements already described separately. Combined heart-lung transplant is recommended in patients with congenital problems affecting these organs, pulmonary hypertension and/or cystic fibrosis. The recipients for the combined transplant are recommended to be less than 55 years old. Survival rates are 79, 66, and 54% at 1 month, 1 year and 3 years, respectively, after transplantation. 

Ierano, T., Siiipo, A., Sturiale, L., Garozzo, D., Corns, P., Perry, J., Lanzetta, R., Parrilli, M., De Soyza, A., Molinaro, A. Structural elucidation of a novel B. cenocepacia ET-12 lipooligosac-charide isolated from a cystic fibrosis patient after lung transplantation. Eur J Org Chem 7 (2010a) 1299-1306. 

Couetil, J.R, Houssin, D.R, Donsset, B.E., Chevalier, P.G., Gulnvarch, A., Loumet, D., Achkar, A., Carpentier, A.F. Combined heart-lung-liver, double lung-hver, and isolated hver transplantation for cystic fibrosis in children. Transpl. Int. 1997 19 33-39... 

A 29-year-old man received a double lung transplantation for end-stage cystic fibrosis. After uneventful surgery, he was accidentally given ten times the intended dose of ciclosporin (30 instead of 3 mg/kg) and 18 hours later became anuric. His blood ciclosporin concentration was 4100 ng/ml. Hemodialysis was required for 6 weeks. A renal biopsy 7 weeks later showed typical features of acute tubular necrosis and lesions that resembled chronic nephrotoxicity. Renal function was stiU abnormal when he died from another cause 14 weeks after the accidental overdose. 

A 17-year-old man with cystic fibrosis who took itraconazole after a lung-Uver transplant had high trough concentrations of tacrolimus, despite the relatively low dosage (0.1-0.3 mg/kg/day) (113). 

The pharmacokinetics of tobramycin in patients with cystic fibrosis is significantly altered after lung transplantation, and early and close drug monitoring is recommended (43). 

Bauldoff GS, Nunley DR, Manzetti JD. Use of aerosolized colistin sodium in cystic fibrosis awaiting lung transplantation. Transplantation 64 748-752, 1997. 

Saiman L, Mehar F, Niu WW. Antibiotic susceptibility of multiply resistant Pseudomonas aeruginosa isolated from patients with cystic fibrosis, including candidates for transplantation. Clin Infect Dis 23 532-537, 1996. 

Yankaskas JR, Westerman JH, Thompson JT, et al. Improved results of lung transplantation for patients with cystic fibrosis. J Thorac Cardiovasc Surg 1995 109 224-234. 

Other etiologies Congenital, idiopathic, hereditary (trypsinogen gene mutations), cystic fibrosis, inflammatory bowel disease, peptic ulcer disease, solid organ transplantation (liver, kidney, heart), refeeding... 

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