Long-term outcome of lung transplantation for cystic fibrosis ? Danish results

Bech, Bo

doi:10.1016/j.ejcts.2004.08.015

Cited by 38 publications

(34 citation statements)

References 23 publications

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“…Lung transplantation is currently the only treatment available for end-stage respiratory insufficiency, and today, CF patients are the second largest group of lung transplant recipients in Europe (13). In a recent study of lung transplant CF patients from our center, 51 Cr-EDTA clearance declined from normal levels of ϳ100 -32 ml/min 6 months after transplantation and decreased further after 6 years (13).…”

Section: Other Complicationsmentioning

confidence: 99%

“…treatment with dialysis or kidney transplantation (13). Cyclosporine treatment might be the main reason for the drop in kidney function.…”

Section: Other Complicationsmentioning

confidence: 99%

“…The patients received immunosuppressive treatment, including cyclosporine and azathoprine, adjusted according to renal function, leukocytes, and platelets. Methylprednisolone was administered at a daily dose of 0.1 mg/kg (13). Two nontransplanted patients were treated with prednisolone and cyclosporine due to allergic broncho-pulmonary aspergillosis.…”

Section: Diabetic Retinopathymentioning

confidence: 99%

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Cystic Fibrosis–Related Diabetes

et al. 2006

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OBJECTIVE -Cystic fibrosis (CF)-related diabetes has been regarded as a mild form of diabetes with a low risk of severe diabetes complications. The prevalence of CF-related diabetes increases with age, resulting in a 50% prevalence of diabetes at age 30 years. We sought to investigate whether microvascular complications in CF-related diabetes appear with a relevant frequency.RESEARCH DESIGN AND METHODS -Thirty-eight patients aged 30 (range 18 -55) years with CF-related diabetes for 20 (0 -31) years were screened for diabetes complications. Because of chronic pulmonary infections, the majority of patients were regularly treated with aminoglycoside and cyclosporine given frequently.RESULTS -Since the pharmacological treatment of lung transplant patients could influence metabolical regulation and renal function, the results are given separately for nontransplanted (n ϭ 29) and transplanted (n ϭ 9) CF patients. Nine patients (27%) had retinopathy, two of which had proliferative retinopathy and needed laser treatment. Lung transplantation did not affect the prevalence of retinopathy. In nontransplanted patients, nine had hypertension, three microalbuminuria, and one elevated creatinine. None had macroalbuminuria. In transplanted patients, eight of nine had hypertension, two had microalbuminuria, and none had macroalbuminuria. Seven of nine lung transplant patients had elevated plasma creatinine, and severely reduced glomerular filtration rate was significantly more frequent.CONCLUSIONS -A high frequency of diabetic retinopathy was found in patients with insulin-treated CF-related diabetes, stressing the need for a regular screening program as in type 1 diabetes. Severely impaired kidney function was common in lung transplant patients, probably secondary to cyclosporine treatment. Diabetes Care 29:2660 -2663, 2006C ystic fibrosis (CF) is the most frequent autosomal recessive inheritable disease in the Caucasian population in Denmark, affecting 1 in 4,700 children. The disease is characterized by recurring lung infections, reduced function of the exocrine pancreas, intestinal obstruction, and liver diseases. Median life expectancy is increasing, presently ϳ30 years. Diabetes occurs with increasing frequency as the patient grows older; ϳ50% have developed diabetes at age 30 years. The pathogenesis of CF-related diabetes is unknown.Previously, treatment of patients with CF-related diabetes did not aim for strict metabolic control due to the short life expectancy. However, ϳ20 cases of retinopathy, nephropathy, and neuropathy in CF patients with diabetes have been reported worldwide (1-6). Background and proliferative retinopathy in CF patients with diabetes was first reported in 1986 (1,2). Since then, at least four cases of proliferative retinopathy have been reported, with two cases leading to blindness (3). All cases had a poor metabolic control and associated nephropathy (1-3).Patients with CF are at risk of developing secondary renal disease as a result of nephrotoxic medications, primarily aminoglycosides and cyclo...

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Section: Other Complicationsmentioning

confidence: 99%

“…treatment with dialysis or kidney transplantation (13). Cyclosporine treatment might be the main reason for the drop in kidney function.…”

Section: Other Complicationsmentioning

confidence: 99%

Section: Diabetic Retinopathymentioning

confidence: 99%

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Cystic Fibrosis–Related Diabetes

et al. 2006

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“…54,72,163,187,250,283,287,288 En nuestra serie el tiempo medio de soporte extracorpóreo fue de 198 ± 115 (r:…”

Section: Influencia De La Cecunclassified

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

Deu

Romero

Román

et al. 2018

Archivos de Bronconeumología

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“…La longévité est encore prolongée par la réussite d'une transplantation pulmonaire. Le taux de survie à 5 ans des patients atteints d'une mucoviscidose est généralement plus élevé que celui des patients transplantés en raison d'autres maladies, probablement parce qu'ils sont plus jeunes et particulièrement s'ils sont également en bon état nutritionnel [59,60] .…”

Section: éVolution Cliniqueunclassified

Soins des patients atteints d’une mucoviscidose

Strandvik¹

2006

Ann Nestlé [Fr]

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La mucoviscidose, la maladie pédiatrique autosomique héréditaire la plus fréquente, n’atteint plus uniquement des enfants, car son traitement moderne a permis aux patients de vivre plus longtemps avec une meilleure qualité de vie. Voici plus de 16 ans, l’identification du gène responsable et de la mutation la plus fréquente, ΔF508, a suscité l’espoir général d’une guérison rapide de cette maladie. Ces découvertes ont motivé une forte intensification des recherches sur la physiopathologie, mais, à ce jour, les améliorations de la prise en charge clinique ont été totalement dues au développement des traitements traditionnels. L’amélioration de la situation des patients est liée à une meilleure supplémentation en enzymes pancréatiques et à un traitement nutritionnel plus intensif, en prêtant une attention particulière aux anomalies du métabolisme des lipides. Le traitement respiratoire a permis de mieux préserver la fonction respiratoire des patients adultes, grâce à l’accès à un ensemble d’antibiotiques efficaces et au développement de nouvelles strategies, à des mucolytiques ainsi que des incitations à l’activité physique dès le plus jeune âge en association à des modifications de la kinésithérapie et à l’amélioration des résultats de la transplantation pulmonaire en tant que traitement de dernier recours. Une atteinte hépatique est plus souvent reconnue et plus fréquemment traitée à long terme par l’acide ursodésoxycholique, bien que l’effet préventif de ce produit vis-à-vis de la progression de l’hépatopathie n’ait pas été totalement évalué. Le dépistage néonatal est de plus en plus fréquent, ce qui suscite de nouveaux problèmes à court terme, tels ceux de nature psychologique liés à la détection de porteurs ou de sujets atteints d’une forme très peu sévère, et tels que l’évaluation des résultats à long terme afin de démontrer que le statut clinique s’améliore et que la survie augmente encore. Les limitations de survie encore observées semblent principalement dues au mystère sous-tendant la colonisation chronique des voies aériennes par des micro-organismes.

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Long-term outcome of lung transplantation for cystic fibrosis ? Danish results

Cited by 38 publications

References 23 publications

Cystic Fibrosis–Related Diabetes

Cystic Fibrosis–Related Diabetes

El trasplante pulmonar en la fibrosis quística y la influencia de la circulación extracorpórea

Soins des patients atteints d’une mucoviscidose

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