Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis

Staufer, Katharina; Halilbasic, Emina; Hillebrand, Peter; Harm, S.; Schwarz, Štefan; Jaksch, Péter; Kivaranovic, Danijel; Klepetko, Walter; Trauner, Michael; Kazemi-Shirazi, Lili

doi:10.1177/2050640618778381

“…Nonetheless, a significant proportion of patients still fail to achieve normal growth and good nutritional status. In other studies, Colombo [27] and Staufer [28] remarked the importance of nutritional status as an important prognostic factor in patients with CF prior to LT. We found a significant difference in HbA1c among the three groups, with a worse metabolic profile in LRs. is is probably due to the role played by immunosuppressants in both renal function decline and metabolic profile derangement, leading to a greater cardiovascular risk.…”

Section: Discussionmentioning

confidence: 93%

Adult Patients Affected by Cystic Fibrosis in Therapy with Cystic Fibrosis Transmembrane Regulator Modulators and Lung Transplant: Renal Function, Metabolic and Nutritional Status

Lai

¹

,

Mazzaferro

²

,

Mitterhofer

³

et al. 2020

Journal of Nutrition and Metabolism

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Background. Cystic fibrosis (CF) is one of the most frequent genetic diseases. The discovery and implementation of new therapies prolonged the survival of CF patients in the last years. Evaluation of long-term complications could be useful to improve the outcome of these patients. Aim of the Study. To evaluate renal function, metabolic, nutritional, and inflammatory status in CF patients on cystic fibrosis transmembrane regulator (CFTR) modulators therapy as well as lung transplant recipients (LRs) and patients on conservative therapy (control group). Materials and Methods. We performed a prospective, longitudinal study on 69 CF patients. Clinical and laboratory parameters (metabolic and nutritional indices and inflammatory markers) were evaluated in all patients before starting CFTR therapy or transplant (T0) and after 3 years (T1). Results. We enrolled 69 CF patients (42 males). Patients were distributed into three groups. The average age was 35.01 ± 10.57 years for the control group (group 0), 32.47 ± 9.40 years for patients on CFTR modulators therapy (group 1), and 38.93 ± 7.14 years for LRs (group 2). At T1, we showed a significant difference among the three groups in terms of renal function indices: creatinine, eGFR, serum nitrogen as well as serum uric acid, sodium, and potassium ( p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , p < 0.001 , and p < 0.001 , respectively), particularly in LRs patients. Significant differences were found in nutritional status parameters among the three groups: total protein, serum albumin, serum fibrinogen, serum transferrin, and white blood cell counts ( p < 0.001 , p = 0.037 , p = 0.04 , p = 0.003 , and p = 0.007 , respectively), particularly in LRs compared with other groups. Moreover, we found significant differences in metabolic profile (HbA1c, p = 0.026 ) and inflammatory status, with a significant difference in C-reactive protein values, neutrophil counts, and neutrophil-lymphocyte ratio (NLR) among the three groups ( p < 0.001 , p = 0.005 , and p = 0.026 , respectively). Conclusions. Our study showed a reduced renal function and poor nutritional status in LRs, along with worse metabolic control. Moreover, we showed a lower inflammatory status in patients on CFTR modulators therapy. Therefore, we suggest early and careful monitoring of renal function, metabolic, and nutritional parameters in CF patients, whether they are on conservative therapy, CFTR modulators therapy, and LRs patients.

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“…Primary outcome measures included MMT-8 [20] and Functional Index-2 (FI-2) [20]. Secondary outcome measures included the Health Assessment Questionnaire (HAQ) [20], Medical Outcomes Study 36-item Short Form Health Survey (SF-36) [20,21], Fatigue Impact Scale (FIS) [22], Beck's Depression Inventory-II (BDI-II) [23], force vector area (FVA) assessed by indirect static posturography [24], basal metabolic rate (BMR) [25], and muscle fitness (extracellular mass to body cell mass ratio [ECM/BCM]) assessed by bioelectric impedance [26,27]. Safety was assessed by (a) Borg Category-Ratio (CR)-10 perceived exertion scale [28,29]; (b) erythrocyte sedimentation rate (ESR), serum C-reactive protein (CRP), creatine kinase (CK), lactate dehydrogenase (LD), myoglobin, and selected inflammatory cytokines (interleukin [IL]-1β, IL-6, tumor necrosis factor [TNF]) or chemokines (IL-8, monocyte chemoattractant protein-1 [MCP-1, CCL2]) which play a substantial role in the pathophysiology of IIM [1,3,[30][31][32][33][34]; and (c) mRNA expression of these cytokines/chemokines assessed in the muscle biopsy of volunteers from the IG (n = 7) at week 0 and 24.…”

Section: Outcome Measuresmentioning

confidence: 99%

The effect of a 24-week training focused on activities of daily living, muscle strengthening, and stability in idiopathic inflammatory myopathies: a monocentric controlled study with follow-up

Špiritović

¹

,

Heřmánková

²

,

Oreská

³

et al. 2021

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Background The structural and functional changes of the skeletal muscles in idiopathic inflammatory myopathies (IIM) caused by inflammation and immune changes can be severely disabling. The objective of this study was to assess the effect of a 24-week program combining a supervised training of activities of daily living (ADL), resistance, and stability with home exercise for improving muscle function, compared to a daily home-based exercise representing the regular outpatient care. Methods Fifty-seven patients with IIM were consecutively and non-selectively enrolled in an intervention (IG, n = 30) or control (CG, n = 27) group. Both groups were provided a standard-of-care pharmacological treatment and follow-up. Only the IG underwent the supervised intervention twice a week for 1 h per session. At baseline, 12, 24, and 48 weeks, all patients were assessed by an assessor blinded to the intervention for primary outcomes: muscle strength (Manual Muscle Testing of eight muscle groups [MMT-8]) and endurance (Functional Index-2 [FI-2]), and secondary outcomes: stability and body composition. Secondary outcomes also included questionnaires evaluating disability (Health Assessment Questionnaire [HAQ]), quality of life (Short Form 36 [SF-36]), depression (Beck’s Depression Inventory-II [BDI-II]), and fatigue (Fatigue Impact Scale [FIS]), and analysis of the systemic and local inflammatory response and perceived exertion to assess the safety of the intervention. Results Twenty-seven patients in the IG and 23 in the CG completed the entire program and follow-up. At week 24, compared to deterioration in the CG, we found a significant improvement in the IG in muscle strength (mean % improvement compared to baseline by 26%), endurance (135%), disability (39%), depression (26%), stability (11%), and basal metabolism (2%) and a stabilization of fitness for physical exercise. The improvement was clinically meaningful (a 24-week change by >20%) in most outcomes in a substantial proportion of patients. Although the improvement was still present at 48 weeks, the effect was not sustained during follow-up. No significant increase in the systemic or local expression of inflammatory markers was found throughout the intervention. Conclusions This 24-week supervised intervention focused on ADL training proved to be safe and effective. It not only prevented the progressive deterioration, but also resulted in a significant improvement in muscle strength, endurance, stability, and disability, which was clinically meaningful in a substantial proportion of patients. Trial registration ISRCTN35925199 (retrospectively registered on 22 May 2020).

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“…In patients with cystic fibrosis, weight loss often occurs secondary to maldigestion, malabsorption, and associated malnutrition. Malnutrition in patients with cystic fibrosis is reportedly associated with worse survival and pulmonary function after LT [ 31 , 32 ]. Whether the low BMI in patients with PPFE results from malnutrition and affects post-LT outcomes should be investigated in further studies.…”

Section: Clinical Characteristics Of Ppfementioning

confidence: 99%

Lung Transplantation for Pleuroparenchymal Fibroelastosis

Shiiya

¹

,

Sato

²

2021

JCM

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Pleuroparenchymal fibroelastosis (PPFE), a new disease entity associated with interstitial pneumonia, is characterized by fibrosis and elastosis involving the pleura and subpleural lung parenchyma, predominantly in the upper lobe. As the awareness of this disease entity has increased, many studies have revealed the prevalence and incidence, clinical and pathological characteristics, and disease course of PPFE. Patients with PPFE reportedly have several unique clinical characteristics—including an extremely low body mass index with a slender body and chest wall deformity, known as “flat chest”. As this disease progresses, shrinking of the lungs often causes life-threatening complications, such as pneumothorax, and associated air leak syndrome. Lung transplantation is considered the only effective treatment for patients with advanced PPFE; however, little is known about the influences of the characteristics of PPFE on the outcome of lung transplantation. This review focuses on the unique clinicopathologic characteristics of PPFE and associated outcomes of lung transplantation for these patients.

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Impact of nutritional status on pulmonary function after lung transplantation for cystic fibrosis

Cited by 18 publications

References 30 publications

Adult Patients Affected by Cystic Fibrosis in Therapy with Cystic Fibrosis Transmembrane Regulator Modulators and Lung Transplant: Renal Function, Metabolic and Nutritional Status

Adult Patients Affected by Cystic Fibrosis in Therapy with Cystic Fibrosis Transmembrane Regulator Modulators and Lung Transplant: Renal Function, Metabolic and Nutritional Status

The effect of a 24-week training focused on activities of daily living, muscle strengthening, and stability in idiopathic inflammatory myopathies: a monocentric controlled study with follow-up

Lung Transplantation for Pleuroparenchymal Fibroelastosis

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