The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis

Tilanus, T. B. M.; Groothuis, Jan T.; TenBroek-Pastoor, J. M. C.; Feuth, Ton; Heijdra, Yvonne F.; Slenders, J. P. L.; Doorduin, Jonne; Engelen, Baziel G. van; Kampelmacher, M. J.; Raaphorst, Joost

doi:10.1186/s12931-017-0624-8

Cited by 66 publications

(52 citation statements)

References 32 publications

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“…To date, no precise indicators have been established to optimize PEG placement time, although some scales and functional parameters have already been tested [ 23 ]. In line with what has been established as indicators for the onset of ventilatory support, namely the presence of inherent symptoms to hypoventilation and functional impairment with FVC < 70% and PImax < 60 cmH 2 O, a CPF < 300 L/min was also suggested as a good indicator of the need for short-term ventilatory support, and CPF < 270 L/min as indicator for cough assistance [ 9 , 24 ]. In this study, we now present a functional and clinical indicator for PEG placement, with 100% S if ALSFRS-R-B ≤ 8 and 100% E if ALSFRS-R-B ≤ 6, in the same line as already described by Rooney et al [ 13 ], regarding its discriminatory power in spinal and bulbar phenotypes.…”

Section: Discussionsupporting

confidence: 52%

Functional and Endoscopic Indicators for Percutaneous Endoscopic Gastrostomy (PEG) in Amyotrophic Lateral Sclerosis Patients

Condé

Martins

Rodrigues

et al. 2018

JCM

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(1) Background: Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative condition, whose bulbar involvement compromises language, swallowing, and airway protection. When oral nutrition is no longer adequate, percutaneous endoscopic gastroscopy (PEG) may be indicated. However, as exact timing is still debatable, we tried to find it. (2) Methods: A prospective cohort study was performed using fiber-optic endoscopic evaluation of swallowing (FEES), functional evaluation scales (ALS Functional Rating Scale-Revised (ALSFRS-R) and bulbar sub-score (ALSFRS-R-B)), lung function tests (like Forced Vital Capacity (FVC), Cough Peak Flow (CPF)) and anthropometric data. (3) Results: Twenty-three patients were enrolled (mean 65.4 ± 9.1 years, 60.9% males), 12 with spinal-onset. During the study period, 58 FEES were performed (1–4/patients). Even before formal PEG indication, suggestions were given to correct the alterations found. PEG was placed in 12 patients, on average 21.8 months after diagnosis (FVC = 69.9% ± 26.7%, ALSFRS-R-B = 7.7 ± 3.7, ALSFRS-R = 28.9 ± 12.3), and being 91.7% under ventilatory support. ALSFRS-R-B, CPF, FVC, and ALSFRS-R showed significant discriminant ability for PEG placement. Sensitivity and specificity were, respectively, ALSFRS-R-B ≤ 8 (100/90.9), CPF ≤ 205 (83.3), FVC ≤ 74 (83.3/74.2), and ALSFRS-R < 29 (83.3/65.1). (4) Conclusions: FEES provide additional information beyond formal PEG indication. ALSFRS-R-B score ≤ 8 was found as a best functional and noninvasive indicator for PEG placement in ALS patients.

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Section: Discussionsupporting

confidence: 52%

Functional and Endoscopic Indicators for Percutaneous Endoscopic Gastrostomy (PEG) in Amyotrophic Lateral Sclerosis Patients

Condé

Martins

Rodrigues

et al. 2018

JCM

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“…Hypoxia and hypoxemia can strongly stimulate the immune system in ALS patients 54 , an activation of the immune systeme due to declining respiratory function can therefore not be excluded. However declining respiratory function also represents ALS disease progression 55 . It is therefore challenging to conclude whether the Th1/Th17 shift is a side effect following progressing neurodegeneration due to the accumulation of toxic products or a pathomechanistic e.g.…”

Section: Discussionmentioning

confidence: 99%

Peripheral proinflammatory Th1/Th17 immune cell shift is linked to disease severity in amyotrophic lateral sclerosis

Jin

Günther

Akgün

et al. 2020

Sci Rep

110

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Neuroinflammation is involved in the pathogenesis of amyotrophic lateral sclerosis (ALS), but only limited data are available on systematic peripheral and central immune cell profiles in ALS. We studied detailed immune profiles of 73 ALS patients and 48 healthy controls (controls) in peripheral blood by fluorescence-activated cell sorting as well as cytokine expression profiles in serum. In a subgroup of 16 ALS patients and 10 controls we additionally studied cerebrospinal fluid (CSF) samples. In peripheral blood, T cell subtypes presented a shift towards pro-inflammatory Th 1 and Th 17 cells whereas anti-inflammatory Th2 and T regulatory cells were decreased. Important players in innate immunity including distinct monocyte (Mo) and natural killer (NK) cell subtypes were changed in ALS patients compared to controls. Pro-inflammatory serum cytokines such as interleukin (IL)-1 beta, IL-6 and interferon-gamma (IFN-gamma) were increased and the anti-inflammatory cytokine IL-10 was decreased. Correlation analysis revealed moderate negative correlations between Th1 and Th17 to the ALS functional rating scale revised (ALSFRS-R) and to forced vital capacity. In CSF samples, no relevant alteration of the immune profile was found. In conclusion, the immune profile in ALS was shifted towards a Th1/Th17 cell-mediated pro-inflammatory immune response and correlated to disease severity and progression. Large prospective studies are needed to confirm these findings. Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease, which affects the upper and lower motoneurons (MN) leading to progressive muscle weakness, paralysis and ultimately death due to respiratory failure. The pathophysiological mechanisms are still unresolved, although increasing knowledge gained in the last two decades. Multiple studies corroborated that numerous pathological processes are involved in MN degeneration and neuroinflammation gained increasing attention and had deemed as a pathological hallmark of ALS 1-3. It is commonly accepted that the non-neuronal neighbors play a significant role in the degenerative process of this vulnerable cell type 4. One of the most impressive pathohistological characteristics during disease progression is activation and increase abundance of microglial cells in the central nervous system (CNS) shown in animal models of ALS 5 as well as in human studies 6-8. Microglial cells are considered to be the resident mononuclear phagocytes in the CNS and can be renewed by bone marrow cells under special circumstances 9,10. It is supposable that specialized peripheral immune cell subsets are attracted to the central compartment to contribute and perpetuate the pathological processes in ALS disease by local inflammation or activation of resident cells. Therefore, neuroinflammation is not limited to the CNS, it needs a systemic inflammatory response to enable the local inflammation. In general, a systemic inflammatory response is very complex, a plethora of different immune cells are involved, diverse cytokines a...

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“…It started in the 60s with the predictive studies performed by Black et al 2,3 for the values of Maximal Inspiratory Pressure (MIP) and Maximal Expiratory Pressure (MEP). Since then, other authors have conducted researches of the MRP in different countries with healthy people [4][5][6] and in pathological conditions 7,8 . Considering the studies with breast cancer patients, scarce literature reporting postoperative strength assessments of the respiratory muscles in post-surgery [8][9] and after radiotherapy [10][11][12][13][14] were found.…”

Section: Introductionmentioning

confidence: 99%

Estudo das Pressões Respiratórias Máximas após Exposição à Radioterapia de Mama em Mulheres: um Estudo-Piloto

Ávila

Ferraz

Barbosa

et al. 2020

Rev. Brasileira.De.Cancerologia

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Introdução: No câncer de mama, a radioterapia pós-operatória é amplamente usada para reduzir a incidência de recorrência local da doença. Entretanto, a irradiação das estruturas torácicas implica riscos, especialmente para os pulmões. Objetivo: Estudar as pressões respiratórias máximas (PRM) após exposição à radioterapia de mama em mulheres. Método: Estudo prospectivo observacional realizado no Hospital de Câncer Alfredo Abrão em Campo Grande, estado de Mato Grosso do Sul – MS. A amostra deste estudo foi composta por mulheres (N=8) expostas à radioterapia de mama após cirurgia de quadrantectomia. Avaliou-se a força dos músculos respiratórios por meio da pressão inspiratória máxima (Pimáx) e pressão expiratória máxima (Pemáx), utilizando-se do aparelho portátil denominado manovacuômetro. O exame foi realizado antes da primeira sessão de radioterapia e após a 25ª sessão, correspondendo ao último dia de tratamento radioterápico. Também foram avaliados o peso e a altura para medir o índice de massa corporal, os sintomas respiratórios clínicos de dispneia com o uso da Escala de Dispneia Medical Research Council e a caracterização de tosse com os Critérios Comuns de Toxicidade Pulmonar, em classificação da pneumonite clínica. Resultados: Os valores da Pimáx resultaram em 95,90±23,86 e 81,20±23,12 (média±desvio padrão da média - p=0,035) em relação ao percentual ideal, caracterizando diminuição significativa ao se comparar antes e após a exposição à radioterapia. Observou-se nível de significância de p<0,05, teste t-student e pareado. Conclusão: O estudo das PRM antes e após a exposição à radioterapia de mama evidenciou diminuição significativa da Pimáx.

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The predictive value of respiratory function tests for non-invasive ventilation in amyotrophic lateral sclerosis

Cited by 66 publications

References 32 publications

Functional and Endoscopic Indicators for Percutaneous Endoscopic Gastrostomy (PEG) in Amyotrophic Lateral Sclerosis Patients

Functional and Endoscopic Indicators for Percutaneous Endoscopic Gastrostomy (PEG) in Amyotrophic Lateral Sclerosis Patients

Peripheral proinflammatory Th1/Th17 immune cell shift is linked to disease severity in amyotrophic lateral sclerosis

Estudo das Pressões Respiratórias Máximas após Exposição à Radioterapia de Mama em Mulheres: um Estudo-Piloto

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