scholarly journals Macular sub-layer thinning and association with pulmonary function tests in Amyotrophic Lateral Sclerosis

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Joseph M. Simonett ◽  
Russell Huang ◽  
Nailah Siddique ◽  
Sina Farsiu ◽  
Teepu Siddique ◽  
...  
2015 ◽  
Vol 77 (3-4) ◽  
Author(s):  
B. Chandrasoma ◽  
D. Balfe ◽  
T. Naik ◽  
A. Elsayegh ◽  
M. Lewis ◽  
...  

Background. Amyotrophic Lateral Sclerosis (ALS) is a progressive neurodegenerative disorder affecting both the upper and lower motor neurons. Deteriorating pulmonary function as a reflection of progressive respiratory muscle weakness is a common feature, accounting for the majority of deaths. The aim of the study was to describe a trend in initial pulmonary function tests (PFT) of Amyotrophic Lateral Sclerosis (ALS) patients, in addition, differentiating between the types of disease onset, bulbar, limb muscle, and a combination. Methods. Initial PFT were gathered from 32 consecutive patients in our clinic with the diagnosis of ALS, they were categorized by the type of disease onset. Values obtained were referenced to the 95% confidence limits for normality. Results. There was evidence of significant reductions in both the FEV1 (64.7% predicted) and FVC (61.2%), with preservation of the FEV1/FVC (81.7%). The MVV was significantly reduced(43%). Total lung capacity was 93.2%, the residual volumes was increased at 145.7%. Subgroup analysis failed to show significant differences between types of disease onset. In the bulbar onset group (versus the limb group) there was a trend for the MVV to be further reduced (p=0.15) and the RV to be higher (157.4% versus 135.9%, P=0.24). Conclusions. ALS is a devastating disease that invariably leads to respiratory failure. Abnormal spirometric variables such as the FVC and MVV, likely reflect inspiratory muscle weakness and increased RV likely reflect expiratory muscle weakness. The type of disease onset did not result in a different pattern of PFT abnormalities.


2018 ◽  
Vol 6 (3) ◽  
pp. 16-19
Author(s):  
Gajanan V Patil ◽  
◽  
Atish Pagar ◽  
U S Patil ◽  
M K Parekh ◽  
...  

2013 ◽  
Vol 9 (1) ◽  
pp. 3-10
Author(s):  
Linus Grabenhenrich ◽  
Cynthia Hohmann ◽  
Remy Slama ◽  
Joachim Heinrich ◽  
Magnus Wickman ◽  
...  

2005 ◽  
Vol 37 (4) ◽  
pp. 550-556
Author(s):  
MELISSA R. MAZAN ◽  
EDWARD P. INGENITO ◽  
LARRY TSAI ◽  
ANDREW HOFFMAN

CHEST Journal ◽  
2008 ◽  
Vol 134 (4) ◽  
pp. 49S
Author(s):  
Ibrahim H. Abou Daya ◽  
Muhammad U. Anwer ◽  
Gilda Diaz-Fuentes ◽  
Steve Blum ◽  
Latha Menon

Lupus ◽  
2021 ◽  
pp. 096120332110103
Author(s):  
Alfonso Ragnar Torres Jimenez ◽  
Nayma Ruiz Vela ◽  
Adriana Ivonne Cespedes Cruz ◽  
Alejandra Velazquez Cruz ◽  
Alma Karina Bernardino Gonzalez

Shrinking Lung Syndrome (SLS) is a rare and little known complication associated with Systemic Lupus Erythematosus (SLE), characterized by progressive and unexplainable dyspnea, pleuritic pain, small pulmonary volumes and elevation of the diaphragm on chest X-rays as well as restrictive pattern on pulmonary function tests. Objective To describe clinical, radiological and treatment characteristics in pediatric patients with SLS. Material and methods This is a descriptive and retrospective study in patients under 16 years old with the diagnosis of SLE complicated by SLS at the General Hospital. National Medical Center La Raza. Clinical, radiological and treatment variables were analyzed. Results are shown in frequencies and percentages. Results Data from 11 patients, 9 females and 2 males were collected. Mean age at diagnosis of SLS was 12.2 years. Age at diagnosis of SLE was 11.1 years. SLEDAI 17.3. Renal desease 72%, hematological 91%, lymphopenia 63%, mucocutaneous 72%, neurological 9%, arthritis 54%, serositis 91%, fever 81%, secondary antiphospholipid syndrome, low C3 72%, low C4 81%, positive ANA 91%, positive anti-DNA 91%. Regarding clinical manifestations of SLE: cough 81%, dyspnea 91%, hipoxemia 81%, pleuritic pain 71%, average oxygen saturation 83%. Chest X-rays findings: right hemidiaphragm affection 18%, left 63%, bilateral 18%. Elevated hemidiaphragm 91%, atelectasis 18%, pleural effusion 91%, over one third of the cardiac silhouette under the diphragm 36%, bulging diaphragm 45%, 5th. anterior rib that crosses over the diaphragm 91%. M-mode ultrasound: diaphragmatic hypomotility 100%, pleural effusion 63%. Pulmonary function tests: restrictive pattern in 45% of the cases. Treatment was with supplementary oxygen 100%, intubation 18%, antibiotics 100%, steroids 100%, intravenous immunoglobulin 54%, plasmapheresis 18%, cyclophosphamide 54% and rituximab 18%. The clinical course was favorable in 81%. Conclusions SLS should be suspected in patients with SLE and active disease who present hipoxemia, pleuritic pain, cough, dyspnea, pleural effusion and signs of restriction on chest X-rays. Therefore, a diaphragmatic M-mode ultrasound should be performed in order to establish the diagnosis.


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