scholarly journals Letter: Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency (Ann Rehabil Med 2020;44:165-70)

2020 ◽  
Vol 44 (5) ◽  
pp. 409-410
Author(s):  
Mikaela I. Poling ◽  
Craig R. Dufresne
Keyword(s):  
Pulmonary Function ◽  
Respiratory Insufficiency ◽  
Function Evaluation ◽  
Recurrent Pneumonia ◽  
Chronic Respiratory Insufficiency

scholarly journals Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency

2020 ◽  
Vol 44 (2) ◽  
pp. 165-170 ◽  
Author(s):  
Jihyun Park ◽  
Seong-Woong Kang ◽  
Won Ah Choi ◽  
Yewon Lee ◽  
Han Eol Cho
Keyword(s):  
Pulmonary Function ◽  
Respiratory Insufficiency ◽  
Function Evaluation ◽  
Functional Evaluation ◽  
Recurrent Pneumonia ◽  
Severe Scoliosis ◽  
Restrictive Lung Disease ◽  
Non Invasive ◽  
Chronic Respiratory Insufficiency

Freeman-Sheldon syndrome (FSS) is a rare distal arthrogryposis syndrome. There are few reports on the respiratory insufficiency of FSS. Additionally, there is no detailed information on pulmonary functional evaluation. A 17-year-old male patient with FSS developed respiratory failure, leading him to be admitted to hospital several times for evaluation and treatment. Of those times he was admitted, two were due to pneumonia. His pulmonary functions were indicative of a restrictive lung disease potentially caused by severe scoliosis. After a non-invasive ventilatorwas applied correctly to the patient, pulmonary hypertension was normalized. His pulmonary function has been maintained for 13 years. Since receiving proper respiratory care, which includes assisted coughing methods, the patient has not developed pneumonia. It is important to properly evaluate the pulmonary function of patients who have FSS and scoliosis to eliminate the risk of long-term respiratory complications.

scholarly journals Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis

2013 ◽  
Vol 71 (3) ◽  
pp. 146-152 ◽  
Author(s):  
Guilherme Fregonezi ◽  
Palomma Russelly Saldanha Araújo ◽  
Tathiana Lindemberg Ferreira Macêdo ◽  
Mario Emilio Dourado Junior ◽  
Vanessa Regiane Resqueti ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscle Strength ◽  
Early Diagnosis ◽  
Pulmonary Function ◽  
Healthy Subjects ◽  
Respiratory Muscle ◽  
Function Evaluation ◽  
Respiratory Muscle Strength ◽  
Respiratory Dysfunction ◽  
Lateral Sclerosis

ObjectiveIt was study the relationship between respiratory muscle strength and forced vital capacity (FVC) in patients with amyotrophic lateral sclerosis (ALS) versus healthy subjects.MethodsPulmonary function and respiratory muscle strength [maximal inspiratory (PImax), maximal expiratory (PEmax) and sniff nasal inspiratory pressure (SNIP)] were assessed in patients with ALS and healthy subjects, matched using cutoffs established in the literature for impaired pulmonary function and respiratory muscle weakness.ResultsTwenty-eight ALS patients and 28 healthy subjects were studied. We found sensitivity and specificity for PImax, PEmax and SNIP of 75/58%, 81/67% and 75/67%. The Receiver Operating Characteristic curve (ROC curve) indicated that the variables PImax, PEmax and SNIP can identify differences in respiratory muscle strength between ALS and healthy individuals at 0.89, 0.9 and 0.82, respectively. A positive correlation was recorded between FVC (%) versus SNIP, PImax and PEmax.ConclusionIn ALS, monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of FVC.

scholarly journals Dyspnea on Exercise Is Associated with Overall Symptom Burden in Patients with Chronic Respiratory Insufficiency

2021 ◽  
Vol 2 (1) ◽  
pp. 48-53
Author(s):  
Heidi A. Rantala ◽  
Sirpa Leivo-Korpela ◽  
Juho T. Lehto ◽  
Lauri Lehtimäki
Keyword(s):  
Respiratory Insufficiency ◽  
Symptom Burden ◽  
Chronic Respiratory Insufficiency

scholarly journals NOVOSTI V OBRAVNAVI PLJUČNE BOLEZNI PRI OTROCIH IN MLADOSTNIKIH S CISTIČNO FIBROZO

2015 ◽  
Vol 84 (2) ◽  
Author(s):  
Marina Praprotnik ◽  
Ana Kotnik Pirš ◽  
Barbara Salobir ◽  
Majda Oštir ◽  
Matjaž Turel ◽  
...  
Keyword(s):  
Cystic Fibrosis ◽  
Lung Transplantation ◽  
Lung Disease ◽  
Respiratory Insufficiency ◽  
Clinical Skills ◽  
Holistic Approach ◽  
Patient Registries ◽  
Recent Developments ◽  
Chronic Respiratory Insufficiency ◽  
Infection And Inflammation

Cystic fibrosis (CF) is a multi-organ disease,  affecting mostly lungs and gastrointestinal tract. Data from patient registries show that the survival of patients with CF has progressively improved over the past several decades, as a result of advances in antibiotic treatment, supplementation of pancreatic enzymes, better nutrition and a holistic approach to treatment in CF centres.The purpose of this review is to survey recent developments in the treatment of lung disease  in children and adolescents with CF.We describe newborn screening for CF.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue.Lung disease is the most common cause of morbidity and mortality in CF patients. Emerging new therapies are targeted at all points in the pathogenesis of lung disease, from drugs that treat infection and inflammation in the airways to gene transfer studies  and to drugs that augment airway surface liquid height. A number of antibacterial agents formulated for inhalation are at various stages of study and there are several anti-inflammatory candidate drugs in  clinical trials.  The most important development  in the recent years is  modulation of the abnormal protein that causes CF, the cystic fibrosis transmembrane regulator (CFTR), where drugs are targeted at specific defects in the transcription, processing or functioning.When chronic respiratory insufficiency occurs, lung transplantation becomes a very important issue. The role of the CF nurse, who has responsibilities in educating and teaching clinical skills to patients and families, is described.

scholarly journals Dyspnea is associated with overall symptom burden in patients with chronic respiratory insufficiency

2020 ◽  
Author(s):  
Heidi Anniina Rantala ◽  
Sirpa Leivo-Korpela ◽  
Juho T. Lehto ◽  
Lauri Lehtimäki
Keyword(s):  
Respiratory Insufficiency ◽  
Depression Scale ◽  
Symptom Burden ◽  
Symptom Assessment ◽  
Assessment System ◽  
Chronic Obstructive ◽  
Obstructive Pulmonary Disease ◽  
Edmonton Symptom Assessment System ◽  
Symptom Screening ◽  
Chronic Respiratory Insufficiency

Abstract Objective Patients with chronic respiratory insufficiency suffer from many symptoms together with dyspnea. We evaluated the association of dyspnea with other symptoms in patients with chronic respiratory insufficiency due to chronic obstructive pulmonary disease or interstitial lung disease. Results This retrospective study included 101 patients. Dyspnea was assessed with modified Medical Research Council dyspnea questionnaire (mMRC) and other symptoms with Edmonton Symptom Assessment System (ESAS) and Depression Scale (DEPS). Patients with mMRC 4 (most severe dyspnea) compared to those with mMRC 0–3 reported higher median (IQR) symptom scores on ESAS in e.g. dry mouth (7.0 (4.0–8.0) vs. 3.0 (1.0–6.0), P < 0.001), tiredness (6.0 (3.0–7.0) vs. 3.0 (1.0–5.0), P < 0.001) and anxiety (3.0 (0.0-5.5) vs. 1.0 (0.0–3.0), P = 0.007). Patients with mMRC 4 were more likely to reach the DEPS threshold for depression compared to those with mMRC 0–3 (42.1% vs. 20.8%, P = 0.028). In conclusion, patients with chronic respiratory insufficiency need comprehensive symptom screening with relevant treatment, as they suffer from many severe symptoms worsening with increased dyspnea.

scholarly journals Non-invasive respiratory unit in the Hungarian health care system

2012 ◽  
Vol 153 (23) ◽  
pp. 918-921 ◽  
Author(s):  
András Lorx ◽  
Dóra Bartusek ◽  
György Losonczy ◽  
János Gál
Keyword(s):  
Health Care ◽  
Intensive Care ◽  
Hospital Stay ◽  
Respiratory Insufficiency ◽  
First Year Experience ◽  
First Year ◽  
Non Invasive ◽  
High Dependency ◽  
Chronic Respiratory Insufficiency ◽  
General Wards

Treating patients with acute or chronic respiratory insufficiency still poses a major load on the healthcare system. Though there is evidence that treating these patients in high dependency respiratory units results in a shortening of hospital stay, reduces the need of intubation, and decreases mortality. In the Hungarian routine these patients are treated in general wards until the development of global respiratory insufficiency, when they are transferred to intensive care units. The authors present their first year experience on their novel Non-invasive Respiratory Unit established at Semmelweis University. Orv. Hetil., 2012, 153, 918–921.

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