Annals of Rehabilitation Medicine

Response: 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): Jihyun Park, Seong-Woong Kang, Won Ah Choi, Yewon Lee, Han Eol Cho; Ann Rehabil Med 2020;44(5):411-413. Published online October 29, 2020; DOI: https://doi.org/10.5535/arm.20110.R

PDF

4,084 View
72 Download

Precise Pulmonary Function Evaluation and Management of a Patient With Freeman-Sheldon Syndrome Associated With Recurrent Pneumonia and Chronic Respiratory Insufficiency: Jihyun Park, Seong-Woong Kang, Won Ah Choi, Yewon Lee, Han Eol Cho; Ann Rehabil Med 2020;44(2):165-170. Published online April 29, 2020; DOI: https://doi.org/10.5535/arm.2020.44.2.165

Abstract
PDF

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.

Citations

Citations to this article as recorded by

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)
Mikaela I. Poling, Craig R. Dufresne
Annals of Rehabilitation Medicine.2020; 44(5): 409. CrossRef
Response: 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)
Jihyun Park, Seong-Woong Kang, Won Ah Choi, Yewon Lee, Han Eol Cho
Annals of Rehabilitation Medicine.2020; 44(5): 411. CrossRef

6,050 View
161 Download
2 Web of Science
2 Crossref

Improvement of Peak Cough Flow After the Application of a Mechanical In-exsufflator in Patients With Neuromuscular Disease and Pneumonia: A Pilot Study: Ji Ho Jung, Hyeon Jun Oh, Jang Woo Lee, Mi Ri Suh, Jihyun Park, Won Ah Choi, Seong-Woong Kang; Ann Rehabil Med 2018;42(6):833-837. Published online December 28, 2018; DOI: https://doi.org/10.5535/arm.2018.42.6.833

Abstract
PDF

Objective
To investigate and demonstrate persistent increase of peak cough flow after mechanical in-exsufflator application, in patients with neuromuscular diseases and pneumonia.
Methods
A mechanical in-exsufflator was applied with patients in an upright or semi-upright sitting position (pressure setting, +40 and −40 cmH2O; in-exsufflation times, 2–3 and 1–2 seconds, respectively). Patients underwent five cycles, with 20–30 second intervals to prevent hyperventilation. Peak cough flow without and with assistive maneuvers, was evaluated before, and 15 and 45 minutes after mechanical in-exsufflator application.
Results
Peak cough flow was 92.6 L/min at baseline, and 100.4 and 100.7 L/min at 15 and 45 minutes after mechanical in-exsufflator application, respectively. Assisted peak cough flow at baseline, 15 minutes, and 45 minutes after mechanical in-exsufflator application was 170.7, 179.3, and 184.1 L/min, respectively. While peak cough flow and assisted peak cough flow increased significantly at 15 minutes after mechanical in-exsufflator application compared with baseline (p=0.030 and p=0.016), no statistical difference was observed between 15 and 45 minutes.
Conclusion
Increased peak cough flow after mechanical in-exsufflator application persists for at least 45 minutes.

Citations

Citations to this article as recorded by

Pearls and pitfalls of respiratory testing in a patient with amyotrophic lateral sclerosis and COPD
Stephen W. Littleton, Franco Laghi
Breathe.2023; 19(2): 230043. CrossRef
The use of cough peak flow in the assessment of respiratory function in clinical practice- A narrative literature review
M. Brennan, M.J. McDonnell, N. Duignan, F. Gargoum, R.M. Rutherford
Respiratory Medicine.2022; 193: 106740. CrossRef
Comparison of two mechanical insufflation-exsufflation devices in patients with amyotrophic lateral sclerosis: a preliminary study
Antonello NICOLINI, Paola PRATO, Laura BECCARELLI, Bruna GRECCHI, Giancarlo GARUTI, Paolo BANFI, Francesco D’ABROSCA
Panminerva Medica.2022;[Epub] CrossRef
Analysis of Pneumothorax in Noninvasive Ventilator Users With Duchenne Muscular Dystrophy
Han Eol Cho, Justin Byun, Won Ah Choi, Myungsang Kim, Kyeong Yeol Kim, Seong-Woong Kang
Chest.2021; 159(4): 1540. CrossRef

6,634 View
168 Download
5 Web of Science
4 Crossref

Long-Term Outcome of Amyotrophic Lateral Sclerosis in Korean Subjects: Mi Ri Suh, Won Ah Choi, Young-Chul Choi, Jang Woo Lee, Jung Hwa Hong, Jihyun Park, Seong-Woong Kang; Ann Rehabil Med 2017;41(6):1055-1064. Published online December 28, 2017; DOI: https://doi.org/10.5535/arm.2017.41.6.1055

Abstract
PDF

Objective

To report the latest long-term outcome of amyotrophic lateral sclerosis (ALS) and to analyze the predictors of prognosis.

Methods

Subjects who were diagnosed with ALS between January 2005 and December 2009 at a single institute were followed up until death or up to December 2014. Data regarding age, sex, date of onset, date of diagnosis, presence of bulbar symptoms on onset, date of initiation of non-invasive ventilation (NIV), and the date of tracheostomy were collected. Survival was assessed using Kaplan-Meier curves and multivariate analyses of the risk of death were performed using the Cox proportional hazards model.

Results

Among 212 suspicious subjects, definite ALS was diagnosed in 182 subjects. The survival rate at 3 and 5 years from onset was 61.5% and 40.1%, respectively, and the survival rate at 3 and 5 years post-diagnosis was 49.5% and 24.2%, respectively. Further, 134 patients (134/182, 73.6%) were initiated on NIV, and among them, 90 patients (90/182, 49.5%) underwent tracheostomy. Male gender and onset age of ≥65 years were independent predictors of adverse survival.

Conclusion

The analysis of long term survival in ALS showed excellent outcomes considering the overall poor prognosis of this disease.

Citations

Citations to this article as recorded by

A fact‐finding survey of medical care provided to neuromuscular disease patients at the National Center of Neurology and Psychiatry in Japan
Akiko Hanai, Keisuke Yorimoto, Ryo Ohkubo, Tadashi Tsukamoto, Katsuhiro Mizuno, Yuji Takahashi
Neurology and Clinical Neuroscience.2023; 11(1): 32. CrossRef
Interleukin 6 (IL6) level is a biomarker for functional disease progression within IL6R358Ala variant groups in amyotrophic lateral sclerosis patients
Marlena Wosiski-Kuhn, James B. Caress, Michael S. Cartwright, Gregory A. Hawkins, Carol Milligan
Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration.2021; 22(3-4): 248. CrossRef
What is the Adequate Cuff Volume for Tracheostomy Tube? A Pilot Cadaver Study
Dong Min Kim, Myung Jun Shin, Sung Dong Kim, Yong Beom Shin, Ho Eun Park, Young Mo Kim, Jin A Yoon
Annals of Rehabilitation Medicine.2020; 44(5): 402. CrossRef