Introduction Chronic diseases that may lead to restrictive ventilatory dysfunction are characterised by reduced lung volume either because of an alteration in lung parenchyma or because of a disease of the pleura, chest wall or neuromuscular apparatus.
Aim to asses the role of spirometry in managing chronic diseases associated with a decline of FVC in order to establish the moment of therapeutic intervention (surgery or continuous positive pressure ventilation) for improving the quality of life of the patients.
Method A retrospective study conducted at ‘Dr. Victor Gomoiu’ Children’s Hospital including 46 hospitalised patients. For all patients 74 spirometries were made between ianuary 2015 to december 2016. The patients had chronic diseases that in time may lead to a restrictive pattern, defined as a decline of FVC under 80% predicted. We classified the study group in three categories: neuromuscular disorders (Muscular Dystrophies, Spinal Amiotrophy and Myasthenia gravis) – 30 patients, rare syndromes (Sdr McLeod, Sdr Dejerine Sotos, Mucopolysaccharidoses, etc) −10 patients and rib cage deformities – 6 patients.
Results The study group included 34 boys and 12 girls with ages ranging from 4 to 17 years old.
Spirometry was normal at entry with a median FCV 88% from predicted, whereas the maximal FVC was 119.4% from predicted and the minimal FCV was 33% from predicted.
41.3% of the patients had restrictive ventilatory dysfunction and that means 12 patients from the group of neuromuscular disorders, 6 patients from the group of rare syndromes and one patient from the group with chest wall deformities.
The median of age in the study group was 11 years old, similar to that of the nineteenth children with restrictive ventilatory dysfunction.
Eight children made several spirometries during the study and we observed a decline of 11% FVC/year whereas the second spirometry showed a median FVC 65% from predicted.
Conclusions Periodically monitoring of the lung function by a personalised plan is being required in managing treatment options or paliative care for patients with chronic diseases that may lead to restrictive pulmonary dysfunction.
Spirometry is very important especially for patients with neuromuscular disorders because they have a more rapidly decline in FCV. As shown in our study, the rate of decline in respiratory function corelates with the neuromuscular pathology.
- restrictive ventilatory dysfunction
- neuromuscular disorders
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