EVALUATION OF THE RESPIRATORY SYSTEM DURING FATIGUE TEST AND
OXIDATIVE STRESS IN INDIVIDUAL& WITH CONGENITAL GENERALIZED LIPODYSTROPHY
fatigue, respiratory system and congenital generalized lipodystrophy
INTRODUCTION: Congenital generalized lipodystrophy (LGC) or Berardinelli-Seip Syndrome is an
autosomal recessive genetic syndrome, inbred, ultra rare, characterized by the absence of adipose
tissue, first reported in Brazil in 1954. In the state of Rio Grande do Norte, Northeastern Brazil, there
is a high prevalence of the syndrome, with pulmonary infections being the main cause of death in
these patients. The previously published literature demonstrates that the respiratory system could
be impacted by the disease, however, no aspect of the respiratory system has been systematically
studied. OBJECTIVES: To assess potential changes in the respiratory system in subjects with LGC and a
healthy group during inspiratory muscle fatigue testing. METHODOLOGY: The research is a crossover
study with a quantitative approach, in which individuals with LGC and healthy of both genders will be
studied. A pilot study was carried out with two healthy subjects to test the feasibility of the respiratory
fatigue test protocol. The subjects were initially submitted to pulmonary function tests and respiratory
muscle strength assessment. Then 10 nasal inspiratory pressure (SNIP) maneuvers were performed
before the test, followed by the endurance test and 10 post SNIP maneuvers. The endurance test was
performed with a linear pressure load valve at 80% of the maximum inspiratory pressure (PIMáx).
During the protocol, the subject was monitored by optoelectronic plethysmography, surface
electromyography and by near infrared spectroscopy (NIRS) to assess tissue oxygenation of the
stemocleidomastoid muscle.
From the SNIP maneuvers, variables related to relaxation and contraction properties of the
respiratory muscles. The data are analyzed in median and interquartile range (25% -75%)
RESULTS: A total of 2 subjects participated in the study, 1 man and 1 woman, aged 24.5 (24-26.5)
years, BMI 23 , 55kg / m2 (21.85-25.26). There was a decrease in the peak pressure of the SNIP
maneuvers post in relation to the maneuver pre in both subjects. The maximum relaxation rate
(MRR) decreased in the 2 first post SNIP maneuvers, returning to baseline values from the third.
The maximum rate of pressure development (MRPD) decreased in all post maneuvers in relation
to the pre. In relation to the shortening speed of the global inspiratory muscles (/ NCWm), there
was an increase in the speed after this in relation to the pre-test. The activation of the respiratory
muscles (RMS) was calculated as a percentage in relation to the pre SNIP and there was a decrease
in the post maneuvers in all the studied muscles, as well as there was a linear drop in the median
frequency during the performance of the fatigue test. The duration of the tests was divided into
intervals (20%, 40%, 60%, 80%, 100%) for the analysis of tissue oxygenation variables. There
was an increase in the concentration of oxygenated hemoglobin (H02B), deoxygenated
hemoglobin (HHB) and total hemoglobin (THB) in all percentages of the test duration, this
increase being more evident in the last east interval (100%).