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      A New Method for Measuring Bell-Shaped Chest Induced by Impaired Ribcage Muscles in Spinal Muscular Atrophy Children

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          Abstract

          The involvement of the respiratory muscular pump makes SMA children prone to frequent hospitalization and morbidity, particularly in type 1. Progressive weakness affects ribcage muscles resulting in bell-shaped chest that was never quantified. The aims of the present work were: (1) to quantify the presence of bell-shaped chest in SMA infants and children and to correlate it with the action of ribcage muscles, assessed by the contribution of pulmonary ribcage to tidal volume (ΔV RC, p); (2) to verify if and how the structure of the ribcage and ΔV RC, p change after 1-year in SMA type 2. 91 SMA children were studied in supine position during awake spontaneous breathing: 32 with type 1 (SMA1, median age: 0.8 years), 51 with type 2 (SMA2, 3.7 years), 8 with type 3 (SMA3, 5.4 years) and 20 healthy children (HC, 5.2 years). 14 SMA2 showed negative ΔV RC, p (SMA2px), index of paradoxical inspiratory inward motion. The bell-shaped chest index was defined as the ratio between the distance of the two anterior axillary lines at sternal angle and the distance between the right and left 10th costal cartilage. If this index was < < 1, it indicated bell shape, if ~1 it indicated rectangular shape, while if >> 1 an inverted triangle shape was identified. While the bell-shaped index was similar between HC (0.92) and SMA3 (0.91), it was significantly ( p < 0.05) reduced in SMA2 (0.81), SMA2px (0.74) and SMA1 (0.73), being similar between the last two. There was a good correlation (Spearman's rank correlation coefficient, ρ = 0.635, p < 0.001) between ribcage geometry and ΔV RC, p. After 1 year, ΔV RC, p reduced while bell-shaped chest index did not change being significantly lower than HC. The shape of the ribcage was quantified and correlated with the action of ribcage muscles in SMA children. The impaired ribcage muscles function alters the ribcage structure. HC and SMA3 show an almost rectangular ribcage shape, whereas SMA2, SMA2px and SMA1 are characterized by bell-shaped chest. In SMA, therefore, a vicious cycle starts since infancy: the disease progressively affects ribcage muscles resulting in reduced expansion of lung and ribcage that ultimately alters ribcage shape. This puts the respiratory muscles at mechanical disadvantage.

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          Most cited references37

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          An expanded version of the Hammersmith Functional Motor Scale for SMA II and III patients.

          To develop and evaluate an expanded version of the Hammersmith Functional Motor Scale allowing for evaluation of ambulatory SMA patients.
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            Chest wall and lung volume estimation by optical reflectance motion analysis.

            Estimation of chest wall motion by surface measurements only allows one-dimensional measurements of the chest wall. We have assessed on optical reflectance system (OR), which tracks reflective markers in three dimensions (3-D) for respiratory use. We used 86 (6-mm-diameter) hemispherical reflective markers arranged circumferentially on the chest wall in seven rows between the sternal notch and the anterior superior iliac crest in two normal standing subjects. We calculated the volume of the entire chest wall and compared inspired and expired volumes with volumes obtained by spirometry. Marker positions were recorded by four TV cameras; two were 4 m in front of and two were 4 m behind the subject. The TV signals were sampled at 100 Hz and combined with grid calibration parameters on a personal computer to obtain the 3-D coordinates of the markers. Chest wall surfaces were reconstructed by triangulation through the point data, and chest wall volume was calculated. During tidal breathing and vital capacity maneuvers and during CO2-stimulated hyperpnea, there was a very close correlation of the lung volumes (VL) estimated by spirometry [VL(SP)] and OR [VL(OR)]. Regression equations of VL(OR) (y) vs. VL(SP) (x, BTPS in liters) for the two subjects were given by y = 1.01x-0.01 (r = 0.996) and y = 0.96x + 0.03 (r = 0.997), and by y = 1.04x + 0.25 (r = 0.97) and y = 0.98x + 0.14 (r = 0.95) for the two maneuvers, respectively. We conclude spirometric volumes can be estimated very accurately and directly from chest wall surface markers, and we speculate that OR may be usefully applied to calculations of chest wall shape, regional volumes, and motion analysis.
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              Sex differences in thoracic dimensions and configuration.

              The volume of adult female lungs is typically 10-12% smaller than that of males who have the same height and age. In this study, we investigated how this volume difference is distributed between the rib cage and the diaphragm abdomen compartments. Internal rib cage dimensions, diaphragm position relative to spine, and diaphragm length were compared in 21 normal male and 19 normal female subjects at three different lung volumes using anterior-posterior and lateral chest radiographs. At all lung volumes examined, females had smaller radial rib cage dimensions in relationship to height than males, a greater inclination of ribs, a comparable diaphragm dome position relative to the spine, and a shorter diaphragm length. Female subjects exhibited a greater inspiratory rib cage muscle contribution during resting breathing than males, presumably reflecting an improved mechanical advantage conferred to these muscles by the greater inclination of ribs. Because of a greater inclination of ribs, female rib cages could accommodate a greater volume expansion. The results suggest a disproportionate growth of the rib cage in females relative to the lung, which would be well suited to accommodate large abdominal volume displacements as in pregnancy.
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                Author and article information

                Contributors
                Journal
                Front Neurol
                Front Neurol
                Front. Neurol.
                Frontiers in Neurology
                Frontiers Media S.A.
                1664-2295
                13 September 2018
                2018
                : 9
                : 703
                Affiliations
                [1] 1Dipartimento di Elettronica, Informazione e Bioingegneria, Politecnico di Milano , Piazza Leonardo Da Vinci, Milan, Italy
                [2] 2IRCCS Fondazione Don Carlo Gnocchi , Milan, Italy
                [3] 3Fondazione IRCCS Cà' Granda Ospedale Maggiore Policlinico, SAPRE-UONPIA , Neuropsichiatria dell'Infanzia e dell'Adolescenza, Milan, Italy
                [4] 4UO Neurologia dello Sviluppo, Fondazione IRCCS Istituto Neurologico Carlo Besta , Milan, Italy
                Author notes

                Edited by: Genevieve Gourdon, Institut National de la Santé et de la Recherche Médicale (INSERM), France

                Reviewed by: Giuseppe Piscosquito, Fondazione Salvatore Maugeri, Telese (IRCCS), Italy; Maria Grazia Biferi, Center of Research in Myology, France

                *Correspondence: Antonella LoMauro antonella.lomauro@ 123456polimi.it

                This article was submitted to Neuromuscular Diseases, a section of the journal Frontiers in Neurology

                Article
                10.3389/fneur.2018.00703
                6146085
                30271372
                305279c2-8c0e-412f-90e5-d388fc10c71e
                Copyright © 2018 LoMauro, Banfi, Mastella, Alberti, Baranello and Aliverti.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 28 March 2018
                : 03 August 2018
                Page count
                Figures: 7, Tables: 1, Equations: 0, References: 45, Pages: 11, Words: 7566
                Categories
                Neurology
                Original Research

                Neurology
                bell-shaped chest,opto-electronic plethysmography,breathing pattern,paradoxical breathing,sma,ribcage muscles

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