Evidence map›Paper›PMID 39804450›Full record

ArticleIndian journal of pediatrics2025

Pulmonary Dysfunction in Children with Dystrophinopathy: A Cross-Sectional Study.

Ankit Kumar Meena, Gautam Kamila, Vaishak Anand, Biswaroop Chakrabarty, Kana Ram Jat, S K Kabra, R M Pandey, Sheffali Gulati

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Article in Indian journal of pediatrics, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.

0numbers the graph read from it
0cells of the map it votes in
4citing papers in PubMed
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1 · What the graph read from it

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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

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3 · Its place in the literature

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4 citing papers in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Ankit Kumar MeenaCentre of Excellence and Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Gautam KamilaCentre of Excellence and Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Vaishak AnandCentre of Excellence and Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Biswaroop ChakrabartyCentre of Excellence and Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
Kana Ram JatDivision of Pediatric Pulmonology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
S K KabraDivision of Pediatric Pulmonology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
R M PandeyDepartment of Biostatistics, All India Institute of Medical Sciences, New Delhi, India.
Sheffali GulatiCentre of Excellence and Advanced Research for Childhood Neurodevelopmental Disorders, Child Neurology Division, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India. sheffaligulati@gmail.com.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectivesTo observe the prevalence of impaired pulmonary function during various phases of the disease course in children aged 5-18 y with dystrophinopathy. The correlation between different parameters of pulmonary dysfunction and motor function was also studied.

methodsOne hundred and thirty-three confirmed cases of Duchenne muscular dystrophy (DMD), fulfilling predefined inclusion and exclusion criteria were evaluated. A pulmonary function test, the six-minute walk test (6MWT), and a functional assessment of muscular strength using the Vignos and Brooke functional scale were performed on all children. Pulmonary dysfunction was defined as < 80% of the predicted forced vital capacity (FVC).

resultsThe overall prevalence of pulmonary dysfunction was 48.12% (95% CI: 39.8-56.54). The prevalence (95% CI) of pulmonary dysfunction in age groups 5-7.5 y, 7.5-10 y, and 10-18 y was 50% (33.63-66.37), 39.29% (27.58-52.37), and 57.78% (43.3-71.03) respectively. There was a significant association of mean walk duration (r = 0.2509; p = 0.019), walk distance (r = 0.21; p = 0.028), Brooke functional score (r = -0.30; p = 0.0006), and Vignos functional score (r = -0.21; p = 0.019) with percentage predicted FVC. Children with deletion of R17, 18 and 19 spectrin-like repeats were found to have a significantly high proportion of pulmonary dysfunction (p = 0.006). No statistically significant differences were found between children with a deletion involving ABD or hinge region 3 and those without.

conclusionsImpaired pulmonary function can be seen quite early in the disease course, and its prevalence is comparable to those of children with a long-standing disease.

Indexed as

Lung DiseasesMuscular Dystrophy, DuchenneAdolescentChildChild, PreschoolCross-Sectional StudiesFemaleHumansMaleMuscle StrengthPrevalenceRespiratory Function TestsVital CapacityWalk TestBrooke scaleDuchenne muscular dystrophy (DMD)DystrophinopathyPulmonary function testSix-minute walk testVignos scale

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.