Evidence map›Paper›PMID 39973453›Full record

ArticleJournal of neuromuscular diseases

Nutritional status of patients with nemaline myopathy and related congenital myopathies in Finland: A pilot study.

Vilma-Lotta Lehtokari, Minna Similä, Marianne Tammepuu, Pirjo Isohanni, Mari Auranen, Sinikka Hiekkala, Carina Wallgren-Pettersson, Sonja Strang-Karlsson

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Article in Journal of neuromuscular diseases. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

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1citing papers in PubMed
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1 · What the graph read from it

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3 · Its place in the literature

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1 citing paper in PubMed.

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4 · The record

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5 · Who and what money

Authors and funding

8 authors.

Vilma-Lotta LehtokariFolkhälsan Research Center, Helsinki, Finland.ORCID 0000-0002-8276-0972
Minna SimiläClinical Nutrition Unit, Internal Medicine and Rehabilitation, Helsinki University Hospital, University of Helsinki, Helsinki, Finland.
Marianne TammepuuDepartment of Pediatric Neurology, Children's Hospital, Pediatric Research Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Pirjo IsohanniDepartment of Pediatric Neurology, Children's Hospital, Pediatric Research Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.ORCID 0000-0002-0156-1614
Mari AuranenClinical Neurosciences, Neurology, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Sinikka HiekkalaThe Finnish Association of People with Physical Disabilities, Helsinki, Finland.
Carina Wallgren-PetterssonFolkhälsan Research Center, Helsinki, Finland.
Sonja Strang-KarlssonDepartment of Clinical Genetics, HUS Diagnostic Center, Helsinki University Hospital and University of Helsinki, Finland.ORCID 0000-0002-8594-2077

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundThe muscle weakness in patients with nemaline myopathy (NM) and related disorders (NMR) often affects the muscles used for eating and dining, and some scientific evidence of poorer nutritional status of patients with myopathy exists. However, comprehensive research on the nutritional status of persons with NM or NMR has not been done.

objectivesOur aim was to conduct a comprehensive cross-sectional pilot study among adult Finnish patients with NM or NMR to investigate their food consumption, nutrient intakes, selected nutrient-related laboratory parameters in blood, and self-assessed functioning of dining and eating and of the gastrointestinal tract. We also aimed to test the suitability of the methods selected.

methodsThe methods included a questionnaire to investigate eating-related functioning, a food frequency questionnaire, food records, and laboratory analyses from blood samples.

resultsOf 32 invited patients, 20 returned the survey and food frequency questionnaire. Food records were returned by 17, and blood samples were obtained from 16. Food consumption as well as nutrient and energy intake were highly variable between the individuals. Low energy intakes, low consumption of healthy foods, such as fruits, vegetables, and whole grains as well as low intakes of vitamin D, calcium, dietary fiber, vitamin C, folate, and iron were observed. Low nutrient intakes were seen especially in non-ambulatory participants, who also reported challenges in eating and dining related functioning. The laboratory parameters did not indicate severe undernourishment in any of the participants.

conclusionsEvaluation of food consumption and nutrient intakes were needed to find patients with risk of undernourishment. The results underline the importance of monitoring adequate intake of calcium and vitamin D in this group of patients, especially because of the immobility-induced risk of osteoporosis. Non-ambulatory patients reported more challenges in eating and dining. This, however, did not determine the nutritional status of the patient.

Indexed as

Myopathies, NemalineNutritional StatusAdultAgedCross-Sectional StudiesEnergy IntakeFemaleFinlandHumansMaleMiddle AgedPilot ProjectsYoung Adultdistal myopathyfood frequency questionnairefood recordsnemaline myopathynutrient intakenutritional statusnutrition-related laboratory parameters

Identifiers

PMID39973453
PMCPMC11949308

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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.