دورية أكاديمية

Body mass index in type 2 spinal muscular atrophy: a longitudinal study

التفاصيل البيبلوغرافية
العنوان: Body mass index in type 2 spinal muscular atrophy: a longitudinal study
المؤلفون: Ferrantini G., Coratti G., Onesimo R., Lucibello S., Turrini I., Cicala G., Caprarelli M., Pera M. C., Giorgio V., D'Amico A., Leoni C., Pane M., Mercuri E.
المساهمون: Ferrantini, Gloria, Coratti, Giorgia, Onesimo, Roberta, Lucibello, Simona, Bompard, S., Turrini, Ida, Cicala, Gianpaolo, Caprarelli, Michela, Pera, Maria Carmela, Bravetti, C., Berti, B., Giorgio, Valentina, Bruno, C., Brolatti, N., Panicucci, C., D'Amico, Adele, Longo, A., Leoni, Chiara, Sansone, V. A., Albamonte, E., Messina, S., Sframeli, M., Bertini, E., Pane, Marika, Mercuri, Eugenio Maria
بيانات النشر: Springer Science and Business Media Deutschland GmbH
سنة النشر: 2022
المجموعة: Università Cattolica del Sacro Cuore: PubliCatt
مصطلحات موضوعية: Body mass index, Children, Neonate, Nutritional status, Spinal muscular atrophy, Settore MED/39 - NEUROPSICHIATRIA INFANTILE, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA
الوصف: The aim of this retrospective study was to review body mass index (BMI) in a large cohort of Italian pediatric type 2 spinal muscular atrophy (SMA) patients, aged between 0 and 20years and to establish possible differences in relation to a number of variables such as ventilation, motor function, and survival motor neuron 2 gene copies. Cross-sectional data were collected from 102 patients for a total of 344 visits. Standard growth charts for height and weight were used as reference, with age adjusted BMI calculated using the Center for Disease and Prevention Children’s BMI Tool. In the 344 visits, weight ranged between 3.90 and 83kg, and the BMI between 8.4 and 31.6 with a BMI/age z-scores < − 2SD present in 28% and BMI/age z-scores > + 2SD in 9% of the measurements. The BMI/age z-scores were relatively stable < 5years of age with an increasing number of patients < − 2SD after the age of 5, and a wider range of BMI/age z-scores after the age of 13. A difference on the BMI/age z-scores was found among the different age subgroups (< 5, 5–12, ≥ 13years). A multivariate analysis in 58 patients with longitudinal assessments showed that baseline BMI/age z-scores and gender were significantly contributing to the changes while other variables were not. Conclusion: Our results confirm that careful surveillance of weight and BMI/age z-scores is needed in type 2 SMA. Further studies, including assessments of chewing and swallowing and of lean/fat body mass, will help to better understand the possible mechanisms underlying weight issues.What is Known:• Feeding difficulties have been reported in a few studies and were invariably found in patients with type 1 SMA.• Type 2 SMA patients often have low BMI with a relevant number of patients requiring tube feeding.What is New:• Reduction in BMI/age z-score overtime appeared to depend on baseline BMI/age z-score and gender.• Patients with a low BMI/age z-score were at higher risk of developing further reduction.
نوع الوثيقة: article in journal/newspaper
اللغة: English
العلاقة: info:eu-repo/semantics/altIdentifier/pmid/35048179; info:eu-repo/semantics/altIdentifier/wos/WOS:000744366600001; issue:N/A; firstpage:N/A; lastpage:N/A; issueyear:2022; journal:EUROPEAN JOURNAL OF PEDIATRICS; https://hdl.handle.net/10807/197863Test; info:eu-repo/semantics/altIdentifier/scopus/2-s2.0-85123253073
DOI: 10.1007/s00431-021-04325-3
الإتاحة: https://doi.org/10.1007/s00431-021-04325-3Test
https://hdl.handle.net/10807/197863Test
رقم الانضمام: edsbas.EC89E488
قاعدة البيانات: BASE