Nutrition status survey of type 2 and 3 spinal muscular atrophy in Chinese population

ABSTRACT

Objective

To evaluate the nutritional status of children with SMA types II and III in a Chinese population.

Methods

We performed a retrospective medical record review of prospectively collected data from children with SMA types II and III in a single centre. We analysed data including clinical parameters, anthropometrics, and 24-hour dietary intake records in our clinic.

Results

We analysed the anthropometric data from 86 children with 69 (80%) SMAII and 17 (20%) SMAIII; 47 (55%) were female, mean age was 5.22 ± 3.73 years. The WAZ of the SMAII (n = 69) and SMAIII (n = 17) were −0.48 (IQR −1.69, 0.57) vs −0.53 (IQR −1.60, 0.55), P = 0.926; the HAZ were −0.62 (IQR −1.4, 0.3) vs −0.6 (IQR −1.61, 0.4), P=0.72; the BMIZ were −0.51 (IQR −1.53, 0.99) vs −0.08 (IQR −1.625, 1.125), P = 0.537.The dietary intake of 51 children was compared to the Chinese Dietary Reference Intakes (DRIs). The actual energy intake in SMAII was similar to the DRIs, but which in SMAIII was less than the DRIs (1312.4 ± 329.5 kcal vs. 1655 ± 640.1 kcal, P = 0.028). The protein intake in SMAII and SMAIII was higher than the DRIs (55 ± 16.3 g/d vs 30.2 ± 4.6 g/d, P < 0.05; 56.8 ± 18.1 g/d vs 41.5 ± 17.5 g/d, p = 0.22), and calcium intake was lower than the recommendation (507.7 ± 177.8 mg/d vs 731.7 ± 123.4 mg/d, P < 0.05; 478.4 ± 207.4 mg/d vs 478.4 ± 207.4 mg/d, P = 0.01). Swallowing on the Neuromuscular Disease Status Scale was 7.41 ± 0.5.

Conclusions

Children with SMAII and SMAIII were at risk for malnutrition and low calcium intake.

KEYWORDS:

• Spinal muscular atrophy
• diet survey
• undernutrition
• obesity

Acknowledgements

We thank all of the families and children who participated in our survey. We thank all doctors of the Department of Neurology of Children’s Hospital of Fudan University.

Disclosure statement

No potential conflict of interest was reported by the authors.

Additional information

Notes on contributors

Yiyao Zhou

Yiyao Zhou is a graduate student in nutrition, currently a clinical dietitian of clinical nutrition department at the Children’s Hospital of Fudan University.

Jieyu Chen

Jieyu Chen is a clinical dietitian of clinical nutrition department at the Children’s Hospital of Fudan University. She is engaging in the nutritional treatment of endocrine and neurosurgical diseases in children.

Xiaoyan Gong

Xiaoyan Gong is a clinical dietitian of clinical nutrition department at the Children’s Hospital of Fudan University. Her research focuses on nutritional guidance on ketogenic diet and biliary atresia.

Zhongying Lu

Zhongying Lu: is a clinical dietitian of clinical nutrition department at the Children’s Hospital of Fudan University. Her research focuses on nutritional guidance on ketogenic diet and immune deficiency disease.

Haimei Hua

Haimei Hua is a clinical dietitian, currently director of clinical nutrition department at the Children’s Hospital of Fudan University. She is engaging in nutritional therapy related to kidney disease in children.

Xiaomei Zhu

Xiaomei Zhu is a neurologist and a master of medicine, and currently a Ph.D. student, researching associations between neurology and neuromuscular diseases in children.

Peng Shi

Peng Shi is a MS in Statistics and Epidemiology, currently a research fellow in the Department of Data Management and Statistics in Children's Hospital of Fudan University.

Xihua Li

Xihua Li is a M.D., PhD, currently director of department of neuromuscular disorder, Children's Hospital of Fudan University. Her research focuses on rare neuromuscular disorders, neurogenetic diseases and neonatal myopathy. She is currently the principal investigator of internationally collaborated multi-centre clinical trials for spinal muscular atrophy and Duchenne muscular dystrophy.

Shuizhen Zhou

Shuizhen Zhou is a M.D., Ph.D., currently director of the Department of Neurology, Children's Hospital of Fudan University. She has currently engaged in pediatric epilepsy standard diagnosis and treatment, refractory epilepsy genetic etiology diagnosis and genetic counseling, refractory epilepsy ketogenic diet treatment, pediatric nervous system problems, and etc.

Yi Wang

Yi Wang is a M.D., Ph.D., Vice President of Children's Hospital of Fudan University. Her research focuses on the early diagnosis, prevention and treatment of childhood epilepsy, neuroprotective mechanism and intervention as well as the clinical epidemiology and translational research of autism spectrum disorders.

Tian Qian

Tian Qian is a M.D., Ph.D., currently deputy director of clinical nutrition department at the Children’s Hospital of Fudan University. She has been focusing in nutritional status of children with neurodevelopmental disorders.