Dietary intake and growth deficits in Rett syndrome-A cross-section study / Lee-Chin WONG in Autism Research, 14-7 (July 2021)  

Public ISBD [article]  inAutism Research > 14-7 (July 2021) . - p.1512-1521 Titre : Dietary intake and growth deficits in Rett syndrome-A cross-section study Type de document : texte imprimé Auteurs : Lee-Chin WONG, Auteur ; Yen-Tsz CHEN, Auteur ; Shu-Mei TSAI, Auteur ; Yen-Ju LIN, Auteur ; Chia-Jui HSU, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Hsiu-Yu SHEN, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur Article en page(s) : p.1512-1521 Langues : Anglais (eng) Mots-clés : Autism Spectrum Disorder  Body Height  Eating  Humans  Methyl-CpG-Binding Protein 2/genetics  Mutation  Rett Syndrome/complications/genetics  Rett syndrome  clinical severity  dietary intakes  dystonia  growth deficit  nutrition Index. décimale : PER Périodiques Résumé : Growth deficit is a common comorbidity and one of the supportive criteria in Rett syndrome (RTT). This study aimed to investigate the impact of dystonia, dietary intakes, and clinical severities on growth patterns in a Taiwanese cohort of RTT. We recruited 44 RTT patients with MECP2 mutation for analysis. For individuals ≤18 years of age, in comparison to the RTT-specific growth chart which comprised American RTT cohort, the body height was right-shifted to a higher percentile, whereas the body weight was left-shifted to a lower percentile. Furthermore, the body mass index was significantly decreased when compared to RTT-specific growth chart (p = 0.01). Higher degree of overall disease severity (odd ratio = 1.159; 95% CI = 1.063-1.264; p = 0.001) and hand use impairment (odd ratio = 2.017; 95% CI = 1.037, 3.921; p = 0.039) were associated with more severe growth patterns. All individuals had dystonia at certain variable degrees. The dystonia worsened with age (p < 0.001) but did not have significant impact on growth deficit. Most of our cohort had adequate protein (97.37%) and energy (58.97%) intakes. The fiber intakes were generally low, with about 38 (97.4%) individuals did not meet the daily reference intakes of fiber. The protein intake was significantly lower in individuals with severe growth deficit (p = 0.04). Our study shows that ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. Further, disease severity, genotypes, and nutrition exert important impacts on RTT-growth pattern. LAY SUMMARY: Growth impairment is an important issue in Rett syndrome and the underlying patho-mechanism is multifactorial. Higher degree of overall disease severity and hand use impairment were associated with more severe growth pattern deficits. Although all individuals had dystonia at certain variable degrees and the dystonia worsened with age, but it did not have significant impact on growth deficit. Nutritional intakes may partially affect growth. Furthermore, ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. En ligne : http://dx.doi.org/10.1002/aur.2508 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=449 [article] Dietary intake and growth deficits in Rett syndrome-A cross-section study [texte imprimé] / Lee-Chin WONG, Auteur ; Yen-Tsz CHEN, Auteur ; Shu-Mei TSAI, Auteur ; Yen-Ju LIN, Auteur ; Chia-Jui HSU, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Hsiu-Yu SHEN, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur . - p.1512-1521. Langues : Anglais (eng) in Autism Research > 14-7 (July 2021) . - p.1512-1521 Mots-clés : Autism Spectrum Disorder  Body Height  Eating  Humans  Methyl-CpG-Binding Protein 2/genetics  Mutation  Rett Syndrome/complications/genetics  Rett syndrome  clinical severity  dietary intakes  dystonia  growth deficit  nutrition Index. décimale : PER Périodiques Résumé : Growth deficit is a common comorbidity and one of the supportive criteria in Rett syndrome (RTT). This study aimed to investigate the impact of dystonia, dietary intakes, and clinical severities on growth patterns in a Taiwanese cohort of RTT. We recruited 44 RTT patients with MECP2 mutation for analysis. For individuals ≤18 years of age, in comparison to the RTT-specific growth chart which comprised American RTT cohort, the body height was right-shifted to a higher percentile, whereas the body weight was left-shifted to a lower percentile. Furthermore, the body mass index was significantly decreased when compared to RTT-specific growth chart (p = 0.01). Higher degree of overall disease severity (odd ratio = 1.159; 95% CI = 1.063-1.264; p = 0.001) and hand use impairment (odd ratio = 2.017; 95% CI = 1.037, 3.921; p = 0.039) were associated with more severe growth patterns. All individuals had dystonia at certain variable degrees. The dystonia worsened with age (p < 0.001) but did not have significant impact on growth deficit. Most of our cohort had adequate protein (97.37%) and energy (58.97%) intakes. The fiber intakes were generally low, with about 38 (97.4%) individuals did not meet the daily reference intakes of fiber. The protein intake was significantly lower in individuals with severe growth deficit (p = 0.04). Our study shows that ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. Further, disease severity, genotypes, and nutrition exert important impacts on RTT-growth pattern. LAY SUMMARY: Growth impairment is an important issue in Rett syndrome and the underlying patho-mechanism is multifactorial. Higher degree of overall disease severity and hand use impairment were associated with more severe growth pattern deficits. Although all individuals had dystonia at certain variable degrees and the dystonia worsened with age, but it did not have significant impact on growth deficit. Nutritional intakes may partially affect growth. Furthermore, ethnicity should be considered when comparing RTT individuals' growth pattern to the RTT-specific growth chart. En ligne : http://dx.doi.org/10.1002/aur.2508 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=449

Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study / Chia-Jui HSU ; Yen-Tzu WU ; Hsu-Feng CHU ; Jui-Hsiang LIN ; Hsin-Pei WANG ; Su-Ching HU ; Ying-Chieh TSAI ; Wen-Che TSAI ; Wang-Tso LEE in Autism, 28-9 (September 2024)  

Public ISBD [article]  inAutism > 28-9 (September 2024) . - p.2267-2281 Titre : Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study Type de document : texte imprimé Auteurs : Chia-Jui HSU, Auteur ; Yen-Tzu WU, Auteur ; Hsu-Feng CHU, Auteur ; Jui-Hsiang LIN, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Ying-Chieh TSAI, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur Article en page(s) : p.2267-2281 Langues : (en) Mots-clés : dystonia  microbiota-gut-brain axis  probiotics  Rett syndrome Index. décimale : PER Périodiques Résumé : This pilot study investigates the feasibility and assesses the impact of Lactobacillus plantarum PS128 probiotics on the neurological function in Rett syndrome. We conducted a randomized, double-blind, and placebo-controlled trial on Rett syndrome with MECP2 mutation aged between 1 and 50 years in Taiwan. In this pilot study, twice-daily L. plantarum PS128 or placebo was administered for 16 weeks. In addition to feasibility, we also assessed the changes utilizing the Mullen Scales of Early Learning. In total, 36 participants were finally randomized into L. plantarum PS128 (n = 18) or placebo (n = 18) groups. At the end of intervention, the retention rates were 100% for L. plantarum PS128 and 94.44% for placebo, with withdrawal rates of 5.56% for the placebo group. Both groups tolerated well, except for one L. plantarum PS128 participant who reported loose stool. The probiotic group showed a change of 2.19+3.76, while the placebo group had 0.85+5.09 (p = 0.051) in the total age-equivalent scores of Mullen Scales of Early Learning. There was a significant difference in the change of the total score on the Burke-Fahn-Marsden Movement Scale between probiotc group and placebo group ( 12.19+12.12 vs 4.59+4.20, p = 0.020). In leg dystonia, the probiotic group exhibited a change of 4.11+5.11 compared with 0.38+1.50 in the placebo group (p = 0.008). Our findings affirm the feasibility of L. plantarum PS128 in Rett syndrome. Future clinical trials are mandatory to further explore its long-term impact on Rett syndrome. Lay abstract Rett syndrome often involves gastrointestinal symptoms and gut microbiota imbalances. We conducted a study to explore the feasibility of probiotic Lactobacillus plantarum PS128 and the impact on neurological functions in Rett syndrome. The results of our investigation demonstrated that the supplementation of probiotic L. plantarum PS128 was feasible and well tolerated, with 100% retention rate and 0% withdrawal rate. In addition, there was only one participant who had loose stool after taking L. plantarum PS128. Further, there was a tendency to enhance overall cognitive developmental level, as assessed using Mullen Scales of Early Learning. In addition, it significantly improved dystonia, as assessed using the Burke-Fahn-Marsden Movement Scale, in comparison with the placebo group. This study provides a strong foundation for future research and clinical trials exploring the potential of L. plantarum PS128 probiotics as a complementary therapy for individuals with Rett syndrome. En ligne : https://dx.doi.org/10.1177/13623613231225899 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=535 [article] Investigating the impact of probiotic on neurological outcomes in Rett syndrome: A randomized, double-blind, and placebo-controlled pilot study [texte imprimé] / Chia-Jui HSU, Auteur ; Yen-Tzu WU, Auteur ; Hsu-Feng CHU, Auteur ; Jui-Hsiang LIN, Auteur ; Hsin-Pei WANG, Auteur ; Su-Ching HU, Auteur ; Ying-Chieh TSAI, Auteur ; Wen-Che TSAI, Auteur ; Wang-Tso LEE, Auteur . - p.2267-2281. Langues : (en) in Autism > 28-9 (September 2024) . - p.2267-2281 Mots-clés : dystonia  microbiota-gut-brain axis  probiotics  Rett syndrome Index. décimale : PER Périodiques Résumé : This pilot study investigates the feasibility and assesses the impact of Lactobacillus plantarum PS128 probiotics on the neurological function in Rett syndrome. We conducted a randomized, double-blind, and placebo-controlled trial on Rett syndrome with MECP2 mutation aged between 1 and 50 years in Taiwan. In this pilot study, twice-daily L. plantarum PS128 or placebo was administered for 16 weeks. In addition to feasibility, we also assessed the changes utilizing the Mullen Scales of Early Learning. In total, 36 participants were finally randomized into L. plantarum PS128 (n = 18) or placebo (n = 18) groups. At the end of intervention, the retention rates were 100% for L. plantarum PS128 and 94.44% for placebo, with withdrawal rates of 5.56% for the placebo group. Both groups tolerated well, except for one L. plantarum PS128 participant who reported loose stool. The probiotic group showed a change of 2.19+3.76, while the placebo group had 0.85+5.09 (p = 0.051) in the total age-equivalent scores of Mullen Scales of Early Learning. There was a significant difference in the change of the total score on the Burke-Fahn-Marsden Movement Scale between probiotc group and placebo group ( 12.19+12.12 vs 4.59+4.20, p = 0.020). In leg dystonia, the probiotic group exhibited a change of 4.11+5.11 compared with 0.38+1.50 in the placebo group (p = 0.008). Our findings affirm the feasibility of L. plantarum PS128 in Rett syndrome. Future clinical trials are mandatory to further explore its long-term impact on Rett syndrome. Lay abstract Rett syndrome often involves gastrointestinal symptoms and gut microbiota imbalances. We conducted a study to explore the feasibility of probiotic Lactobacillus plantarum PS128 and the impact on neurological functions in Rett syndrome. The results of our investigation demonstrated that the supplementation of probiotic L. plantarum PS128 was feasible and well tolerated, with 100% retention rate and 0% withdrawal rate. In addition, there was only one participant who had loose stool after taking L. plantarum PS128. Further, there was a tendency to enhance overall cognitive developmental level, as assessed using Mullen Scales of Early Learning. In addition, it significantly improved dystonia, as assessed using the Burke-Fahn-Marsden Movement Scale, in comparison with the placebo group. This study provides a strong foundation for future research and clinical trials exploring the potential of L. plantarum PS128 probiotics as a complementary therapy for individuals with Rett syndrome. En ligne : https://dx.doi.org/10.1177/13623613231225899 Permalink : https://www.cra-rhone-alpes.org/cid/opac_css/index.php?lvl=notice_display&id=535

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