The use of two Comfort Young Child Formulas in the dietary management of toddlers with functional constipation: a randomized controlled trial.

BACKGROUND: Pharmacological intervention with laxatives is the conventional treatment for functional constipation (FC). Data to support the dietary management of FC is lacking. This study compared the efficacy of two Comfort young child formulas (YCFs) with regards to the maintenance of healthy stooling parameters in toddlers with a history of constipation. It was registered in the Netherlands Trial Registry [identifier: NL7420 (NTR7653)], registration date 20/09/2018. METHODS: Ninety-five healthy toddlers, aged 12 to 32 months, diagnosed with FC (Rome III criteria) were randomized to receive one of two study formulas after pharmacological treatment. For the first month of the intervention, subjects received a laxative in a decreasing maintenance dose alongside a test or control formula (maintenance phase). Subsequently, subjects only consumed formula for another month (post-maintenance phase). Stooling parameters were obtained weekly using the Bristol Stool Scale and the modified Rome III Questionnaire on Paediatric Gastrointestinal Symptoms for infants and toddlers. Differences in percentages of hard stools (primary outcome) and other stooling parameters were analysed using analysis of covariance and Chi-Square methods. RESULTS: Both formulas resulted in similar overall percentage of hard stools during the intervention period, respectively 5.02% in the test and 2.99% in the control group (n.s.). In the test group, percentages dropped from 7.11% at the end of the maintenance phase, to 3.92% at the end of the post-maintenance phase. In contrast, the percentage of hard stools in the control group was similar at the end of the maintenance (3.18%) and post-maintenance phase (2.83%; n.s.). No difference was found in the overall stool frequency between groups. At the end of the maintenance phase, only 22% and 19% of toddlers consuming the test and control formulae, respectively, met 2 or more of the criteria for FC. At the end of the study, this percentage of subjects decreased further to 9% in the test group, which tended to be lower compared to the 21% found in the control (p = 0.107). No laxative use was reported in either study group during the post-maintenance phase. CONCLUSION: Both Comfort YCF support the maintenance of improved stooling over time in toddlers with a history of constipation. The percentage of subjects suffering from functional constipation tended to be lower after the intervention period when receiving the formula with intact protein.

The Effects of High-Protein Diet and Resistance Training on Glucose Control and Inflammatory Profile of Visceral Adipose Tissue in Rats.

High-protein diets (HPDs) are widely accepted as a way to stimulate muscle protein synthesis when combined with resistance training (RT). However, the effects of HPDs on adipose tissue plasticity and local inflammation are yet to be determined. This study investigated the impact of HPDs on glucose control, adipocyte size, and epididymal adipose inflammatory biomarkers in resistance-trained rats. Eighteen Wistar rats were randomly assigned to four groups: normal-protein (NPD; 17% protein total dietary intake) and HPD (26.1% protein) without RT and NPD and HPD with RT. Trained groups received RT for 12 weeks with weights secured to their tails. Glucose and insulin tolerance tests, adipocyte size, and an array of cytokines were determined. While HPD without RT induced glucose intolerance, enlarged adipocytes, and increased TNF-α, MCP-1, and IL1-ß levels in epididymal adipose tissue (p < 0.05), RT diminished these deleterious effects, with the HPD + RT group displaying improved blood glucose control without inflammatory cytokine increases in epididymal adipose tissue (p < 0.05). Furthermore, RT increased glutathione expression independent of diet (p < 0.05). RT may offer protection against adipocyte hypertrophy, pro-inflammatory states, and glucose intolerance during HPDs. The results highlight the potential protective effects of RT to mitigate the maladaptive effects of HPDs.

Effect of administration of high-protein diet in rats submitted to resistance training.

PURPOSE: Although there is limited evidence regarding the pathophysiological effects of a high-protein diet (HD), it is believed that this type of diet could overload the body and cause damage to the organs directly involved with protein metabolism and excretion. The aim of this study was to verify the effects of HD on biochemical and morphological parameters of rats that completed a resistance training protocol (RT; aquatic jump) for 8 weeks. METHODS: Thirty-two adult male Wistar rats were divided into four groups (n = 8 for each group): sedentary normal protein diet (SN-14%), sedentary high-protein diet (SH-35%), trained normal protein diet (TN-14%), and trained high-protein diet (TH-35%). Biochemical, tissue, and morphological measurements were made. RESULTS: Kidney (1.91 ± 0.34) and liver weights (12.88 ± 1.42) were higher in the SH. Soleus muscle weight was higher in the SH (0.22 ± 0.03) when compared to all groups. Blood glucose (123.2 ± 1.8), triglycerides (128.5 ± 44.0), and HDL cholesterol levels (65.7 ± 20.9) were also higher in the SH compared with the other experimental groups. Exercise reduced urea levels in the trained groups TN and TH (31.0 ± 4.1 and 36.8 ± 6.6), respectively. Creatinine levels were lower in TH and SH groups (0.68 ± 0.12; 0.54 ± 0.19), respectively. HD negatively altered renal morphology in SH, but when associated with RT, the apparent damage was partially reversed. In addition, the aquatic jump protocol reversed the damage to the gastrocnemius muscle caused by the HD. CONCLUSIONS: A high-protein diet promoted negative metabolic and morphological changes, while RT was effective in reversing these deleterious effects.

Hepatic Glycogen Storage Diseases Toward One Global Collaborative Network

Abstract The third international meeting of the Scandinavian Association for Glycogen Storage Disease focused on hepatic glycogen storage disease and was organized for health-care professionals, patient representatives, and representatives from the industry. This report highlights dilemmas in dietary management, differences in monitoring strategies, and challenges with rare disease care, research, and patient participation.

Structured Dietary Management Dramatically Improves Marked Transaminitis, Metabolic and Clinical Profiles in Glycogen Storage Disease Type IXa

Abstract Glycogen storage disease type IXa (GSD IXa) presents in childhood with hepatomegaly, poor growth, and ketotic hypoglycemia. Clinical course is usually mild, often not requiring treatment with attenuation of symptoms with increasing age. The phenotypic spectrum has recently expanded to include more severe involvement with hepatic fibrosis or cirrhosis warranting dietary therapy. We report a 2-year-old boy with a severe phenotype of GSD IXa presenting with a massive hepatomegaly, significant transaminitis, recurrent ketotic hypoglycemia, and short stature. Aggressive dietary management with regular feeds, frequent uncooked cornstarch doses, and protein supplementation resulted in clinical improvements including enhanced growth velocity, energy levels, overall well-being, and reduction in hepatomegaly with restitutions in biochemical parameters. We concur with a recent report which proposed that GSD IXa is not always a mild condition but instead part of an expanding phenotypic spectrum warranting intensive dietary management to optimize metabolic control and quality of life.

Doença Celíaca no município de São Paulo: a disponibilidade de um mercado específico / Celiac Disease in São Paulo: the availability of a specific market

Celiac Disease is a genetic enteropathy characterized by permanent intolerance to gluten. Specific and restrictive, gluten-free diet may present a growing demand for products such as breads, pasta, sausages and beverages with special formulations. Whereas no similar studies were found in the literature, the aims of this investigation were to identify the repertoire of gluten-free products available in the market of São Paulo and to estimate the cost difference between a diet consisting of conven¬tional products and other with gluten-free products. Characterized as an exploratory qualitative and quantitative study, it was based on market research. Visits were made to specialty stores in areas of higher population density and/or that concentrate the largest number of establishments in São Paulo. Interviews were carried out with members of associations, universities, hospitals or research and treatment centers of celiac disease. The interview was semi-structured with open questions approaching aspects of the market of gluten-free products and celiac disease. Data analysis was obtained by calculating the percentage difference between the products found and formulated diets. The gluten-free diet represents a significant impact on the cost of food, especially when it contains products such as pasta and flour. In São Paulo, a diet with gluten-free products can be approximately 44% more expensive than a diet with conventional products.

La enfermedad celíaca es una enteropatía de origen genético caracterizada por la intolerancia permanente al gluten. Específica y restrictiva, la dieta del enfermo celíaco puede presentar una demanda creciente de productos tales como panes, pastas, embutidos y bebidas alcohólicas con formulaciones especiales. Considerando que no se encontraron estudios similares en la literatura, los objetivos de esta investigación fueron: identificar el repertorio de productos sin gluten disponibles en el mercado paulistano y estimar la diferencia de coste entre una dieta constituida de productos convencionales y otra sin gluten. Caracterizándose como un estudio exploratorio de tipo cualitativo y cuantitativo, se fundamentó en un estudio de mercado. Se realizaron visitas a las tiendas especializadas en las regiones de mayor densidad poblacional y/o que concentran el mayor número de establecimientos en el municipio de São Paulo, y se entrevistó a los responsables de asociaciones, Universidades, Hospitales o Centros de investigación y tratamiento de enfermos Celíacos. Al responsable se le realizó una entrevista semi-estructurada con preguntas abiertas, donde se abordaban aspectos del mercado de productos sin gluten y de la enfermedad celíaca. El análisis de los datos se obtuvo mediante el cálculo de la diferencia porcentual entre los productos encontrados y las dietas preparadas. La dieta sin gluten representa un impacto significativo en el coste alimenticio, especialmente cuando contiene productos como pasta y harina. En la ciudad de São Paulo, una dieta con productos sin gluten puede ser aproximadamente 44% más cara que una dieta con productos convencionales.

Doença Celíaca é uma enteropatia de origem genética, caracterizada pela intolerância permanente ao glúten. Específica e restritiva, a dieta do Celíaco pode apresentar uma demanda crescente de produtos, como pães, massas, embutidos e bebidas alcoólicas com formulações especiais. Considerando-se que não foram encontrados estudos semelhantes na literatura, os objetivos desta investigação foram identificar o repertório de produtos livres de glúten disponíveis no mercado paulistano e estimar a diferença de custo entre uma dieta constituída de produtos convencionais e outra isenta de glúten. Caracterizando-se como um estudo qualitativo e quantitativo exploratório, fundamentou-se em uma pesquisa de mercado. Foram realizadas visitas a lojas especializadas nas regiões de maior densidade demográfica e/ou que concentram maior número de estabelecimentos no município de São Paulo, e entrevistas com os responsáveis por associações, Universidades, Hospitais ou Centros de Investigação e Tratamento de Celíacos. Com o responsável, foi realizada uma entrevista semiestruturada com perguntas abertas, abordando aspectos sobre o mercado de produtos sem glúten e a Doença Celíaca. A análise dos dados foi obtida a partir do cálculo da diferença percentual entre os produtos encontrados e as dietas elaboradas. A dieta isenta de glúten representa um impacto significativo no custo com alimentação, especial¬mente quando contém produtos como macarrão e farinhas. Na cidade de São Paulo, uma dieta com produtos livres de glúten pode ser aproximadamente 44% mais cara do que uma dieta com produtos convencionais.

Tratamento de erros inatos do metabolismo: [revisão] / Treatment of inborn errors of metabolism: [review]

OBJETIVO: Esta revisão teve por objetivo abordar a situação atual do tratamento dos distúrbios do metabolismo intermediário (principalmente dos aminoácidos, ciclo da uréia e ácidos orgânicos) e das doenças relacionadas a duas organelas subcelulares (lisossomos e peroxissomos). FONTES DOS DADOS: Na abordagem do tratamento dos distúrbios do metabolismo intermediário, foi dada prioridade às principais formas de manejo da intoxicação, em virtude da importância para o pediatra do tratamento de quadros agudos e com risco de vida. O artigo apresenta também uma visão geral do tratamento das doenças lisossômicas e peroxissômicas, com ênfase na terapia de reposição enzimática, uma modalidade de tratamento de uso crescente com a qual o pediatra deve se familiarizar. SÍNTESE DOS DADOS: As principais medidas para manejo da intoxicação presente em muitos erros inatos do metabolismo intermediário foram apresentadas (restrição de aporte de substrato através da dieta ou através de inibição enzimática, remoção do produto tóxico, estímulo da atividade enzimática residual, reposição do produto deficiente). O material elaborado sobre terapia para doenças lisossômicas e peroxissômicas inclui tabelas informativas sobre os tratamentos disponíveis. CONCLUSÕES: O tratamento dos erros inatos do metabolismo é uma situação complexa e que deve ser abordada por uma equipe multidisciplinar, na qual o pediatra é peça-chave. Este capítulo contém informações práticas relativas ao manejo de alguns erros inatos do metabolismo e proporciona ao pediatra uma visão geral dos desenvolvimentos recentes ocorrido nessa área da medicina.

OBJECTIVE: To describe the current state of treatment for disorders of intermediate metabolism (primarily of amino acids, urea cycle and organic acids) and for diseases related to two subcellular organelles (lysosomes and peroxisomes). SOURCES: In covering the treatment of disorders of intermediate metabolism, priority was given to the most important methods for managing intoxication, in view of the importance for pediatricians to treat acute and life-threatening cases. The article also provides a general overview of the treatment for lysosomal and peroxisomal diseases, with emphasis on enzyme replacement therapy, which is a treatment modality that is growing in use and with which pediatricians should make themselves familiar. SUMMARY OF THE FINDINGS: The most important measures used to manage the intoxication present in many inborn errors of intermediate metabolism were presented (restriction of substrate build-up by means of diet or enzymatic inhibition, removal of toxic products, stimulation of residual enzyme activity, replacement of the deficient product). The section on treatment for lysosomal and peroxisomal diseases includes tables providing information on the treatments available. CONCLUSIONS: Treating inborn errors of metabolism is a complex task that should be performed by a multidisciplinary team of which the pediatrician is the key member. This article provides practical information relating to the management of some inborn errors of metabolism and provides pediatricians with a general overview of recent developments in this area of medicine.