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Impaired Antioxidant Defence Status Is Associated With Metabolic-Inflammatory Risk Factors in Preterm Children With Extrauterine Growth Restriction: The BIORICA Cohort Study.

Introduction: An impaired antioxidant status has been described during foetal growth restriction (FGR). Similarly, the antioxidant defence system can be compromised in preterm children with extrauterine growth restriction (EUGR). The aim of this prospective study was to evaluate the antioxidant status in prepubertal children with a history of prematurity without FGR, with and without EUGR, compared to a healthy group. Methods: In total, 211 children were recruited and classified into three groups: 38 with a history of prematurity and EUGR; 50 with a history of prematurity and adequate extrauterine growth (AEUG); and 123 control children born at term. Catalase (CAT), superoxide dismutase (SOD), glutathione peroxidase (GPx) and glutathione reductase (GR) activities were assessed in lysed erythrocytes with spectrophotometric methods. Plasma levels of the antioxidants α-tocopherol, retinol and ß-carotene were determined through solvent extraction and ultra-high-pressure liquid chromatography coupled to mass spectrometry. Results: Children with the antecedent of EUGR and prematurity had lower CAT activity than the other two groups and lower GPx activity than the control children. Lower SOD, GPx and GR activities were observed in the AEUG group compared to the controls. However, higher concentrations of α-tocopherol and ß-carotene were found in the EUGR group compared to the other groups; retinol levels were also higher in EUGR than in AEUG children. In EUGR and AEUG children, enzymatic antioxidant activities and plasma antioxidants were associated with metabolic syndrome components and pro-inflammatory biomarkers. Conclusions: This study reveals, for the first time, that the EUGR condition and prematurity appear to be linked to an impairment of the antioxidant defence status, which might condition an increased risk of adverse metabolic outcomes later in life.

Plasma Adipokines Profile in Prepubertal Children with a History of Prematurity or Extrauterine Growth Restriction.

Adipose tissue programming could be developed in very preterm infants with extrauterine growth restriction (EUGR), with an adverse impact on long-term metabolic status, as was studied in intrauterine growth restriction patterns. The aim of this cohort study was to evaluate the difference in levels of plasma adipokines in children with a history of EUGR. A total of 211 school age prepubertal children were examined: 38 with a history of prematurity and EUGR (EUGR), 50 with a history of prematurity with adequate growth (PREM), and 123 healthy children born at term. Anthropometric parameters, blood pressure, metabolic markers and adipokines (adiponectin, resistin, leptin) were measured. Children with a history of EUGR showed lower values of adiponectin (µg/mL) compared with the other two groups: (EUGR: 10.6 vs. PREM: 17.7, p < 0.001; vs. CONTROL: 25.7, p = 0.004) and higher levels of resistin (ng/mL) (EUGR: 19.2 vs. PREM: 16.3, p =0.007; vs. CONTROL: 7.1, p < 0.001. The PREM group showed the highest values of leptin (ng/mL), compared with the others: PREM: 4.9 vs. EUGR: 2.1, p = 0.048; vs. CONTROL: 3.2, p = 0.029). In conclusion, EUGR in premature children could lead to a distinctive adipokines profile, likely associated with an early programming of the adipose tissue, and likely to increase the risk of adverse health outcomes later in life.

Necesidades sanitarias y socioeducativas de niños con enfermedades raras de tipo metabólico y sus familias: estudio cualitativo en un hospital de tercer nivel / Health and socio-educational needs of the families and children with rare metabolic diseases: Qualitative study in a tertiary hospital

INTRODUCCIÓN: Las enfermedades raras suponen un reto para la salud pública debido a la escasa información sobre su magnitud. Entre ellas destacan los errores congénitos del metabolismo. El presente estudio pretende valorar la calidad de vida y las posibles necesidades sanitarias, socioeducativas y económicas de los pacientes pediátricos con diagnóstico de ER de tipo metabólico y de sus familiares o cuidadores principales, atendidos habitualmente en un hospital de tercer nivel. MATERIAL Y MÉTODO: Se desarrolló un cuestionario basado en las necesidades y expectativas recogidas fundamentalmente en el Plan Andaluz para las Enfermedades Raras. Se analizaron variables de los órdenes sociosanitario, económico y educativo en 65 pacientes pediátricos con errores congénitos del metabolismo. RESULTADOS: Los encuestados manifestaron escasas posibilidades para afrontar el gasto de la medicación (61%), alimentación especial (86%) y otras prestaciones sanitarias (79%). El 43% consideraron que la calidad de vida familiar se afectó bastante desde la aparición de la enfermedad. En el 61,5% la cuidadora principal fue la madre, frente al 1,5% de casos en los que fue el padre. El cuidador principal redujo su jornada laboral o abandonó su trabajo en el 77% de los casos. CONCLUSIONES: El tratamiento multidisciplinar se ve afectado por la imposibilidad de las familias para hacer frente a su elevado coste, junto a una difícil accesibilidad a dichos recursos. Además, existe gran repercusión en la calidad de vida de los pacientes y sus cuidadores. Por tanto, deberían evaluarse los resultados de los planes gubernamentales de apoyo sanitario y socioeconómico a pacientes con enfermedades raras, y conseguir una respuesta real a sus necesidades

INTRODUCTION: Rare diseases are a challenge for public health due to the lack of information on their magnitude. These include inborn errors of metabolism. The objective of this study was to assess the quality of life and social, health, economic, and educational needs of a group of paediatric patients with inborn errors of metabolism attended to in a hospital. MATERIAL AND METHOD: A questionnaire was developed based on the needs and expectations, based mainly on the Andalusian Plan for Rare Diseases. An analysis was performed on the variables of health, socioeconomic, and educational needs of 65 paediatric patients with inborn errors of metabolism. RESULTS: The respondents showed few possibilities to cope with medication (61%), special diet (86%), and other health benefits (79%). Just under half of them (43%) believed that the quality of family life had been greatly reduced since the onset of the disease. The main caregiver was the mother in 61.5% of cases, compared to 1.5% of cases in which it was the father. The primary caregivers had to reduce their working hours or give up their job in 77% of cases. CONCLUSIONS: The multidisciplinary treatment is affected by the inability of families to cope with a high cost, as well as with difficult access to these resources. In addition, there is great impact on the quality of life of patients, and their caregivers. Therefore, there is a need to evaluate the results of government health and socio-economic support plans for patients with rare diseases, and make a real response to their needs

[Health and socio-educational needs of the families and children with rare metabolic diseases: Qualitative study in a tertiary hospital]. / Necesidades sanitarias y socioeducativas de niños con enfermedades raras de tipo metabólico y sus familias: estudio cualitativo en un hospital de tercer nivel.

INTRODUCTION: Rare diseases are a challenge for public health due to the lack of information on their magnitude. These include inborn errors of metabolism. The objective of this study was to assess the quality of life and social, health, economic, and educational needs of a group of paediatric patients with inborn errors of metabolism attended to in a hospital. MATERIAL AND METHOD: A questionnaire was developed based on the needs and expectations, based mainly on the Andalusian Plan for Rare Diseases. An analysis was performed on the variables of health, socioeconomic, and educational needs of 65 paediatric patients with inborn errors of metabolism. RESULTS: The respondents showed few possibilities to cope with medication (61%), special diet (86%), and other health benefits (79%). Just under half of them (43%) believed that the quality of family life had been greatly reduced since the onset of the disease. The main caregiver was the mother in 61.5% of cases, compared to 1.5% of cases in which it was the father. The primary caregivers had to reduce their working hours or give up their job in 77% of cases. CONCLUSIONS: The multidisciplinary treatment is affected by the inability of families to cope with a high cost, as well as with difficult access to these resources. In addition, there is great impact on the quality of life of patients, and their caregivers. Therefore, there is a need to evaluate the results of government health and socio-economic support plans for patients with rare diseases, and make a real response to their needs.

Prepubertal children with a history of extra-uterine growth restriction exhibit low-grade inflammation.

Intra-uterine growth restriction (IUGR) may induce significant metabolic and inflammatory anomalies, increasing the risk of obesity and CVD later in life. Similarly, alterations in the adipose tissue may lead to metabolic changes in children with a history of extra-uterine growth restriction (EUGR). These mechanisms may induce alterations in immune response during early life. The aim of the present study was to compare pro-inflammatory markers in prepubertal EUGR children with those in a reference population. A total of thirty-eight prepubertal children with a history of EUGR and a reference group including 123 healthy age- and sex-matched children were selected. Perinatal data were examined. In the prepubertal stage, the concentrations of inflammatory biomarkers were measured in both groups. The serum concentrations of C-reactive protein (CRP) and plasma concentrations of hepatocyte growth factor (HGF), IL-6, IL-8, monocyte chemotactic protein type 1 (MCP-1), neural growth factor, TNF-α and plasminogen activator inhibitor type 1 were determined. The plasma concentrations of inflammatory biomarkers CRP, HGF, IL-8, MCP-1 and TNF-α were higher in the EUGR group than in the reference group (P< 0·001). After adjustment for gestational age, birth weight and length, blood pressure values and TNF-α concentrations remained higher in the EUGR group than in the reference group. Therefore, further investigations should be conducted in EUGR children to evaluate the potential negative impact of metabolic, nutritional and pro-inflammatory changes induced by the EUGR condition.

Changes in plasma adipokines in prepubertal children with a history of extrauterine growth restriction.

OBJECTIVE: Because nutritional support in perinatal life has been associated with metabolic programming, children with a history of extrauterine growth restriction (EUGR) might display alterations in the adipocyte and in the secretion of adipokines. The aim of this study was to assess adiponectin, resistin, and leptin concentrations in prepubertal children with a history of EUGR, and to determine the potential correlation between these adipokines and metabolic parameters. METHODS: This case-control study sample included 38 prepubertal children with a history of EUGR and a control group of 123 healthy children of similar age and sex. Anthropometric measures and blood pressure were assessed. Biochemical markers and blood adipokine concentrations (adiponectin, resistin, and leptin) were evaluated. RESULTS: Adiponectin concentration was significantly lower in the EUGR group compared with controls (EUGR: 11.49 ± 6.07 versus control: 25.72 ± 10.13 µg/mL), and resistin concentration was higher (EUGR: 20332.95 ± 6401.25 versus control: 8056.31 ± 3823.63 pg/mL), even after adjustment for gestational age, weight, and size at birth. Systolic blood pressure was associated with adipokines concentrations in the EUGR group (P < 0.001). In EUGR children adiponectin was associated with high-density lipoprotein cholesterol (P = 0.042), whereas resistin was associated with carbohydrate metabolism parameters (P < 0.001). CONCLUSIONS: Early postnatal malnutrition in EUGR children could program adipose tissue. Plasma adipokines can be measured in childhood to identify precocious changes that may be associated with a higher risk for metabolic syndrome or cardiovascular disease later in life.

Nutritional assessment in neonatal and prepubertal children with a history of extrauterine growth restriction.

BACKGROUND: Nutritional deficit during perinatal stage may induce significant alterations in adipose tissue and increase the risk of obesity, metabolic syndrome and cardiovascular disease in children with a history of extrauterine growth restriction (EUGR). AIMS: To describe the nutritional status in neonatal and prepubertal with a history of EUGR and establish an association between EUGR and later conditions. STUDY DESIGN: Descriptive, analytical, observational case-control study. SUBJECTS: The study included a sample of 38 prepubertal children with a history of EUGR, and 123 gender-and-age matched controls. OUTCOME MEASURES: The EUGR group was asked to answer a food frequency questionnaire. Analysis of body composition in both groups included anthropometric measurements, assessment of blood pressure and biochemical markers. RESULTS: Newborns with EUGR received parenteral feeding with a standard nutritional regime and long-chain fatty acid support for 41 ± 23 days; enteral feeding with a special formula for premature infants was initiated at 7 ± 11 days of life. At the prepubertal stage, daily fiber and fatty acid intake in children who had experienced EUGR in the neonatal stage was below the recommended intake. In the EUGR group, the intake of vegetables, fruits and olive oil was below dietary recommendations, while the intake of butchery, fatty meats, pastries and snacks was above the recommendations for the Spanish population. CONCLUSIONS: Appropriate nutrition education strategies should be developed for children with a history of EUGR to prevent later associated pathologies, as neonatal nutritional support and feeding during childhood are associated with an increase in diseases in this risk group.

Profile of oxidant and antioxidant activity in prepubertal children related to age, gender, exercise, and fitness.

Tissue damage resulting from oxidative stress induced by a pathological condition might have more serious consequences in children than in adults. Researchers have not yet identified particular markers - alone or in combination with others - of oxidative stress, or their role in pediatric diseases. The aim of this study was to identify gender-based biomarkers for measuring oxidative stress. Oxidative biomarkers were studied in 138 healthy Spanish children (85 boys, 53 girls) 7 to 12 years of age, at the prepubertal (Tanner I) stage, independent of body mass index (BMI), age, fitness (measured by 20-m shuttle run test), and physical activity (measured by participation in an after-school exercise program). The oxidative biomarkers measured were lipid peroxidation products, total nitrites, protein carbonyls, and oxidized glutathione (GSSG). The antioxidant biomarkers measured were total glutathione (TG), reduced glutathione (GSH), superoxide dismutase activity (SOD), and glutathione peroxidase activity. In the study population, height, weight, waist circumference, and BMI were lower in girls than in boys. For oxidative biomarkers, boys had higher levels of protein carbonyl than girls (p < 0.001). In spite of this, girls had higher levels of GSSG (p < 0.001) and TG (p = 0.001), and a lower GSH/GSSG ratio (p < 0.001) than boys. For the antioxidant response, girls had higher levels of SOD (p = 0.002) than boys. All analyses were adjusted for BMI, age, fitness, and physical activity. In conclusion, prepubertal girls had higher oxidative stress than boys, in addition to higher levels of SOD, independent of age, BMI, fitness, and physical activity.

Are birth plans associated with improved maternal or neonatal outcomes?

PURPOSE: The purpose of this study was to determine whether birth plans are associated with improved obstetric and neonatal outcomes. STUDY AND DESIGN: This retrospective case-control study (N = 182) was conducted at a hospital in Córdoba, Spain, between August 2008 and September 2011. Obstetric and neonatal outcomes were compared between groups (women with and without birth plans). Chi-square statistics and Student's t-tests were used for statistical analysis. RESULTS: Women with birth plans were older and had a higher academic background than the control group. There were no significant differences between groups for any of the obstetric outcomes or 5-minute Apgar scores; however, there was a significant difference in umbilical artery cord blood pH values (p = .019). The percent of babies with umbilical cord blood pH < 7.24 among nulliparous women with birth plans (14.7%, n = 5) was lower than among babies of nulliparous women without birth plans (37.5%, n = 26). CLINICAL IMPLICATIONS: Findings of this study provide evidence that birth plans may be helpful and are not associated with any negative obstetric or neonatal outcomes. Mothers with birth plans and their babies have at least as good outcomes as those without a birth plan. Babies of nulliparous women with birth plans had better umbilical cord blood pH values than babies of nulliparous women without birth plans. Healthcare professionals can promote the use of birth plans as a resource to enhance communication of women's desires for labor and birth.

Evaluation of metabolic risk in prepubertal girls versus boys in relation to fitness and physical activity.

BACKGROUND: Low levels of cardiorespiratory fitness (CRF) and physical activity (PA) are associated with a risk of the development of metabolic syndrome. Contradictory findings are reported in the literature regarding the influence of sex and CRF and PA on metabolic changes. OBJECTIVE: The aim of this study was to analyze the effects of CRF and PA on lipid and carbohydrate metabolism biomarkers in boys and girls. METHODS: A total of 82 prepubertal boys and 55 girls (7-12 years of age) were classified according to sex, low or high CRF, and performance or nonperformance of PA. Anthropometric and blood pressure (BP) measurements, plasma lipid profile values, glucose and insulin levels, and homeostasis model assessment for insulin resistance were analyzed. RESULTS: The percentage of boys with high CRF and performance of PA was higher than that of girls (P < 0.05). When children of the same sex were compared, higher values for body mass index and waist circumference z-scores were found for boys with low CRF compared with boys with high CRF (P < 0.001) without differences between girls, and in all groups classified by PA. Systolic and diastolic BPs were higher in boys than in girls, in both CRF and PA groups (P < 0.05). In the low CRF and no PA groups, girls had higher plasma glucose, total cholesterol, and low-density lipoprotein cholesterol levels than boys, with higher high-density lipoprotein cholesterol and apolipoprotein A levels (P < 0.05). CONCLUSIONS: Sex in relation to CRF and PA could affect the plasma lipid profile. These changes in girls are associated with low CRF and low levels of PA. Considering these results, we suggest the need to improve CRF and promote PA, especially in girls, to reduce metabolic risk.

Non-traditional markers of metabolic risk in prepubertal children with different levels of cardiorespiratory fitness.

OBJECTIVE: To assess classical and non-classical metabolic risk biomarkers in prepubertal children with different levels of cardiorespiratory fitness (CRF). DESIGN: CRF was assessed by the 20 m shuttle run test. To estimate physical activity, participants were observed while engaged in an after-school programme. Additionally, a short test based on a validated questionnaire was used to obtain information about physical activity practice and sedentary habits. Anthropometric parameters, blood pressure, and classical and non-traditional metabolic risk biomarkers--plasma lipid profile, glucose and insulin, homeostasis model assessment-insulin resistance index (HOMA-IR), plasma uric acid, transaminases and C-reactive protein (CRP)--were measured. SETTING: The study was conducted in local elementary schools in Córdoba, Spain. SUBJECTS: One hundred and forty-one healthy children (eighty-eight boys, fifty-three girls) aged 7-12 years, in Tanner stage I, were recruited. They were divided into two groups after they performed the 20 m shuttle run test: equal or higher cardiovascular fitness (EHCF) group and low cardiovascular fitness (LCF) group. RESULTS: The LCF group displayed significantly higher TAG (P = 0.004) and lower HDL cholesterol levels (P = 0.001), as well as significantly lower values for the non-traditional lipid marker apo-A1 (P = 0.001) compared with the EHCF group. The LCF children displayed higher plasma glucose (P = 0.003) and insulin levels, higher HOMA-IR scores (P < 0.001) and higher plasma uric acid and CRP levels (P < 0.05). After adjustment for BMI, age and sex, no statistically significant differences were found between groups for the biomarkers analysed. CONCLUSIONS: The study provides new information to understand the role not only of weight status but also of the level of CRF on the metabolic health profile of prepubertal children.