Epidemiologic trend of pediatric inflammatory bowel disease in Latin America: The Latin American Society for Pediatric Gastroenterology, Hepatology and Nutrition (LASPGHAN) Working Group.

INTRODUCTION AND AIMS: The primary aim was to explore the epidemiologic trend of pediatric inflammatory bowel disease in Latin America, and the secondary aims were to obtain an overview of the diagnostic/therapeutic focus of the members of the LASPGHAN and examine the relation of case frequency to year, during the study period. MATERIALS AND METHODS: Latin American pediatric gastroenterologists participated in an online survey, conducted through the SurveyMonkey platform, that investigated the yearly frequency of new inflammatory bowel disease patients within the time frame of 2005-2016, their disease variety, the gastrointestinal segments affected, and the diagnostic and treatment methods utilized. The correlation of new case frequency with each study year was evaluated. RESULTS: A total of 607 patients were studied. The diagnoses were ulcerative colitis in 475 (78.3%) cases, Crohn's disease in 104 (17.1%), and inflammatory bowel disease D unclassified in 28 (4.6%). The trend in ulcerative colitis was a lineal increase in the frequency of new cases related to each study year, with a significant correlation coefficient. Pancolitis was found in 67.6% of the patients. The diagnostic methods included clinical data, endoscopy, and biopsies in more than 99% of the cases, and imaging studies were indicated selectively. Drug regimens were limited to 5-aminosalicylic acid derivatives, azathioprine, 6-mercaptopurine, infliximab, and adalimumab. CONCLUSIONS: Pediatric inflammatory bowel disease in Latin America appears to have increased during the years included in the study period, with a predominance of moderate or severe ulcerative colitis. That lineal trend suggests the predictive likelihood of a gradual increase in the coming years, with possible epidemiologic and clinical implications.

Epidemiologic trend of pediatric inflammatory bowel disease in Latin America: The Latin American Society for Pediatric Gastroenterology, Hepatology and Nutrition (LASPGHAN) Working Group. / Tendencia epidemiológica de la enfermedad intestinal inflamatoria en pacientes pediátricos en América Latina: Grupo de Trabajo en Enfermedad Intestinal Inflamatoria, Sociedad Latinoamericana de Gastroenterología, Hepatología y Nutrición Pediátrica (SLAGHNP).

INTRODUCTION AND OBJECTIVES: The primary aim was to explore the epidemiologic trend of pediatric inflammatory bowel disease in Latin America, and the secondary aims were to obtain an overview of the diagnostic/therapeutic focus of the members of the LASPGHAN and examine the relation of case frequency to year, during the study period. MATERIALS AND METHODS: Latin American pediatric gastroenterologists participated in an online survey, conducted through the SurveyMonkey platform, that investigated the yearly frequency of new inflammatory bowel disease patients within the time frame of 2005 to 2016, their disease variety, the gastrointestinal segments affected, and the diagnostic and treatment methods utilized. The correlation of new case frequency with each study year was evaluated. RESULTS: A total of 607 patients were studied. The diagnoses were ulcerative colitis in 475 (78.3%) cases, Crohn's disease in 104 (17.1%), and inflammatory bowel disease D unclassified in 28 (4.6%). The trend in ulcerative colitis was a lineal increase in the frequency of new cases related to each study year, with a significant correlation coefficient. Pancolitis was found in 67.6% of the patients. The diagnostic methods included clinical data, endoscopy, and biopsies in more than 99% of the cases, and imaging studies were indicated selectively. Drug regimens were limited to 5-aminosalicylic acid derivatives, azathioprine, 6-mercaptopurine, infliximab, and adalimumab. CONCLUSIONS: Pediatric inflammatory bowel disease in Latin America appears to have increased during the years included in the study period, with a predominance of moderate or severe ulcerative colitis. That lineal trend suggests the predictive likelihood of a gradual increase in the coming years, with possible epidemiologic and clinical implications.

Cytokine distribution in mothers and breastfed children after omega-3 LCPUFAs supplementation during the last trimester of pregnancy and the lactation period: A randomized, controlled trial.

OBJECTIVE: To determine whether maternal diet supplementation with omega-3 long chain polyunsaturated fatty acids (omega-3 LC-PUFAs) during the last trimester of pregnancy and the breastfeeding period influences the levels of inflammatory cytokines in mother and infants. MATERIAL AND METHOD: This registered, double-blind randomized study included 46 pregnant women, who were randomly allocated to either an experimental group receiving 400mL/day of a fish oil-enriched dairy drink [320mg docosahexaenoic acid (DHA) + 72mg eicoapentaenoic acid] (FO group, n = 24) or to a control group receiving 400mL/day of a non-supplemented dairy drink (CT group, n = 22), from week 28 of pregnancy until the fourth month of lactation. During the study, maternal dietary patterns were monitored by a nutritionist, who encouraged compliance with current recommendations of fatty acids intake. DHA concentrations and cytokine levels (GM-CSF, IL-2, IL-4, IL-6, IL-10, INF-γ and TNF-α) were measured in maternal plasma at the moment of recruitment and in maternal (n = 46) and infant (n = 46) plasma at birth and 2.5 months after birth. RESULTS: Maternal plasmatic IL-4 levels were higher in FO than in CT subjects (p = 0.009). Additionally, a tendency was observed to higher IL-10 and IL-2 in the FO group. Plasmatic IL-6 however, was higher in CT mothers (p = 0.001). TNF-α was higher in CT infants at birth and 2.5 months after birth (p = 0.005). An analysis of possible relationships between DHA and the concentrations of different cytokines revealed negative correlation between maternal plasmatic IL-6 and DHA (higher plasmatic DHA corresponded to lower IL-6). CONCLUSIONS: Maternal dietary omega-3 LC-PUFAs supplementation during critical periods like pregnancy, lactation and early newborn development may influence the levels of certain inflammatory cytokines, reducing pro-inflammatory cytokines and promoting an anti-inflammatory "environment".

Tyrosinemia type II: Mutation update, 11 novel mutations and description of 5 independent subjects with a novel founder mutation.

BACKGROUND: Tyrosinemia type II, also known as Richner-Hanhart Syndrome, is an extremely rare autosomal recessive disorder, caused by mutations in the gene encoding hepatic cytosolic tyrosine aminotransferase, leading to the accumulation of tyrosine and its metabolites which cause ocular and skin lesions, that may be accompanied by neurological manifestations, mostly intellectual disability. AIMS: To update disease-causing mutations and current clinical knowledge of the disease. MATERIALS AND METHODS: Genetic and clinical information were obtained from a collection of both unreported and previously reported cases. RESULTS: We report 106 families, represented by 143 individuals, carrying a total of 36 genetic variants, 11 of them not previously known to be associated with the disease. Variants include 3 large deletions, 21 non-synonymous and 5 nonsense amino-acid changes, 5 frameshifts and 2 splice variants. We also report 5 patients from Gran Canaria, representing the largest known group of unrelated families sharing the same P406L mutation. CONCLUSIONS: Data analysis did not reveal a genotype-phenotype correlation, but stressed the need of early diagnosis: All patients improved the oculocutaneous lesions after dietary treatment but neurological symptoms prevailed. The discovery of founder mutations in isolated populations, and the benefits of early intervention, should increase diagnostic awareness in newborns.

Breastfeeding during the first 6 months of life, adiposity rebound and overweight/obesity at 8 years of age.

BACKGROUND: The question of whether breastfeeding protects the child from obesity is a still debated issue; however, the relationship between early adiposity rebound and higher risk of obesity is well known. This study was aimed at evaluating whether breastfeeding (without formula supplement) during the first 6 months of life delays the time of adiposity rebound and consequently reduces the rates of obesity at the age of 8. METHODS: This retrospective cohort study included 1812 children born in Gran Canaria in 2004, with follow-up until they were 8 years of age. Anthropometrical data had been taken during routine visits to the doctor and were extracted from medical record databases. Only children with breastfeeding for the first 6 months of life (173 children) and children without breastfeeding (192 children) were included. Children with mixed feeding and children whose data were not available were excluded. RESULTS: No body mass index (BMI) differences were found between children with breastfeeding (17.7) or without breastfeeding (17.3) during the first 6 months of life. The percentages of children with normal weight, overweight and obesity were similar in both groups, as well as the age of adiposity rebound breastfeeding 3.61 years; formula 3.64 years). Early adiposity rebound was associated with increased BMI at the age of 8, both in male and female children. CONCLUSIONS: Breastfeeding during the first 6 months of life was not demonstrated to delay the age of the adiposity rebound, in our study.

Effect of Zinc Intake on Growth in Infants: A Meta-analysis.

A systematic review and meta-analysis of available randomized controlled trials (RCTs) was conducted to evaluate the effect of zinc (Zn) intake on growth in infants. Out of 5500 studies identified through electronic searches and reference lists, 19 RCTs were selected after applying the exclusion/inclusion criteria. The influence of Zn intake on growth was considered in the overall meta-analysis. Other variables were also taken into account as possible effect modifiers: doses of Zn intake, intervention duration, nutritional status, and risk of bias. From each select growth study, final measures of weight, length, mid upper arm circumference (MUAC), head circumference, weight for age z-score (WAZ), length for age z-score (LAZ), and weight for length z-score (WLZ) were assessed. Pooled ß and 95% confidence interval (CI) were calculated. Additionally, we carried out a sensitivity analysis. Zn intake was not associated with weight, length, MUAC, head circumference, and LAZ in the pooled analyses. However, Zn intake had a positive and statistically effect on WAZ (ß = 0.06; 95%CI 0.02 to 0.10) and WLZ (ß = 0.05; 95%CI 0.01 to 0.08). The dose-response relationship between Zn intake and these parameters indicated that a doubling of Zn intake increased WAZ and WLZ by approximately 4%. Substantial heterogeneity was present only in length analyses (I(2) = 45%; p = 0.03). Zn intake was positively associated with length values at short time (four to 20 weeks) (ß = 0.01; CI 95% 0 to 0.02) and at medium doses of Zn (4.1 to 8 mg/day) (ß = 0.003; CI 95% 0 to 0.01). Nevertheless, the effect magnitude was small. Our results indicate that Zn intake increases growth parameters of infants. Nonetheless, interpretation of these results should be carefully considered.

[Quantitative analysis of nutrient intake in children under 3 years old. ALSALMA study]. / Análisis cuantitativo de la ingesta de nutrientes en niños menores de 3 años. Estudio ALSALMA.

OBJECTIVE: The objective of the study was to analyze the nutritional patterns of children under three years of age and to compare the results against the recommendations for energy and nutrient intake. PATIENTS AND METHODS: In this cross-sectional epidemiological study, parents completed a dietary diary on their food intake of their children on 4 non-consecutive days. The percentage of children with mean intakes below the recommendations for each age and nutrient was analyzed using the "Estimated Average Requirement (EAR) cut-point method." RESULTS: A total of 186 pediatricians included 1701 children in the study. A total of 95.9% (n=1320) of the children between 7 and 36 months had a protein consumption more than twice that of the Recommended Daily Allowances. The deficiencies observed (% < EAR) in the age groups 13-24 months and 25-36 months, respectively, were: vitamin D in 81.7% and 92.1%; vitamin E in 39.3% and 53.4%; folic acid in 12.5% and 14.8%; calcium in 10.1% and 5.5%; iodine in 27.1% and 31%. It was observed that a higher percentage in the daily intake of proteins (P=.013) and of carbohydrates (P<.0001), and a lower percentage of total lipids (P<.0001), were related to a greater body mass index, regardless of energy intake. CONCLUSIONS: The study presents a very detailed view of the eating patterns of Spanish children less than three years of age. The encouragement of healthy feeding should be directed towards the correction of the dietary imbalances detected, in order to promote the future health of children.

[Evaluation of diet and nutrient intake in children under three years old. ALSALMA pilot study]. / Evaluación de la alimentación y consumo de nutrientes en menores de 3 años. Estudio piloto ALSALMA.

OBJECTIVE: The present study evaluates energy and nutrient intake in Spanish children under three years of age, and compares the results with the current recommendations in order to identify possible inadequate nutrient intake. PATIENTS AND METHODS: A cross-sectional pilot study. The mothers completed a diet diary for four non-consecutive days, recording the products and amounts consumed by their children. Nutrient intake was calculated, and the results were compared with the dietary reference intakes (DRI) for each age group. RESULTS: A total of 188 children (93 boys and 95 girls) aged 0-6 (n=41), 7-12 (n=24), 13-24 (n=57), and 25-36 months (n=66) were included. Statistically significant differences in DRI were observed for most of the nutrients analyzed. Protein intake, in particular was 376% of DRI in children between 1-3 years of age. By age groups, 96% of the children aged 7-12 months, 88% of the children aged 13-24 months, and 97% of the children aged 25-36 months showed protein intakes more than two-fold DRI. CONCLUSIONS: Nutrient intake differed from the DRI, particularly as regards proteins. A new study is required to determine whether the observed study deviations could be representative of the national population of this age group, as well as the possible effects on child health.

Analysis of the Spanish national registry for pediatric home enteral nutrition (NEPAD): implementation rates and observed trends during the past 8 years.

BACKGROUND/OBJECTIVES: The home enteral nutrition (HEN) provides nutritional support to children with chronic diseases who are nutritionally compromised and allows them to be discharged more quickly from hospitals. In 2003, a web-based registry (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition -NEPAD-) was created with the objective of gathering information about pediatric HEN practices in Spain. AIM: The aim of this study was to report the implementation of the NEPAD (Nutrición Enteral Pediátrica Ambulatoria y Domiciliaria, Pediatric Ambulatory and Home Enteral Nutrition) registry of pediatric HEN in Spain and to analyze data evolution trends from 2003 to 2010. SUBJECTS/METHODS: The data from the Spanish NEPAD registry were analyzed according to the following variables: demographic data, diagnosis, indication for HEN, nutritional support regime and administration route. RESULTS: Over the study period, 952 patients (1048 episodes) from 20 Spanish hospitals were included in the NEPAD registry. The most frequent indication for HEN was decreased oral intake (64%), and neurological disease was the most prevalent illness. HEN was delivered via a nasogastric tube in 573 episodes (54.7%), by gastrostomy in 375 episodes (35.8%), oral feeding in 77 episodes (7.3%) and by jejunal access in 23 episodes (2.2%). Significant differences in the mode of administration were observed based on the pathology of the child (χ(2), P<0.0001). The cyclic feeding was the most widely used technique for the administration of HEN. Most of the patients used a pump and a polymeric formula. Transition to oral feeding was the primary reason for discontinuation of this type of support. CONCLUSIONS: Since the NEPAD registry was established in Spain, the number of documented patients has increased more than 25-fold. Many children with chronic illness benefit from HEN, mainly those suffering from neurological diseases.

[Nutritional problems perceived by pediatricians in Spanish children younger than 3 years]. / Problemas nutricionales percibidos por los pediatras en niños Españoles menores de 3 años.

INTRODUCTION: Parents are most responsible for nutritional education of children, and pediatricians must advise and help them with their doubts. The purpose of this study was to know the relevance of nutritional topics in daily practice and the main nutritional problems perceived by pediatricians in children under 3 years of age. METHODS: Descriptive, cross-sectional study performed in 2 stages. First stage consisted on discussion meetings with 30 random-selected pediatricians from Madrid and Barcelona. Results were used to design the on-line questionnaire of the second stage (76 questions related to nutrition in children under 3 years). A random and representative sample of Spanish pediatricians was selected for this stage. RESULTS: One hundred and fifty one pediatricians, among 258 sampled, completed the questionnaire. They referred to see a mean of 588 patients/month. The main perceived nutritional problems in the first year of life were iron and vitamin deficiencies and poor weight gain. In the next months, excess in carbohydrates and lipid intake and overweight were the main problems perceived. Parents were considered the main actors regarding their children's nutritional health, but their concern in these questions significantly reduced with children's age (p < 0,0001). Factors considered to have the greatest relevance in the acquisition of good nutritional habits were to provide a diet adjusted for children's requirements and to observe the appropriate sleeping hours. The proportion of children who receive nutritional counseling varied from 88% (0-6 months) to 61% (24-36 months). CONCLUSIONS: Despite of the relevance given by pediatricians, educational intervention regarding nutritional health is not ideal. Nutritional problems perceived by pediatricians varied with children's age.

[Epidemiological study of the metabolic diseases with homocystinuria in Spain]. / Estudio epidemiológico de las enfermedades metabólicas con homocistinuria en España.

OBJECTIVES: To determine the prevalence of homocystinuria in Spain and to establish the measures and mechanisms to ensure its prevention, diagnosis and treatment. MATERIAL AND METHODS: A national cross-sectional survey was conducted by means of a questionnaire sent to 35 hospitals in which children and adult patients are treated. RESULTS: Using the questionnaires submitted by 25 physicians from 16 centres, 75 patients were identified: 41 transsulphuration defects (one deceased), 27 remethylation (six deaths) and 7 without a syndromic diagnosis. The age at diagnosis varied widely, and 18 cases had more than one sibling affected. The more severe clinical manifestations involved the patients with remethylation defects. There was a high percentage of cognitive impairment, followed by lens diseases. Almost half of the patients had neurological disorders. There was increased vascular involvement in CBS-deficient adults. The therapeutic options most used were, folic acid, hydroxycobalamin and betaine. CONCLUSIONS: In view of these results and especially the small number of CBS deficiencies detected, we conclude that there is a need to introduce newborn screening for classical homocystinuria and ensure implementation of an appropriate diagnostic workup in all patients at risk.

[Guidelines for the treatment of functional constipation]. / Recomendaciones para el tratamiento del estreñimiento funcional.

Constipation is common in childhood. It can affect around 5-30% of the child population, depending on the criteria used for diagnosis. The currently recommended treatment is based on three main points: a) explanation, b) disimpaction and c) maintenance therapy consisting of diet changes, behavioural modification, and the use of laxatives. In the last decades treatment on disimpaction have changed radically from the rectal route to the oral route with polyethylene glycol 3350 (PEG), the most used and accepted regimen nowadays. Treatment and care should take into account the individual needs and preferences of the patient. Good communication is essential, to allow patients to reach informed decisions about their care. Maintenance therapy consists of dietary interventions, toilet training, and laxatives to obtain daily painless defaecation in order to prevent re-accumulation of stools. Maintenance therapy should be start as soon as the child's bowel is disimpacted. Early intervention with oral laxatives may improve complete resolution of functional constipation. Enemas using phosphate, mineral oil, or normal saline are effective in relieving rectal impaction, but carry the risk of mechanical trauma and are not recommended for maintenance therapy in the paediatric population. Among osmotic agents, polyethylene glycol 3350 plus electrolyte solutions appear to be the first-line drug treatment to use in children of any age, as it is safe, effective, and well-tolerated. Recommended doses ranges from 0.25 to 1.5g/kg. Advances in the understanding of the gastrointestinal enteric nervous system and epithelial function have led to the development of new substances that bind to serotonin receptors or are chloride channel activators.

Methylmalonic acidaemia: examination of genotype and biochemical data in 32 patients belonging to mut, cblA or cblB complementation group.

Methylmalonic acidaemia (MMA) is a genetic disorder caused by defects in methylmalonyl-CoA mutase or in any of the different proteins involved in the synthesis of adenosylcobalamin. The aim of this work was to examine the biochemical and clinical phenotype of 32 MMA patients according to their genotype, and to study the mutant mRNA stability by real-time PCR analysis. Using cellular and biochemical methods, we classified our patient cohort as having the MMA forms mut (n = 19), cblA (n = 9) and cblB (n = 4). All the mut (0) and some of the cblB patients had the most severe clinical and biochemical manifestations, displaying non-inducible propionate incorporation in the presence of hydroxocobalamin (OHCbl) in vitro and high plasma odd-numbered long-chain fatty acid (OLCFA) concentrations under dietary therapy. In contrast, mut (-) and cblA patients exhibited a milder phenotype with propionate incorporation enhanced by OHCbl and normal OLCFA levels under dietary therapy. No missense mutations identified in the MUT gene, including mut (0) and mut (-) changes, affected mRNA stability. A new sequence variation (c.562G>C) in the MMAA gene was identified. Most of the cblA patients carried premature termination codons (PTC) in both alleles. Interestingly, the transcripts containing the PTC mutations were insensitive to nonsense-mediated decay (NMD).

[Child subnutrition, health and poverty, integral intervention programme]. / Desnutrición infantil, salud y pobreza: intervención desde un programa integral.

Childood is considered a transcendental stage in the evolutionary process of man, characterized by two phenomenon: growth and development, for which a suitable nutrition is fundamental. The damage that causes undernourishment that is suffered in childhood is greatly lamented by society, since in this stage it is the child's brain that suffers the greatest impact, in which irreversible metabolisms and structural alterarions will occur. Nevertheless, infant undernourishment is not only a problem of lack of fodds, but is also a deeper social conflict, that must be considered at the time of offereing solutions. The Corporation for Childhood Nutrition (CONIN), founded in Chile in 1975, has the mission to help in the recovery of the children from 0 to 3 years who present primary or secondary undernourishment. From 1993, this proyect was extended to the province of Mendoza, Argentina, as the CONIN Foundation (Cooperative for Childhood Nutrition), where this experience was completed by creating Undernourishment Prevention Centres. At the moment this project has been developed in different provinces from the country, in Paraguay and in the near future in Peru, sustaining its work on three pillars: teaching, attendance and investigation. CONIN develops a intervention strategy of great impact, in their area of influence, which is used on the low income population. This in turn causes high social repercusions, as the family and its surrounding are reinforced as a basis for the correct physical and intellectual development of the child, together with an adequate nutrients contribution, allowing the child to develop his genetic potential.

Assessment of the DQ heterodimer test in the diagnosis of celiac disease in the Canary Islands (Spain).

BACKGROUND: Celiac disease is a multifactorial disorder of the proximal small intestine associated with a permanent intolerance to gluten. The HLA-DQ(alpha1*0501, beta1*02) heterodimer is strongly associated with this disease. MATERIALS AND METHODS: The authors studied a sample of 354 unrelated Caucasoid individuals: 118 patients with celiac disease and 236 control subjects. All patients and controls subjects were born in Gran Canaria (Canary Islands) at least two generations ago. The authors typed the HLA-DQA1 and DQB1 genes by DNA methods. The positive and negative predictive values of the test were studied. RESULTS: The mean age at diagnosis was 25.4 months, with a statistically significant proportion of females (64.4%, P < 0.002). For DQB1 gene, the susceptibility allele found was DQB1*02 (relative risk [RR] = 7.60, confidence interval [CI]: 5.35-10.78), whereas for the DQA1 gene, the susceptibility alleles found were DQA1*0501 (RR = 2.99, CI: 2.16-4.14) and DQA1*0201 (RR = 1.88, CI: 1.25-2.82). The presence of the DQ(alpha1*0501, beta1*02) heterodimer was strongly associated with the disease (92.4% in the patients group vs. 21.6% in control subjects). HLA-DQ8 heterodimer was absent in the authors' patients. DQB1*02 homozygous subjects presented a higher relative risk for celiac disease. There was no correlation of DQB1*02 dosage with age at onset below 12 years of age or with gender distribution. Sensitivity, specificity, and the positive and negative predictive values of the test were 92.4%, 78.4%, 68.1%, and 95.4%, respectively. CONCLUSIONS: The presence of the DQ2 (DQA1*0501/DQB1*02) heterodimer is strongly associated with celiac disease in the population studied by the authors. The value of this test derives from its ability to exclude disease when a negative result occurs.

Determinants of nutrient intake among children and adolescents: results from the enKid Study.

BACKGROUND: Although adequacy of nutrient intake has been studied considerably in children and adolescents across Europe, the factors associated with nutritional risk have rarely been addressed. This study was developed in order to explore the nutritional intakes of Spanish children and the factors influencing the risk of nutritional inadequacy. OBJECTIVES: To evaluate socio-economic and lifestyle variables associated with nutritional adequacy in Spanish children and adolescents. METHODS: A cross-sectional study utilising face-to-face interviews. A random sample of 3,534 individuals aged 2-24 years were interviewed by a team of 43 dieticians in the subjects' homes. Interviews included two 24-hour recalls (a second 24-hour recall in 25% of the sample) and other questions, including lifestyle. Weight and height were measured in all subjects. Under-reporters (18%) were excluded from the present analysis. An unconditional logistic regression analysis was used to identify variables associated with greater nutritional risk. RESULTS: The participation rate was 68%. Twenty percent of males and 50% of females were classified as being at high nutritional risk. Variables associated with increased nutritional risk were: age between 14 and 24 years, being female, low social class, low educational level of the mother, having more than one sibling, smoking, watching TV during meals, sedentary habits at leisure time, infrequent meals and a poor quality breakfast. One dietary factor closely associated with nutritional risk was a failure to consume ready-to-eat cereals. CONCLUSIONS: Nutritional risk during infancy and adolescence is associated with socio-economic and educational variables of the family, and some lifestyle factors including physical activity and the quality of the breakfast meal.

[Current indications of esophageal pH-monitoring]. / Indicaciones actuales de la monitorización de la pHmetría esofágica.

Regurgitation is a common manifestation in infants below the age of 1 year and a frequent cause of visits to general practitioners and pediatricians. Accurate diagnosis of this condition requires a good choice of pH monitoring equipment and optimal technique when performing this procedure. The present article is a consensus statement of the Working Group on Gastroesophageal Reflux of the Spanish Society of Pediatric Gastroenterology, Hepatology and Nutrition (SSPGHN) and reflects the conclusions reached in the VII Congress of the SSPGHN (Vigo, 2000). In general, esophageal pH monitoring is indicated in the following situations: (i) when symptoms of gastroesophageal reflux are present and evolution is not favorable, despite appropriate treatment; (ii) when trying to determine the relationship between gastroesophageal reflux and non-digestive symptoms and (iii) when evaluating the results of treatment, whether medical or surgical.

[Diagnostic approach and treatment of inherited mitochondrial fatty acid oxidation disorders]. / Aproximación diagnóstica y tratamiento de los errores innatos de la oxidación mitocondrial de los ácidos grasos.

Inherited mitochondrial fatty acid oxidation disorders are a complex set of genetically-based diseases in which up to 22 different entities are currently recognized. Their incidence is probably underestimated because a high level of diagnostic suspicion is required for their detection. Their clinical spectrum and prognosis are variable. In recent years knowledge of these diseases and improved treatment have reduced associated mortality. A common characteristic of all these diseases is hypoketotic hypoglycemia, although this is not constant and does not appear in the short-chain disorders and, sometimes, does not even appear in the medium-chain disorders. Cardiac or skeletal myopathy combined and/or hepatic involvement at periods of metabolic decompensation are typical, since these tissues depend on fatty acid oxidation. Diagnosis has been simplified by the study of acylcarnitines in blood, even in periods of metabolic stability. Determination of acylglycines, organic acids, carnitines, free fatty acids and 3-hydroxy-fatty acids, together with enzymic and genetic studies, complete the diagnosis. In certain circumstances, a provocation test should be carried out. Treatment basically consists of avoiding fasting, restricting fatty acid uptake and increasing carbohydrate uptake, depending on the type of metabolic disorder. Pharmacological treatment may also be added (carnitine, riboflavine or carbamylglutamate).

[Surgical options in the treatment of short bowel syndrome with loss of the ileocecal valve. Intestinal valve, intestinal duplication]. / Opciones quirúrgicas en el tratamiento del síndrome de intestino corto con pérdida de la válvula ileocecal. Válvula intestinal, desdoblamiento intestinal.

Morbidity and mortality are especially increased in pediatric patients affected of short bowel syndrome in whom ileocecal valve was resected. This report concerns 13 infants and children with short gut syndrome with ileocecal valve resected treated in the last 4 years. In two of them we performed intestinal duplication and in seven an antireflux intestinal valve with a segment of bowel. With that procedure we achieved a significant shortening of the hospital stay as well as reduction in time and quantity of total parenteral nutrition. The rest of the patients were treated with conservative management.