Dietary management of infants and young children with feeding difficulties and unsatisfactory weight gain using a nutritionally complete hypercaloric infant formula. practical considerations from clinical cases.

INTRODUCTION: A decrease in weight velocity and feeding difficulties in infants may be caused by an inadequate caloric intake and underlying medical conditions. CASE DESCRIPTION: By focusing on four clinical cases, this article illustrates the temporary use of a special infant formula in orally-fed and enterally-fed infants with unsatisfactory weight gain and special medical conditions such as gastrointestinal and neurological disorders. The formula was a nutritionally complete hypercaloric infant formula containing partially hydrolyzed whey protein. It was used after full consideration of all feeding options including breastfeeding. CONCLUSION: Implementing appropriate feeding behaviors, adapted to age and potential comorbidities, is an essential prerequisite for therapeutic management. The use of a nutritionally complete hypercaloric infant formula can be helpful to manage unsatisfactory weight gain and feeding difficulties in infants.

DGAT1 mutations leading to delayed chronic diarrhoea: a case report.

BACKGROUND: Early-onset chronic diarrhoea often indicates a congenital disorder. Mutation in diacylglycerol o-acyltransferase 1 (DGAT1) has recently been linked to early-onset chronic diarrhoea. To date, only a few cases of DGAT1 deficiency have been reported. Diarrhoea in those cases was severe and developed in the neonatal period or within 2 months after birth. CASE PRESENTATION: Here, we report a female patient with DGAT1 mutations with delayed-onset chronic diarrhoea. The patient had vomiting, hypoalbuminemia, hypertriglyceridemia, and failure to thrive at early infancy. Her intractable chronic diarrhoea occurred until she was 8 months of age. A compound heterozygous DGAT1 mutation was found in the patient, which was first found in the Chinese population. Her symptoms and nutrition status improved after nutritional therapy, including a fat restriction diet. CONCLUSIONS: This case expanded our knowledge of the clinical features of patients with DGAT1 mutations. Intractable diarrhoea with delayed onset could also be a congenital disorder.

Changes in Age at Diagnosis and Nutritional Course of Celiac Disease in the Last Two Decades.

The frequency of celiac disease (CD) has increased along time, with relevant changes reported in geographical variations, clinical presentation and nutritional repercussions. In recent years, some celiac patients are presenting overweight/obesity, but it is unclear how frequent this is and to what extent undernutrition remains a concern. This is relevant because CD tends to be overlooked in overweight patients. With this in mind, we assessed age at diagnosis, clinical characteristics and nutritional status of 155 celiac patients diagnosed between 1994-2017 in four pediatric hospitals in Santiago, Chile. Since 2003, the number of patients diagnosed has increased (p < 0.0033), coinciding with antitransglutaminase and antiendomysial antibodies becoming available to public health systems. In 2000, 4.5% of patients were asymptomatic at diagnosis, suggesting that active search is not routinely applied. Gastrointestinal symptoms plus failure to thrive were significantly more frequent under 2 years (p = 0.0001). Nutritional status has improved at diagnosis and during follow up, but undernutrition remains more frequent in children <2 and <5 years (p < 0.002 and p < 0.0036, respectively). Overweight at diagnosis was reported in 2002 and obesity in 2010. After initiating treatment, since 2010, patients changing from undernourishment to overweight has sometimes been observed after only 6 months on a gluten-free diet.

Nocturnal enteral nutrition is therapeutic for growth failure in Fanconi-Bickel syndrome.

Fanconi-Bickel syndrome (FBS) is a rare autosomal recessive disorder characterised by impaired glucose liver homeostasis and proximal renal tubular dysfunction. It is caused by pathogenic variants in SLC2A2 coding for the glucose transporter GLUT2. Main clinical features include hepatomegaly, fasting hypoglycaemia, postprandial hyperglycaemia, Fanconi-type tubulopathy occasionally with rickets, and a severe growth disorder. While treatment for renal tubular dysfunction is well established, data regarding optimal nutritional therapy are scarce. Similarly, detailed clinical evaluation of treated FBS patients is lacking. These unmet needs were an incentive to conduct the present pilot study. We present clinical findings, laboratory parameters and molecular genetic data on 11 FBS patients with emphasis on clinical outcome under various nutritional interventions. At diagnosis, the patients' phenotypic severity could be classified into two categories: a first group with severe growth failure and rickets, and a second group with milder signs and symptoms. Three patients were diagnosed early and treated because of family history. All patients exhibited massive glucosuria at diagnosis and some in both groups had fasting hypoglycaemic episodes. Growth retardation improved drastically in all five patients treated by intensive nutritional intervention (nocturnal enteral nutrition) and uncooked cornstarch with final growth parameters in the normal range. The four severely affected patients who were treated with uncooked cornstarch alone did not catch up growth. All patients received electrolytes and l-carnitine supplementation to compensate for the tubulopathy. This is one of the largest series of FBS on therapeutic management with evidence that nocturnal enteral nutrition rescues growth failure.

Failure to Thrive: A Practical Guide.

Children with very low weight for age or height and those who do not maintain an appropriate growth pattern may have failure to thrive (FTT), also known as weight faltering. If confirmed by repeated valid measurements, FTT should prompt a search for causes of undernutrition, including neglect, family food insecurity, and underlying medical conditions. Inadequate caloric intake is the most common cause of FTT, but inadequate nutrient absorption or increased metabolism is also possible. Difficulty attaining or maintaining appropriate weight is the first indication of FTT, and sustained undernutrition can impede appropriate height, head circumference, and the development of cognitive skills or immune function in extreme cases. Early identification and management of the issues causing undernutrition are critical. In most cases, an appropriate growth velocity can be established with outpatient management based on proper nutrition and family support. Primary care physicians can effectively treat most children with FTT, and subspecialist consultation or hospitalization is rarely indicated.

Milk-sensitive eosinophilic gastroenteritis in a 2-month-old boy.

Eosinophilic gastroenteritis is a rare disease of unknown aetiology, characterised by eosinophilic infiltration of the gastrointestinal wall with various gastrointestinal manifestations. Clinical presentation and radiological findings are non-specific and there is an overlap with more frequent childhood diseases requiring a high degree of clinical suspicion for accurate diagnosis. We describe a 2-month-old boy with prolonged diarrhoea, vomiting and food refusal. Diagnosis was settled by histology. The treatment with elemental diet was successful, with clinical resolution and catch-up growth.

Caloric intake during the perioperative period and growth failure in infants with congenital heart disease.

Infants with congenital heart disease have impaired weight gain during the first several months of life. Efforts have focused on improving weight gain and nutritional status during the first months of life. Close examination of the data suggests that the immediate postoperative period is problematic. Etiology of this early growth failure should be identified to develop effective interventions. This is a retrospective study of neonates who underwent modified systemic-to-pulmonary artery shunt, including Norwood palliation, at Children's Healthcare of Atlanta between January 2009 and July 2011. We analyzed growth from time of surgical intervention to hospital discharge. Measures of calculated weight-for-age Z-score (WAZ score) were performed using the World Health Organization's Anthro Software (version 3.2.2, January 2011; WHO, Geneva, Switzerland). Seventy-three patients were identified. Eight patients did not meet inclusion criteria. Complete data were collected on the remaining 65 patients. Median caloric intake patients received was 50.4 [interquartile range (IQR) 41.6 to 63.6] calories/kg/day while exclusively on parental nutrition. At hospital discharge, the median WAZ score was -2.0 (IQR -2.7 to -1.2) representing an overall median WAZ score decrease of -1.3 (IQR -1.7 to -0.7) from time of shunt palliation to hospital discharge. Despite studies showing poor weight gain in infants with congenital heart disease after neonatal palliation, this study reports the impact of hospital-based nutritional practices on weight gain in infants during the immediate postoperative period. Our data demonstrate that actual caloric intake during the cardiac intensive care unit stay is substantially below what is recommended.

Higher protein intake strategies in human milk fortification for preterms infants feeding. Auxological and neurodevelopmental outcome.

Postnatal growth restriction and failure to thrive still remain a major problem in Extremely Low Birth Weight (ELBW) infants . The goal for the nutritional care of these infants is to achieve rate of growth similar to those of the fetus in utero at the equivalent gestational age. Human milk fortified remains the best food for all these preterms. Two groups of preterm of weight 580-1250 g and gestational age 23-32 wk, were fed with different protein intake in the human/maternal milk fortified ( 3,5 g Kg-1 per day and 4,8 g Kg-1 per day in the control and intervention group respectively).The feeding tolerance, intrahospital growth, neurological outcome and anthropometric data until 12 months of corrected age, were evaluated. The protein supplemented group (PSG) showed an intrahospital highter growth rate ( mostly in head circumference, p 0,02, and length growth, p 0,04) only in the preterms with 580-980 g and 23-30 wk. In the same preterms, Griffith Development Mental Score at 3 and 12 months corrected age showed higher score than in the control group in the Performance (p 0,04) and Hearing/Language (p 0,03) items. The auxological evaluation in the postdischarge period showed in the PSG group mean z-score values for length higher than those in the control group at 9 (p 0,04) months of corrected age.

Nutritional and metabolic findings in patients with Prader-Willi syndrome diagnosed in early infancy.

OBJECTIVE: Early treatment (growth hormone and nutritional support) improves development in infants with Prader-Willi syndrome. This study aimed to evaluate the nutritional and metabolic condition of nine patients who were diagnosed and treated in early infancy. METHODS: Nine patients were hospitalized at the age of \xe2\u20ac\xa810 days to 11 months because of severe feeding difficulties, failure to thrive, or developmental delay. The diagnosis of Prader-Willi syndrome was confirmed by fluorescence in situ hybridization or other molecular genetic techniques. Nutritional and metabolic investigations including urinary organic acid analysis, blood amino acid, and acylcarnitine profiles were performed. RESULTS: The diagnosis was made at the mean age of 6.3 months. A deletion of the paternal gene in the 15q11-13 region was detected in all patients. Eight patients had ketosis, seven had malnutrition, five had hyperammonemia, three had liver dysfunction, three had low blood cholesterol level, and two had hypoglycemia. All patients had reduction of serum multiple amino acids and free carnitine. Significant arginine deficiency was found in all patients. Six patients had mildly elevated blood long-chain and very long-chain acylcarnitine. After supplementation with l-arginine, medium-chain fatty acids, l-carnitine, and vitamins, all patients responded with improvement of motor development and nutritional conditions. Four patients were almost caught up on physical and psychomotor development. CONCLUSIONS: Patients with Prader-Willi syndrome are in bad metabolic condition in the early period. Early diagnosis and individual nutritional interventions may improve the nutritional and developmental progress and decrease death rate in infancy.

Specialised milks: a guide to the right choices.

Specialist milks are prescribed if a baby is preterm, has faltering growth, a suspected food allergy, or other medical condition that precludes them from having one of the usual formula milks. Because there are many different types of specialist milks available--and their suitability for different conditions and age groups varies--it is vital to refer a child for specialist help.

Laparoendoscopic gastrostomy tube placement: our all-in-one technique.

BACKGROUND AND AIMS: Gastrostomy tube placement may be performed in numerous ways: percutaneous endoscopic gastrostomy (PEG), laparoscopic PEG, and laparoscopic gastrostomy tube placement. In this paper, we propose a new technique of laparoendoscopic gastrostomy tube (LEGT) placement, using a three-suture technique to pexy the stomach to the abdominal wall. This paper outlines the LEGT technique and its benefits over previously reported techniques. PATIENTS AND METHODS: Five children were included in this study during 2007. Their ages ranged from 15 months to 14 years. Under general anesthesia, a gastroscope was placed in the stomach and laparoscopic visualization was obtained through a 5-mm umbilical port. Two small incisions were made transabdominally for working instruments. Using laparoscopic visualization, three sutures were placed around a proposed gastrostomy site in the stomach. These sutures were pulled externally, using a GraNee needle (R-Med, Inc., Oregon, OH). Under visualization by a gastroscope, the gastrostomy button was placed in the center of these three sutures at the proposed gastrostomy tube site. Once the gastrostomy balloon was inflated, the three sutures were pulled taught and tied subcutaneously to pexy the stomach to the abdominal wall. Inspection with the gastroscope and laparoscope ensured proper gastrostomy tube placement. RESULTS: All patients had no complications intra- or postoperatively and at 2 months of follow-up. An optimal cosmetic result was obtained, and there was no need for suture removal or a second procedure. CONCLUSIONS: The laparoscopic visualization of the LEGT avoids accidental gastrocolic fistula formation and allows primary placement of the gastrostomy button without need for subsequent procedures. The endoscopic visualization, if the LEGT ensures the gastrostomy tube, is placed within the gastric lumen. Additionally, the three-suture technique gives an optimal fixation of the stomach to the abdominal wall, gives outstanding cosmetic results, avoids accidental disruption of sutures, and has no need for suture removal at a postoperative visit. LEGT thus serves as a one-stop shopping approach for gastrostomy in children.

Acompanhamento nutricional de criança portadora de anemia falciforme na Rede de Atenção Básica à Saúde / Nutritional follow-up of children with sickle cell anemia treated in a Primary Care Unit

OBJETIVO: Relatar estudo de caso de atendimento nutricional a criança com diagnóstico de anemia falciforme. DESCRIÇÃO DO CASO: Realizaram-se quatro atendimentos nutricionais pela equipe do Internato de Nutrição da Universidade do Estado do Rio de Janeiro (Uerj) no período de julho a setembro de 2005 a criança de 1 a 4 meses, feminina, negra, com anemia falciforme. Na avaliação do estado nutricional e do ganho de peso, empregaram-se os seguintes indicadores antropométricos: comprimento/idade, peso/idade e peso/comprimento, e um indicador de impacto nutricional. A análise dietética compreendeu a avaliação da ingestão energética e de macronutrientes observada na primeira consulta após 30 dias de intervenção. COMENTÁRIOS: Ao longo do período analisado, a baixa estatura para a idade (z=−1,32) evoluiu para adequação (z=0,87), enquanto o peso em relação ao comprimento manteve-se inadequado (z=−2,53). O ganho de peso foi 50% inferior ao incremento esperado. O consumo energético inicialmente inadequado (60% das recomendações) alcançou, após 30 dias, 117%. A Estratégia em Saúde da Família vem sendo recomendada como importante ferramenta para monitorar as condições nutricionais, bem como para melhorar a atenção prestada. Entretanto, considerando o modelo de atenção primária local, observa-se haver necessidade de capacitação, especialmente no que tange às peculiaridades inerentes à condição de nutrição e de saúde dos portadores de anemia falciforme.

OBJECTIVE: To report the nutritional follow-up of a black baby girl, one year and four months old, with homozygous sickle cell anemia. CASE DESCRIPTION: From July-September 2005, the infant attended four nutritional appointments at the Nutrition Internship Program from the State University of Rio de Janeiro, Rio de Janeiro, Brazil. The nutritional status was evaluated by the anthropometric indexes: length/age, weight/age and weight/length, and by one indicator of nutritional impact. Analysis of the energy dietary intakes and macronutrients ingestion was performed in two opportunities: at the first nutritional evaluation and 30 days after the first intervention. COMMENTS: At the first nutritional evaluation, the child presented low stature/age (z=−1,32), that became adequate during the follow-up (z=0,87). The low weight/length (z=−2,53) was similar at the first and final evaluations. The weight gain during the follow-up period was only 50% of the expected increment. The energy dietary intake was 66% of the recommended energy intake at the first appointment and 117% after 30 days. The Family Health Strategy is recommended as an important tool to monitor nutritional status and improve nutritional care. There is a need of developing professional skills regarding nutritional care of patients with sickle cell anemia.

Nutritional problems related to food allergy in childhood.

Food allergy is becoming an increasing problem worldwide with an estimated 6-8% of children affected at some point in their childhood. The perceived prevalence of food allergy is even higher with an estimated 20% of children adhering to some form of elimination diet. Against this background, accurate diagnosis is essential to prevent the imposition of unnecessarily restrictive diets on young children. Raising clinical awareness amongst health professionals as to the clinical characteristics, epidemiology, investigation, and management of food allergic disorders is key to tackling this growing problem. In this article, three separate cases of children with poor nutrition and secondary morbidity are presented, highlighting the varying scenarios in which these conditions can be encountered. In the first child, the features clinically displayed were hypocalcemic seizures and rickets due to prolonged breast feeding, poor weaning, and inadequate dietary supplementation. The second case reveals the dangers of complementary diagnostic allergy testing leading to poor nutrition as a consequence of an unsupervised elimination diet. The last report describes a child with multiple food allergies, failure to thrive, and protein losing enteropathy to highlight the diversity of nutritional problems faced by allergists and to underline the importance of specialist dietetic input in the management of a child with food allergy.

Randomized comparison of a nutrient-dense formula with an energy-supplemented formula for infants with faltering growth.

INTRODUCTION: The practice of supplementing standard infant formula with energy for infants with faltering growth has been widespread. This increases energy density but disturbs the protein : energy ratio, and increases risks of microbial contamination and errors in feed preparation. This study aimed to compare the effectiveness of a nutrient-dense formula (NDF) with an energy-supplemented formula (ESF) in infants with faltering growth. METHODS: In an open, parallel, randomized study, 49 infants with faltering growth were randomized to receive a NDF (4.2 kJ mL(-1)) or an ESF (4.2 kJ mL(-1)), for 6 weeks. Anthropometry, biochemistry, feed intake, stool and vomit frequency were collected. RESULTS: No significant differences in tolerance, feed volumes or energy intakes were recorded but the NDF group received 42% more protein and 15-40% more vitamins and minerals. Blood urea concentration in the ESF group fell by 50% over the trial period, suggesting a suboptimal protein : energy ratio in the ESF feed. The NDF group retained a normal mean blood urea concentration, a higher urinary potassium concentration and did not have the significant fall in length z-score seen in the ESF group. CONCLUSION: Increasing the energy content of normal infant formula without also increasing protein and micronutrients should not be practiced in infants with faltering growth.

The efficacy of amino acid-based formulas in relieving the symptoms of cow's milk allergy: a systematic review.

The aim of this systematic review was to evaluate the efficacy of amino acid-based formulas (AAF) in patients with cow's milk allergy (CMA). Studies were identified using electronic databases and bibliography searches. Subjects eligible for inclusion were patients of any age with CMA or symptoms suggestive of it. Comparisons of interest were AAF vs. extensively hydrolysed formula (eHF), AAF vs. soy-based formula (SF) and AAF vs. cow's milk or cow's milk-based formula. Outcomes of interest were gastrointestinal (GI), dermatological, respiratory and behavioural symptoms as well as growth. A total of 20 studies [three head-to-head randomized controlled trials (RCTs), three cross-over challenge RCTs, seven clinical trials (CTs) and seven case reports (CRs)] were included in the review. In infants with confirmed or suspected CMA, the use of an AAF was shown to be safe and efficacious. Findings from RCT comparisons of AAF with eHF showed that both formulas are equally efficacious at relieving the symptoms of CMA in confirmed or suspected cases. However, infants in specific subgroups (e.g. non-IgE mediated food-induced gastro-enterocolitis-proctitis syndromes with failure to thrive, severe atopic eczema, or with symptoms during exclusive breastfeeding) were more likely overall to benefit from AAF, as intolerance to eHF may occur. In such cases, symptoms persisting despite eHF feeding usually remit on AAF, and catch-up growth may be seen. Meta-analysis of the findings was not possible due to lack of homogenous reporting of outcomes in the original trials. This systematic review shows clinical benefit from use of AAF in both symptoms and growth in infants and children with CMA who fail to tolerate eHF. Further studies are required to determine the relative medical or economic value of initial treatment with AAF in infants at high risk of eHF intolerance.

Should high-energy infant formula be given at full strength from its first day of usage?

OBJECTIVE: To evaluate the feeding tolerance of a high-energy (1 kcal mL(-1)) infant formula (HEIF) for infants with faltering growth, weighing 2.5-8 kg, when administered at full strength from day 1 compared with stepwise introduction. METHODS: Thirty infants (aged 2-43 weeks) from a supraregional children's hospital requiring a high-energy formula for faltering growth were randomly allocated to receive either a 1 kcal mL(-1) ready to feed infant formula at full strength from day 1 or the same formula in diluted form, graded to full strength over 3 days. Bowel actions, vomits and volume of feed taken were recorded daily for 2 weeks. Weight, length, head circumference and mid-upper arm circumference were recorded at recruitment and after 2 weeks. RESULTS: The number of bowel actions in the first 2 days was significantly higher for the full strength group than for the stepwise group (P = 0.02); younger infants (<12 weeks) had more bowel actions over the first 2 days on the HEIF at full strength (Spearman's correlation = -0.5; P = 0.04); and infants with a total higher energy intake (kJ kg(-1)) had more frequent bowel actions over the first 4 days (Spearman's correlation = 0.48; P = 0.01). There was no significant difference between groups for growth or vomiting. CONCLUSIONS: High-energy infant formula was generally well tolerated in infants under the age of 12 months with faltering growth when administered at full strength from day 1. However, younger infants (<12 weeks of age) may benefit from a graded introduction in order to avoid increased bowel frequency.