Academy of Nutrition and Dietetics Nutrition Research Network: A Home Garden Intervention Improves Child Length-for-Age Z-Score and Household-Level Crop Count and Nutritional Functional Diversity in Rural Guatemala.

Home gardens may help address childhood malnutrition in low- and middle-income countries. In this quasi-experimental pilot study, the Academy of Nutrition and Dietetics, in collaboration with Maya Health Alliance, evaluated the feasibility of augmenting a standard-of-care nutrition-specific package for Maya children with length-for-age z score ≤-2 (stunting) in rural Guatemala with a nutrition-sensitive home garden intervention. Two agrarian municipalities in Guatemala were included. Families of 70 children with stunting from 1 municipality received the standard-of-care package (food supplementation, multiple micronutrient powders, monthly nutrition home visits, group nutrition classes). Families of 70 children with stunting from another municipality received the standard-of-care package plus a home garden intervention (garden materials, monthly agricultural home visits, agriculture classes). Maternal and child dietary diversity, household food insecurity, child growth, and agricultural indicators were collected at baseline and 6 months later and were analyzed using mixed linear and logistic regression models. Compared with the standard-of-care group, the garden intervention group had improved child (odds ratio [OR] 3.66, 95% CI 0.89-15.10, P = 0.07) and maternal dietary diversity (OR 2.31, 95% CI 0.80-6.65, P = 0.12) and decreased food insecurity (OR 0.38, 95% CI 0.11-1.35, P = 0.14); however, these effects were not statistically significant. Participation in gardens predicted a higher length-for-age z-score (change difference [CD] 0.22 SD, 95% CI 0.05-0.38, P = 0.009), greater crop species count (CD 2.97 crops, 95% CI 1.79-4.16, P < 0.001), and greater nutritional functional diversity (CD 0.04 points, 95% CI 0.01-0.07, P = 0.006) than standard-of-care alone. Home garden interventions are feasible in rural Guatemala and may have potential benefits for child growth when added to other nutrition-specific interventions.

Potential Benefits of Bovine Colostrum in Pediatric Nutrition and Health.

Bovine colostrum (BC), the first milk produced from cows after parturition, is increasingly used as a nutritional supplement to promote gut function and health in other species, including humans. The high levels of whey and casein proteins, immunoglobulins (Igs), and other milk bioactives in BC are adapted to meet the needs of newborn calves. However, BC supplementation may improve health outcomes across other species, especially when immune and gut functions are immature in early life. We provide a review of BC composition and its effects in infants and children in health and selected diseases (diarrhea, infection, growth-failure, preterm birth, necrotizing enterocolitis (NEC), short-bowel syndrome, and mucositis). Human trials and animal studies (mainly in piglets) are reviewed to assess the scientific evidence of whether BC is a safe and effective antimicrobial and immunomodulatory nutritional supplement that reduces clinical complications related to preterm birth, infections, and gut disorders. Studies in infants and animals suggest that BC should be supplemented at an optimal age, time, and level to be both safe and effective. Exclusive BC feeding is not recommended for infants because of nutritional imbalances relative to human milk. On the other hand, adverse effects, including allergies and intolerance, appear unlikely when BC is provided as a supplement within normal nutrition guidelines for infants and children. Larger clinical trials in infant populations are needed to provide more evidence of health benefits when patients are supplemented with BC in addition to human milk or formula. Igs and other bioactive factors in BC may work in synergy, making it critical to preserve bioactivity with gentle processing and pasteurization methods. BC has the potential to become a safe and effective nutritional supplement for several pediatric subpopulations.

High Early Parenteral Lipid in Very Preterm Infants: A Randomized-Controlled Trial.

OBJECTIVE: To determine whether high early parenteral soybean oil lipid intake in very low birth weight (VLBW) infants in the first week after birth decreases the proportion of weight loss and subsequently the incidence of extrauterine growth restriction (EUGR). STUDY DESIGN: This was a randomized controlled trial of appropriate for gestational- ge VLBW infants. Lipid intake in the control group started at 0.5-1 g/kg per day and increased daily by 0.5-1 g/kg per day till reaching 3 g/kg per day. The intervention group was started on 2 g/kg per day that increased to 3 g/kg per day the following day. RESULTS: Of the 176 infants assessed for eligibility, 83 were included in the trial. Infants in the intervention group were started on lipid sooner (13.8 ± 7.8 vs 17.5 ± 7.8 hour; P = .03) and had higher cumulative lipid intake in the first 7 days of age (13.5 ± 4.2 vs 10.9 ± 3.5 g/kg per day; P = .03). Infants in the intervention group had a lower percentage of weight loss (10.4 vs 12.7%; P = .02). The mean triglyceride level was higher in the intervention group (1.91 ± 0.79 vs 1.49 ± 0.54 mmol/L; P = .01), however, hypertriglyceridemia was similar between the 2 groups. The incidence of EUGR was lower in the intervention group (38.6% vs 67.6%; P = .01). Head circumference z score was higher in the intervention group (-1.09 ± 0.96 vs -1.59 ± 0.98; P = .04). CONCLUSIONS: In VLBW infants, provision of a high early dose of parenteral lipid in the first week of age results in less weight loss and lower incidence of EUGR. TRIAL REGISTRATION: Clinicaltrials.gov: NCT03594474.

Comparative effectiveness of East Asian traditional medicine for treatment of idiopathic short stature in children: A protocol for systematic review and network meta-analysis.

BACKGROUND: There are many East Asian traditional medicine (EATM) therapies that are widely used and effective for idiopathic short stature (ISS) in children. However, the comparative effectiveness of these therapies remains unclear. We describe the methods that will be used to comparatively evaluate the efficacy and safety of EATM therapies for the treatment of pediatric ISS. METHODS AND ANALYSIS: Fourteen electronic English, Korean, Chinese, and Japanese databases will be searched up to August 2020 for relevant randomized controlled trials of various EATMs for the treatment of pediatric ISS, without language or publication status restrictions. The primary outcome will be growth-related anthropometric indicators, and acceptability, measured through drop-outs that occur during treatment for any reason. We will conduct a pairwise meta-analysis for direct comparisons if multiple studies use the same types of intervention, comparison, and outcome measure. A frequentist network meta-analysis will be performed to summarize the available direct and indirect evidence regarding various EATM options for pediatric ISS. The risk of bias for the included studies will be evaluated using the Cochrane Collaboration's risk of bias tool. CONCLUSIONS: The findings of this review will provide evidence for the comparative effectiveness and ranks of current EATMs and help to inform clinical practitioners, patients, and policy makers in decision making. ETHICS AND DISSEMINATION: Ethical approval is not required because individual patient data are not included. The findings of this systematic review will be disseminated through a peer-reviewed publication or conference presentations. PROTOCOL REGISTRATION NUMBER: OSF (URL: https://osf.io/s4vp7), PROSPERO CRD42020187160.

Improvement of a massage chair (BEG-100) on height growth in children with average: Human subjects research.

BACKGROUND: Studies that examine the growth effects of massage at ages beyond infancy are rare. A recently developed massage chair (BEG-100) targets teenagers who want to increase their growth. The chair can stretch areas around the knee after fixating the knee and ankle areas. METHODS: This is a clinical study that included 38 children aged 11 years whose heights ranged from 145.0 to 155.0 cm. We aim to observe the potential of a 24-week massage for growth promotion. We will also evaluate the changes in height percentile, height, height standard deviation score, bone age, height standard deviation score for bone age, growth rate, predicted height estimated from bone age, seated height-to-standing height ratio, weight, and body mass index. To confirm safety, the associated adverse events will be investigated. OBJECTIVE: The aim of this study was to investigate the growth-promoting effect of BEG-100 in children with average height. TRIAL REGISTRATION: KCT0004673 (Clinical Research Information Service).

New approach to simplifying and optimising acute malnutrition treatment in children aged 6-59 months: the OptiMA single-arm proof-of-concept trial in Burkina Faso.

The Optimising treatment for acute MAlnutrition (OptiMA) strategy trains mothers to use mid upper arm circumference (MUAC) bracelets for screening and targets treatment to children with MUAC < 125 mm or oedema with one therapeutic food at a gradually reduced dose. This study seeks to determine whether OptiMA conforms to SPHERE standards (recovery rate > 75 %). A single-arm proof-of-concept trial was conducted in 2017 in Yako district, Burkina Faso including children aged 6-59 months in outpatient health centres with MUAC < 125 mm or oedema. Outcomes were stratified by MUAC category at admission. Multivariate survival analysis was carried out to identify variables predictive of recovery. Among 4958 children included, 824 (16·6 %) were admitted with MUAC < 115 mm or oedema, 1070 (21·6 %) with MUAC 115-119 mm and 3064 (61·8 %) with MUAC 120-124 mm. The new dosage was correctly implemented at all visits for 75·9 % of children. Global recovery was 86·3 (95 % CI 85·4, 87·2) % and 70·5 (95 % CI 67·5, 73·5) % for children admitted with MUAC < 115 mm or oedema. Average therapeutic food consumption was 60·8 sachets per child treated. Recovery was positively associated with mothers trained to use MUAC prior to child's admission (adjusted hazard ratio 1·09; 95 % CI 1·01, 1·19). OptiMA was successfully implemented at the scale of an entire district under 'real-life' conditions. Programme outcomes exceeded SPHERE standards, but further study is needed to determine if increasing therapeutic food dosages for the most severely malnourished will improve recovery.

Managing Paediatric Growth Disorders: Integrating Technology Into a Personalised Approach

Long-term growth management can be challenging for patients, families and healthcare professionals (HCP). Personalised optimal responses to growth hormone (GH) therapy depend on the creation of a good working relationship between the patient and family and the HCPs responsible for care. Current unmet needs in growth management will be discussed, focusing on the likelihood of a poor growth response and its identification and management with emphasis on the importance of good adherence to GH therapy. Digital tools are now available to record injections and communicate accurate adherence data to the HCP and patient. Psychological barriers to good adherence will be covered, with techniques identified to change behaviour and improve outcome. Motivational interviewing is a valuable skill in this respect and should be taught to both medical and nursing HCPs to enhance the quality of the relationship with the patient and family. Key messages are, firstly, the importance of personalised care with the HCP using acquired psychological skills to prevent and manage poor adherence. Secondly, a human-eHealth partnership is necessary to maximise the benefit of new digital tools to aid in successful growth management.

Serum Adipokines, Growth Factors, and Cytokines Are Independently Associated with Stunting in Bangladeshi Children.

Growth in young children is controlled through the release of several hormonal signals, which are affected by diet, infection, and other exposures. Stunting is clearly a growth disorder, yet limited evidence exists documenting the association of different growth biomarkers with child stunting. This study explored the association between different growth biomarkers and stunting in Bangladeshi children. A quasi-experimental study was conducted among 50 stunted (length-for-age Z-score (LAZ) < -2 SD) and 50 control (LAZ ≥ -2 SD) children, aged 12-18 months, residing in a Bangladeshi slum. The enrolled stunted children received an intervention package, which included food supplementation for three months, psychosocial stimulation for six months, and routine clinical care on community nutrition center at the study field site. The controls received routine clinical care only. All children were clinically screened over the study period. Length, weight, fasting blood and fecal biomarkers were measured. All biomarkers levels were similar in both groups except for oxyntomodulin at enrolment. Leptin (adjusted odds ratio, AOR: 4.0, p < 0.01), leptin-adiponectin ratio (AOR 5.07 × 108, p < 0.01), insulin-like growth factor-1 (IGF-1) (AOR 1.02, p < 0.05), and gamma interferon (IFN-γ) (AOR 0.92, p < 0.05) levels were independently associated with stunting at enrolment. Serum leptin, leptin-adiponectin ratio, interleukin-6 (IL-6), IL-10, tumor necrosis factor-alpha (TNF-α), and fecal alpha-1-antitrypsin (AAT) levels increased significantly (p < 0.001), while IFN-γ levels significantly decreased among stunted children after six months of intervention. Leptin, leptin-adiponectin ratio, IGF-1, and IFN-γ are independently associated with stunting in Bangladeshi children. This trial was registered at clinicaltrials.gov as NCT02839148.

Extrauterine Growth Restriction in Low Birth Weight Infants.

Extrauterine growth restriction (EUGR) affects a significant number of very low birth weight (VLBW) infants and has the potential to impact neurodevelopmental outcome as well as other aspects of long-term health. More aggressive nutritional approaches have reduced the incidence of postnatal growth failure but many questions remain about the expected rate of growth for very preterm infants, the best ways to measure growth velocity, and the optimal approaches to supporting growth. This article examines some of the outstanding issues regarding postnatal growth failure and summarizes current practice recommendations.

Growth failure in Crohn's disease children: may the first treatment have a role?

Introduction: Growth failure in children is a frequent feature of childhood-onset Crohn's disease (CD), and stunting can persist into adulthood. Growth is an important outcome by which to judge the effectiveness of therapies in children; currently available studies in CD children have focused on the short-term impact of treatments on growth, and there are limited data regarding the long-term effects of treatments upon growth. Areas covered: We designed the present article to review whether the first treatment performed in newly diagnosed CD children may have a role on the future growth course. We conducted a systematic literature search to identify relevant studies published on the PubMed database from January 2002 up to now. We found only six surveys that documented mid-term growth course in newly diagnosed CD patients. Expert commentary: In the last years there have been relevant advances in the clinical management of CD children; however, there is a lack of knowledge about the best strategy to reverse growth failure. Children treated with enteral nutrition have appropriate height and weight gain but do not reverse the growth course. Further surveys are required to better explore not only clinical outcomes but also long-term growth course following each therapeutic strategy.

Treatment and rehabilitation outcomes of children affected with nodding syndrome in Northern Uganda: a descriptive case series.

INTRODUCTION: Nodding Syndrome (NS) is a neurological disorder affecting children 5-15 years at onset in East Africa. A major criterion for diagnosis is atonic seizure with dorso-ventral "nodding" of the head. Comorbidities include psychological and behavioral abnormalities, malnutrition, cognitive decline, school dropout and other seizure types. We aimed to describe the presentations and rehabilitation outcomes of NS children at Hope for HumaNs (HfH) centre in Gulu from September 2012 to October 2013. METHODS: Data was obtained from a retrospective review of 32 NS children's medical records at HfH center. Ethical approval was obtained from Gulu University IRB. Data analysis was conducted using WHO AnthroPlus, SPSS and Excel software. RESULTS: Growth statistics showed steady improvement over time using local nutrition and multivitamin supplementation. Severe and moderate stunting was reduced from a combined total of 54.8% to 7.7% and 12.8% respectively. Severe and moderate wasting was reduced from 29.1% to 2.6% and 5.1% respectively. Three groups of NS children were identified and compared in the review; Low seizure occurrence averaging <2 seizures/month (28.1%); Moderate averaging 2-4 seizures/month (34.4%) and High averaging >4 seizures/month (37.5%). CONCLUSION: NS is a neurological disorder of unknown etiology. Treatment with regular high quality local nutrition, multivitamin supplementation, anti-seizures, regular follow up and illness prevention; children's seizures can be reduced or stopped completely. The debilitating malnutrition and stunting of NS children in Uganda could be partially independent of the syndrome but attributable to poor nutrition. NS as observed is not "invariably fatal" but rather a treatable neurological disorder.

Desempenho cognitivo de pré-escolares com baixa estatura em tratamento de recuperação nutricional / Cognitive performance of stunted pre-school children undergoing nutritional recovery treatment

RESUMO Objetivo: Determinar se o tratamento de crianças com baixa estatura, ofertado em um centro especializado, influencia seu desempenho cognitivo. Métodos: Foram analisados dois grupos de crianças advindas de famílias vulneráveis, um com crianças com baixa estatura em tratamento no Centro de Recuperação e Educação Nutricional (CREN) e outro de crianças eutróficas de uma creche municipal localizada na mesma região do CREN. No CREN, as crianças são tratadas em semi-internato (9 horas/dia, 5 dias/semana), recebendo suporte médico, nutricional e psicopedagógico. Todas foram submetidas ao Teste de Triagem do Desenvolvimento de Denver-II e avaliadas quanto ao índice de estatura-para-idade em 3 momentos distintos: no início do acompanhamento e após 6 e 12 meses. A classificação socioeconômica das crianças, de acordo com os Critérios de Classificação Econômica Brasil, foi feita no início do acompanhamento. Calcularam-se razões de prevalência para a análise transversal da linha de base, por meio de uma regressão de Poisson, e razões de prevalência agrupadas para a análise longitudinal, por meio de um modelo de estimativas de equações generalizadas, ambas ajustadas por idade, sexo e classe socioeconômica. Resultados: Ao todo, 74 crianças foram analisadas, 37 em cada grupo. Não houve diferenças de idade, sexo e classe socioeconômica entre os grupos. Na análise longitudinal, o grupo CREN apresentou melhor desempenho no domínio pessoal-social (razão de prevalência agrupada: 0,89; intervalo de confiança de 95% - IC95%: 0,82-0,95), sem diferenças significativas para os demais domínios. Conclusão: O tratamento ofertado pelo CREN melhorou satisfatoriamente as habilidades sociais das crianças tratadas, sem alterar os demais domínios.

ABSTRACT Objective: To determine if the treatment of stunted children offered at a specialized center influences their cognitive performance. Methods: Two groups of children from vulnerable families were selected, one consisting of stunted children being treated at the Nutrition Education and Recovery Center (CREN), and the other group of eutrophic children from a local, public day care center. At CREN, children are treated in a day-hospital system (9 hours/day, 5 days/week), receiving medical, nutritional and psycho-pedagogical support. All children were submitted to the Denver-II Development Screening Test and had their development and the height-for-age index assessed at 3 moments: at the beginning of the follow-up, and after 6 and 12 months. The socioeconomic status, according to the Brazilian Economic Classification Criteria, was assessed at the beginning of the follow-up. Data were treated by prevalence ratios for cross-sectional baseline analysis, using the Poisson regression, and by pooled prevalence ratios for longitudinal analysis, using a generalized equation estimation model, both adjusted by age, sex and economic status. Results: Seventy-four children were included, 37 for each group. There were no differences in age, sex and socioeconomic status between groups. In the longitudinal analysis, the CREN group showed better performance in the personal-social domain (pooled prevalence ratio: 0.89; 95% confidence interval - 95%IC 0.82-0.95), with no differences in the other domains. Conclusions: The treatment offered at CREN satisfactorily improved the social skills of the treated children, without changing other domains.

Unique presentation of transient zinc deficiency from low maternal breast milk zinc levels.

We report full-term siblings with a unique clinical presentation of polycyclic papulosquamous plaques secondary to transient zinc deficiency due to low maternal breast milk zinc levels. We present this case to highlight this unique presentation of zinc deficiency in breastfed infants.

COGNITIVE PERFORMANCE OF STUNTED PRE-SCHOOL CHILDREN UNDERGOING NUTRITIONAL RECOVERY TREATMENT. / DESEMPENHO COGNITIVO DE PRÉ-ESCOLARES COM BAIXA ESTATURA EM TRATAMENTO DE RECUPERAÇÃO NUTRICIONAL.

OBJECTIVE: To determine if the treatment of stunted children offered at a specialized center influences their cognitive performance. METHODS: Two groups of children from vulnerable families were selected, one consisting of stunted children being treated at the Nutrition Education and Recovery Center (CREN), and the other group of eutrophic children from a local, public day care center. At CREN, children are treated in a day-hospital system (9 hours/day, 5 days/week), receiving medical, nutritional and psycho-pedagogical support. All children were submitted to the Denver-II Development Screening Test and had their development and the height-for-age index assessed at 3 moments: at the beginning of the follow-up, and after 6 and 12 months. The socioeconomic status, according to the Brazilian Economic Classification Criteria, was assessed at the beginning of the follow-up. Data were treated by prevalence ratios for cross-sectional baseline analysis, using the Poisson regression, and by pooled prevalence ratios for longitudinal analysis, using a generalized equation estimation model, both adjusted by age, sex and economic status. RESULTS: Seventy-four children were included, 37 for each group. There were no differences in age, sex and socioeconomic status between groups. In the longitudinal analysis, the CREN group showed better performance in the personal-social domain (pooled prevalence ratio: 0.89; 95% confidence interval - 95%IC 0.82-0.95), with no differences in the other domains. CONCLUSIONS: The treatment offered at CREN satisfactorily improved the social skills of the treated children, without changing other domains.

OBJETIVO: Determinar se o tratamento de crianças com baixa estatura, ofertado em um centro especializado, influencia seu desempenho cognitivo. MÉTODOS: Foram analisados dois grupos de crianças advindas de famílias vulneráveis, um com crianças com baixa estatura em tratamento no Centro de Recuperação e Educação Nutricional (CREN) e outro de crianças eutróficas de uma creche municipal localizada na mesma região do CREN. No CREN, as crianças são tratadas em semi-internato (9 horas/dia, 5 dias/semana), recebendo suporte médico, nutricional e psicopedagógico. Todas foram submetidas ao Teste de Triagem do Desenvolvimento de Denver-II e avaliadas quanto ao índice de estatura-para-idade em 3 momentos distintos: no início do acompanhamento e após 6 e 12 meses. A classificação socioeconômica das crianças, de acordo com os Critérios de Classificação Econômica Brasil, foi feita no início do acompanhamento. Calcularam-se razões de prevalência para a análise transversal da linha de base, por meio de uma regressão de Poisson, e razões de prevalência agrupadas para a análise longitudinal, por meio de um modelo de estimativas de equações generalizadas, ambas ajustadas por idade, sexo e classe socioeconômica. RESULTADOS: Ao todo, 74 crianças foram analisadas, 37 em cada grupo. Não houve diferenças de idade, sexo e classe socioeconômica entre os grupos. Na análise longitudinal, o grupo CREN apresentou melhor desempenho no domínio pessoal-social (razão de prevalência agrupada: 0,89; intervalo de confiança de 95% - IC95%: 0,82-0,95), sem diferenças significativas para os demais domínios. CONCLUSÃO: O tratamento ofertado pelo CREN melhorou satisfatoriamente as habilidades sociais das crianças tratadas, sem alterar os demais domínios.

Growth assessment and risk factors of malnutrition in children with cystic fibrosis.

OBJECTIVES: To evaluate the nutritional status, to screen for the presence of malnutrition, and to study the possible risk factors associated with malnutrition in patients with cystic fibrosis (CF). METHODS: A retrospective cross-sectional review of medical records of all diagnosed CF patients in the Pediatric Department, Salmaniya Medical Complex, Manama, Kingdom of Bahrain, between January 1984 and May 2015 was conducted. Demographic and anthropometric data were collected from records of last visit to CF clinic. Nutritional status and risk factors of malnutrition were assessed. RESULTS: All records of 109 CF patients were reviewed. Forty-seven pediatric patients were included in the study. All included patients were on pancreatic enzyme replacement and 42 (89%) received high-calorie supplementation. Growth failure was noted in 34 (72%) patients, 19 (56%) were wasted and stunted, 8 (23.5%) were wasted only, and 7 (20.5%) were stunted. Low birth weight (p=0.032), and the presence of gastroesophageal reflux disease (GERD) (p=0.039) were the significant risk factors for malnutrition. CONCLUSIONS: Most CF patients in Bahrain (72%) are malnourished. Low birth weight and the presence of GERD are risk factors.

Endurance exercise and growth hormone improve bone formation in young and growth-retarded chronic kidney disease rats.

BACKGROUND: Childhood chronic kidney disease (CKD) is associated with both short stature and abnormal bone mineralization. Normal longitudinal growth depends on proper maturation of epiphyseal growth plate (EGP) chondrocytes, leading to the formation of trabecular bone in the primary ossification centre. We have recently shown that linear growth impairment in CKD is associated with impaired EGP growth hormone (GH) receptor signalling and that exercise improved insulin-like growth factor I (IGF-I) signalling in CKD-related muscle atrophy. METHODS: In this study, 20-day-old rats underwent 5/6 nephrectomy (CKD) or sham surgery (C) and were exercised with treadmill, with or without GH supplementation. RESULTS: CKD-related growth retardation was associated with a widened EGP hypertrophic zone. This was not fully corrected by exercise (except for tibial length). Exercise in CKD improved the expression of EGP key factors of endochondral ossification such as IGF-I, vascular endothelial growth factor (VEGF), receptor activator of nuclear factor kappa-B ligand (RANKL) and osteocalcin. Combining GH treatment with treadmill exercise for 2 weeks improved the decreased trabecular bone volume in CKD, as well as the expression of growth plate runt-related transcription factor 2, RANKL, metalloproteinase 13 and VEGF, while GH treatment alone could not do that. CONCLUSIONS: Treadmill exercise improves tibial bone linear growth, as well as growth plate local IGF-I. When combined with GH treatment, running exercise shows beneficial effects on trabecular bone formation, suggesting the potential benefit of this combination for CKD-related short stature and bone disease.

Endocrine disorders in Fanconi anemia: recommendations for screening and treatment.

CONTEXT: Endocrine problems are common in patients with Fanconi anemia (FA). About 80% of children and adults with FA have at least one endocrine abnormality, including short stature, GH deficiency, abnormal glucose or insulin metabolism, dyslipidemia, hypothyroidism, pubertal delay, hypogonadism, or impaired fertility. The goal of this report is to provide an overview of endocrine abnormalities and guidelines for routine screening and treatment to allow early diagnosis and timely intervention. EVIDENCE ACQUISITION: This work is based on a comprehensive literature review, including relevant articles published between 1971 and 2014, and proceedings of a Consensus Conference held by the Fanconi Anemia Research Fund in 2013. EVIDENCE SYNTHESIS: The panel of experts collected published evidence and discussed its relevance to reflect current information about the endocrine care of children and adults with FA before the Consensus Conference and through subsequent deliberations that led to the consensus. CONCLUSIONS: Individuals with FA should be routinely screened for endocrine abnormalities, including evaluation of growth; glucose, insulin, and lipid metabolism; thyroid function; puberty; gonadal function; and bone mineral metabolism. Inclusion of an endocrinologist as part of the multidisciplinary patient care team is key to providing comprehensive care for patients with FA.

Growth in children with congenital diaphragmatic hernia during the first year of life.

PURPOSE: Infants with congenital diaphragmatic hernia (CDH) have high rates of mortality and long-term morbidity, including poor growth and failure to thrive. The aim of this study was to describe growth patterns during the first year of life in infants with congenital diaphragmatic hernia in a non-ECMO cohort. METHODS: Medical records of infants with CDH admitted to our center between January 2005 and December 2011 were reviewed. Infants with anthropometric measurements at 3, 6 and 12months were included. Anthropometric measurements were obtained for the first year of life. Logistic regression analyses were performed to find predictive associations with failure to thrive (FTT). RESULT: Of the 45 survivors, 38 were seen twice (84%) and 24 (53%) were seen on three occasions to age 12months. Poor growth was observed with weight being most affected. FTT was present in 63% during the first six months of life. Days of mechanical ventilation were the only predictor of FTT. Besides poor weight gain, height and head circumference were also reduced. However, catch-up growth occurred during the second half of infancy and at age 12months failure to thrive had reduced by two thirds to 21%. CONCLUSIONS: Poor growth is a common early finding in CDH patients, which improves during infancy. This emphasizes the importance of close follow-up and aggressive nutritional management in CDH patients.

Preterm nutrition and the lung.

Experimental and clinical evidence show that fetal and neonatal nutrition and metabolism can markedly modulate pulmonary growth, development, and function, as well as long-term lung health and disease risks. Intrauterine growth restriction has been linked to an increased risk for respiratory distress syndrome and chronic lung disease, while excessive fetal growth reduced forced expiratory volume. Postnatal undernutrition adversely affected pulmonary function in animal models and was associated to a higher risk of chronic lung disease in very low birth weight infants. The supply of specific nutrients to very low birth weight infants, including fluids, protein, carbohydrates, inositol, docosahexaenoic acid, calcium, phosphorus and the vitamins A and E has been associated with lung development and function and deserves further evaluation. In infants with evolving or established chronic lung disease, excess fluid administration and high intravenous glucose infusion rates should be avoided and the provision of vitamin A be considered. Opportunities exist for further research relating to neonatal nutrition and lung health, for example exploring optimal strategies and effects of providing vitamin A, docosahexaenoic acid and intravenous lipid emulsions.

Growth in children with chronic kidney disease: role of nutrition, growth hormone, dialysis, and steroids.

PURPOSE OF REVIEW: Children with chronic kidney disease (CKD) have impaired growth that leads to short stature in adulthood. The problem persists even with successful transplantation and steroid withdrawal protocols. The aim of this review is to provide an overview of the pressing issues related to growth failure in children with CKD both before and after transplantation. RECENT FINDINGS: Although great strides have been made in dialysis and transplantation, the incidence of abnormal adult height in children growing up with CKD remains as high as 45-60%. The lack of catch-up growth and resultant short stature is a critical issue for self-esteem and quality of life in many children with CKD. Aggressive daily dialysis, improved nutrition, treatment of metabolic bone disease, and the use of recombinant human growth hormone provide some hope for catch-up growth in select patients. SUMMARY: The causes of growth failure in the setting of CKD are multifactorial. Attention to all the details by optimizing nutritional, bone and mineral metabolism, correcting metabolic acidosis and anemia, achieving excellent blood pressure control, reversing cardiovascular complications such as left ventricular hypertrophy, and minimizing the use of corticosteroids is the current standard of care. Aggressive daily dialysis can reverse many of the uremic derangements. For patients not yet on dialysis or for those after renal transplant, early institution of recombinant human growth hormone can promote growth. Improved understanding of the mechanisms of hormone resistance may offer novel targets or measurements of treatment effectiveness.