Undernutrition in children & critical windows of opportunity in Indian context.

It is intriguing to note that majority of the wasting among the under 5 yr in India is present at birth. The National Family Health Survey 4 (NFHS-4) data analysis shows 31.9 per cent wasting at birth, which is decreasing to 17.7 per cent in the under five children; clearly suggesting that any reduction in wasting should come from improvement in foetal growth. In addition, children with both severe wasting and severe stunting, in whom the risk of mortality increases many folds, are <1 per cent in almost all the States; and these are the children in whom special care is required under the community-based management of severe acute malnutrition. This article presents an overview of nutrition status in children, their antecedents, and the critical phases; especially, nutrition status before pregnancy that plays a crucial role in all the nutrition status indicators of children. More attention on the critical phases is crucial to maximize the benefits from national programmes.

Environmental Enteric Dysfunction and Growth Failure/Stunting in Global Child Health.

Approximately 25% of the world's children aged <5 years have stunted growth, which is associated with increased mortality, cognitive dysfunction, and loss of productivity. Reducing by 40% the number of stunted children is a global target for 2030. The pathogenesis of stunting is poorly understood. Prenatal and postnatal nutritional deficits and enteric and systemic infections clearly contribute, but recent findings implicate a central role for environmental enteric dysfunction (EED), a generalized disturbance of small intestinal structure and function found at a high prevalence in children living under unsanitary conditions. Mechanisms contributing to growth failure in EED include intestinal leakiness and heightened permeability, gut inflammation, dysbiosis and bacterial translocation, systemic inflammation, and nutrient malabsorption. Because EED has multiple causal pathways, approaches to manage it need to be multifaceted. Potential interventions to tackle EED include: (1) reduction of exposure to feces and contact with animals through programs such as improved water, sanitation, and hygiene; (2) breastfeeding and enhanced dietary diversity; (3) probiotics and prebiotics; (4) nutrient supplements, including zinc, polyunsaturated fatty acids, and amino acids; (5) antiinflammatory agents such as 5-aminosalicyclic acid; and (6) antibiotics in the context of acute malnutrition and infection. Better understanding of the underlying causes of EED and development of noninvasive, practical, simple, and affordable point-of-care diagnostic tools remain key gaps. "Omics" technologies (genomics, epigenomics, transcriptomics, proteomics, and metabolomics) and stable isotope techniques (eg, 13C breath tests) targeted at children and their intestinal microbiota will enhance our ability to successfully identify, manage, and prevent this disorder.

Byler disease: early natural history.

OBJECTIVES: Byler disease, originally described in Amish kindred, results from mutations in ATPase Class I Type 8b Member 1 (ATP8b1). Specific clinical reports of Amish Byler disease were last published 40 years ago. These investigations were directed at the present detailed clinical understanding of the early course of hepatic manifestations of Byler disease. METHODS: This study analyzed routine clinical practice and outcomes of children with Byler disease (defined by homozygous c.923G>T mutation in ATP8b1), who initially presented to Children's Hospital of Pittsburgh of UPMC between January 2007 and October 2014. Data were analyzed to the earlier of 24 months of age or partial external biliary diversion. RESULTS: Six children presented between 1 and 135 days of life: 2 presented with newborn direct hyperbilirubinemia, 2 had complications of coagulopathy, 1 had failure to thrive and rickets, and 1 sibling was identified by newborn genetic testing. Intensive fat-soluble vitamin supplementation was required to prevent insufficiencies in vitamins D, E, and K. Hyperbilirubinemia was variable both over time and between children. Serum bile acid levels were elevated, whereas γ-glutamyltranspeptidase levels were low normal. Scratching behavior (pruritus) was intractable in 4 of 6 children with onset between 6 and 12 months of age. Features of portal hypertension were not observed. Partial external biliary diversion was used during the second year of life in 4 children. CONCLUSIONS: Detailed analysis of Byler disease revealed varied disease presentation and course. Nutritional issues and pruritus dominated the clinical picture in the first 2 years of life.

Total body sodium depletion and poor weight gain in children and young adults with an ileostomy: a case series.

Patients with high-output small bowel ostomies are at risk for total body sodium depletion (TBSD), defined as a urine sodium level <10 mmol/L. Failure to thrive (FTT) as a consequence of TBSD has been reported in neonates with ileostomies; however, this has not been well described in older children. The records of all children beyond the age of infancy with a small bowel ostomy cared for in our Children's Intestinal Rehabilitation Program from 2010-2012 were reviewed. Four patients between the ages of 18 months and 19 years were identified as having TBSD. All 4 patients experienced unintentional weight loss, despite adequate energy intake based on calculated needs, which was associated with a urine sodium level ≤10 mmol/L. With the supplementation of sodium, either enteral or intravenous, all patients demonstrated improved weight gain and correction of TBSD. The following cases suggest that the relationship between TBSD and FTT may extend well beyond the neonatal period and possibly into adulthood. We advise that patients of all ages with high stoma output have routine urine sodium levels checked, particularly in the setting of weight loss or poor gain. Furthermore, instances of TBSD should be treated with sodium supplementation. Further research is needed to better understand the relationship between TBSD and FTT and to establish intervention guidelines.

The clinical and biochemical presentation of vitamin D deficiency and insufficiency in children and adolescents.

AIM: To evaluate the clinical and biochemical findings of the children and adolescents with vitamin D deficiency and insufficiency in order to determine the clinical and biochemical presentation differences between age groups. METHODS: This retrospective study included a review of medical reports of 543 patients (aged between 1-17 years) who were referred to our hospital between October 2011 and May 2012 with symptoms related to vitamin D deficiency or insufficiency. The patients were divided into four groups by age: 1-3 years (Group 1), 4-6 years (Group 2), 7-11 years (Group 3) and 12-17 years (Group 4). Patients diagnosed with vitamin D deficiency or insufficiency were evaluated as to their clinical and biochemical findings. RESULTS: Gender distribution were not statistically different between the four groups. The mean ages of Groups 1-4 were 1.9±0.7, 5.1±0.9, 8.9±1.3, 13.1±1.1, respectively. Major complaints on admission were muscle weakness (91%), low weight gain (failure to thrive) (89%), head deformity (frontal bossing) (35.6%), bone deformity (enlargement of wrist and ankles) (29.7%) for Group 1. Muscle weakness (76%) and low weight gain (failure to thrive) (68%) for Group 2. Leg and chest pain were the major symptoms in Group 3 (57% and 28%, respectively) and in Group 4 (26% and 55%, respectively) as well as high rates of obesity (31% and 63%). The biochemical findings of vitamin D deficiency mostly appeared in the first group who developed vitamin D deficiency due to the lack of vitamin D supplementation. However, in older children, the majority of the patients had low 25 hydroxyvitamin D (25 OHD) values without evidence of biochemical findings of osteomalacia. CONCLUSION: Depending on the degree of deficiency and insufficiency, and the age of the patients, the clinical and biochemical findings varied widely. Children under the age of 3 who either never received vitamin D supplementation or who had been receiving supplementation that was stopped too early were at a greater risk for developing clinically and biochemically proved vitamin D deficiency. In older children, low vitamin D levels mostly resulted in subtle complaints without abnormal biochemical findings.

The adolescent child with short bowel syndrome: new onset of failure to thrive and need for increased nutritional supplementation.

BACKGROUND: Proper nutrition for children with short bowel syndrome (SBS) is challenging. An underappreciated SBS cohort is the group of adolescents in which nutritional demand is markedly increased. We identified several children that previously had weaned off parenteral nutrition but needed re-initiation of parenteral nutrition or increased enteral nutritional supplementation (NS) during adolescence. This study aims to identify characteristics of adolescent SBS patients to better understand their nutritional status. METHODS: A single institution, retrospective review of SBS patients over 10 years was done. Patients were analyzed for NS need, remaining bowel length, height, weight, and essential nutrient levels. RESULTS: Sixteen adolescent SBS patients were identified for analysis. Of these, ten did not require increased NS upon reaching adolescence (group A), and six required increased NS during adolescence (group B). Group B patients required a mean increase in NS of 29.6 kcal/kg per day. Group B patients had significantly shorter remaining bowel length. NS resulted in minimal height increase, but more substantial gains in weight and pubertal development. CONCLUSIONS: Adolescents with SBS may require NS beyond what can be provided enterally. These patients are at risk for nutritional deficiencies, and an aggressive approach to monitoring and supplementing these deficiencies is warranted, especially during this critical period of growth.

Preoperative steroid-related complications in Japanese pediatric patients with ulcerative colitis.

PURPOSE: This study was designed to clarify a limit for steroid therapy in patients with ulcerative colitis through analyzing the preoperative major steroid-related complications and to define when alternative therapies, including surgery, should be performed in pediatric ulcerative colitis patients. METHODS: The medical records of 28 pediatric and 57 adult patients with ulcerative colitis who underwent total proctocolectomy and ileal J-pouch-anal anastomosis were reviewed. The relationship between the preoperative dose of glucocorticoids and major steroid-related complications, as well as the surgery variables, was evaluated. RESULTS: Significantly higher incidences of growth retardation, osteoporosis, glaucoma, and cataracts were noted in pediatric patients than in adult patients. In pediatric patients, major steroid-related complications occurred at a significantly lower preoperative total dosage of glucocorticoids/body weight (mg/kg) or preoperative total dosage of glucocorticoids/body surface area (mg/m2) than in adult patients. A similar surgical procedure was performed in both pediatric and adult patients. The presence of major steroid-related complications can lower a patient's long-term quality of life. CONCLUSIONS: Evidence-based guidelines for the recommended dose of glucocorticoids according to body weight or body surface area are needed. To allow patients to feel well and maintain a good quality of life, early introduction of alternative treatments, including surgery, should be considered.