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Exposure to nutritional imbalance during early life can influence disease risk lifelong and across generations. In this long‐term conditioning, epigenetics constitutes a key mechanism. They bridge environmental cues and the expression of genes involved in the setting of long‐standing biological regulations in numerous organs and species. Epigenetic marks are proposed as innovative diagnostic biomarkers and potential targets in the prevention of diseases. However, a number of uncertainties make them difficult to use in clinical approaches in the context of early exposure to nutritional challenge. In conclusion, active investigations in this field are still needed before clinical applications are considered. [ABSTRACT FROM AUTHOR]

Aim: Extremely low birthweight (ELBW) neonates require a high protein intake, but this can be challenging in the very rare cases when they also have phenylketonuria (PKU). This is due to a lack of suitable parenteral nutrition or enteral formula. Our aim was to analyse tolerance to phenylalanine in these infants.Material: There are approximately 110 000 children born in the Czech Republic each year. A neonatal screening programme from 2005 to 2020 found that 320 neonates had PKU, including 30 premature neonates with a birth weight of less than 2500 g.Results: This study focused on three neonates who were born with ELBWs of 720, 740 and 950 g, respectively. Phenylalanine levels normalised in ELBW neonates with PKU within 1 week of the introduction of low-phenylalanine parenteral or enteral nutrition. The tolerance to phenylalanine was very high (70-110 mg/kg) in the first months of life, due to a rapid weight gain, but significantly decreased during infancy.Conclusion: Extremely low birthweight neonates with PKU need special dietary management. Regular assessments of phenylalanine are necessary during the first weeks of life to allow prompt dietary adjustments that reflect rapid weight gain and transitory high tolerance to phenylalanine. [ABSTRACT FROM AUTHOR]

Aim: Improved survival rates for premature infants have also increased the population at risk of necrotising enterocolitis (NEC). This study evaluated the outcomes of surgically treated NEC and identified risk factors for mortality, intestinal failure (IF) and IF associated liver disease (IFALD). Methods: This was a retrospective observational study of 131 infants with surgically treated NEC from 1976 to 2016 in a Swedish tertiary referral centre: 20 in 1976–1996, 33 in 1997–2006 and 78 in 2007–2016. Data were extracted from medical records, and the Cox regression model was used to identify risk factors. Results: When the first and last periods were compared, they showed decreases in both gestational age, from 30 to 26 weeks, and mortality rates, from 45% to 29%. IF was found in 67 patients (56%), IFALD in 41 patients (34%) and short bowel syndrome (SBS) in 13 (19%). The incidence of IF was high, even in infants without SBS. Low gestational age was an independent risk factor for mortality. No risk factors were identified for IF or IFALD. Conclusion: Survival rates for NEC improved from 1976–2016, despite a decrease in gestational age. Clinicians should be particularly aware of the risk of infants without SBS developing IF. [ABSTRACT FROM AUTHOR]

Aim: Extensive ongoing research on probiotics and infant formulas raises a number of safety questions. One concern is the potential influence of d-lactic acid-containing preparations on the health of infants and children. The aim of this review was to summarise the available knowledge on the ingestion of d-lactic acid-producing bacteria, acidified infant formulas and fermented infant formulas as a potential cause of paediatric d-lactic acidosis.Methods: A Medline database search was performed in July 2017, with no restrictions on the language, article type or publication date. The 1715 search results were screened for clinical trials, review articles, case series and case reports of relevance to the topic.Results: We identified five randomised controlled trials from 2005 to 2017 covering 544 healthy infants and some case reports and experimental studies. No clinically relevant adverse effects of d-lactic acid-producing probiotics and fermented infant formulas were described in healthy children. However, a harmless, subclinical accumulation of d-lactate was theoretically possible. The only known cases of paediatric d-lactic acidosis occurred in patients with short bowel syndrome or, historically, in infants fed with acidified formulas.Conclusion: Our main finding was that probiotics and fermented formulas did not cause d-lactic acidosis in healthy children. [ABSTRACT FROM AUTHOR]

Aim: Children with ultra-short bowel syndrome (USBS) have not been extensively studied to date because the condition is rare. The aim of the study was to assess the nutritional status of children with USBS receiving home parenteral nutrition, using citrulline serum concentration and cholestasis.Methods: We studied 17 patients with USBS, with a median age of 6.6 years and median duration of parenteral nutrition of 6.6 years. The study was carried out at The Children's Memorial Health Institute, Warsaw, from January 2014 to January 2015.Results: The median standard deviation score (SDS) was -1.2 for body mass according to chronological age, -1.72 according to height and -0.59 according to height for age. Patients requiring seven days per week parenteral nutrition had a citrulline concentration below 10 μmol/L. Decreased bone-mineral density was observed in 87% of the patients. Low values of 25-hydroxyvitamin D were found in 53% of the children. None of the patients had elevated conjugated bilirubin levels above 34.2 μmol/L.Conclusion: Children with USBS were growth deficient according to their chronological age, with frequent abnormal bone mineralisation and vitamin D deficiency. Children requiring parenteral nutrition seven days a week had citrulline concentrations below 10 μmol/L. Cholestasis was not seen. [ABSTRACT FROM AUTHOR]

Aim Necrotising enterocolitis ( NEC) is associated with high morbidity and mortality. The aim of this study was to identify predictors of intestinal failure ( IF), morbidity and mortality following NEC. Methods We performed a retrospective study of all neonates treated for NEC stage II or greater at a tertiary referral NICU between 2000 and 2009. Demographic data, need for surgery, residual bowel length and rates of bacteraemia, cholestasis, IF and mortality were analysed. Results During the 10-year period, 301 patients were referred with NEC and 152 had surgical intervention. Overall mortality was 32%. Of the 230 infants who survived >42 days, 97 (42%) had IF at 42 days, decreasing to 15% at >90 days. The rate of IF was significantly higher in the surgical group than the medical group ( OR 2.04, 95% CI, 1.25-3.35, p < 0.004), but 23% of the medically treated infants with NEC also developed IF. There was a significant relationship between IF and gram-negative bacteraemia, the need for surgery, cholestasis, liver failure and mortality. Conclusion Intestinal failure occurred in a significant proportion of infants with NEC. Predictors for IF among infants with NEC were low birthweight, low gestational age, need for surgical intervention and gram-negative bacteraemia. [ABSTRACT FROM AUTHOR]

Blockage of the renin-angiotensin system (RAS) during pregnancy is known to cause serious dysfunction of many organs of the foetus and newborn. Angiotensin II is an angiogenic factor and angiotensin-converting enzyme (ACE) inhibitors prevent retinal neovascularisation and their use in preventing proliferative retinopathy of prematurity has been suggested. We report on twin girls born after 32 gestational weeks, who were exposed to blockage of the RAS during gestation, had severely reduced retinal vasculature and developed severe retinopathy of prematurity (ROP) despite receiving very little extra oxygen. Conclusion: It is likely that ACE inhibitors and angiotensin receptor blockers not only reduce pathological neovascularisation but also normal vascularisation of the eyes and other organs. [ABSTRACT FROM AUTHOR]

The term “auto-brewery syndrome” has been frequently used to describe patients who show features of alcohol intoxication because of abnormal yeast proliferation after ingesting carbohydrate-rich meals. We present a case of a 3-y-old girl with short bowel syndrome (SBS) who demonstrated signs of alcohol intoxication on repeated occasions. A blood test indicated an ethanol concentration of 15 mmol/l, and cultures from gastric fluid and faeces showed the presence of Candida kefyr . An association was found between the introduction of a carbohydrate-rich fruit drink and the occurrence of symptoms. Conclusion: The possibility of endogenous ethanol fermentation should be considered in patients with SBS and the diagnosis of auto-brewery syndrome added to the differential diagnosis list for D-lactic acidosis. Management includes both antifungal treatment and special diet modification. [ABSTRACT FROM AUTHOR]

Aim: Improved survival rates for premature infants have also increased the population at risk of necrotising enterocolitis (NEC). This study evaluated the outcomes of surgically treated NEC and identified risk factors for mortality, intestinal failure (IF) and IF associated liver disease (IFALD).Methods: This was a retrospective observational study of 131 infants with surgically treated NEC from 1976 to 2016 in a Swedish tertiary referral centre: 20 in 1976-1996, 33 in 1997-2006 and 78 in 2007-2016. Data were extracted from medical records, and the Cox regression model was used to identify risk factors.Results: When the first and last periods were compared, they showed decreases in both gestational age, from 30 to 26 weeks, and mortality rates, from 45% to 29%. IF was found in 67 patients (56%), IFALD in 41 patients (34%) and short bowel syndrome (SBS) in 13 (19%). The incidence of IF was high, even in infants without SBS. Low gestational age was an independent risk factor for mortality. No risk factors were identified for IF or IFALD.Conclusion: Survival rates for NEC improved from 1976-2016, despite a decrease in gestational age. Clinicians should be particularly aware of the risk of infants without SBS developing IF. [ABSTRACT FROM AUTHOR]

Short bowel syndrome (SBS) is characterized by a state of malabsorption following extensive resection of the small bowel, resulting in insufficient nutritive supply requiring artificial nutrition with long-term parenteral nutrition. Here we present an illustrative case report of a premature infant born with gastroschisis and SBS, who was treated with enteral refeeding via rectum. The infant developed during the period of rectal feeding with jejunostomy loses bowel lengthening and could be fed orally within a few months after birth. Rectal feeding with ostomy loses could stimulate bowel growth and adaptation in neonatal SBS. Conclusion: The purpose of this report is to describe an illustrative case of short bowel syndrome due to gastroschisis and to share a novel technique of rectal feeding to stimulate bowel growth and adaptation. [ABSTRACT FROM AUTHOR]