Pseudohypoaldosteronism type I is caused by a peripheral resistance to aldosterone and can present with electrolyte abnormalities, poor growth, or dehydration. Although a rare disease, several case reports have been published regarding Pseudohypoaldosteronism type I in neonates and infants. We report a case of failure to thrive and hyponatremia in an infant with hypoplastic left heart syndrome who was subsequently found to have Pseudohypoaldosteronism type I.

In this paper we set out to explore the prevalence of child undernutrition found in community studies in affluent societies, but a preliminary literature review revealed that, in the absence of a gold standard method of diagnosis, the prevalence largely depends on the measure, threshold and the growth reference used, as well as age. We thus go on to explore describe the common clinical ‘syndromes’ of child undernutrition: wasting, stunting and failure to thrive (weight faltering) and how we have used data from two population-based cohort studies, this paper to explore how much these different ‘syndromes’ overlap and the extent to which they reflect true undernutrition. This analysis revealed that when more than one definition is applied to the same children, a majority are below the lower threshold for only one measure. However, those with both weight faltering and low BMI in infancy, go on in later childhood to show growth and body composition patterns suggestive of previous undernutrition. In older children there is even less overlap and most children with either wasting or low fat seem to be simply growing at one extreme of the normal range. We conclude that in affluent societies the diagnosis of undernutrition is only robust when it relies on a combination of both, that is decline in weight or BMI centile and wasting.

Although failure to thrive (FTT) is generally thought to be a nutritional problem, dietary intake in children with FTT has been little researched. We describe two community-based studies of dietary intake and eating behaviour in FTT. The first study of ninety-seven children with FTT identified by population screening found that only a minority of case children were associated with neglect, organic illness or deprivation, even though dietary information suggested an underlying nutritional cause in the majority. Limited case-control data suggested significantly delayed weaning and less liking for food in general among the case children. The second study found diminished appetite, delayed progression onto solid foods and fewer foods eaten by forty-four children referred with FTT compared with forty-five controls, but was unable to detect a significant difference in energy intake, even allowing for varying body composition and other confounding variables. This study also revealed the bias introduced by recruiting case children by referral rather than screening, and that despite rigorous matching procedures the controls were not representative of the general population. These studies suggest a wide range of differences in eating behaviour and feeding patterns between children with FTT and controls, but suggest that measurements of nutrient totals may not be robust. Studies of FTT based on referred children can be misleading, as can comparison with controls, unless allowance can be made for important confounding social variables.

Adequate nutrition is crucial to the management of children and infants with cardiac disease. Difficulties with feeding are extremely common, and maintaining an adequate caloric intake, in order to achieve sustained growth, is often not possible without nutritional support. We retrospectively reviewed our experience between 1995 and 1999 in treating 37 children with cardiac disease who underwent percutaneous endoscopic construction of a gastrostomy to augment nutritional needs. We stratified the patients into those with cyanotic heart disease, when saturations of oxygen were less than 95%; those with non-cyanotic heart disease with saturations greater than 95%, and those with minor cardiac disease associated with a systemic disorder. Each group was compared to control children matched for age, sex, and diagnosis. We evaluated the variation in standard deviation score for body weight over a median period of follow-up of 295 days. Improvements in the standard deviation score for body weight occurred in each of the groups, whereas children in the control groups demonstrated a decrease in standard deviation score for body weight. The median change of the score for body weight was significantly higher in patients managed with gastrostomy compared to controls. We conclude that supplementation using a gastrostomy tube allows the safe delivery of the caloric intake needed to support malnourished children with cardiac disease.

Background: The clinical symptoms of heart failure in infants with left-to-right shunts are thought to be explained by well-known hemodynamic disturbances such as pulmonary hypertension and overcirculation, but previous studies have not, thus far, found the expected correlations with hemodynamic and clinical parameters. Based on the neurohormonal model of heart failure, we hypothesised that the clinical symptoms of infants with left-to-right shunts are also related to neurohormonal disorders. Methods: We compared various neurohormonal and hemodynamic parameters measured invasively in 70 infants with left-to-right shunts to the respiratory rate and gain in weight over a corresponding period of time. Heart rate correlated significantly with respiratory rate (r = 0.62***, p < 0.001) and gain in weight (r =−0.31*, p = 0.015), but more conventional measures of severity, such as the ratio of pulmonary to systemic flows, failed to show comparable correlations with clinical symptoms. Respiratory rate was related to levels of norepinephrine (r = 0.47***, p < 0.001) and plasma renin activity (r = 0.65***, p < 0.001). The important impact of autonomic imbalance on respiratory rate was underlined by an analysis of variability of heart rate in 26 infants that showed significantly reduced values for the domains of time and frequency. We were not able to find a conclusive multiple regression model with which to explain the symptom "failure to thrive". Conclusions: A increased heart rate, reduced variability in heart rate, and elevated levels of norepinephrine and renin are significant predictors of clinical symptoms such as tachypnea in infants with congenital cardiac malformations. The neurohormonal hypothesis, in which heart failure is interpreted not only as a hemodynamic derangement but also as a neurohormonal disorder, may be valid for infants with congenital cardiac malformations.

The developmental sequelae of infant failure to thrive (FTT) were examined in an unreferred group of 6-year-olds with a history of severe nonorganic growth retardation, sampled from a 1-year birth cohort in an inner-city area of South London. Children who failed to thrive in infancy (weight below the third centile for at least 3 months) and their pairwise matched comparisons were originally studied at 15 months, and 42 cases and 42 controls (89.5% of the sample) were followed up. At 6 years, previously growth-retarded children were considerably smaller than matched comparisons, in terms of body mass index (BMI), and height and weight for age z scores. History of FTT explained substantial variance in weight and BMI at 6 years, with maternal height also contributing to variation in height for age. Child cognitive functioning at 6 years was examined using the McCarthy Scales: cases had more limited quantitative and memory skills than comparisons, but there was no intergroup variation in general cognitive performance. In contrast to analyses of physical development, failure to thrive did not account for cognitive functioning; maternal IQ was the sole significant predictor of performance on all indices of child cognitive abilities. At 15 months, earlier growth faltering was linked to limitations in mental development, but these findings were not confirmed by the follow-up data: the timing of FTT was not related to cognitive abilities at 6 years. Results correspond to past research indicating that nonorganic failure to thrive is associated with persistent limitations in physical stature. There was little evidence of cognitive disadvantage for case group children at school age, suggesting that the adverse effects of early malnutrition on cognitive functioning appear to diminish over time.

A 1-year cohort of children born at term in Newcastle-upon-Tyne in 1987/8 was screened for failure to thrive in infancy using a conditional longitudinal standard which identified those whose weight gain was in the lowest 5%. A group of 136 cases and 136 controls (from the same GP practice and living in a neighbourhood with the same deprivation index) was followed up at 7–9 years of age, when 79% of cases and 87% of controls were successfully studied. Cases at 7–9 were significantly shorter (mean 126.0 cm, SD 5.6) than controls (mean 130.7 cm, SD 5.9); adjusted for parental heights a difference of 4.4 cm (95% CI 2.92 to 5.90 cm) remained. They had smaller head circumferences (mean 51.9 cm, SD 1.8) than controls (mean 52.8 cm, SD 1.7), were significantly lighter (medians 23.8 kg, IR 21.5 to 26.9 in cases, and 27.9 kg, IR 25.2 to 31.6 in controls) and had a lower body mass index (median 14.9, IR 14.1 to 16.0 in cases, and 16.3, IR 15.3 to 17.8 in controls). Despite these large growth differences, no statistically significant differences in cognitive outcomes were found. Mean IQ was 87.6 (SD 17.4) in cases and 90.6 (SD 17.1) in controls; after adjustment for organic cause and mother's IQ the mean difference was 1.7 IQ points (95%CI −5.2 to 1.9). There were no statistically significant differences in the reading, spelling, or reading comprehension scores; the mean standardised reading score was 93.5 (SD 16.2) for cases and 94.5 (SD 15.6) for controls. These results show that when carefully defined by velocity-based anthropometric criteria, nonorganic failure to thrive in infancy is followed by persisting stunting and wasting, and a reduced head circumference, but is not associated with cognitive or educational disadvantages at school age.

The case of a vallecular cyst in a neonate is described. The presentation was with failure to thrive. This previously unreported mode of presentation is discussed, and aspects of the management are emphasized. In particular, the value of flexible nasopharyngoscopy in assessing the neonatal laryngopharynx is highlighted.

Failure to thrive (FTT) in infants with congenital heart disease (CHD) can be attributed to their low energy intakes and high resting energy expenditures. Energy intake, energy expenditure and growth were studied in infants with CHD on normal formula feeds and then on feeds supplemented with glucose polymer to see whether supplementation improved energy retention and growth. Mean gross energy intakes increased by 31.7% on high-energy feeding and mean weight gain improved from 1.3 g/kg per d on control to 5.8 g/kg per d on high-energy feeding. Resting oxygen consumption (νo2 ml/kg per min) was not significantly different on the two feeding regimens, although respiratory quotient rose on high-energy feeding reflecting the increased carbohydrate intake. Estimated energy costs of growth on high-energy feeding fell within the previously described range for normal infants. It is recommended that infants with CHD known to be associated with FTT are fed on high-energy diets from the time of diagnosis in order to optimize growth.