No abstract

Seven cases of cystic fibrosis complicated by chronic salt depletion and failure to thrive were studied. After replacement of the salt deficit, the metabolic abnormalities resolved, and weight gain was rapid. This should be considered as a differential diagnosis in children who have been diagnosed as having cystic fibrosis, but who fail to thrive despite standard treatment.Metabolic alkalosis in association with low serum electrolyte concentrations (hyponatraemia, hypokalaemia, and hypochloraemia) is uncommon in infancy. In the United Kingdom the more common causes included pyloric stenosis, gastric drainage without electrolyte replacement, and-less common-chloride losing nephropathy, the use of thiazide diuretics, and Bartter's syndrome. Acute salt loss in cystic fibrosis is well known, but the gradual development of abnormally low serum electrolyte concentrations, metabolic alkalosis, and failure to thrive without severe dehydration is less widely recognised. Though most cases have been reported from North America, the syndrome has also been described in the United Kingdom.

ObjectiveTo report mortality in babies enrolled on a community-based programme, Care of Next Infant Plus (CONI PLUS), which primarily supports parents anxious because of previous sudden unexpected death in infancy (SUDI) in their extended family or following an apparent life threatening event (ALTE) in their baby.DesignProspective observational study from 1996 to 2010 in the UK.ResultsOf 6487 babies enrolled, 37 died (5.7 per 1000). There were 2789 (43.0%) SUDI related babies of whom, six died suddenly and unexpectedly (2.15 per 1000). Four babies were sharing a sofa at night or a bed with parent(s) who smoked or had consumed alcohol. Of the 1882 (29.0%) babies enrolled following an ALTE, five died suddenly and unexpectedly (2.66 per 1000): four unexplained and one due to infection. None occurred while sharing a sleep surface, and at least three died during the day. The remaining 1816 (28%) babies were enrolled for other reasons. Seven died suddenly and unexpectedly (3.85 per 1000), two were unexplained and none associated with bed sharing.ConclusionsThe number of SUDI deaths in babies enrolled on CONI PLUS is higher than expected from UK averages. Deaths in babies enrolled because of family history of SUDI were mostly associated with inappropriate sharing of a sleep surface at night and mostly outside the peak age range for sudden infant death. The opposite is true for those enrolled following an ALTE. The number of deaths is small but findings suggest a different mechanism for death in these two groups.