Salt solutions are routinely used in salt losing conditions like congenital adrenal hyperplasia, pseudohypoaldosteronism, cystic fibrosis and polyuric salt wasting kidney disease. Problems with administration (of a liquid to an increasingly solid diet), compliance and the behavioural impact of altering food preferences abound.
We describe the case of a 2-year-old boy DA with aldosterone synthetase deficiency who is currently on fludrocortisone and sodium chloride supplementation of 10 mmol/day. DA, the 2nd child of Pakistani parents presented to the ED at 5 weeks of age with an apparent life threatening event. There was no significant antenatal or family history.
Investigations done at this time showed a low sodium and high potassium suggesting CAH. Later, normal 17 OHP and cortisol with inappropriately low aldosterone levels and a urinary steroid profile consistent with aldosterone synthetase deficiency led to the diagnosis.
The family were going to Pakistan for a prolonged visit over the summer and were not happy to be weaned off the sodium before their departure. They did recognise the difficulty of carrying large quantities of solution with attendant issues of spillage and breakage. Enquiries made at various Fast Food Outlets revealed that freely available, standardised salt sachets contain 1 g of salt (16.6 mmol of NaCl) which would result in 2/3rd sachet providing DAs daily requirements. It seemed reasonable to ask the parents to sprinkle the contents of one sachet divided over 3 meals in the course of a day to account for salt being lost on the plate or DA not finishing the meal he was offered. The family are now back in the UK after a long, hot summer in Pakistan and are continuing this as a convenient, safe and non medical solution to DAs salt requirements. A recent clinic visit showed normal electrolytes and continued growth along the 50th centile.
DAs modest requirements of 10 mmol/day of salt has an annual NHS cost of £294 (100 ml bottle with 1 mmol/ml and 5.85 g of salt costs £6.66) taking into account dispensing costs but not shelf life and breakage. As children get older with salt drinks becoming an unpalatable alternative and the food industry comes under increasing pressure to reduce salt in manufactured foods and pre-packaged meals, the ubiquitous salt sachet may come back into currency as the solution to salt solutions!
05 - 07 Nov 2008
British Society for Paediatric Endocrinology and Diabetes