Severe growth retardation is found commonly in cystinotic children. Contributing factors include nausea, vomiting, polyuria, abdominal pain, esophageal dysmotility, hypothyroidism, pancreatic insufficiency and eventually renal failure. Treatment with cysteamine has shown a small improvement in growth (Gahl). Our hypothesis is that chronic intravascular volume depletion contributes to growth delay in children with tubular damage from cystinosis.

Seven patients were treated with cysteamine and general supportive therapy with electrolyte supplements. Nightly enteral feeds were initiated through a gastrostomy feeding tube in four of the patients studied. The formula was high volume and low in solute with minerals added to correct electrolyte abnormalities. Growth parameters were taken from before and after enteral feeds were initiated. Table

Table 1
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Early intervention with high volume low solute nutrition via gastrostomy with appropriate electrolyte supplement allowed catch-up growth in 2 children and normal growth velocity in 2 others while children without supplementation continued with short stature. Chronic intravascular volume depletion may be a major factor in growth failure in children with cystinosis.