Background Juvenile dermatomyositis is a systemic vasculopathy, affecting mainly the skin and muscles. Gastrointestinal manifestations, including poor gut motility and gastrointestinal vasculopathy are recognised. We report a case with GI involvement which did respond to initial therapies, but with an unexpected sequale.
Case report A 12-year-old girl was seen with a history of joint pain for 18 months, a rash and increasing difficulty with activities of daily life. She had significant weight loss and constipation. On examination, she was thin and pale, Gottren's papules were noted, she had painful restriction of movement in several joints and reduced power in her legs and shoulders. She was ANA positive (1 in 100), but otherwise had normal autoantibodies, viral titres and electrolytes. She was started on methylprednisolone and her symptoms rapidly improved, including her appetite. Blood tests 3 days later showed a phosphate of 0.69 mmol/l and ALT increased to 167 IU/l. In combination with her significantly reduced body mass index, she was diagnosed with refeeding syndrome. She was started on phosphate supplements and a semi-elemental formula, with daily electrolyte monitoring and then subsequent increases in feed volumes. Her electrolytes and liver function normalised and she suffered no further problems.
Discussion This is the first reported case of a child with refeeding syndrome associated with juvenile dermatomyositis. The refeeding syndrome is encountered in patients who have been nutritionally depleted. In this state, patients deplete glycogen stores and move to fat metabolism. Despite normal serum electrolyte levels, total body stores are low. On the resumption of diet, carbohydrate metabolism begins and is associated with an intracellular influx of electrolytes and drop in serum levels. While initially asymptomatic, spasms, tetany and arrhythmias may follow, with ultimate cardiac and respiratory failure. While there is limited trial data to guide management of patients with refeeding syndrome, there are a number of well recognised risk factors and consensus guidelines.
Conclusions Although it presents with almost no clinical features, refeeding syndrome can have significant morbidity and mortality. As it can occur in children who are nutritionally depleted for any reason, screening and prompt recognition of patients at risk is needed.