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Letter to the Editor
Retinopathy associated with pancreatitis in a child with maple syrup urine disease
  1. JOHN DANIAS,
  2. EDWARD L RAAB,
  3. ALAN H FRIEDMAN
  1. Department of Ophthalmology, Mt Sinai Medical Center, CUNY, New York, USA
  1. Dr Friedman, Mt Sinai Medical Center, Box 1183, 1 Gustave L Levy Place, New York, NY 10029, USA.

https://doi.org/10.1136/bjo.82.7.841

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    Editor,—Retinopathy associated with pancreatitis is an uncommon condition first described in 1975.1 To date, fewer than 50 cases have been reported, all involving adults. We report a case of pancreatitis with retinopathy in a young child with maple syrup urine disease, a rare metabolic disorder.

    case report

    A 7 year old Indian female presented with maple syrup urine disease (MSUD) diagnosed in infancy. She has been maintained on a special diet since then, enjoying normal development. However, during December 1995 she was admitted to the hospital for management of acute gastroenteritis and dehydration. Laboratory studies included serum amylase and lipase which were within normal limits. She received hyperalimentation with glucose and intralipids through a femoral catheter. On her third hospital day she was noted to have mental status changes which improved with hydration. However, on her sixth hospital day her mental status deteriorated again. Her abdomen became diffusely tender. Laboratory studies …

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