Severe rickets in Lowe syndrome: treatment with continuous nasogastric infusion.
A boy with Lowe syndrome who manifested renal Fanconi syndrome by severe hypophosphatemic rickets, failure to thrive, and metabolic acidosis failed to improve with conventional bolus therapy of phosphate and bicarbonate. He was then placed on home continuous nasogastric infusion of phosphate and bicarbonate in addition to caloric supplementation. Rapid reversal of metabolic acidosis and hypophosphatemia was achieved. There was significant improvement in clinical and radiological signs of rickets and in bone mineral content. After 9 months therapy, his ponderal age improved dramatically from 7.5 to 29.5 months and his statural age from 16 to 26 months. We conclude that continuous nasogastric infusion of phosphorus and bicarbonate is a useful alternative mode of therapy in patients with renal Fanconi syndrome who are resistant to conventional bolus therapy.
Redfield
V A
VA
Department of Pediatrics, Children's Hospital Medical Center, University of Cincinnati College of Medicine, OH 45267-0541.
Mimouni
F
F
Strife
F C
FC
Tsang
R C
RC
D000138:Acidosis / Q000628:therapy; D001639:Bicarbonates / Q000008:administration & dosage; D002675:Child, Preschool; D005198:Fanconi Syndrome / Q000628:therapy*; D006801:Humans; D007263:Infusions, Parenteral; D007441:Intubation, Gastrointestinal; D008297:Male; D009800:Oculocerebrorenal Syndrome / Q000150:complications*; D010710:Phosphates / Q000008:administration & dosage / Q000097:blood
D001639:Bicarbonates; D010710:Phosphates