title: Lens opacities in glycogenoses type I and III.
abstract: OBJECTIVE
  The glycogen storage diseases (GSD) or glycogenoses comprise several inherited diseases caused by abnormalities of the enzymes that regulate the synthesis or degradation of glycogen. This report presents lens opacities not previously described in patients with type I or III GSD.
  
  
  METHODS
  Eleven patients with type I and III GSD.
  
  
  METHODS
  We examined a series of 11 consecutive patients (aged 13-40 years) with type I and III GSD by full ophthalmologic examination.
  
  
  RESULTS
  We found changes of the lens on 7 of 11 patients (aged 23-40 years) with glycogenoses I and III. In 6 patients, the lens showed multiple, bilateral, punctate, and peripheral opacities; only 1 patient showed a posterior subcapsular opacity in both eyes. We did not observe changes in the cornea and the posterior pole correlated to the accumulation of glycogen and lipids.
  
  
  CONCLUSIONS
  In this series, we found that 60% of patients with type I and III GSD show lens opacities. These opacities are bilateral, peripheral, multiple, and small; they do not give any visual disturbance. Considering that subjects with age ranging from 13 to 23 years had no lens opacities, we postulate that they could progressively develop over time because of exposure to recurrent attacks of hypoglycemia, which lead to a progressive depletion of hexokinase.
authors.affiliation: Eye Clinic, San Paolo Hospital, University of Milan, Milan. Electronic address: davideallegrini@yahoo.it.
authors.forename: Davide
authors.initials: D
authors.lastname: Allegrini
authors.affiliation: Eye Clinic, San Paolo Hospital, University of Milan, Milan.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Autelitano
authors.affiliation: Eye Clinic, San Paolo Hospital, University of Milan, Milan.
authors.forename: Paolo
authors.initials: P
authors.lastname: Fogagnolo
authors.affiliation: Eye Clinic, San Paolo Hospital, University of Milan, Milan; Unit of Ophthalmology(,) Maggiore della Carità Hospital, Novara.
authors.forename: Stefano
authors.initials: S
authors.lastname: De Cillà
authors.affiliation: Unit of Pediatric Ophthalmology, Niguarda-Cà Granda Hospital.
authors.forename: Elena
authors.initials: E
authors.lastname: Piozzi
authors.affiliation: Unit of Pediatric Ophthalmology, Niguarda-Cà Granda Hospital.
authors.forename: Marco
authors.initials: M
authors.lastname: Mazza
authors.affiliation: Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy.
authors.forename: Sabrina
authors.initials: S
authors.lastname: Paci
authors.affiliation: Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Montanari
authors.affiliation: Department of Pediatrics, San Paolo Hospital, University of Milan, Milan, Italy.
authors.forename: Enrica
authors.initials: E
authors.lastname: Riva
authors.affiliation: Eye Clinic, San Paolo Hospital, University of Milan, Milan.
authors.forename: Luca
authors.initials: L
authors.lastname: Rossetti
mesh_terms: D000293:Adolescent; D000328:Adult; D002386:Cataract / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D003402:Creatine Kinase / Q000097:blood; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000097:blood / Q000150:complications*; D006010:Glycogen Storage Disease Type III / Q000097:blood / Q000150:complications*; D006801:Humans; D007328:Insulin / Q000097:blood; D019344:Lactic Acid / Q000097:blood; D008055:Lipids / Q000097:blood; D008297:Male; D014792:Visual Acuity / Q000502:physiology; D055815:Young Adult
chemical_list: D007328:Insulin; D008055:Lipids; D019344:Lactic Acid; D003402:Creatine Kinase