ABSTRACT
The configuration and progression of the ERG in two children with mucopolysaccharidosis (MPS) I H/S (Hurler-Scheie syndrome) and MPS I H (Hurler syndrome) is described. Physical examination, biochemical analysis, ophthalmic examination and electroretinography were performed. The Hurler-Scheie patient (case 1) showed negative scotopic but normal photopic ERGs, which remained unchanged over 2 years. The Hurler patient (case 2) showed negative scotopic and photopic ERGs which did not alter after bone marrow transplantation (BMT). One year after BMT, further b-wave amplitude reduction had caused the ERGs to become more negative. The electronegative configuration of the ERGs suggests that, in these cases of MPS, the primary retinal abnormality in MPS I may be faulty synaptic transmission from photoreceptors to more proximal elements, deficient bipolar responsivity, or Muller cell disease. Further degradation with time suggests the defect to be progressive with BMT causing little or no improvement. In the Hurler-Scheie syndrome case, the defect appears to spare the cone system and to show little or no progression.
Subject(s)
Electroretinography , Mucopolysaccharidosis I/physiopathology , Retina/physiopathology , Child, Preschool , Female , Humans , Infant , Male , Mucopolysaccharidosis I/geneticsABSTRACT
PURPOSE: To report a case of bilateral endogenous endophthalmitis due to group G Streptococcus after a dental procedure. METHODS: Case report of a 69-year-old woman who presented with pain, decreased vision, bilateral uveitis, and a unilateral hypopyon 1 week after treatment for an abscessed tooth. RESULTS: Bilateral endophthalmitis was diagnosed, and group G Streptococcus was cultured from the vitreous samples. CONCLUSIONS: To our knowledge, this is the second reported case of endogenous endophthalmitis following a dental procedure. Furthermore, it was due to group G Streptococcus, which is a rare cause of this condition.
