Literature DB >> 7443310

Diagnosis of lysosomal storage diseases by the ultrastructural study of conjunctival biopsies.

J Libert.   

Abstract

This ultrastructural study of conjunctival biopsies of 150 patients affected with various lysosomal storage disease allows us to demonstrate particular lesions in all the infantile phenotypes investigated so far. These alterations appear before the first clinical signs, suggest the possibility of such a disorder, and are often specific enough to propose an accurate diagnosis either because the lysosomes have a typical appearance or because their topographic distribution among the different conjunctival cell types is characteristic. As the morphologic approach is an obligatory step in the recognition of lysosomal storage diseases, we suggest concurrent use of conjunctival biopsy for the early screening and diagnosis of these disorders, as it is a very simple, easy, harmless technique, whose results are generally superior to those obtained by other methods.

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Year:  1980        PMID: 7443310

Source DB:  PubMed          Journal:  Pathol Annu        ISSN: 0079-0184


  5 in total

Review 1.  The efficacy of conjunctival biopsy as a screening technique in lysosomal storage disorders.

Authors:  M L Mazow
Journal:  Trans Am Ophthalmol Soc       Date:  1987

2.  Skeletal muscle pathology of mannosidosis in two siblings with spastic paraplegia.

Authors:  H Kawai; H Nishino; Y Nishida; K Yoneda; Y Yoshida; T Inui; K Masuda; S Saito
Journal:  Acta Neuropathol       Date:  1985       Impact factor: 17.088

3.  Neuronal ceroid lipofuscinosis. Ocular histopathologic and electron microscopic studies in the late infantile, juvenile, and adult forms.

Authors:  E I Traboulsi; W R Green; M W Luckenbach; Z C de la Cruz
Journal:  Graefes Arch Clin Exp Ophthalmol       Date:  1987       Impact factor: 3.117

4.  Infantile form of sialic acid storage disorder: clinical, ultrastructural, and biochemical studies in two siblings.

Authors:  M Tondeur; J Libert; E Vamos; F Van Hoof; G H Thomas; G Strecker
Journal:  Eur J Pediatr       Date:  1982-10       Impact factor: 3.183

5.  Beta-mannosidosis: lesions of the distal peripheral nervous system.

Authors:  J A Malachowski; M Z Jones
Journal:  Acta Neuropathol       Date:  1983       Impact factor: 17.088

  5 in total

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