.: OKULISTYKA :.

Wydanie 2/2009
str. 29

Keratopatie metaboliczne - obraz kliniczny najczęstszych jednostek chorobowych

Metabolic Keratopathies - Clinical Manifestations of the Most Frequent Diseases

Robert Sulak, Anna M. Ambroziak, Anna Kamińska

Katedra i Klinika Okulistyki II Wydziału Lekarskiego Warszawskiego Uniwersytetu Medycznego Samodzielny Publiczny Kliniczny Szpital Okulistyczny w Warszawie Kierownik: prof. dr hab. n. med. Jerzy Szaflik

Summary: Some metabolic disorders are accompanied by ophthalmological symptoms. Cornea is described to be the most frequent part of the eye affected by these diseases. Cystinosis, Wilson’s disease, multiple myeloma, Waldenstoem ‘s macroglobulinemia and mucopolysaccharidoses can be classified as the most common metabolic diseases causing ophthalmological problems. Cystinosic is characterized by accumulation of cystein in result of disturbed lisosomal transport. Deposition of immunoproteins refers to multiple myeloma, Waldenstoem ‘s macroglobulinemia and leukemia. On account of deficiency or malfunctioning of specific lysosomal enzymes mucopolysaccharidoses lead to abnormal accumulation of certain complex carbohydrates in vacuoles of various tissues including cornea. Excessive copper incrustation in tissues is caused by Wilson’s disease. Corneal deposits cause visual acuity deterioration. Therapy of metabolic keratopathy is based on systemic metabolic disorders treatment. In more advanced cases corneal transplantation is required.

Słowa kluczowe: choroby metaboliczne, cystynoza, choroba Wilsona, szpiczak mnogi, makroglobulinemia Waldenströma, mukopolisacharydozy.

Keywords: metabolic diseases, cystinosis, Wilson’s disease, multiple myeloma, Waldenstoem‘s macroglobulinemia.

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