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November 1955

Ocular Manifestations of Hereditary Primary Systemic Amyloidosis

Author Affiliations

Ann Arbor, Mich.
University of Michigan Medical School.

AMA Arch Ophthalmol. 1955;54(5):660-664. doi:10.1001/archopht.1955.00930020666004

Hereditary primary systemic amyloidosis, an unusual expression of the amyloid problem, is characterized by a protean symptomatology that remarkably mimics the commonplace and the obscure. To date only four reports have appeared in the world literature describing in some detail the clinical, genetic, and pathologic constellation.* Recently three reports have appeared which emphasize the remarkable involvement of the ocular apparatus.†

As to the genesis of this universal and morbid process, much speculation has appeared in the literature in the past halfcentury. On the one hand, it has been held that the pathophysiology represents the endresult of an immunological reaction, and, on the other, it has been regarded as an anomaly of the connective tissue ground substance. For the greater part, these observations have been based upon, with their known limitation, fundamental animal experimental data. It has recently been suggested that secondary (parenchymatous) and primary (mesenchymal) systemic amyloidosis might represent similar

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