Hereditary endotheliopathy, retinopathy, nephropathy, and stroke
Other Names for this Disease
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At this time there is no effective treatment for hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS). Treatment of HERNS is largely palliative, which means that it is aimed at decreasing pain and suffering by providing treatments for relief of symptoms along with comfort and support. In some cases, aspirin may be recommended. Laser treatment to prevent retinal hemorrhage may be beneficial to some affected individuals. A continuous maintenance dose of corticosteroids may be prescribed to manage cerebral edema (swelling in the brain).
Last updated: 10/9/2013
- Dichgans M, Meschia JF. Cerebrovascular disorders. In: Rimoin DL et al. Principle and Practice of Medical Genetics, 5th ed. Philadelphia, PA: Churchill Livingston Elsevier; 2002;
- Jen JC, Baloh RW. Herediatry endotheliopathy with retinopathy, nephropathy, and stroke (HERNS). In: Bogousslavsky J, Caplan L., eds. Uncommon Causes of Stroke. 2nd ed. Cambridge University Press; 2001;
- Seifried C, Sitzer M, Jen J, Auburer G. HERNS. A rare, hereditary, multisystem disease with cerebral microangiopathy. Nervenartz. 2005;
- Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition.