Turner-Kieser Syndrome

Turner-Kieser syndrome (also known as hereditary arthroosteoonychodysplasia, hereditary onychoarthrosis, or hereditary onychoarthroosteodysplasia) is a rare genetic disorder characterized by congenital defects of the nails and bones.

This disease was first described by the American physician Joseph W. Turner and the German physician Wilhelm Kieser in 1938, after whom the syndrome was named.

The main symptoms of Turner-Kieser syndrome:

1. Deformations and underdevelopment of fingernails and toenails
2. Osteoporosis and other bone development defects
3. Short height
4. Deformities of the feet and other joints

The cause of the disease is mutations in the ROR2 and FAM20C genes, which play an important role in the normal development of the skeleton and nails. Turner-Kieser syndrome is inherited in an autosomal recessive manner.

Diagnosis is based on the clinical picture and gene analysis. Treatment is mainly symptomatic and aimed at correcting bone and joint deformities. The prognosis for life is favorable, but disability is possible due to severe skeletal abnormalities.

Familial Turner-Kieser syndrome is a rare hereditary disease in which the patient develops various dysfunctions of the musculoskeletal system. The main symptoms are arthrosis, ankylosis and onychohypertrophy on the toes. In this article we will look at what Turner-Kieser syndrome is, how to treat it, and what consequences this disease can cause.

Turner–Kieser syndrome (synonym: Arthroostoochodnisaplasia) is a progressive hereditary osteo-dystrophic lesion of the musculoskeletal system, especially often observed in childhood and adolescence. Povy