Was ist Proteus Syndrom?

Was ist Proteus Syndrom?

Das Proteus-Syndrom (PS) ist eine sehr seltene und komplexe Krankheit mit hamartösem Überwuchs/’Riesenwuchs‘. Kennzeichnend ist ein fortschreitender Überwuchs des Skeletts, der Haut, des Fettgewebes und des Zentralnervensystems.

Was sind vergrößerte Extremitäten?

Als Akromegalie (aus altgriechisch ἄκρος akros, ‚äußerst‘, + μέγας megas, ‚groß‘) wird eine ausgeprägte chronische, durch einen Überschuss an Wachstumshormon (Somatotropin) verursachte Vergrößerung von Akren (distale Teile (periphere Enden) der Extremitäten und vorspringende Teile des Körpers) bezeichnet.

What is the life expectancy of someone with Proteus syndrome?

The prognosis of this condition is poor as the mortality rate for those affected is high. It has been noted that about a median survival age for the affected is only 2.5 days. It’s considered a lucky case if the affected child has survived at least 6 months of life and more luckier if they have reached their teens.

What are the symptoms of Proteus syndrome?

Noncancerous (benign) tumors

Abnormal curvature of the spine ( scoliosis)

Fatty growths (lipomas) or areas of tissue loss ( atrophy)

Malformed blood vessels,which increases the risk of blood clots and pulmonary embolism (the most common cause of death in people with Proteus syndrome)

What are the effects of Proteus syndrome?

The overgrowth or growth caused by Proteus syndrome can severely alter a person’s appearance. When one or more limb is affected, it can greatly impact a person’s mobility. This, combined with neurological effects, may limit a person’s ability to live independently.

Is there a cure for Proteus syndrome?

There is no cure for Proteus syndrome. Each person who has the condition will have different symptoms, and those symptoms will vary in severity. All patients with Proteus syndrome will need to work with a team of healthcare professionals to manage their medical care.