Open Access
Table 2.
Classification of “Idiopathic osteolysis” according to Hardegger et al. [5]. Patients with Gorham-Stout syndrome do not show renal involvement or systemic symptoms.
| Type | Typical age of manifestation | Location of manifestation | Nephropathy | Prognosis | |
|---|---|---|---|---|---|
| 1 | Hereditary multicentric osteolysis with dominant inheritance | Juvenile | Carpotarsal osteolysis, less often with affection of the radius and ulna | No | Good, disease arrest in adolescence |
| 2 | Hereditary multicentric osteolysis with recessive transmission | Juvenile | According to type 1, in addition severe generalized osteoporosis | No | Good, disease arrest in adolescence |
| 3 | Non hereditary multicentric osteolysis with nephropathy | Juvenile | Mainly carpometacarpal, rare tarsal affection, malignant hypertension | Yes, proteinuria in progressive renal pathology | Often unfavourable |
| 4 | Gorham-Stout syndrome | Age-independent | Typical: shoulder girdle, pelvis, facial skull | No | Mostly good. When in spine or chylothorax: lethality up to 50% |
| 5 | Winchester syndrome (hereditary, autosomal recessive) | Juvenile | Carpotarsal osteolysis and contractures, short stature, osteoporosis, corneal deterioration | No | Progressive |
Current usage metrics show cumulative count of Article Views (full-text article views including HTML views, PDF and ePub downloads, according to the available data) and Abstracts Views on Vision4Press platform.
Data correspond to usage on the plateform after 2015. The current usage metrics is available 48-96 hours after online publication and is updated daily on week days.
Initial download of the metrics may take a while.