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Table 1 Classification of “idiopathic osteolysis” according to Hardegger et al

From: A report of two children with Gorham-Stout disease

Type Typical age at manifestation Location of the manifestation Nephropathy Prognosis
I
Hereditary multicentric osteolysis with dominant inheritance
Juvenile Carpotarsal osteolysis, sometimes affecting the radius and ulna No Good, self-limiting in adolescence
II
Hereditary multicentric osteolysis with recessive transmission
Juvenile Consistent with type I, in addition to generalized osteoporosis No Good, self-limiting in adolescence
III
Nonhereditary multicentric osteolysis with nephropathy
Juvenile Mainly carpometacarpal, tarsal involvement is rare, malignant hypertension Yes, proteinuria in progressive renal pathology Unfavorable
IV
Gorham-Stout syndrome
Independent of age Typical: shoulder, pelvis, facial skull bones No Usually good. When there is spinal involvement or chylothorax, mortality rises more than 50%
V
Winchester syndrome (hereditary, autosomal recessive)
Juvenile Carpotarsal osteolysis and contractures, short stature, osteoporosis, corneal deterioration No Progressive