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Osteosclerosis with ichthyosis and fractures

MedGen UID:
331568
Concept ID:
C1833697
Disease or Syndrome
Synonym: CORTICAL THICKENING OF LONG BONES WITH BOWING AND ICHTHYOSIS
 
Monarch Initiative: MONDO:0008160
OMIM®: 166740

Clinical features

From HPO
Tibial bowing
MedGen UID:
332360
Concept ID:
C1837081
Finding
A bending or abnormal curvature of the tibia.
Recurrent fractures
MedGen UID:
42094
Concept ID:
C0016655
Injury or Poisoning
The repeated occurrence of bone fractures (implying an abnormally increased tendency for fracture).
Increased bone mineral density
MedGen UID:
10502
Concept ID:
C0029464
Disease or Syndrome
An abnormal increase of bone mineral density, that is, of the amount of matter per cubic centimeter of bones which is often referred to as osteosclerosis. Osteosclerosis can be detected on radiological examination as an increased whiteness (density) of affected bones.
Femoral bowing
MedGen UID:
347888
Concept ID:
C1859461
Finding
Bowing (abnormal curvature) of the femur.
Cortical thickening of long bone diaphyses
MedGen UID:
870679
Concept ID:
C4025133
Anatomical Abnormality
Abnormal thickening of the cortex of the diaphyseal region of long bones.
Ichthyosis
MedGen UID:
7002
Concept ID:
C0020757
Disease or Syndrome
An abnormality of the skin characterized the presence of excessive amounts of dry surface scales on the skin resulting from an abnormality of keratinization.

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