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Epiphyseal dysplasia, Baumann type

MedGen UID:
322764
Concept ID:
C1835830
Disease or Syndrome
Synonym: Epiphyseal Dysplasia, Baumann Type
 
Monarch Initiative: MONDO:0012558
OMIM®: 610797

Clinical features

From HPO
Pes planus
MedGen UID:
42034
Concept ID:
C0016202
Anatomical Abnormality
A foot where the longitudinal arch of the foot is in contact with the ground or floor when the individual is standing; or, in a patient lying supine, a foot where the arch is in contact with the surface of a flat board pressed against the sole of the foot by the examiner with a pressure similar to that expected from weight bearing; or, the height of the arch is reduced.
Ulnar deviation of finger
MedGen UID:
68543
Concept ID:
C0231679
Finding
Bending or curvature of a finger toward the ulnar side (i.e., away from the thumb). The deviation is at the metacarpal-phalangeal joint, and this finding is distinct from clinodactyly.
Genu valgum
MedGen UID:
154364
Concept ID:
C0576093
Anatomical Abnormality
The legs angle inward, such that the knees are close together and the ankles far apart.
Carpal bone aplasia
MedGen UID:
324464
Concept ID:
C1836219
Finding
Congenital absence of a carpal bone.
Aplasia of metacarpal bones
MedGen UID:
335431
Concept ID:
C1846473
Finding
Developmental defect associated with absence of one or more metacarpal bones.
Clinodactyly of the 5th finger
MedGen UID:
340456
Concept ID:
C1850049
Congenital Abnormality
Clinodactyly refers to a bending or curvature of the fifth finger in the radial direction (i.e., towards the 4th finger).
Long fingers
MedGen UID:
346836
Concept ID:
C1858091
Finding
The middle finger is more than 2 SD above the mean for newborns 27 to 41 weeks EGA or above the 97th centile for children from birth to 16 years of age AND the five digits retain their normal length proportions relative to each other (i.e., it is not the case that the middle finger is the only lengthened digit), or, Fingers that appear disproportionately long compared to the palm of the hand.
Growth abnormality
MedGen UID:
808205
Concept ID:
C0262361
Finding
Epiphyseal dysplasia
MedGen UID:
95932
Concept ID:
C0392476
Congenital Abnormality
Metaphyseal irregularity
MedGen UID:
325478
Concept ID:
C1838662
Finding
Irregularity of the normally smooth surface of the metaphyses.
Joint hypermobility
MedGen UID:
336793
Concept ID:
C1844820
Finding
The capability that a joint (or a group of joints) has to move, passively and/or actively, beyond normal limits along physiological axes.
Narrow pelvis bone
MedGen UID:
336266
Concept ID:
C1848103
Finding
Reduced side to side width of the pelvis.
Hypoplasia of the femoral head
MedGen UID:
384014
Concept ID:
C1856920
Anatomical Abnormality
Underdevelopment of the femoral head.
Marked delay in bone age
MedGen UID:
401468
Concept ID:
C1868549
Finding
Absent metacarpal epiphyses
MedGen UID:
867155
Concept ID:
C4021513
Anatomical Abnormality
Absence of the epiphyses of the metacarpal bones, which are normally located at the distal ends of the metacarpals.

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