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Skeletal dysplasia with delayed epiphyseal and carpal bone ossification

MedGen UID:
356650
Concept ID:
C1866939
Disease or Syndrome
Synonym: Skeletal Dysplasia With Delayed Epiphyseal And Carpal Bone Ossification
 
Monarch Initiative: MONDO:0008430
OMIM®: 182255

Clinical features

From HPO
Delayed ossification of carpal bones
MedGen UID:
374771
Concept ID:
C1841684
Finding
Ossification of carpal bones occurs later than age-adjusted norms.
Short middle phalanx of finger
MedGen UID:
337690
Concept ID:
C1846950
Finding
Short (hypoplastic) middle phalanx of finger, affecting one or more fingers.
Short 1st metacarpal
MedGen UID:
376561
Concept ID:
C1849311
Finding
A developmental defect characterized by reduced length of the first metacarpal (long bone) of the hand.
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).
Broad middle phalanx of finger
MedGen UID:
867025
Concept ID:
C4021383
Anatomical Abnormality
Increased width of the middle phalanx of finger.
Short stature
MedGen UID:
87607
Concept ID:
C0349588
Finding
A height below that which is expected according to age and gender norms. Although there is no universally accepted definition of short stature, many refer to "short stature" as height more than 2 standard deviations below the mean for age and gender (or below the 3rd percentile for age and gender dependent norms).
Skeletal dysplasia
MedGen UID:
98053
Concept ID:
C0410528
Disease or Syndrome
A general term describing features characterized by abnormal development of bones and connective tissues.
Cone-shaped epiphysis
MedGen UID:
351282
Concept ID:
C1865037
Finding
Cone-shaped epiphyses (also known as coned epiphyses) are epiphyses that invaginate into cupped metaphyses. That is, the epiphysis has a cone-shaped distal extension resulting from increased growth of the central portion of the epiphysis relative to its periphery.
Delayed epiphyseal ossification
MedGen UID:
351324
Concept ID:
C1865200
Finding

Recent clinical studies

Diagnosis

Tchio Tchoumba MA, Bai Y, Jin R, Yu X, Male M
Orthop Surg 2020 Feb;12(1):333-336. Epub 2019 Dec 6 doi: 10.1111/os.12546. PMID: 31808622Free PMC Article
Kim OH, Cho TJ, Song HR, Chung CY, Miyagawa S, Nishimura G, Superti-Furga A, Unger S
Skeletal Radiol 2009 Aug;38(8):803-11. Epub 2009 Mar 11 doi: 10.1007/s00256-009-0671-4. PMID: 19277648

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