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Spondylometaphyseal dysplasia, A4 type(SMDA4)

MedGen UID:
324620
Concept ID:
C1836862
Disease or Syndrome
Synonym: Spondylometaphyseal Dysplasia, Type A4
SNOMED CT: Spondylometaphyseal dysplasia A4 type (782912001)
Modes of inheritance:
Autosomal recessive inheritance
MedGen UID:
141025
Concept ID:
C0441748
Intellectual Product
Source: Orphanet
A mode of inheritance that is observed for traits related to a gene encoded on one of the autosomes (i.e., the human chromosomes 1-22) in which a trait manifests in individuals with two pathogenic alleles, either homozygotes (two copies of the same mutant allele) or compound heterozygotes (whereby each copy of a gene has a distinct mutant allele).
 
Monarch Initiative: MONDO:0012185
OMIM®: 609052
Orphanet: ORPHA168555

Definition

The spondylometaphyseal dysplasias are a relatively common, heterogeneous group of disorders characterized by spinal and metaphyseal changes of variable pattern and severity. The classification of spondylometaphyseal dysplasias of Maroteaux and Spranger (1991) was based on changes of the femoral neck and the shape of vertebral anomalies. In this classification, type A4 referred to a form with severe metaphyseal changes of the femoral neck and ovoid, flattened vertebral bodies with anterior tongue-like deformities. [from OMIM]

Clinical features

From HPO
Brachydactyly
MedGen UID:
67454
Concept ID:
C0221357
Congenital Abnormality
Digits that appear disproportionately short compared to the hand/foot. The word brachydactyly is used here to describe a series distinct patterns of shortened digits (brachydactyly types A-E). This is the sense used here.
Coxa valga
MedGen UID:
116080
Concept ID:
C0239137
Finding
Coxa valga is a deformity of the hip in which the angle between the femoral shaft and the femoral neck is increased compared to age-adjusted values (about 150 degrees in newborns gradually reducing to 120-130 degrees in adults).
Irregular patellae
MedGen UID:
324624
Concept ID:
C1836870
Finding
An alteration of the normally relatively smooth margins of the kneecap in radiographic images leading to an irregular contour.
Osteoporotic metatarsal
MedGen UID:
324626
Concept ID:
C1836873
Finding
Decrease in mass and density of the metatarsal bones.
Flat acetabular roof
MedGen UID:
373340
Concept ID:
C1837485
Finding
Flattening of the superior part of the acetabulum, which is a cup-shaped cavity at the base of the hipbone into which the ball-shaped head of the femur fits. The acetabular roof thereby appears horizontal rather than arched, as it normally does.
Sclerotic humeral metaphysis
MedGen UID:
871045
Concept ID:
C4025509
Disease or Syndrome
Severe short stature
MedGen UID:
3931
Concept ID:
C0013336
Disease or Syndrome
A severe degree of short stature, more than -4 SD from the mean corrected for age and sex.
Disproportionate short-limb short stature
MedGen UID:
342370
Concept ID:
C1849937
Finding
A type of disproportionate short stature characterized by a short limbs but an average-sized trunk.
Pectus carinatum
MedGen UID:
57643
Concept ID:
C0158731
Finding
A deformity of the chest caused by overgrowth of the ribs and characterized by protrusion of the sternum.
Dolichocephaly
MedGen UID:
65142
Concept ID:
C0221358
Congenital Abnormality
An abnormality of skull shape characterized by a increased anterior-posterior diameter, i.e., an increased antero-posterior dimension of the skull. Cephalic index less than 76%. Alternatively, an apparently increased antero-posterior length of the head compared to width. Often due to premature closure of the sagittal suture.
Narrow greater sciatic notch
MedGen UID:
154353
Concept ID:
C0566888
Finding
A narrowing of the sacrosciatic notch, i.e., the deep indentation in the posterior border of the hip bone at the point of union of the ilium and ischium.
Broad ischia
MedGen UID:
324622
Concept ID:
C1836868
Finding
Increased width of the ischium, which forms the lower and back part of the hip bone.
Osteoporotic tarsals
MedGen UID:
324625
Concept ID:
C1836872
Finding
Reduction in bone mineral density affecting any or all of the tarsal bones, seven bones of the foot comprising the calcaneus, talus, cuboid, navicular, and the cuneiform bones.
Metaphyseal irregularity
MedGen UID:
325478
Concept ID:
C1838662
Finding
Irregularity of the normally smooth surface of the metaphyses.
Hypoplasia of the capital femoral epiphysis
MedGen UID:
374176
Concept ID:
C1839254
Finding
Underdevelopment of the proximal epiphysis of the femur.
Platyspondyly
MedGen UID:
335010
Concept ID:
C1844704
Finding
A flattened vertebral body shape with reduced distance between the vertebral endplates.
Metaphyseal widening
MedGen UID:
341364
Concept ID:
C1849039
Finding
Abnormal widening of the metaphyseal regions of long bones.
Ovoid vertebral bodies
MedGen UID:
344549
Concept ID:
C1855665
Finding
When viewed in lateral radiographs, vertebral bodies have a roughly rectangular configuration. This term applies if the vertebral body appears rounded or oval.
Enlargement of the costochondral junction
MedGen UID:
346535
Concept ID:
C1857180
Finding
Abnormally increased size of the costochondral junctions, which are located between the distal part of the ribs and the costal cartilages, which are bars of hyaline cartilage that connect the ribs to the sternum.
Metaphyseal sclerosis
MedGen UID:
765440
Concept ID:
C3552526
Finding
Abnormally increased density of metaphyseal bone.
Irregular capital femoral epiphysis
MedGen UID:
866530
Concept ID:
C4020825
Anatomical Abnormality
Irregular surface of the normally relatively smooth capital femoral epiphysis.
Costochondral joint sclerosis
MedGen UID:
867230
Concept ID:
C4021588
Finding
Abnormal increase in density of the tissue at the costochondral junctions.
Spondylometaphyseal dysplasia
MedGen UID:
1674850
Concept ID:
C4759767
Disease or Syndrome
A heterogeneous group of disorders associated with walking and growth disturbances that become evident during the second year of life. Characteristics are platyspondyly (flattened vertebrae) and marked hip and knee metaphyseal lesions. The different forms of spondylometaphyseal dysplasia are distinguished by the localisation and severity of involvement of the affected metaphyses.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSpondylometaphyseal dysplasia, A4 type
Follow this link to review classifications for Spondylometaphyseal dysplasia, A4 type in Orphanet.

Recent clinical studies

Etiology

Verloes A, Lepage P, Baumann C, Maroteaux P, Merrer ML
Am J Med Genet 2002 Dec 15;113(4):362-6. doi: 10.1002/ajmg.b.10738. PMID: 12457408

Diagnosis

Zhang X, Liang H, Liu W, Li X, Zhang W, Shang X
Medicine (Baltimore) 2019 Jul;98(30):e16485. doi: 10.1097/MD.0000000000016485. PMID: 31348255Free PMC Article
Verloes A, Lepage P, Baumann C, Maroteaux P, Merrer ML
Am J Med Genet 2002 Dec 15;113(4):362-6. doi: 10.1002/ajmg.b.10738. PMID: 12457408

Clinical prediction guides

Zhang X, Liang H, Liu W, Li X, Zhang W, Shang X
Medicine (Baltimore) 2019 Jul;98(30):e16485. doi: 10.1097/MD.0000000000016485. PMID: 31348255Free PMC Article

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