Houston-Harris sub type
Achondrogenesis type Ia, also known as the Houston-Harris subtype, is a subtype of achondrogenesis. It is an extremely rare lethal skeletal dysplasia (chondrodysplasia) with a characteristic severe disarrangement of endochondral ossification.
Clinical presentation
Some of the common clinical features include:
- low birth weight
- skeletal abnormalities
- very short limbs
- short neck
- incomplete ossification of bones - particularly the skull
- congenital cardiac anomalies
- breathing problems
- small chest
- underdeveloped lungs
- protuberant abdomen
Pathology
The growth plate cartilage completely lacks columnar-zone formation and shows chondrocyte expansion due to intracellular vacuoles.
See also
- achondrogenesis
- type I:
- type Ia: Houston-Harris subtype
- type Ib: Parenti-Fraccaro subtype
- type II: Langer-Saldino syndrome
- type I:
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