Bone dysplasia, lethal Holmgren type

Orpha code: 1842OMIM code: 211120

Definition

A rare lethal bone dysplasia characterized at birth by low birth weight, a rhizomelic dwarfism, bent femora and short chest producing asphyxia. The initial cases could have been diagnosed as Desbuquois syndrome, or a recessive Larsen syndrome. There has been no further description in the literature since 1988.

Disease data
Classification

Malformation syndrome

Synonyms
Autosomal recessive lethal chondrodysplasia, round femoral inferior epiphysis type
Autosomalna recesywna śmiertelna chondrodysplazja, typ okrągłe nasady kości udowych
ORPHA code
1842
OMIM code
211120
ICD10 code
Q77.8
ICD11 code
-

No additional description.

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