Phocomelia, Schinzel type

Orpha code: 2879OMIM code: 276820

Definition

Schinzel phocomelia syndrome, also called limb/pelvis hypoplasia/aplasia syndrome, is characterized by skeletal malformations affecting the ulnae, pelvic bones, fibulae and femora. As the phenotype is similar to that described in the malformation syndrome known as Al-Awadi/Raas-Rothschild syndrome, they are thought to be the same disorder.

Disease data
Classification

Malformation syndrome

Synonyms
Al Awadi-Raas-Rothschild syndrome
Aplazja/hipoplazja kończyn i miednicy
Ciężki ubytek kończyn
Wrodzony brak kości łokciowej i strzałkowej
Zespół Al Awadi, Raasa i Rothschilda
Aplasia/hypoplasia of limbs and pelvis
Congenital absence of ulna and fibula
Severe limb deficit
ORPHA code
2879
OMIM code
276820
ICD10 code
Q87.2
ICD11 code
LD2F.1Y

No additional description.

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