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Carpenter syndrome

ORPHA:65759Malformation syndromeAutosomal recessiveAntenatal, Childhood, Neonatal

Ассоциированные гены (2)

MEGF8
multiple EGF like domains 8
Disease-causing germline mutation(s) in
OMIM: 604267
RAB23
RAB23, member RAS oncogene family
Disease-causing germline mutation(s) (loss of function) in
OMIM: 606144

Фенотипы (40)

Облигатный (100%)3
HP:0001156Brachydactyly
HP:0001770Toe syndactyly
HP:0006101Finger syndactyly
Очень частый (80–99%)18
HP:0000028Cryptorchidism
HP:0000098Tall stature
HP:0000256Macrocephaly
HP:0000263Oxycephaly
HP:0000275Narrow face
HP:0000286Epicanthus
HP:0000316Hypertelorism
HP:0000929Abnormal skull morphology
HP:0001159Syndactyly
HP:0001249Intellectual disability
HP:0001357Plagiocephaly
HP:0001363Craniosynostosis
HP:0001513Obesity
HP:0003241External genital hypoplasia
HP:0004209Clinodactyly of the 5th finger
HP:0004279Short palm
HP:0005487Prominent metopic ridge
HP:0010442Polydactyly
Частый (30–79%)12
HP:0000262Turricephaly
HP:0000445Wide nose
HP:0000457Depressed nasal ridge
HP:0000481Abnormal cornea morphology
HP:0000486Strabismus
HP:0001162Postaxial hand polydactyly
HP:0001841Preaxial foot polydactyly
HP:0002676Cloverleaf skull
HP:0002857Genu valgum
HP:0011304Broad thumb
HP:0012243Abnormal reproductive system morphology
HP:0030680Abnormal cardiovascular system morphology
Периодический (5–29%)7
HP:0001537Umbilical hernia
HP:0001643Patent ductus arteriosus
HP:0001748Polysplenia
HP:0001762Talipes equinovarus
HP:0002751Kyphoscoliosis
HP:0010044Short 4th metacarpal
HP:0100490Camptodactyly of finger

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы