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XYLT1-CDG

ORPHA:370930DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

XYLT1
xylosyltransferase 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 608124

Фенотипы (29)

Частый (30–79%)23
HP:0000252Microcephaly
HP:0000343Long philtrum
HP:0000664Synophrys
HP:0000885Broad ribs
HP:0000894Short clavicles
HP:0001027Soft, doughy skin
HP:0001061Acne
HP:0001373Joint dislocation
HP:0001382Joint hypermobility
HP:0001510Growth delay
HP:0001763Pes planus
HP:0002240Hepatomegaly
HP:0002342Intellectual disability, moderate
HP:0002673Coxa valga
HP:0003015Flared metaphysis
HP:0003026Short long bone
HP:0004322Short stature
HP:0005616Accelerated skeletal maturation
HP:0011304Broad thumb
HP:0012471Thick vermilion border
HP:0030084Clinodactyly
HP:0100864Short femoral neck
HP:0500011Moon facies
Периодический (5–29%)6
HP:0000175Cleft palate
HP:0000520Proptosis
HP:0000545Myopia
HP:0001007Hirsutism
HP:0001956Truncal obesity
HP:0004482Relative macrocephaly

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