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Isolated exencephaly

ORPHA:563612Clinical subtypeAntenatal

Ассоциированные гены (2)

VANGL2
VANGL planar cell polarity protein 2
Major susceptibility factor in
OMIM: 600533
MTHFR
methylenetetrahydrofolate reductase
Major susceptibility factor in
OMIM: 607093

Фенотипы (16)

Частый (30–79%)9
HP:0000520Proptosis
HP:0000929Abnormal skull morphology
HP:0001274Agenesis of corpus callosum
HP:0001360Holoprosencephaly
HP:0001561Polyhydramnios
HP:0002683Abnormality of the calvaria
HP:0005466Hypoplasia of the frontal bone
HP:0007360Aplasia/Hypoplasia of the cerebellum
HP:0012443Abnormality of brain morphology
Периодический (5–29%)7
HP:0000369Low-set ears
HP:0005280Depressed nasal bridge
HP:0009800Maternal diabetes
HP:0010627Anterior pituitary hypoplasia
HP:0011756Posterior pituitary agenesis
HP:0011821Abnormality of facial skeleton
HP:0030244Maternal fever in pregnancy

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы