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Hydrolethalus

ORPHA:2189Malformation syndromeAutosomal recessiveAntenatal, Neonatal

Ассоциированные гены (2)

HYLS1
HYLS1 centriolar and ciliogenesis associated
Disease-causing germline mutation(s) in
OMIM: 610693
KIF7
kinesin family member 7
Disease-causing germline mutation(s) in
OMIM: 611254

Фенотипы (28)

Очень частый (80–99%)8
HP:0000238Hydrocephalus
HP:0000278Retrognathia
HP:0000347Micrognathia
HP:0001162Postaxial hand polydactyly
HP:0001274Agenesis of corpus callosum
HP:0001331Absent septum pellucidum
HP:0001561Polyhydramnios
HP:0001622Premature birth
Частый (30–79%)13
HP:0000358Posteriorly rotated ears
HP:0000175Cleft palate
HP:0000176Submucous cleft hard palate
HP:0000193Bifid uvula
HP:0000369Low-set ears
HP:0000490Deeply set eye
HP:0001601Laryngomalacia
HP:0002086Abnormality of the respiratory system
HP:0004408Abnormality of the sense of smell
HP:0030680Abnormal cardiovascular system morphology
HP:0030690Gingival cleft
HP:0100333Unilateral cleft lip
HP:0100682Tracheal atresia
Периодический (5–29%)7
HP:0000028Cryptorchidism
HP:0000528Anophthalmia
HP:0000568Microphthalmia
HP:0002139Arrhinencephaly
HP:0002323Anencephaly
HP:0002983Micromelia
HP:0011027Abnormality of the fallopian tube

Эпидемиология (3)

Prevalence at birth
1-9 / 100 000
Finland
Point prevalence
<1 / 1 000 000
Finland
Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы