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Linear nevus sebaceus syndrome

ORPHA:2612DiseaseNot applicableChildhood, Infancy, Neonatal

Ассоциированные гены (3)

HRAS
HRas proto-oncogene, GTPase
Disease-causing somatic mutation(s) in
OMIM: 190020
KRAS
KRAS proto-oncogene, GTPase
Disease-causing somatic mutation(s) in
OMIM: 190070
NRAS
NRAS proto-oncogene, GTPase
Candidate gene tested in
OMIM: 164790

Фенотипы (31)

Очень частый (80–99%)21
HP:0000269Prominent occiput
HP:0000506Telecanthus
HP:0000568Microphthalmia
HP:0000612Iris coloboma
HP:0000995Melanocytic nevus
HP:0001048Cavernous hemangioma
HP:0001249Intellectual disability
HP:0001250Seizure
HP:0001252Hypotonia
HP:0001315Reduced tendon reflexes
HP:0001347Hyperreflexia
HP:0001596Alopecia
HP:0002007Frontal bossing
HP:0002119Ventriculomegaly
HP:0002353EEG abnormality
HP:0002816Genu recurvatum
HP:0003422Vertebral segmentation defect
HP:0004422Biparietal narrowing
HP:0007360Aplasia/Hypoplasia of the cerebellum
HP:0009720Adenoma sebaceum
HP:0100555Asymmetric growth
Частый (30–79%)6
HP:0000324Facial asymmetry
HP:0000478Abnormality of the eye
HP:0000504Abnormality of vision
HP:0001357Plagiocephaly
HP:0002132Porencephaly
HP:0007400Irregular hyperpigmentation
Периодический (5–29%)4
HP:0001305Dandy-Walker malformation
HP:0001510Growth delay
HP:0002514Cerebral calcification
HP:0007370Aplasia/Hypoplasia of the corpus callosum

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы