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Syndromic recessive X-linked ichthyosis

ORPHA:281090DiseaseX-linked recessiveChildhood

Ассоциированные гены (1)

STS
steroid sulfatase
Role in the phenotype of
OMIM: 300747

Фенотипы (21)

Очень частый (80–99%)3
HP:0000962Hyperkeratosis
HP:0000966Hypohidrosis
HP:0008064Ichthyosis
Частый (30–79%)5
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0002381Aphasia
HP:0007018Attention deficit hyperactivity disorder
HP:0007957Corneal opacity
Периодический (5–29%)13
HP:0000028Cryptorchidism
HP:0000083Renal insufficiency
HP:0000122Unilateral renal agenesis
HP:0000135Hypogonadism
HP:0000717Autism
HP:0001250Seizure
HP:0001339Lissencephaly
HP:0002488Acute leukemia
HP:0002577Abnormal stomach morphology
HP:0004298Abnormality of the abdominal wall
HP:0004322Short stature
HP:0010866Abdominal wall defect
HP:0100617Testicular seminoma

Эпидемиология (1)

Point prevalence
1-9 / 100 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы