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X-linked intellectual disability-psychosis-macroorchidism syndrome

ORPHA:3077Malformation syndromeX-linked dominantChildhood

Ассоциированные гены (1)

MECP2
methyl-CpG binding protein 2
Disease-causing germline mutation(s) in
OMIM: 300005

Фенотипы (27)

Очень частый (80–99%)7
HP:0006801Hyperactive deep tendon reflexes
HP:0000053Macroorchidism
HP:0000718Aggressive behavior
HP:0000737Irritability
HP:0000752Hyperactivity
HP:0001250Seizure
HP:0002360Sleep abnormality
Частый (30–79%)11
HP:0001337Tremor
HP:0001635Congestive heart failure
HP:0002039Anorexia
HP:0002061Lower limb spasticity
HP:0002342Intellectual disability, moderate
HP:0002395Lower limb hyperreflexia
HP:0004322Short stature
HP:0007302Bipolar affective disorder
HP:0010864Intellectual disability, severe
HP:0011188Focal EEG discharges with secondary generalization
HP:0000718Aggressive behavior
Периодический (5–29%)9
HP:0001297Stroke
HP:0001300Parkinsonism
HP:0001513Obesity
HP:0002136Broad-based gait
HP:0002322Resting tremor
HP:0002362Shuffling gait
HP:0002751Kyphoscoliosis
HP:0025403Stooped posture
HP:0100852Abnormal fear/anxiety-related behavior

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