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Micrognathia-recurrent infections-behavioral abnormalities-mild intellectual disability syndrome

ORPHA:476126Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (1)

TRIO
trio Rho guanine nucleotide exchange factor
Disease-causing germline mutation(s) (loss of function) in
OMIM: 601893

Фенотипы (55)

Очень частый (80–99%)3
HP:0001256Intellectual disability, mild
HP:0001263Global developmental delay
HP:0002465Poor speech
Частый (30–79%)14
HP:0000164Abnormality of the dentition
HP:0000324Facial asymmetry
HP:0000347Micrognathia
HP:0000348High forehead
HP:0000664Synophrys
HP:0000678Dental crowding
HP:0000708Atypical behavior
HP:0000729Autistic behavior
HP:0001182Tapered finger
HP:0002650Scoliosis
HP:0002719Recurrent infections
HP:00046912-3 toe syndactyly
HP:0008872Feeding difficulties in infancy
HP:0011451Congenital microcephaly
Периодический (5–29%)38
HP:0000020Urinary incontinence
HP:0000218High palate
HP:0000286Epicanthus
HP:0000343Long philtrum
HP:0000486Strabismus
HP:0000646Amblyopia
HP:0000706Unerupted tooth
HP:0000718Aggressive behavior
HP:0000722Compulsive behaviors
HP:0000733Abnormal repetitive mannerisms
HP:0000742Self-mutilation
HP:0000767Pectus excavatum
HP:0001155Abnormality of the hand
HP:0001250Seizure
HP:0001328Specific learning disability
HP:0001337Tremor
HP:0001344Absent speech
HP:0001508Failure to thrive
HP:0001674Complete atrioventricular canal defect
HP:0001763Pes planus
HP:0002033Poor suck
HP:0002066Gait ataxia
HP:0002360Sleep abnormality
HP:0002808Kyphosis
HP:0003072Hypercalcemia
HP:0003196Short nose
HP:0004209Clinodactyly of the 5th finger
HP:0004279Short palm
HP:0005484Secondary microcephaly
HP:0006889Intellectual disability, borderline
HP:0007018Attention deficit hyperactivity disorder
HP:0007970Congenital ptosis
HP:0009659Partial absence of thumb
HP:0010035Aplasia of the 1st metacarpal
HP:0011471Gastrostomy tube feeding in infancy
HP:0011908Unilateral radial aplasia
HP:0012450Chronic constipation
HP:0200006Slanting of the palpebral fissure

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