← Назад

Macrocephaly-intellectual disability-autism syndrome

ORPHA:210548DiseaseAutosomal dominantInfancy, Neonatal

Ассоциированные гены (2)

PTEN
phosphatase and tensin homolog
Disease-causing germline mutation(s) in
OMIM: 601728
HEPACAM
hepatic and glial cell adhesion molecule
Disease-causing germline mutation(s) in
OMIM: 611642

Фенотипы (24)

Очень частый (80–99%)2
HP:0000256Macrocephaly
HP:0000729Autistic behavior
Частый (30–79%)3
HP:0000750Delayed speech and language development
HP:0001249Intellectual disability
HP:0001270Motor delay
Периодический (5–29%)7
HP:0000957Cafe-au-lait spot
HP:0001012Multiple lipomas
HP:0001054Numerous nevi
HP:0001252Hypotonia
HP:0005280Depressed nasal bridge
HP:0031447Penile freckling
HP:0200008Intestinal polyposis
Очень редкий (1–4%)12
HP:0000316Hypertelorism
HP:0001177Preaxial hand polydactyly
HP:0001250Seizure
HP:0001397Hepatic steatosis
HP:0001829Foot polydactyly
HP:0002007Frontal bossing
HP:0002890Thyroid carcinoma
HP:0003196Short nose
HP:0006781Hurthle cell thyroid adenoma
HP:0007018Attention deficit hyperactivity disorder
HP:0011800Midface retrusion
HP:0032203Lymphoid nodular hyperplasia

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