← Назад

PHIP-related behavioral problems-intellectual disability-obesity-dysmorphic features syndrome

ORPHA:589905DiseaseAutosomal dominantAntenatal, Infancy, Neonatal

Ассоциированные гены (1)

PHIP
PHIP subunit of CUL4-Ring ligase complex
Disease-causing germline mutation(s) in
OMIM: 612870

Фенотипы (38)

Очень частый (80–99%)3
HP:0000400Macrotia
HP:0001249Intellectual disability
HP:0012758Neurodevelopmental delay
Частый (30–79%)18
HP:0000233Thin vermilion border
HP:0000316Hypertelorism
HP:0000343Long philtrum
HP:0000348High forehead
HP:0000455Broad nasal tip
HP:0000463Anteverted nares
HP:0000539Abnormality of refraction
HP:0000540Hypermetropia
HP:0000664Synophrys
HP:0000957Cafe-au-lait spot
HP:0001182Tapered finger
HP:0001252Hypotonia
HP:0004209Clinodactyly of the 5th finger
HP:0004324Increased body weight
HP:0007018Attention deficit hyperactivity disorder
HP:0008872Feeding difficulties in infancy
HP:0012378Fatigue
HP:0100710Impulsivity
Периодический (5–29%)17
HP:0000028Cryptorchidism
HP:0000286Epicanthus
HP:0000403Recurrent otitis media
HP:0000486Strabismus
HP:0000508Ptosis
HP:0000582Upslanted palpebral fissure
HP:0001250Seizure
HP:0001288Gait disturbance
HP:0001319Neonatal hypotonia
HP:0002019Constipation
HP:0002020Gastroesophageal reflux
HP:0002360Sleep abnormality
HP:0002378Hand tremor
HP:0002761Generalized joint laxity
HP:0002788Recurrent upper respiratory tract infections
HP:00046912-3 toe syndactyly
HP:0007874Almond-shaped 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