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Multiple benign circumferential skin creases on limbs

ORPHA:2505DiseaseAutosomal dominant, Autosomal recessiveInfancy, Neonatal

Ассоциированные гены (2)

MAPRE2
microtubule associated protein RP/EB family member 2
Disease-causing germline mutation(s) in
OMIM: 605789
TUBB
tubulin beta class I
Disease-causing germline mutation(s) in
OMIM: 191130

Фенотипы (30)

Очень частый (80–99%)3
HP:0000969Edema
HP:0001072Thickened skin
HP:0007522Increased number of skin folds
Частый (30–79%)2
HP:0000175Cleft palate
HP:0007400Irregular hyperpigmentation
Периодический (5–29%)25
HP:0000358Posteriorly rotated ears
HP:0000377Abnormal pinna morphology
HP:0000023Inguinal hernia
HP:0000028Cryptorchidism
HP:0000045Abnormality of the scrotum
HP:0000046Small scrotum
HP:0000047Hypospadias
HP:0000252Microcephaly
HP:0000271Abnormality of the face
HP:0000286Epicanthus
HP:0000343Long philtrum
HP:0000347Micrognathia
HP:0000482Microcornea
HP:0000488Retinopathy
HP:0000568Microphthalmia
HP:0001249Intellectual disability
HP:0001263Global developmental delay
HP:0001537Umbilical hernia
HP:0001635Congestive heart failure
HP:0002230Generalized hirsutism
HP:0003011Abnormality of the musculature
HP:0004322Short stature
HP:0006768Localized neuroblastoma
HP:0100559Lower limb asymmetry
HP:0100560Upper limb asymmetry

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