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Isolated osteopoikilosis

ORPHA:166119DiseaseAutosomal dominantAdolescent, Adult, Childhood

Ассоциированные гены (1)

LEMD3
LEM domain containing 3
Disease-causing germline mutation(s) (loss of function) in
OMIM: 607844

Фенотипы (33)

Очень частый (80–99%)3
HP:0011001Increased bone mineral density
HP:0011849Abnormal bone ossification
HP:0040068Abnormality of limb bone
Частый (30–79%)6
HP:0004289Sclerotic foci in hand bones
HP:0009106Abnormal pelvis bone ossification
HP:0011314Abnormality of long bone morphology
HP:0040163Abnormal pelvis bone morphology
HP:0100774Hyperostosis
HP:0100925Sclerosis of foot bone
Периодический (5–29%)15
HP:0000620Dacryocystitis
HP:0001376Limitation of joint mobility
HP:0001387Joint stiffness
HP:0001474Sclerotic scapulae
HP:0002653Bone pain
HP:0002823Abnormality of femur morphology
HP:0002829Arthralgia
HP:0004240Sclerotic foci within carpal bones
HP:0012758Neurodevelopmental delay
HP:0030838Hip pain
HP:0030840Ankle pain
HP:0030955Alcoholism
HP:0031051Tarsal sclerosis
HP:0032148Episodic pain
HP:0100569Abnormally ossified vertebrae
Очень редкий (1–4%)9
HP:0000077Abnormality of the kidney
HP:0000175Cleft palate
HP:0000818Abnormality of the endocrine system
HP:0001159Syndactyly
HP:0001627Abnormal heart morphology
HP:0002960Autoimmunity
HP:0007417Discoid lupus rash
HP:0010562Keloids
HP:0100324Scleroderma

Эпидемиология (2)

Point prevalence
1-9 / 100 000
Worldwide
Annual incidence
1-9 / 100 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы