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Short stature-delayed bone age due to thyroid hormone metabolism deficiency

ORPHA:171706DiseaseAutosomal recessiveChildhood, Infancy

Ассоциированные гены (1)

SECISBP2
SECIS binding protein 2
Disease-causing germline mutation(s) in
OMIM: 607693

Фенотипы (18)

Очень частый (80–99%)1
HP:0012379Abnormal enzyme/coenzyme activity
Частый (30–79%)3
HP:0002925Elevated circulating thyroid-stimulating hormone concentration
HP:0031506Increased circulating thyroxine level
HP:0032210Decreased circulating free T3
Периодический (5–29%)14
HP:0000508Ptosis
HP:0000736Short attention span
HP:0001249Intellectual disability
HP:0001510Growth delay
HP:0001513Obesity
HP:0002750Delayed skeletal maturation
HP:0003162Fasting hypoglycemia
HP:0003391Gowers sign
HP:0004322Short stature
HP:0008994Proximal muscle weakness in lower limbs
HP:0009053Distal lower limb muscle weakness
HP:0012548Fatty replacement of skeletal muscle
HP:0031903Abnormal circulating selenium concentration
HP:0040214Abnormal circulating insulin concentration

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы