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Hypothyroidism due to TSH receptor mutations

ORPHA:90673DiseaseAutosomal dominant, Autosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

TSHR
thyroid stimulating hormone receptor
Disease-causing germline mutation(s) (loss of function) in
OMIM: 603372

Фенотипы (28)

Облигатный (100%)1
HP:0011789Impaired sensitivity to thyroid stimulating hormone
Очень частый (80–99%)2
HP:0000851Congenital hypothyroidism
HP:0002925Elevated circulating thyroid-stimulating hormone concentration
Частый (30–79%)4
HP:0006579Prolonged neonatal jaundice
HP:0025484Increased circulating thyroglobulin concentration
HP:0031219Reduced radioactive iodine uptake
HP:0031507Decreased circulating thyroxine level
Периодический (5–29%)15
HP:0000158Macroglossia
HP:0001254Lethargy
HP:0001265Hyporeflexia
HP:0001537Umbilical hernia
HP:0001662Bradycardia
HP:0002019Constipation
HP:0003265Neonatal hyperbilirubinemia
HP:0004491Large posterior fontanelle
HP:0005930Abnormality of epiphysis morphology
HP:0005990Thyroid hypoplasia
HP:0008223Compensated hypothyroidism
HP:0008828Delayed proximal femoral epiphyseal ossification
HP:0008872Feeding difficulties in infancy
HP:0025429Abnormal cry
HP:0031220Increased radioactive iodine uptake
Очень редкий (1–4%)3
HP:0000969Edema
HP:0001252Hypotonia
HP:0002045Hypothermia
Исключён (0%)3
HP:0000853Goiter
HP:0011437Maternal autoimmune disease
HP:0030057Autoimmune antibody positivity

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