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Variant ABeta2M amyloidosis

ORPHA:314652DiseaseAutosomal dominantAdult

Ассоциированные гены (1)

B2M
beta-2-microglobulin
Disease-causing germline mutation(s) in
OMIM: 109700

Фенотипы (24)

Частый (30–79%)3
HP:0001917Renal amyloidosis
HP:0012185Constrictive median neuropathy
HP:0012622Chronic kidney disease
Периодический (5–29%)21
HP:0000157Abnormality of the tongue
HP:0002176Spinal cord compression
HP:0002756Pathologic fracture
HP:0003365Arthralgia of the hip
HP:0005244Gastrointestinal infarctions
HP:0007141Sensorimotor neuropathy
HP:0010286Abnormal salivary gland morphology
HP:0011805Abnormal skeletal muscle morphology
HP:0011915Cardiovascular calcification
HP:0012065Multiple bony cystic lesions
HP:0012280Hepatic amyloidosis
HP:0012309Cutaneous amyloidosis
HP:0012332Abnormal autonomic nervous system physiology
HP:0012664Reduced left ventricular ejection fraction
HP:0025015Abnormal vascular morphology
HP:0030834Shoulder pain
HP:0030836Wrist pain
HP:0030839Knee pain
HP:0030843Cardiac amyloidosis
HP:0031368Intestinal perforation
HP:0100292Amyloidosis of peripheral nerves

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