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AL amyloidosis

ORPHA:85443DiseaseNot applicableAdult

Фенотипы (55)

Очень частый (80–99%)1
HP:0012378Fatigue
Частый (30–79%)21
HP:0000077Abnormality of the kidney
HP:0000093Proteinuria
HP:0000100Nephrotic syndrome
HP:0001627Abnormal heart morphology
HP:0001639Hypertrophic cardiomyopathy
HP:0001824Weight loss
HP:0002024Malabsorption
HP:0003115Abnormal EKG
HP:0006530Abnormal pulmonary interstitial morphology
HP:0007141Sensorimotor neuropathy
HP:0010702Increased circulating antibody level
HP:0012280Hepatic amyloidosis
HP:0012592Albuminuria
HP:0025077Decreased QRS voltage
HP:0025552Periorbital purpura
HP:0031185Increased circulating NT-proBNP concentration
HP:0031326Monoclonal light chain cardiac amyloidosis
HP:0032613Renal interstitial amyloid deposits
HP:0100639Erectile dysfunction
HP:0410173Increased circulating troponin I concentration
HP:0410174Increased troponin T level in blood
Периодический (5–29%)26
HP:0000083Renal insufficiency
HP:0000217Xerostomia
HP:0000978Bruising susceptibility
HP:0001713Abnormal cardiac ventricle morphology
HP:0001903Anemia
HP:0002094Dyspnea
HP:0002240Hepatomegaly
HP:0002870Obstructive sleep apnea
HP:0003073Hypoalbuminemia
HP:0003155Elevated circulating alkaline phosphatase concentration
HP:0003270Abdominal distention
HP:0005120Abnormal cardiac atrium morphology
HP:0005307Postural hypotension with compensatory tachycardia
HP:0008321Reduced factor X activity
HP:0008652Autonomic erectile dysfunction
HP:0009830Peripheral neuropathy
HP:0010286Abnormal salivary gland morphology
HP:0011675Arrhythmia
HP:0012185Constrictive median neuropathy
HP:0012332Abnormal autonomic nervous system physiology
HP:0012398Peripheral edema
HP:0025389Pulmonary interstitial high-resolution computed tomography abnormality
HP:0030164Jaw claudication
HP:0031246Nonproductive cough
HP:0031595Abnormal P wave
HP:0032550Howell-Jolly bodies
Очень редкий (1–4%)6
HP:0000158Macroglossia
HP:0001609Hoarse voice
HP:0002015Dysphagia
HP:0002239Gastrointestinal hemorrhage
HP:0002578Gastroparesis
HP:0011024Abnormality of the gastrointestinal tract
Исключён (0%)1
HP:0012664Reduced left ventricular ejection fraction

Эпидемиология (81)

Point prevalence
1-9 / 100 000
Germany
Point prevalence
1-9 / 100 000
Worldwide
Annual incidence
1-9 / 100 000
Worldwide
Point prevalence
1-9 / 100 000
Europe
Annual incidence
1-9 / 100 000
Europe
Point prevalence
1-9 / 100 000
Austria
Annual incidence
1-9 / 100 000
Austria
Point prevalence
1-9 / 1 000 000
Belgium
Annual incidence
1-9 / 100 000
Belgium
Point prevalence
1-9 / 100 000
Bulgaria
Annual incidence
1-9 / 100 000
Bulgaria
Point prevalence
1-9 / 100 000
Croatia
Annual incidence
1-9 / 100 000
Croatia
Point prevalence
1-9 / 100 000
Cyprus
Annual incidence
1-9 / 1 000 000
Cyprus
Point prevalence
1-9 / 100 000
Czech Republic
Annual incidence
1-9 / 100 000
Czech Republic
Point prevalence
1-9 / 100 000
Denmark
Annual incidence
1-9 / 100 000
Denmark
Point prevalence
1-9 / 100 000
Estonia
Annual incidence
1-9 / 100 000
Estonia
Point prevalence
1-9 / 100 000
Finland
Annual incidence
1-9 / 100 000
Finland
Point prevalence
1-9 / 100 000
France
Annual incidence
1-9 / 100 000
France
Annual incidence
1-9 / 100 000
Germany
Point prevalence
1-9 / 100 000
Greece
Annual incidence
1-9 / 100 000
Greece
Point prevalence
1-9 / 100 000
Hungary
Annual incidence
1-9 / 100 000
Hungary
Point prevalence
1-9 / 100 000
Iceland
Annual incidence
1-9 / 1 000 000
Iceland
Point prevalence
1-9 / 100 000
Ireland
Annual incidence
1-9 / 1 000 000
Ireland
Point prevalence
1-9 / 100 000
Italy
Annual incidence
1-9 / 100 000
Italy
Point prevalence
1-9 / 100 000
Latvia
Annual incidence
1-9 / 100 000
Latvia
Point prevalence
1-9 / 100 000
Liechtenstein
Annual incidence
1-9 / 100 000
Liechtenstein
Point prevalence
1-9 / 100 000
Lithuania
Annual incidence
1-9 / 100 000
Lithuania
Point prevalence
1-9 / 100 000
Luxembourg
Annual incidence
1-9 / 1 000 000
Luxembourg
Point prevalence
1-9 / 100 000
Malta
Annual incidence
1-9 / 100 000
Malta
Point prevalence
1-9 / 100 000
Netherlands
Annual incidence
1-9 / 100 000
Netherlands
Point prevalence
1-9 / 100 000
Norway
Annual incidence
1-9 / 100 000
Norway
Point prevalence
1-9 / 100 000
Poland
Annual incidence
1-9 / 100 000
Poland
Point prevalence
1-9 / 100 000
Portugal
Annual incidence
1-9 / 100 000
Portugal
Point prevalence
1-9 / 100 000
Romania
Annual incidence
1-9 / 100 000
Romania
Point prevalence
1-9 / 100 000
Slovakia
Annual incidence
1-9 / 1 000 000
Slovakia
Point prevalence
1-9 / 100 000
Slovenia
Annual incidence
1-9 / 100 000
Slovenia
Point prevalence
1-9 / 100 000
Spain
Annual incidence
1-9 / 100 000
Spain
Point prevalence
1-9 / 100 000
Sweden
Annual incidence
1-9 / 100 000
Sweden
Point prevalence
1-9 / 100 000
United Kingdom
Annual incidence
1-9 / 100 000
United Kingdom
Point prevalence
1-9 / 100 000
Brazil
Annual incidence
1-9 / 1 000 000
Brazil
Point prevalence
1-9 / 100 000
Canada
Annual incidence
1-9 / 100 000
Canada
Point prevalence
1-9 / 100 000
Japan
Annual incidence
1-9 / 100 000
Japan
Point prevalence
1-9 / 100 000
Russian Federation
Annual incidence
1-9 / 1 000 000
Russian Federation
Point prevalence
1-9 / 100 000
Korea, Republic of
Annual incidence
1-9 / 1 000 000
Korea, Republic of
Point prevalence
1-9 / 100 000
Taiwan, Province of China
Annual incidence
1-9 / 100 000
Taiwan, Province of China
Point prevalence
1-9 / 100 000
United States
Annual incidence
1-9 / 1 000 000
United States
Annual incidence
1-9 / 1 000 000
Argentina

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы