← Назад

Autosomal recessive methemoglobinemia

ORPHA:621DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (2)

CYB5R3
cytochrome b5 reductase 3
Disease-causing germline mutation(s) in
OMIM: 613213
CYB5A
cytochrome b5 type A
Candidate gene tested in
OMIM: 613218

Фенотипы (26)

Очень частый (80–99%)2
HP:0000961Cyanosis
HP:0012119Methemoglobinemia
Частый (30–79%)4
HP:0000707Abnormality of the nervous system
HP:0001597Abnormality of the nail
HP:0002875Exertional dyspnea
HP:0025118Lip discoloration
Периодический (5–29%)15
HP:0000252Microcephaly
HP:0000565Esotropia
HP:0000592Blue sclerae
HP:0001257Spasticity
HP:0001263Global developmental delay
HP:0001272Cerebellar atrophy
HP:0002283Global brain atrophy
HP:0002305Athetosis
HP:0002451Limb dystonia
HP:0002510Spastic tetraplegia
HP:0006808Cerebral hypomyelination
HP:0006913Frontal cortical atrophy
HP:0007112Temporal cortical atrophy
HP:0010864Intellectual disability, severe
HP:0011344Severe global developmental delay
Очень редкий (1–4%)5
HP:0001250Seizure
HP:0001276Hypertonia
HP:0001518Small for gestational age
HP:0012448Delayed myelination
HP:0012697Small basal ganglia

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