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Charcot-Marie-Tooth disease type 4G

ORPHA:99953DiseaseAutosomal recessiveAdolescent, Childhood

Ассоциированные гены (1)

HK1
hexokinase 1
Disease-causing germline mutation(s) in
OMIM: 142600

Фенотипы (27)

Очень частый (80–99%)12
HP:0001284Areflexia
HP:0001760Abnormal foot morphology
HP:0002495Impaired vibratory sensation
HP:0002936Distal sensory impairment
HP:0003409Distal sensory impairment of all modalities
HP:0003431Decreased motor nerve conduction velocity
HP:0003477Peripheral axonal neuropathy
HP:0007108Demyelinating peripheral neuropathy
HP:0007230Decreased distal sensory nerve action potential
HP:0009053Distal lower limb muscle weakness
HP:0011096Peripheral demyelination
HP:0012078Motor conduction block
Частый (30–79%)10
HP:0001288Gait disturbance
HP:0010830Impaired tactile sensation
HP:0001155Abnormality of the hand
HP:0001761Pes cavus
HP:0001762Talipes equinovarus
HP:0003693Distal amyotrophy
HP:0003701Proximal muscle weakness
HP:0007210Lower limb amyotrophy
HP:0008959Distal upper limb muscle weakness
HP:0009129Upper limb amyotrophy
Периодический (5–29%)5
HP:0002141Gait imbalance
HP:0002505Loss of ambulation
HP:0002650Scoliosis
HP:0007328Impaired pain sensation
HP:0008081Pes valgus

Эпидемиология (2)

Point prevalence
Unknown
Worldwide
Point prevalence
1-5 / 10 000
Czech Republic

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы