← Назад

Dihydropyrimidinuria

ORPHA:38874DiseaseAutosomal recessiveChildhood, Infancy

Ассоциированные гены (1)

DPYS
dihydropyrimidinase
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613326

Фенотипы (20)

Частый (30–79%)6
HP:0001249Intellectual disability
HP:0001250Seizure
HP:0012127Uraciluria
HP:6000118Elevated urinary dihydrouracil level
HP:6000119Elevated urinary dihydrothymine level
HP:6000331Elevated urinary thymine level
Периодический (5–29%)13
HP:0000252Microcephaly
HP:0000256Macrocephaly
HP:0000280Coarse facial features
HP:0000717Autism
HP:0001252Hypotonia
HP:0001508Failure to thrive
HP:0001510Growth delay
HP:0002013Vomiting
HP:0002020Gastroesophageal reflux
HP:0007018Attention deficit hyperactivity disorder
HP:0011473Villous atrophy
HP:0011968Feeding difficulties
HP:0012758Neurodevelopmental delay
Очень редкий (1–4%)1
HP:0002376Developmental regression

Эпидемиология (1)

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