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2p21 microdeletion syndrome

ORPHA:163693DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (4)

SLC3A1
solute carrier family 3 member 1
Role in the phenotype of
OMIM: 104614
PREPL
prolyl endopeptidase like
Role in the phenotype of
OMIM: 609557
CAMKMT
calmodulin-lysine N-methyltransferase
Role in the phenotype of
OMIM: 609559
PPM1B
protein phosphatase, Mg2+/Mn2+ dependent 1B
Role in the phenotype of
OMIM: 603770

Фенотипы (19)

Очень частый (80–99%)14
HP:0000135Hypogonadism
HP:0000527Long eyelashes
HP:0000787Nephrolithiasis
HP:0001252Hypotonia
HP:0001263Global developmental delay
HP:0001508Failure to thrive
HP:0001510Growth delay
HP:0001611Hypernasal speech
HP:0002007Frontal bossing
HP:0002342Intellectual disability, moderate
HP:0003131Cystinuria
HP:0005280Depressed nasal bridge
HP:0200125Mitochondrial respiratory chain defects
HP:0000358Posteriorly rotated ears
Частый (30–79%)3
HP:0001250Seizure
HP:0002901Hypocalcemia
HP:0003128Lactic acidosis
Периодический (5–29%)2
HP:0001558Decreased fetal movement
HP:0001943Hypoglycemia

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