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Renal hypoplasia, bilateral

ORPHA:97362Clinical subtypeAutosomal dominant, Not applicableAll ages

Ассоциированные гены (2)

PAX2
paired box 2
Major susceptibility factor in
OMIM: 167409
PBX1
PBX homeobox 1
Role in the phenotype of
OMIM: 176310

Фенотипы (31)

Очень частый (80–99%)1
HP:0000089Renal hypoplasia
Частый (30–79%)9
HP:0000076Vesicoureteral reflux
HP:0000093Proteinuria
HP:0000107Renal cyst
HP:0000822Hypertension
HP:0001562Oligohydramnios
HP:0002009Potter facies
HP:0011968Feeding difficulties
HP:0012213Decreased glomerular filtration rate
HP:0012622Chronic kidney disease
Периодический (5–29%)21
HP:0000028Cryptorchidism
HP:0000483Astigmatism
HP:0000540Hypermetropia
HP:0000639Nystagmus
HP:0000969Edema
HP:0001254Lethargy
HP:0001508Failure to thrive
HP:0001510Growth delay
HP:0001518Small for gestational age
HP:0001622Premature birth
HP:0001903Anemia
HP:0001942Metabolic acidosis
HP:0002153Hyperkalemia
HP:0002643Neonatal respiratory distress
HP:0002902Hyponatremia
HP:0002907Microscopic hematuria
HP:0003076Glycosuria
HP:0004322Short stature
HP:0012758Neurodevelopmental delay
HP:0025466Beta 2-microglobulinuria
HP:0100520Oliguria

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