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Scalp-ear-nipple syndrome

ORPHA:2036Malformation syndromeAutosomal dominantNeonatal

Ассоциированные гены (1)

KCTD1
potassium channel tetramerization domain containing 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613420

Фенотипы (25)

Очень частый (80–99%)10
HP:0000385Small earlobe
HP:0000951Abnormality of the skin
HP:0001965Abnormality of the scalp
HP:0006709Aplasia/Hypoplasia of the nipples
HP:0008070Sparse hair
HP:0008551Microtia
HP:0009738Abnormality of the antihelix
HP:0011251Underdeveloped antitragus
HP:0011272Underdeveloped tragus
HP:0100783Breast aplasia
Частый (30–79%)9
HP:0000010Recurrent urinary tract infections
HP:0000164Abnormality of the dentition
HP:0000506Telecanthus
HP:0000518Cataract
HP:0000684Delayed eruption of teeth
HP:0000822Hypertension
HP:0001231Abnormal fingernail morphology
HP:0100540Palpebral edema
HP:0100651Type I diabetes mellitus
Периодический (5–29%)6
HP:0000073Ureteral duplication
HP:0000077Abnormality of the kidney
HP:0000625Eyelid coloboma
HP:0000966Hypohidrosis
HP:0005580Duplication of renal pelvis
HP:0012330Pyelonephritis

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