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Multiple endocrine neoplasia type 4

ORPHA:276152DiseaseAutosomal dominant, Not applicableAdult

Ассоциированные гены (1)

CDKN1B
cyclin dependent kinase inhibitor 1B
Disease-causing germline mutation(s) (loss of function) in
OMIM: 600778

Фенотипы (39)

Очень частый (80–99%)6
HP:0000818Abnormality of the endocrine system
HP:0000843Hyperparathyroidism
HP:0002897Parathyroid adenoma
HP:0003072Hypercalcemia
HP:0003165Elevated circulating parathyroid hormone level
HP:0008208Parathyroid hyperplasia
Частый (30–79%)21
HP:0000825Hyperinsulinemic hypoglycemia
HP:0000845Elevated circulating growth hormone concentration
HP:0000854Thyroid adenoma
HP:0001031Subcutaneous lipoma
HP:0002014Diarrhea
HP:0002044Zollinger-Ellison syndrome
HP:0002574Episodic abdominal pain
HP:0002893Pituitary adenoma
HP:0004398Peptic ulcer
HP:0006767Pituitary prolactin cell adenoma
HP:0006772Renal angiomyolipoma
HP:0008256Adrenocortical adenoma
HP:0008283Fasting hyperinsulinemia
HP:0010615Angiofibromas
HP:0011760Pituitary growth hormone cell adenoma
HP:0011761Pituitary null cell adenoma
HP:0012091Abnormality of pancreas physiology
HP:0012197Insulinoma
HP:0030445Pulmonary carcinoid tumor
HP:0100633Esophagitis
HP:0100634Neuroendocrine neoplasm
Периодический (5–29%)11
HP:0003118Increased circulating cortisol level
HP:0006780Parathyroid carcinoma
HP:0007449Confetti-like hypopigmented macules
HP:0008291Pituitary corticotropic cell adenoma
HP:0010783Erythema
HP:0010788Testicular neoplasm
HP:0012030Increased urinary cortisol level
HP:0012334Extrahepatic cholestasis
HP:0030079Cervix cancer
HP:0030688Increased glucagon level
HP:0100570Carcinoid tumor
Очень редкий (1–4%)1
HP:0100522Thymoma

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