Ivemark syndrome

Ivemark syndrome is an autosomal recessive ciliopathy characterized by renal, hepatic, and pancreatic involvement.

Systematic

Congenital abnormalities of the kidney and urinary tract
	Acro-renal-ocular syndrome
	Aplasia of lacrimal and salivary glands
	Autosomal dominant Robinow syndrome 1
	Autosomal recessive Robinow syndrome
	BMP7
	BNAR syndrome
	Brain malformations with urinary tract defects
	Branchio-oculo-facial syndrome
	Branchiootic syndrome
	Branchiootorenal dysplasia
	CHARGE syndrome
	CHD1L
	Congenital anomalies of kidney and urinary tract 1
	Congenital anomalies of kidney and urinary tract 2
	Congenital hypogonadotropic hypogonadism with anosmia 1
	Congenital hypogonadotropic hypogonadism without anosmia 5
	Denys-Drash syndrome
	Fraser syndrome
	Frasier syndrome
	Goldberg-Shprintzen syndrome
	IVIC syndrome
	Ivemark syndrome
		Renal-hepatic-pancreatic dysplasia 1
			NPHP3
		Renal-hepatic-pancreatic dysplasia 2
			NEK8
	Kabuki syndrome
	Lacrimoauriculodentodigital syndrome
	Mowat-Wilson syndrome
	Papillorenal syndrome
	Renal cysts and diabetes (RCAD)
	Renal dysplasia with hypopituitarism and diabetes
	Renal hypodysplasia/aplasia
	Renal tubular dysgenesis
	SERKAL syndrome
	Simpson-Golabi-Behmel syndrome
	Smith-Lemli-Opitz syndrome
	Somatic nephroblastoma
	Susceptibility to cystic renal dysplasia
	Syndromic microphthalmia 6
	Urofacial syndrome
	Vesicoureteral reflux
	WAGR syndrome

References:

1.	Orphanet article Orphanet ID 97548

2.	Wikipedia article Wikipedia EN (Asplenia_with_cardiovascular_anomalies)

Update: June 23, 2025

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