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Oligomeganephronia

MedGen UID:
609100
Concept ID:
C0431694
Disease or Syndrome
Synonyms: oligomeganephronia; Oligomeganephronic hypoplasia; Oligomeganephronic hypoplasia of kidney; Oligomeganephronic renal hypoplasia
SNOMED CT: Oligomeganephronic hypoplasia (18417009); Oligomeganephronia (18417009); Oligomeganephronic hypoplasia of kidney (18417009)
Modes of inheritance:
Non-Mendelian inheritance
MedGen UID:
109109
Concept ID:
C0600599
Genetic Function
Source: Orphanet
A mode of inheritance that depends on genetic determinants in more than one gene.
 
Monarch Initiative: MONDO:0016407
Orphanet: ORPHA2260

Definition

A rare kidney malformation characterized by a reduction of 80% in nephron number and a marked hypertrophy of the glomeruli and tubules. [from ORDO]

Term Hierarchy

Professional guidelines

PubMed

Kalmár T, Turkevi-Nagy S, Bitó L, Kaiser L, Maróti Z, Jakab D, Letoha A, Légrády P, Iványi B
Int J Mol Sci 2023 Dec 14;24(24) doi: 10.3390/ijms242417489. PMID: 38139322Free PMC Article

Recent clinical studies

Etiology

Ren YL, Li Y, Gao J, Zhou XJ, Yang L, Wang SX
BMC Nephrol 2023 Mar 15;24(1):54. doi: 10.1186/s12882-023-03096-3. PMID: 36922764Free PMC Article
Kitakado H, Horinouchi T, Masuda C, Kondo A, Nagai S, Aoto Y, Sakakibara N, Ninchoji T, Yoshikawa N, Nozu K
Pediatr Nephrol 2023 Mar;38(3):757-762. Epub 2022 Jul 21 doi: 10.1007/s00467-022-05687-y. PMID: 35861872
Ishiko S, Horinouchi T, Fujimaru R, Shima Y, Kaito H, Tanaka R, Ishimori S, Kondo A, Nagai S, Aoto Y, Sakakibara N, Nagano C, Yamamura T, Yoshimura M, Nakanishi K, Fujimura J, Kamiyoshi N, Nagase H, Yoshikawa N, Iijima K, Nozu K
Sci Rep 2020 Aug 20;10(1):14026. doi: 10.1038/s41598-020-71101-y. PMID: 32820208Free PMC Article
Bonsib SM
Adv Anat Pathol 2020 Sep;27(5):311-330. doi: 10.1097/PAP.0000000000000269. PMID: 32520748Free PMC Article
Cochat P, Decramer S, Robert-Gnansia E, Dubourg L, Audra P
Transplant Proc 2004 Mar;36(2 Suppl):208S-210S. doi: 10.1016/j.transproceed.2004.01.012. PMID: 15041338

Diagnosis

Hu J, Yang H, Wang X, Ding J, Liao P, Zhu G, Qi C
Am J Med Genet A 2023 Dec;191(12):2850-2855. Epub 2023 Aug 12 doi: 10.1002/ajmg.a.63364. PMID: 37571997
Ren YL, Li Y, Gao J, Zhou XJ, Yang L, Wang SX
BMC Nephrol 2023 Mar 15;24(1):54. doi: 10.1186/s12882-023-03096-3. PMID: 36922764Free PMC Article
Kitakado H, Horinouchi T, Masuda C, Kondo A, Nagai S, Aoto Y, Sakakibara N, Ninchoji T, Yoshikawa N, Nozu K
Pediatr Nephrol 2023 Mar;38(3):757-762. Epub 2022 Jul 21 doi: 10.1007/s00467-022-05687-y. PMID: 35861872
Alves RJ, Oppermann K, Schein LE, Pêgas KL
J Bras Nefrol 2012 Oct-Dec;34(4):392-4. doi: 10.5935/0101-2800.20120030. PMID: 23318829
Schimmenti LA
Eur J Hum Genet 2011 Dec;19(12):1207-12. Epub 2011 Jun 8 doi: 10.1038/ejhg.2011.102. PMID: 21654726Free PMC Article

Therapy

Gatto A, Ferrara P, Leoni C, Onesimo R, Zollino M, Emma F, Zampino G
Am J Med Genet A 2018 Feb;176(2):409-414. Epub 2017 Nov 28 doi: 10.1002/ajmg.a.38554. PMID: 29193639
Cochat P, Decramer S, Robert-Gnansia E, Dubourg L, Audra P
Transplant Proc 2004 Mar;36(2 Suppl):208S-210S. doi: 10.1016/j.transproceed.2004.01.012. PMID: 15041338
Thaisetthawatkul P, Weinstock A, Kerr SL, Cohen ME
J Child Neurol 2001 Nov;16(11):825-31. doi: 10.1177/08830738010160110801. PMID: 11732768

Prognosis

Hu J, Yang H, Wang X, Ding J, Liao P, Zhu G, Qi C
Am J Med Genet A 2023 Dec;191(12):2850-2855. Epub 2023 Aug 12 doi: 10.1002/ajmg.a.63364. PMID: 37571997
Bonsib SM
Adv Anat Pathol 2020 Sep;27(5):311-330. doi: 10.1097/PAP.0000000000000269. PMID: 32520748Free PMC Article
Brenner BM, Garcia DL, Anderson S
Am J Hypertens 1988 Oct;1(4 Pt 1):335-47. doi: 10.1093/ajh/1.4.335. PMID: 3063284
Kusuyama Y, Tsukino R, Oomori H, Kuribayashi K, Katayama H, Koike M, Saito K
Acta Pathol Jpn 1985 Mar;35(2):449-57. doi: 10.1111/j.1440-1827.1985.tb00587.x. PMID: 4024943

Clinical prediction guides

Hu J, Yang H, Wang X, Ding J, Liao P, Zhu G, Qi C
Am J Med Genet A 2023 Dec;191(12):2850-2855. Epub 2023 Aug 12 doi: 10.1002/ajmg.a.63364. PMID: 37571997
Ren YL, Li Y, Gao J, Zhou XJ, Yang L, Wang SX
BMC Nephrol 2023 Mar 15;24(1):54. doi: 10.1186/s12882-023-03096-3. PMID: 36922764Free PMC Article
Kitakado H, Horinouchi T, Masuda C, Kondo A, Nagai S, Aoto Y, Sakakibara N, Ninchoji T, Yoshikawa N, Nozu K
Pediatr Nephrol 2023 Mar;38(3):757-762. Epub 2022 Jul 21 doi: 10.1007/s00467-022-05687-y. PMID: 35861872
Gatto A, Ferrara P, Leoni C, Onesimo R, Zollino M, Emma F, Zampino G
Am J Med Genet A 2018 Feb;176(2):409-414. Epub 2017 Nov 28 doi: 10.1002/ajmg.a.38554. PMID: 29193639
Brenner BM, Garcia DL, Anderson S
Am J Hypertens 1988 Oct;1(4 Pt 1):335-47. doi: 10.1093/ajh/1.4.335. PMID: 3063284

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