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Congenital Renal Hypoplasia

MedGen UID:
884131
•Concept ID:
C4053762
•
Disease or Syndrome

Definition

The underdevelopment of otherwise normal renal parenchyma, characterized by decreased nephron size and possibly a decreased number of nephrons at birth. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital Renal Hypoplasia

Professional guidelines

PubMed

Management of the congenital solitary kidney: consensus recommendations of the Italian Society of Pediatric Nephrology.
La Scola C, Ammenti A, Bertulli C, Bodria M, Brugnara M, Camilla R, Capone V, Casadio L, Chimenz R, Conte ML, Conversano E, Corrado C, Guarino S, Luongo I, Marsciani M, Marzuillo P, Meneghesso D, Pennesi M, Pugliese F, Pusceddu S, Ravaioli E, Taroni F, Vergine G, Peruzzi L, Montini G
Pediatr Nephrol 2022 Sep;37(9):2185-2207. Epub 2022 Jun 17 doi: 10.1007/s00467-022-05528-y. PMID: 35713730Free PMC Article
Genotype-phenotype associations in Fanconi anemia: A literature review.
Fiesco-Roa MO, Giri N, McReynolds LJ, Best AF, Alter BP
Blood Rev 2019 Sep;37:100589. Epub 2019 Jul 16 doi: 10.1016/j.blre.2019.100589. PMID: 31351673Free PMC Article
Turner syndrome: diagnosis and management.
Morgan T
Am Fam Physician 2007 Aug 1;76(3):405-10. PMID: 17708142

Recent clinical studies

Etiology

Can postpyelonephritic renal scarring be prevented?
Nevéus T
Pediatr Nephrol 2013 Feb;28(2):187-90. Epub 2012 Oct 20 doi: 10.1007/s00467-012-2334-2. PMID: 23086593
Diagnosis and complications of renovascular hypertension in children: literature data and clinical observations.
Burnei G, Burnei A, Hodorogea D, Drăghici I, Georgescu I, Vlad C, Gavriliu S
J Med Life 2009 Jan-Mar;2(1):18-28. PMID: 20108487Free PMC Article
Pediatric renal transplantation: 13 years of experience--report from the Chilean Cooperative Multicenter Group.
Rosati P, Pinto V, Delucchi A, Salas P, Cano F, Zambrano P, Lagos E, Rodriguez E, Hevia P, Ramirez K, Quiero X, Azócar M, Rodriguez S, Aguiló J, Varela M, Ferrario M, Ramirez R, Palacios JM, Turu I, Jimenez O, Godoy J, Gaete J, Maluenda X, Villegas R; Chilean Cooperative Multicenter Group
Transplant Proc 2005 Apr;37(3):1569-73. doi: 10.1016/j.transproceed.2004.09.013. PMID: 15866676
Severe vesicoureteral reflux and chronic renal failure: a condition peculiar to male gender? Data from the ItalKid Project.
Marra G, Oppezzo C, Ardissino G, Daccò V, Testa S, Avolio L, Taioli E, Sereni F; ItalKid Project
J Pediatr 2004 May;144(5):677-81. doi: 10.1016/j.jpeds.2004.01.043. PMID: 15127014
Nutritional intervention and growth in children with chronic renal failure.
Reed EE, Roy LP, Gaskin KJ, Knight JF
J Ren Nutr 1998 Jul;8(3):122-6. doi: 10.1016/s1051-2276(98)90002-4. PMID: 9724500

Diagnosis

A novel pathogenic variant c.262delA in PBX1 causing oligomeganephronia identified using whole-exome sequencing and a literature review.
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
Diagnosis and complications of renovascular hypertension in children: literature data and clinical observations.
Burnei G, Burnei A, Hodorogea D, Drăghici I, Georgescu I, Vlad C, Gavriliu S
J Med Life 2009 Jan-Mar;2(1):18-28. PMID: 20108487Free PMC Article
Severe vesicoureteral reflux and chronic renal failure: a condition peculiar to male gender? Data from the ItalKid Project.
Marra G, Oppezzo C, Ardissino G, Daccò V, Testa S, Avolio L, Taioli E, Sereni F; ItalKid Project
J Pediatr 2004 May;144(5):677-81. doi: 10.1016/j.jpeds.2004.01.043. PMID: 15127014
Contemporary incidence of morbidity related to vesicoureteral reflux.
Vallee JP, Vallee MP, Greenfield SP, Wan J, Springate J
Urology 1999 Apr;53(4):812-5. doi: 10.1016/s0090-4295(98)00587-1. PMID: 10197863
Chronic renal failure in children.
Mongeau JG, Robitaille P, Grall MM
Can Med Assoc J 1978 Apr 22;118(8):907-10, 913. PMID: 647561Free PMC Article

Therapy

Successful Treatment of Transplant Renal Artery Thrombosis With Systemic Infusion of Recombinant-Tissue-Plasminogen Activator After Renal Transplant.
Yoshida T, Yanishi M, Nakamoto T, Sugi M, Kinoshita H, Matsuda T
Exp Clin Transplant 2017 Oct;15(5):571-573. Epub 2015 Aug 31 doi: 10.6002/ect.2015.0099. PMID: 26325476
Can postpyelonephritic renal scarring be prevented?
Nevéus T
Pediatr Nephrol 2013 Feb;28(2):187-90. Epub 2012 Oct 20 doi: 10.1007/s00467-012-2334-2. PMID: 23086593
Head circumference in chronic renal failure from birth.
Van Dyck M, Proesmans W
Clin Nephrol 2001 Dec;56(6):S13-6. PMID: 11770805

Prognosis

A novel pathogenic variant c.262delA in PBX1 causing oligomeganephronia identified using whole-exome sequencing and a literature review.
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
Pediatric renal transplantation: 13 years of experience--report from the Chilean Cooperative Multicenter Group.
Rosati P, Pinto V, Delucchi A, Salas P, Cano F, Zambrano P, Lagos E, Rodriguez E, Hevia P, Ramirez K, Quiero X, Azócar M, Rodriguez S, Aguiló J, Varela M, Ferrario M, Ramirez R, Palacios JM, Turu I, Jimenez O, Godoy J, Gaete J, Maluenda X, Villegas R; Chilean Cooperative Multicenter Group
Transplant Proc 2005 Apr;37(3):1569-73. doi: 10.1016/j.transproceed.2004.09.013. PMID: 15866676
Contemporary incidence of morbidity related to vesicoureteral reflux.
Vallee JP, Vallee MP, Greenfield SP, Wan J, Springate J
Urology 1999 Apr;53(4):812-5. doi: 10.1016/s0090-4295(98)00587-1. PMID: 10197863

Clinical prediction guides

A novel pathogenic variant c.262delA in PBX1 causing oligomeganephronia identified using whole-exome sequencing and a literature review.
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
Pathophysiological clinical features of an infant with hypertension secondary to multicystic dysplastic kidney: a case report.
Sugimoto K, Enya T, Joh K, Miyazaki K, Miyazawa T, Ohshima R, Marutani S, Tsukasa T, Okada M
BMC Nephrol 2021 Feb 5;22(1):55. doi: 10.1186/s12882-021-02249-6. PMID: 33546619Free PMC Article
Diagnosis and complications of renovascular hypertension in children: literature data and clinical observations.
Burnei G, Burnei A, Hodorogea D, Drăghici I, Georgescu I, Vlad C, Gavriliu S
J Med Life 2009 Jan-Mar;2(1):18-28. PMID: 20108487Free PMC Article
Renal-coloboma syndrome: report of a novel PAX2 gene mutation.
Chung GW, Edwards AO, Schimmenti LA, Manligas GS, Zhang YH, Ritter R 3rd
Am J Ophthalmol 2001 Dec;132(6):910-4. doi: 10.1016/s0002-9394(01)01231-4. PMID: 11730657
Nutritional intervention and growth in children with chronic renal failure.
Reed EE, Roy LP, Gaskin KJ, Knight JF
J Ren Nutr 1998 Jul;8(3):122-6. doi: 10.1016/s1051-2276(98)90002-4. PMID: 9724500

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