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Tubular luminal dilatation

MedGen UID:
1714243
Concept ID:
C5397640
Finding
Synonym: Tubular ectasia
 
HPO: HP:0032622

Definition

Dilatation (expansion beyond the normal dimension) of the cavity (lumen) of tubules of the kidney. The tubular cross section displays an attenuated brush border (apical PAS positivity greater than 10 percent of the normal expected height, but unequivocally less than normal expected height), resulting in an apparent increase in the size of lumen. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVTubular luminal dilatation

Conditions with this feature

Ventriculomegaly-cystic kidney disease
MedGen UID:
346584
Concept ID:
C1857423
Disease or Syndrome
Ventriculomegaly with cystic kidney disease is a severe autosomal recessive developmental disorder characterized by onset in utero of dilated cerebral ventricles and microscopic renal tubular cysts. The pregnancies of affected individuals are associated with increased alpha-fetoprotein (AFP). Most affected pregnancies have been terminated (summary by Slavotinek et al., 2015). See also 602200 for a disorder characterized by ventriculomegaly and defects of the radius and kidney.
Nephronophthisis-like nephropathy 2
MedGen UID:
1794163
Concept ID:
C5561953
Disease or Syndrome
Nephronophthisis-like nephropathy-2 (NPHPL2) is an autosomal recessive cystic kidney disease characterized by onset of progressive renal insufficiency in the first decades of life. Renal imaging and biopsy show corticomedullary cysts, tubular ectasia, tubular basement membrane disruption, and tubulointerstitial infiltrations. Patients eventually progress to end-stage renal failure, necessitating kidney transplantation or dialysis (summary by Hurd et al., 2013). For a general phenotypic description and a discussion of genetic heterogeneity of nephronophthisis, see NPHP1 (256100).
Hepatorenocardiac degenerative fibrosis
MedGen UID:
1808950
Concept ID:
C5676996
Disease or Syndrome
Hepatorenocardiac degenerative fibrosis (HRCDF) is a primarily fibrotic disease affecting the liver, kidney, and heart, with considerable variability in disease onset and expression. Affected individuals develop degenerative hepatic fibrosis in childhood or early adulthood, with variable later onset of fibrocystic kidney disease and hypertrophic cardiomyopathy (Devane et al., 2022).

Professional guidelines

PubMed

Bak J, Kang HW
Lasers Med Sci 2017 Jul;32(5):993-999. Epub 2017 Mar 29 doi: 10.1007/s10103-017-2199-5. PMID: 28353121

Recent clinical studies

Etiology

Gong H, Sun J, Xue W, Tian P, Ding X, Yan H, Li Y, Zheng J
Exp Biol Med (Maywood) 2014 Jun;239(6):677-85. doi: 10.1177/1535370213517613. PMID: 24737734
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Aust N Z J Med 1993 Feb;23(1):35-41. doi: 10.1111/j.1445-5994.1993.tb00535.x. PMID: 8460972
Moreau P, Albat B, Thevenet A
J Cardiovasc Surg (Torino) 1993 Dec;34(6):465-72. PMID: 8300709
McKeever LS, O'Donnell MJ, Stamato NJ, Cahill JM, Hartmann JR, Marek JC, Brown AS, Enger EL
Am Heart J 1991 Dec;122(6):1515-8. doi: 10.1016/0002-8703(91)90265-j. PMID: 1957744
Sumitomo S, Kumasa S, Tatemoto Y, Ookusa Y, Mori M
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Diagnosis

Waiser J, Knebel F, Rudolph B, Wu K, Müller E, Sanad W, Budde K, Neumayer HH, Halleck F
Transplantation 2015 Jun;99(6):1208-15. doi: 10.1097/TP.0000000000000501. PMID: 25539469
Terada T
Int J Clin Exp Pathol 2012;5(7):690-7. Epub 2012 Sep 5 PMID: 22977666Free PMC Article
Kwiatek MA, Hirano I, Kahrilas PJ, Rothe J, Luger D, Pandolfino JE
Gastroenterology 2011 Jan;140(1):82-90. Epub 2010 Sep 19 doi: 10.1053/j.gastro.2010.09.037. PMID: 20858491Free PMC Article
Ritz E, Zeier M, Geberth S, Waldherr R
Aust N Z J Med 1993 Feb;23(1):35-41. doi: 10.1111/j.1445-5994.1993.tb00535.x. PMID: 8460972
Moreau P, Albat B, Thevenet A
J Cardiovasc Surg (Torino) 1993 Dec;34(6):465-72. PMID: 8300709

Therapy

Waiser J, Knebel F, Rudolph B, Wu K, Müller E, Sanad W, Budde K, Neumayer HH, Halleck F
Transplantation 2015 Jun;99(6):1208-15. doi: 10.1097/TP.0000000000000501. PMID: 25539469
Nakhoul F, Better OS
Clin Nephrol 1995 Aug;44(2):118-20. PMID: 8529299
Voyer LE, Drut R, Méndez JH
Pediatr Nephrol 1994 Oct;8(5):592-4. doi: 10.1007/BF00858136. PMID: 7819009
McKeever LS, O'Donnell MJ, Stamato NJ, Cahill JM, Hartmann JR, Marek JC, Brown AS, Enger EL
Am Heart J 1991 Dec;122(6):1515-8. doi: 10.1016/0002-8703(91)90265-j. PMID: 1957744
Zollinger HU, Mihatsch MJ, Gudat F, Thiel G, Brunner F, Enderlin F
Clin Nephrol 1976 Nov;6(5):483-7. PMID: 791556

Prognosis

Paidas MJ, Mohamed AB, Norenberg MD, Saad A, Barry AF, Colon C, Kenyon NS, Jayakumar AR
Viruses 2021 Aug 27;13(9) doi: 10.3390/v13091703. PMID: 34578284Free PMC Article
Waiser J, Knebel F, Rudolph B, Wu K, Müller E, Sanad W, Budde K, Neumayer HH, Halleck F
Transplantation 2015 Jun;99(6):1208-15. doi: 10.1097/TP.0000000000000501. PMID: 25539469
Kwiatek MA, Hirano I, Kahrilas PJ, Rothe J, Luger D, Pandolfino JE
Gastroenterology 2011 Jan;140(1):82-90. Epub 2010 Sep 19 doi: 10.1053/j.gastro.2010.09.037. PMID: 20858491Free PMC Article
Ritz E, Zeier M, Geberth S, Waldherr R
Aust N Z J Med 1993 Feb;23(1):35-41. doi: 10.1111/j.1445-5994.1993.tb00535.x. PMID: 8460972
Moreau P, Albat B, Thevenet A
J Cardiovasc Surg (Torino) 1993 Dec;34(6):465-72. PMID: 8300709

Clinical prediction guides

Paidas MJ, Mohamed AB, Norenberg MD, Saad A, Barry AF, Colon C, Kenyon NS, Jayakumar AR
Viruses 2021 Aug 27;13(9) doi: 10.3390/v13091703. PMID: 34578284Free PMC Article
Kwiatek MA, Hirano I, Kahrilas PJ, Rothe J, Luger D, Pandolfino JE
Gastroenterology 2011 Jan;140(1):82-90. Epub 2010 Sep 19 doi: 10.1053/j.gastro.2010.09.037. PMID: 20858491Free PMC Article
Nomura A, Morita Y, Maruyama S, Hotta N, Nadai M, Wang L, Hasegawa T, Matsuo S
Am J Pathol 1997 Aug;151(2):539-47. PMID: 9250166Free PMC Article
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Am J Nephrol 1990;10(5):374-88. doi: 10.1159/000168153. PMID: 2080788
Olsen TS, Hansen HE, Olsen HS
Virchows Arch A Pathol Anat Histopathol 1985;406(1):91-104. doi: 10.1007/BF00710560. PMID: 3922113

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