Pediatric Nephrology

, Volume 24, Issue 4, pp 863–867 | Cite as

Congenital unilateral renal tubular dysgenesis and severe neonatal hypertension

  • Danielle Delaney
  • Sean E. Kennedy
  • Vivienne H. Tobias
  • Robert H. Farnsworth
Brief Report


Severe arterial hypertension rarely occurs in the neonatal period but may have life-threatening consequences. It is most often caused by renal parenchymal or vascular abnormality, which, to be accurately diagnosed, may require a combination of imaging modalities. We report on a case of neonatal hypertension presenting as cardiac failure. Initial imaging suggested unilateral renal artery stenosis, but this was not corroborated by magnetic resonance angiography. Surgical nephrectomy was curative for the hypertension and also allowed diagnosis of renal tubular dysgenesis. Unilateral congenital tubular dysgenesis without renal infarction has not been previously reported. We speculate that the condition was secondary to a period of localised hypoperfusion during early foetal life.


Renal artery stenosis Dimercaptosuccinic acid scintigraphy Magnetic resonance angiography Captopril Nephrectomy Cardiac failure 


  1. 1.
    Kiessling SG, Wadhwa N, Kriss VM, Iocono J, Desai NS (2007) An unusual case of severe therapy-resistant hypertension in a newborn. Pediatrics 119:301–304CrossRefGoogle Scholar
  2. 2.
    Flynn JT (2000) Neonatal hypertension: diagnosis and management. Pediatr Nephrol 14:332–341PubMedCrossRefGoogle Scholar
  3. 3.
    Mustafa AE, Bloom DA, Valentini RP, Mattoo TK, Imam AA (2005) MR angiography in the evaluation of a renovascular cause of neonatal hypertension. Pediatr Radiol 36:158–161PubMedCrossRefGoogle Scholar
  4. 4.
    Marcussen N (1991) Atubular glomeruli in renal artery stenosis. Lab Invest 65:558–565PubMedGoogle Scholar
  5. 5.
    Gribouval O, Gonzales M, Neuhaus T, Aziza J, Bieth E, Laurent N, Bouton JM, Feuillet F, Makni S, Ben Amar H, Laube G, Delezoide AL, Bouvier R, Dijoud F, Ollagnon-Roman E, Roume J, Joubert M, Antignac C, Gubler MC (2005) Mutations in genes in the renin-angiotensin system are associated with autosomal recessive renal tubular dysgenesis. Nat Genet 37:964–968PubMedCrossRefGoogle Scholar
  6. 6.
    Voyer LE, Drut R, Mendez JH (1994) Fetal renal maldevelopment with oligohydramnios following maternal use of piroxicam. Pediatr Nephrol 8:592–594PubMedCrossRefGoogle Scholar
  7. 7.
    Kriegsmann J, Coerdt W, Kommoss F, Beetz R, Hallermann C, Muntefering H (2000) Renal tubular dysgenesis (RTD)—an important cause of the oligohydramnion-sequence. Report of 3 cases and review of the literature. Pathol Res Pract 196:861–865PubMedGoogle Scholar
  8. 8.
    Lacoste M, Cai Y, Guicharnaud L, Mounier F, Dumez Y, Bouvier R, Dijoud F, Gonzales M, Chatten J, Delezoide AL, Daniel L, Joubert M, Laurent N, Aziza J, Sellami T, Amar HB, Jarnet C, Frances AM, Daïkha-Dahmane F, Coulomb A, Neuhaus TJ, Foliguet B, Chenal P, Marcorelles P, Gasc JM, Corvol P, Gubler MC (2006) Renal tubular dysgenesis, a not uncommon autosomal recessive disorder leading to oligohydramnios: role of renin-angiotensin system. J Am Soc Nephrol 17:2253–2263PubMedCrossRefGoogle Scholar
  9. 9.
    Pryde PG, Sedman AB, Nugent CE, Barr M Jr (1993) Angiotensin-converting enzyme inhibitor fetopathy. J Am Soc Nephrol 3:1575–1582PubMedGoogle Scholar
  10. 10.
    Saji H, Yamanaka M, Hagiwara A, Ijiri R (2001) Losartan and fetal toxic effects. Lancet 357:363PubMedCrossRefGoogle Scholar
  11. 11.
    Bernstein J, Barajas L (1994) Renal tubular dysgenesis: evidence of abnormality in the renin-angiotensin system. J Am Soc Nephrol 5:224–227PubMedGoogle Scholar
  12. 12.
    Allanson JE, Pantzar JT, MacLeod PM (1983) Possible new autosomal recessive syndrome with unusual renal histopathological changes. Am J Med Genet 16:57–60PubMedCrossRefGoogle Scholar
  13. 13.
    Singh HP, Hurley RM, Myers TF (1992) Neonatal hypertension. Incidence and risk factors. Am J Hypertens 5:51–55PubMedGoogle Scholar
  14. 14.
    Adelman RD (1987) Long-term follow-up of neonatal renovascular hypertension. Pediatr Nephrol 1:35–41PubMedCrossRefGoogle Scholar
  15. 15.
    Cook GT, Marshall VF, Todd JE (1966) Malignant renovascular hypertension in a newborn. J Urol 96:863–866PubMedGoogle Scholar
  16. 16.
    Johal NS, Kraklau D, Cuckow PM (2005) The role of unilateral nephrectomy in the treatment of nephrogenic hypertension in children. BJU Int 95:140–142PubMedCrossRefGoogle Scholar
  17. 17.
    Landing BH, Stela MA, Herta N, Larson EF, Turner M (1994) Labeled lectin studies of renal tubular dysgenesis and renal tubular atrophy of postnatal renal ischaemia and end-stage kidney disease. Pediatr Pathol 14:87–99PubMedCrossRefGoogle Scholar
  18. 18.
    De Paepe ME, Stopa E, Huang C, Hansen K, Luks FI (2003) Renal tubular apoptosis in twin-to-twin transfusion syndrome. Pediatr Dev Pathol 6:215–225PubMedGoogle Scholar
  19. 19.
    Morris S, Akima S, Dahlstrom JE, Ellwood D, Kent A, Falk MC (2004) Renal tubular dysgenesis and neonatal haemochromatosis without pulmonary hypoplasia. Pediatr Nephrol 19:341–344PubMedCrossRefGoogle Scholar
  20. 20.
    Hegde S, Wright C, Shenoy M, Moghal NE, Coulthard MG (2007) Renovascular hypertension commencing during fetal life. Arch Dis Child Fetal Neonatal Ed 92:F301–304PubMedCrossRefGoogle Scholar
  21. 21.
    Selye H (1946) Transformation of the kidney into an exclusively endocrine organ. Nature 158:131CrossRefGoogle Scholar

Copyright information

© IPNA 2008

Authors and Affiliations

  • Danielle Delaney
    • 1
  • Sean E. Kennedy
    • 2
  • Vivienne H. Tobias
    • 3
  • Robert H. Farnsworth
    • 1
  1. 1.Department of Paediatric UrologySydney Children’s HospitalRandwickAustralia
  2. 2.Department of NephrologySydney Children’s HospitalRandwickAustralia
  3. 3.Department of Anatomical Pathology, South Eastern Area Laboratory ServicesSydney Children’s HospitalRandwickAustralia

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