Renal hypoplasia

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Classification according to ICD-10
Q60.3 Renal hypoplasia, unilateral
Q60.4 Renal hypoplasia, bilateral
Q60.5 - Renal hypoplasia, unspecified
ICD-10 online (WHO version 2019)

The renal hypoplasia is a congenital underdevelopment of one or both kidneys . In contrast to kidney dysplasia , the tissue structure in the kidney that is too small is normal.

The change is not to be confused with kidney dysplasia or with an acquired reduction in size of an originally normal kidney ( shrunken kidney ).

distribution

The frequency is given as 2.5%.

root cause

This is due to developmental disorders of the metanephros, the post-kidney.

A cytomegaly infection occurring intrauterine can lead to renal hypoplasia.

Intrauterine growth retardation , gestational diabetes , preeclampsia , eclampsia , intoxication or substance abuse in the mother and premature birth are to be regarded as risk factors .

There are associations with renal coloboma syndrome and Turner syndrome .

In the context of syndromes

Renal hypoplasia can also occur in the context of syndromes :

Classification

A distinction must be made between the following forms:

  • Bilateral renal hypoplasia
  • Unilateral renal hypoplasia
  • Segmental hypoplasia limited to individual sections of a kidney

Clinical manifestations

Kidney hypoplasia can be associated with other developmental disorders.

On both sides

The clinical picture depends on the degree of (impaired) kidney function . Intrauterine is a fruit of lack of water and an increased fetal Β2 microglobulin indicate an unfavorable prognosis . The diagnosis is often made as an "incidental finding" during a routine examination after birth. It can lead to renal hypertension and chronic renal failure occur.

One-sided

If the form is unilateral, there is compensatory hypertrophy of the opposite side, otherwise it is renal dysplasia.

Segmental

With segmental underdevelopment of one or more kidney parts, renal hypertension can also occur.

diagnosis

The diagnosis is usually made by sonography . Kidney hypoplasia can already be detected in utero from around the middle of pregnancy .

Differential diagnosis

The most important differential diagnosis is the much more common renal dysplasia. A morphologically normal kidney that is too small on one side and is morphologically normal can also be a horseshoe kidney . In the case of segmental forms, the much more frequent inflammatory or scarred changes must be delimited.

literature

Individual evidence

  1. a b Urology textbook
  2. a b K. Schärer: Kidney hypoplasia and dysplasia In: K. Schärer, O. Mehls (Ed.): Pediatrische Nephrologie , Springer, 2002, pp. 149–153, ISBN 978-3-642-62621-0 [1 ]
  3. ^ J. Bernstein: Renal hypoplasia and dysplasia. In: CM Edelmann (Ed.): Pediatric kidney disease. Little, Brown, Boston, 1992, pp. 1121-1137
  4. ^ Watkins SL, McDonald RA, Avner ED: Renal dysplasia and miscellaneous cystic disorder. In: TM Barratt, ED Avner, W E. Harmon (Eds.): Pediatric nephrology, 4th Edition, Lippincott, Williams & Wilkins, Baltimore, 1999, pp. 415-425
  5. Renal hypoplasia, congenital cytomegalovirus. In: Orphanet (Rare Disease Database).
  6. a b renal hypoplasia. In: Orphanet (Rare Disease Database).
  7. Bernfried Leiber (founder): The clinical syndromes. Syndromes, sequences and symptom complexes . Ed .: G. Burg, J. Kunze, D. Pongratz, PG Scheurlen, A. Schinzel, J. Spranger. 7., completely reworked. Edition. tape 2 : symptoms . Urban & Schwarzenberg, Munich et al. 1990, ISBN 3-541-01727-9 .
  8. Oligomeganephronia. In: Orphanet (Rare Disease Database).
  9. Lethal fetal brain malformation-duodenal atresia-bilateral renal hypoplasia syndrome. In: Orphanet (Rare Disease Database).
  10. CR Sierra Labarta, A. De Pablo Cardenas, D. Sanchez Zalabardo, JA Cuesta Alcala, JM Martin Cuartero: Left renal hypoplasia with ureteral ectopia to the seminal vesicle. In: Archivos españoles de urología. Vol. 66, No. 9, November 2013, p. 896, PMID 24231304 .
  11. ^ E. Spaggiari, JJ Stirnemann, L. Heidet, S. Dreux, Y. Ville, JF Oury, AL Delezoide, F. Muller: Outcome following prenatal diagnosis of severe bilateral renal hypoplasia. In: Prenatal Diagnosis . Vol. 33, No. 12, December 2013, pp. 1167–1172, doi: 10.1002 / pd.4217 , PMID 23943585 .
  12. a b V. Hofmann, KH Deeg, PF Hoyer: Ultrasound diagnostics in paediatrics and pediatric surgery. Textbook and atlas. Thieme 2005, ISBN 3-13-100953-5 .
  13. Renal hypoplasia, bilateral. In: Orphanet (Rare Disease Database).
  14. Unilateral renal hypoplasia. In: Orphanet (Rare Disease Database).
  15. O. Mehls, B. Klare, K. Möhring, F. Manz: Therapy and prognosis of segmental renal hypoplasia. In: Clinical Pediatrics. Vol. 194, No. 3, 1982 May-Jun, pp. 145-148, doi: 10.1055 / s-2008-1033795 , PMID 7132218 .