Kidney dysplasia

Classification according to ICD-10
Q61.4	Kidney dysplasia
ICD-10 online (WHO version 2019)

The renal dysplasia is a congenital frühembyronale developmental disorder ( malformation ) of one or both kidneys . The disease is characterized by poor kidney tissue development with abnormal kidney architecture.

If the development is maximally disturbed and no kidney is detectable, one speaks of renal agenesis or renal aplasia, if only a size reduction with normal tissue structure is present, it is a renal hypoplasia .

Synonyms are kidney dysgenesis; Renal dysplasia, English renal dysplasia

distribution

The incidence is estimated at 1 in 2,300 births, 1 in 2,400 in adults and 1 in 93 in infants. Combination with other malformations of the urinary tract is found in 57% of the sections.

root cause

A possible cause is a disturbance in the formation of the ureteral bud and the metanephrogenic tissue and / or a urinary stagnation that has already started intrauterine .

pathology

The pathological examination reveals immature glomeruli and epithelial- lined tubules with surrounding rings of connective tissue and smooth muscle cells, often also metaplastic cartilage.

In the context of syndromes

With some syndromes :

Classification

A distinction must be formally following forms:

Bilateral kidney dysplasia

Unilateral kidney dysplasia

Segmental dysplasia limited to individual sections of a kidney

According to comorbidities can be divided into:

Renal dysplasia with normal ostium
- with obstruction
- without obstruction
Renal dysplasia with a lateralized ostium
Renal dysplasia with subvesical obstruction (located below the urinary bladder)
Renal dysplasia in prune belly syndrome

The following classification is used clinically :

simple dysplasia
Multicystic kidney dysplasia (aplastic cystic kidney, English multicystic dysplastic kidney ), occurring spontaneously, mostly unilateral with parenchyma transformed into multiple large cysts

obstructive dysplasia, mostly in urethral valves , also in prune belly syndrome

Polycystic kidney disease , English Polycystic Kidney Disease

genetic (ARPKD), autosomal - recessive , ADPKD Autosomal dominant inheritance , nephronophthisis
Sporadic multicystic kidney dysplasia, solitary kidney cyst, multicystic cortical kidney cyst ( Zellweger syndrome )

Clinical manifestations

Clinical criteria are: In the case of severely impaired kidney function with oligohydramnios , the Potter sequence can occur. If the kidney function is sufficiently preserved, kidney dysplasia is usually symptom-free and is diagnosed either intrauterine or later during a routine check-up, e. B. discovered in a urinary tract infection .

diagnosis

The diagnosis is based on ultrasound imaging with evidence of cysts or an increased echogenic kidney and reduced delimitation of the internal structures. A confirmation can be made biopsy .

Differential diagnosis

Renal hypoplasia and post-inflammatory or post-infectious damage to the kidneys must be differentiated .

Web links

GB Gruber: Developmental disorders of the kidneys and ureters , doi: 10.1007 / 978-3-642-87630-1_10
National Institute of Diabetes and Digestive and Kidney Diseases

Individual evidence

↑ ^a ^b ^c ^d ^e Urology textbook
↑ ^a ^b J. W. Thüroff, H. Schulte-Wissermann (ed.): Pediatric urology in clinic and practice . 2nd Edition. Thieme, 2000, ISBN 3-13-674802-6 .
↑ ^a ^b ^c ^d ^e Renal dysplasia. In: Orphanet (Rare Disease Database).
↑ P. Probst: Frequency, complications and prognosis of kidney dysplasia. In: Journal of Pediatrics. Vol. 113, No. 3, pp. 215-236.
↑ ^a ^b F. C. Sitzmann: Malformations of the kidneys and lower urinary tract. In: Duale Reihe: Pädiatrie , Springer 2007, p. 412 ff., ISBN 978-3-13-125333-0 Online ISBN 978-3-13-190563-5 , doi: 10.1055 / b-0034-10819 .
↑ 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 .
^ V. Hofmann, KH Deeg, PF Hoyer: Ultrasound diagnostics in paediatrics and pediatric surgery. Textbook and atlas. Thieme, 2005, ISBN 3-13-100953-5 .

[Urologie-1] Urology textbook

[Thüroff-2] J. W. Thüroff, H. Schulte-Wissermann (ed.): Pediatric urology in clinic and practice . 2nd Edition. Thieme, 2000, ISBN 3-13-674802-6 .

[Orpha-3] Renal dysplasia. In: Orphanet (Rare Disease Database).

[4] P. Probst: Frequency, complications and prognosis of kidney dysplasia. In: Journal of Pediatrics. Vol. 113, No. 3, pp. 215-236.

[Sitzmann-5] F. C. Sitzmann: Malformations of the kidneys and lower urinary tract. In: Duale Reihe: Pädiatrie , Springer 2007, p. 412 ff., ISBN 978-3-13-125333-0 Online ISBN 978-3-13-190563-5 , doi: 10.1055 / b-0034-10819 .

[Leiber-6] 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 .

[7] V. Hofmann, KH Deeg, PF Hoyer: Ultrasound diagnostics in paediatrics and pediatric surgery. Textbook and atlas. Thieme, 2005, ISBN 3-13-100953-5 .