Kidney dysplasia
Classification according to ICD-10 | |
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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 :
- Beckwith-Wiedemann syndrome
- DiGeorge syndrome
- Fraser Syndrome
- Kallmann syndrome
- Laurence-Moon-Biedl-Bardet syndrome
- Mammorenal Syndrome
- Prune Belly Syndrome
- Renal Coloboma Syndrome
- Renal cysts and diabetes syndrome
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 .
See also
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 .