Nierenzysten und zystische Nierenerkrankungen bei Kindern (AWMF
                    S2k-Leitlinie)

Charlotte Gimpel; Carsten Bergmann; Florian Brinkert; Metin Cetiner; Ulrich Gembruch; Dieter Haffner; Markus Kemper; Jens König; Max Liebau; Rolf Felix Maier; Jun Oh; Lars Pape; Silke Riechardt; Udo Rolle; Rainer Rossi; Joachim Stegmann; Udo Vester; Constantin von Kaisenberg; Stefanie Weber; Franz Schaefer

doi:10.1055/a-1179-0728

Klinische Pädiatrie, Table of Contents

Klin Padiatr 2020; 232(05): 228-248
DOI: 10.1055/a-1179-0728

Diagnostic and Treatment Recommendation

Nierenzysten und zystische Nierenerkrankungen bei Kindern (AWMF S2k-Leitlinie)

Kidney Cysts and Cystic Nephropathies in Children – A Consensus Guideline by 10 German Medical Societies

Charlotte Gimpel

¹Department of Internal Medicine IV, Medical Center – University of Freiburg, Freiburg

²Faculty of Medicine, University of Freiburg, Freiburg im Breisgau

,

Carsten Bergmann

¹Department of Internal Medicine IV, Medical Center – University of Freiburg, Freiburg

²Faculty of Medicine, University of Freiburg, Freiburg im Breisgau

³Medizinische Genetik Mainz, Limbach Genetics, Mainz

,

Florian Brinkert

⁴Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg

,

Metin Cetiner

⁵Department of Pediatrics II, University Hospital Essen, Essen

,

Ulrich Gembruch

⁶Department of Obstetrics and Prenatal Medicine, University Hospital of Bonn, Bonn

,

Dieter Haffner

⁷Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover

,

Markus Kemper

⁸Department of Pediatrics, Asklepios Kliniken Hamburg GmbH, Asklepios Klinik Nord, Standort Heidberg, Hamburg

,

Jens König

⁹Department of General Pediatrics, University Children's Hospital Münster, Münster

,

Max Liebau

¹⁰Department of Pediatrics, University Hospital Cologne, Cologne

¹¹Center for Molecular Medicine, University of Cologne, Cologne

,

Rolf Felix Maier

¹²Department of Pediatrics, University Hospital of Giessen and Marburg, Campus Marburg, Marburg

,

Jun Oh

⁴Department of Pediatrics, University Medical Center Hamburg-Eppendorf, Hamburg

,

Lars Pape

⁷Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover

,

Silke Riechardt

¹³Department of Urology, University Medical Center Hamburg-Eppendorf, Hamburg

,

Udo Rolle

¹⁴Department of Pediatric Surgery, Hospital of the Goethe University Frankfurt, Frankfurt am Main

,

Rainer Rossi

¹⁵Department of Pediatrics, Vivantes Klinikum Neukölln, Berlin

,

Joachim Stegmann

¹⁶Department of Radiology, Catholic Childrenʼs Hospital Wilhelmstift, Hamburg

,

Udo Vester

¹⁷Department of Pediatrics, HELIOS Hospital Duisburg, Duisburg

,

Constantin von Kaisenberg

¹⁸Department of Obstetrics and Gynaecology, Center for Perinatal Medicine, Hannover Medical School, Hannover

,

Stefanie Weber

¹²Department of Pediatrics, University Hospital of Giessen and Marburg, Campus Marburg, Marburg

,

Franz Schaefer

¹⁹Center for Pediatrics and Adolescent Medicine, Division of Pediatric Nephrology, University Hospital Heidelberg, Heidelberg

› Author Affiliations

Abstract

Zusammenfassung

Die Leitlinie wurde im Konsens aller relevanten deutschen Fachgesellschaften zusammen mit Patientenvertretern erstellt. Standard der prä- und postnatalen Bildgebung von Nierenzysten ist die Sonografie, die auch im Abdomen und inneren Genitale extrarenale Manifestationen ausschließen soll. Die MRT hat einzelne Indikationen. Bei Verdacht auf zystische Nierenerkrankungen ist eine kindernephrologische Vorstellung indiziert. Die pränatale Betreuung muss auf sehr unterschiedliche Schweregrade zugeschnitten werden. Bei renalem Oligohydramnion wird eine Entbindung in einem Perinatalzentrum der höchsten Stufe empfohlen. Neugeborenen sollte eine Nierenersatztherapie nicht allein aufgrund des Alters vorenthalten werden. Bei unilateraler multizystischer Nierendysplasie ist keine funktionelle Bildgebung oder Nephrektomie notwendig, aber (wie auch bei uni- oder bilateraler Nierenhypo-/dysplasie mit Zysten) eine langfristige nephrologische Überwachung. Bei der ARPKD (autosomal rezessive polyzystische Nierenerkrankung), Nephronophthise, Bardet-Biedl-Syndrom und HNF1B-Mutationen müssen extrarenale Manifestationen beachtet werden; eine genetische Testung ist hier sinnvoll. Kinder mit tuberöser Sklerose, Tumorprädispositionen (z. B. von Hippel Lindau Syndrom) oder hohem Risiko für erworbene Nierenzysten sollten regelmäßige Nierensonografien erhalten. Auch asymptomatische Kinder von Eltern mit ADPKD (aut. dominanter polyzystischer Nierenerkrankung) sollten regelmäßig auf Hypertonie und Proteinurie untersucht werden. Eine präsymptomatische sonografische oder genetische Diagnostik dieser Minderjährigen sollte nur nach ausführlicher Aufklärung erwogen werden. Einfache (isolierte) Zysten sind bei Kindern sehr selten und eine ADPKD eines Elternteils sollte ausgeschlossen sein. Komplexe Nierenzysten bedürfen weiterer Abklärung.

Abstract

This consensus-based guideline was developed by all relevant German pediatric medical societies. Ultrasound is the standard imaging modality for pre- and postnatal kidney cysts and should also exclude extrarenal manifestations in the abdomen and internal genital organs. MRI has selected indications. Suspicion of a cystic kidney disease should prompt consultation of a pediatric nephrologist. Prenatal management must be tailored to very different degrees of disease severity. After renal oligohydramnios, we recommend delivery in a perinatal center. Neonates should not be denied renal replacement therapy solely because of their age. Children with unilateral multicystic dysplastic kidney do not require routine further imaging or nephrectomy, but long-term nephrology follow-up (as do children with uni- or bilateral kidney hypo-/dysplasia with cysts). ARPKD (autosomal recessive polycystic kidney disease), nephronophthisis, Bardet-Biedl syndrome and HNF1B mutations cause relevant extrarenal disease and genetic testing is advisable. Children with tuberous sclerosis complex, tumor predisposition (e. g. von Hippel Lindau syndrome) or high risk of acquired kidney cysts should have regular ultrasounds. Even asymptomatic children of parents with ADPKD (autosomal dominant PKD) should be monitored for hypertension and proteinuria. Presymptomatic diagnostic ultrasound or genetic examination for ADPKD in minors should only be done after thorough counselling. Simple cysts are very rare in children and ADPKD in a parent should be excluded. Complex renal cysts require further investigation.

Schlüsselwörter

Nierendysplasie - polyzystische Nierenerkrankung - Fetus - Kind - Ziliopathie - Nierenzyste

Key words

kidney dysplasia - multicystic dysplastic kidney (MCDK) - polycystic kidney disease - feuts - child - ciliopathy

Full Text

References

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