{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,30]],"date-time":"2026-07-30T23:00:53Z","timestamp":1785452453319,"version":"3.56.0"},"reference-count":37,"publisher":"Georg Thieme Verlag KG","issue":"04","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Aktuelle Urol"],"published-print":{"date-parts":[[2026,8]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title>\n                  <jats:p>Posteriore Urethralklappen (PUV) sind seltene angeborene Fehlbildungen, die sowohl den oberen als auch den unteren Harntrakt betreffen und langfristig schwerwiegende Komplikationen verursachen k\u00f6nnen. Dank Fortschritten in pr\u00e4nataler Diagnostik, neonatologischer Versorgung sowie kinderurologisch-nephrologischer Therapie erreichen zunehmend mehr Patienten das Erwachsenenalter. F\u00fcr das Langzeit-Outcome sind insbesondere das Ausma\u00df der pr\u00e4natalen Nierendysplasie sowie die fr\u00fchzeitige Erkennung und gezielte Behandlung von Blasendysfunktionen entscheidend. Die Betreuung erwachsener PUV-Patienten stellt dennoch eine besondere Herausforderung dar, da viele Erwachsenenurologen nur begrenzte Erfahrung besitzen und evidenzbasierte Leitlinien zur Langzeitversorgung fehlen. Erwachsene PUV-Patienten bilden eine klar definierte Hochrisikokohorte mit charakteristischen urologischen und renalen Langzeitfolgen. Dazu geh\u00f6ren persistierende oder sekund\u00e4r progrediente Blasenfunktionsst\u00f6rungen mit klinisch relevanten Lower Urinary Tract Symptoms (LUTS) sowie ein erh\u00f6htes Risiko f\u00fcr progressive chronische Nierenerkrankung bis zur terminalen Niereninsuffizienz. Im Fall einer terminalen Niereninsuffizienz kann eine Nierentransplantation erforderlich werden; hierbei ist eine optimierte Blasenfunktion entscheidend, um das Transplantat langfristig zu sch\u00fctzen, diesbez\u00fcglich k\u00f6nnen rekonstruktive Ma\u00dfnahmen, wie eine Harnblasenaugmentation notwendig sein.Die Blasenfunktion ver\u00e4ndert sich lebenslang: Anfangs hypertroph und hyperaktiv, kann sie im Verlauf bis hin zur Atonie dekompensieren (\u201emyogenic failure\u201c). Erwachsene Patienten berichten h\u00e4ufig \u00fcber Miktionsst\u00f6rungen, Restharn, Drangbeschwerden, Inkontinenz und rezidivierende Harnwegsinfektionen, die Lebensqualit\u00e4t, Selbstwert und sexualbezogene Selbstwirksamkeit erheblich beeintr\u00e4chtigen k\u00f6nnen. Die Therapie richtet sich am individuellen urodynamischen Ph\u00e4notyp aus und umfasst konservative, medikament\u00f6se und ggf. interventionelle Ma\u00dfnahmen.Sexualfunktion und Fertilit\u00e4t sind bei den meisten Patienten erhalten; Einschr\u00e4nkungen treten \u00fcberwiegend bei fortgeschrittener CKD oder komplexem Blasenmanagement auf. Psychosoziale Belastungen durch ver\u00e4ndertes K\u00f6rperbild, Inkontinenz, Katheterisierung oder Sorgen bez\u00fcglich Partnerschaft und Familienplanung sind h\u00e4ufig und sollten fr\u00fchzeitig adressiert werden. Die Betreuung von PUV-Patienten erfordert daher eine lebenslange, interdisziplin\u00e4re Nachsorge, eine strukturierte Transition von der Kinder- zur Erwachsenenmedizin sowie enge Abstimmung zwischen Urologie, Nephrologie, Kinderurologie\/-nephrologie und Transplantationsmedizin, um Nierenfunktion, Lebensqualit\u00e4t und Transplantatfunktion langfristig zu sichern.<\/jats:p>","DOI":"10.1055\/a-2864-6465","type":"journal-article","created":{"date-parts":[[2026,6,19]],"date-time":"2026-06-19T23:27:05Z","timestamp":1781911625000},"page":"352-359","source":"Crossref","is-referenced-by-count":0,"title":["Die Harnr\u00f6hrenklappenkrankheit: Eine kinderurologische Erkrankung mit lebenslanger Relevanz"],"prefix":"10.1055","volume":"57","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7974-6694","authenticated-orcid":false,"given":"Aybike","family":"Hofmann","sequence":"additional","affiliation":[{"name":"Marienhaus Clinic Neuwied, Germany","place":["Neuwied"],"department":["Pediatric 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