Zusammenfassung
Durch das verbesserte Langzeitüberleben von Kindern mit chronischen Nierenerkrankungen wird der internistische Nephrologe zunehmend konfrontiert mit jungen Erwachsenen, die teils an seltenen genetischen oder metabolischen Erkrankungen der Nieren und ableitenden Harnwege ledien. Rezidivierende Verläufe, extrarenale Manifestationen und Langzeitkomplikationen chronischer Nierenerkrankungen, wie Kleinwuchs, gestörte psychosoziale Adaptation und erhöhtes kardiovaskuläres Mortalitätsrisiko, machen die internistische Betreuung dieser jungen Menschen zu einer Herausforderung. Die Kenntnis klinischer Klassifikationen, Verläufe und Behandlungsprinzipien sind unabdingbar. Neben den angeborenen Fehlbildungen von Nieren und ableitenden Harnwegen werden das nephrotische Syndrom, das hämolytisch-urämische Syndrom, die nephropathische Zystinose und, als Vertreter der Ziliopathien, die Nephronophthise dargestellt. Rotationen in der Ausbildung zum Kindernephrologen /internistischen Nephrologen können helfen, den Wissensaustausch zu fördern und organisatorische Hemmnisse bei der Transition abzubauen.
Abstract
Due to improved long-term survival of children with chronic kidney diseases (CKD), the adult nephrologist is increasingly facing young adults with rare genetic or metabolic chronic renal illnesses. Disease recurrence, extrarenal manifestations, and long-term complications of CKD, such as growth retardation, disturbed psychosocial adaption, and increased risk of cardiovascular mortality, are challenging for further medical care. Knowledge about clinical classifications, courses, and therapeutic principles of these diseases are, therefore, mandatory. This article depicts not only congenital malformations of the kidneys and the urinary tract, but also describes nephrotic syndrome, hemolytic–uremic syndrome, nephropathic cystinosis, and exemplary for ciliopathies nephronophthisis. Rotations during the training of both pediatric and adult nephrologists have the potential to increase clinical knowledge and reduce organizational barriers of transition.
Literatur
Benz MR, Rüth EM, Kusser F et al (2010) Pipilotta und der Nierendetektiv. Monatsschr Kinderheilk 1:6–7
Caprioli J, Noris M, Brioschi S et al (2006) Genetics of HUS: The impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome. Blood 108:1257–1279
Cameron JS (2001) The continued care of children with renal disease into adult life. Pediatr Nephrol 16:680–685
Cara Fuentes GM, Garcia Meseguer C, Peňa Carrion A et al (2010) Long-term outcome of focal segemntal glomerulosclerosis after pediatric renal transplantation. Pediatr Nephrol 25:529–534
Cochat P, Fargue S, Mestrallet G et al (2009) Disease recurrence in paediatric renal transplantation. Pediatr Nephrol 24:2097–2108
Dorresteijn EM, Kist-van Holthe JE, Levtchenko EN et al (2008) Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome. Pediatr Nephrol 23:2013–2020
Fine RN, Martz K, Stablein D (2010) What have 20 years of data from the North American pediatric renal transplant cooperative study taught us about growth following renal transplantation in infants, children, and adolescents with end-stage renal disease. Pediatr Nephrol 25:739–746
Fink JK, Brouwers P, Barton N et al (1989) Neurologic complications in long-standing nephropathic cystinosis. Arch Neurol 46:543–548
Frei U, Schober-Halstenberg HJ (2006/2007) Nierenersatztherapie in Deutschland. Bericht QuaSi-Niere
Fujisawa M, Iijima K, Ishimura T et al (2002) Long-term outcome of focal segmental glomerulosclerosis after Japanese pediatric renal transplantation. Pediatr Nephrol 17:165–168
Gahl WA, Thoene JG, Schneider JA (2002) Cystinosis. N Engl J Med 347:111–121
Geary DF, Schaefer F (Hrsg) (2008) Comprehensive pediatric nephrology. Mosby Elsevier, Philadelphia
Haffner D, Schaefer F, Nissel R et al (2000) Effect of growth hormone treatment on the adult height of children with chronic renal failure. N Engl J Med 343:923–930
Hildebrandt F, Zhou W (2007) Nephronophthisis-associated ciliopathies. J Am Soc Nephrol 18:1855–1871
Höcker B, Weber LT, Feneberg R et al (2009) Prospective, randomised, multicentre trial on late steroid withdrawal in paediatric renal transplant recipients under cyclosporine microemulsion and mycophenolate mofetil. Transplantation 87:934–941
Hoefele J, Hildebrandt F, Benz MR et al (2007) Nephronophthise. Neueste Erkenntnisse in der molekularen Diagnostik. Nephrologie 5:372–376
Hoefele J, Wolf MT, O’Toole JF et al (2007) Evidence of oligogenic inheritance in nephronophthisis. J Am Soc Nephrol 18:2789–2795
Ichikawa I, Kuwayama F, Pope JC 4th (2002) Pradigm shift from classical anatomic therories to contemporary cell biological views of CAKUT. Kidney Int 61:889–898
Klassen I, Neuhaus TJ, Mueller-Wiefel DE et al (2007) Antenatal oligohydramnios of renal origin: long-term outcome. Nephrol Dial Transplant 22:432–439
Konrad M (2010) Gendiagnostik in der Kindernephrologie. Nephrologie 5:19–24
Lange-Sperandio B, Krüger-Stollfuß I, Hoefele J et al (2010) Zystische Nierenerkrankungen im Kindesalter. Kinder Jugendmed
McDonald SP, Craig JC (2004) Long-term survival of children with end-stage renal disease. N Engl J Med 350:2654–2662
Mekahli D, Liutkus A, Ranchin B et al (2009) Long-term outcome of idiopathic steroid-resistant nephrotic syndrome: a multicenter study. Pediatr Nephrol 24:1525–1532
Nürnberger J, Philipp T, Witzke O et al (2009) Eculizumab for atypical hemolytic-uremic syndrome. N Engl J Med 360:542–544
Odorico JS, Knechtle S, Rayhill SC et al (1996) The influence of native nephrectomy on the incidence of recurrent disease following renal transplantation for primary glomerulonephritis. Transplantation 61:228–234
Oh J, Wunsch R, Turzer M et al (2002) Advanced coronary and carotid arteriopathy in young adults with childhood-onset chronic renal failure. Circulation 106:100–105
Plank C, Kalb V, Hinkes B et al (2008) Cyclopsorin A is superior to cyclophosphamide in children with steroid-resistant nephrotic syndrome – a randomized controlled multicentre trial by the Arbeitsgemeinschaft für Pädiatrische Nephrologie. Pediatr Nephrol 23:1483–1493
Potter DE, Holliday MA, Piel CF et al (1980) Treatment of end-stage renal disease in children: a 15-year experience. Kidney Int 18:103–109
Rosenkranz J, Reichwald-Klugger E, Oh J et al (2005) Psychosocial rehabilitation and satisfaction with life in adults with childhood-onset of end-stage renal disease. Pediatr Nephrol 20:1288–1294
Rüth EM, Kemper MJ, Leumann EP et al (2005) Children with steroid-sensitive nephrotic syndrome come of age: long-term outcome. J Pediatr 147:202–207
Ruf RG, Lichtenberger A, Karle SM et al (2004) Patients with mutations in NPHS2 (podocin) do not respond to standard steroid treatment of nephrotic syndrome. J Am Soc Nephrol 15:722–732
Salomon R, Saunier S, Niaudet P (2009) Nephronophthisis. Pediatr Nephrol 24:2333–2344
Schärer K, Mehls O (Hrsg) (2002) Pädiatrische Nephrologie, Springer, Berlin Heidelberg New York Tokio
Tönshoff B, Schaefer F, Müller-Wiefel DE (2007) Pädiatrische Nephrologie. Monatsschr Kinderheilkd 155:293–303
Vester U, Kranz B, Hoyer PF (2010) The diagnostic value of ultrasound in cystic kidney diseases. Pediatr Nephrol 25:231–240
Watson AR (2005) Problems und pitfalls of transition from paediatric to adult renal care. Pediatr Nephrol 20:113–117
Weber LT (2007) Wichtige Aspekte der Kindernephrologie. In: Keller F, Ketteler M, Schindler R et al (Hrsg) Manuale Nephrologicum Dustri. Orlando, München, S 693–694
Weber S, Gribouval O, Esquivel EL et al (2004) NPHS2 mutation analysis show genetic heterogeneity of steroid-resistant nephrotic syndrome and low post-transplant recurrence. Kidney Int 66:571–579
Weber S, Tönshoff B (2005) Recurrence of focal-segmental glomerulosclerosis in children after renal transplantation: Clinical and genetic aspects. Transplantation 80:128–134
Wingen AM, Fabian-Bach C, Schaefer F et al (1997) Randomised multicentre study of a low-protein diat on the progression of chronic renal failure in children. Lancet 349:1117–1123
Woolf AS (2004) Embryology. In: Avner ED, Harmon WE, Niaudet P (Hrsg) Pediatric nephrology, 5 edn. Lippincott Williams & Williams, Philadelphia, pp 3–24
Wuehl E, Haffner D, Offner G et al (2001) Long term treatment with growth hormon in short children with nephropathic cystinosis. J Paediatrics 138:880–887
Wuehl E, Trivelli A, Picca S et al (2009) Strict blood-pressure control and progression of renal failure in children. N Engl J Med 361:1639–1650
Zimmerhackl LB, Hofer J, Cortina G et al (2010) Prophylactic eculizumab after renal transplantation in atypical hemolytic-uremic syndrome. N Engl J Med 362:1746–1748
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Weber, L. Pädiatrisch nephrologische Erkrankungen nach dem Transfer. Nephrologe 6, 9–21 (2011). https://doi.org/10.1007/s11560-010-0475-5
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DOI: https://doi.org/10.1007/s11560-010-0475-5
Schlüsselwörter
- Pädiatrie
- Nephrologie
- Transition
- Langzeitbetreuung
- Chronische Niereninsuffizienz
- Kongenitale Malformationen