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Klin Padiatr 2005; 217(4): 201-210
DOI: 10.1055/s-2004-820329
Übersichtsarbeit

© Georg Thieme Verlag Stuttgart · New York

Enteraler Eiweißverlust: Pathophysiologie, Ursachen, Diagnostik und Therapie mit Fallbeispielen

Protein-loosing Enteropathy: Report of Four Cases and Review of Etiology, Diagnostic Work-Up and TreatmentL. Bindl1 , S. Buderus1 , C. Bindl1 , M. J. Lentze1
  • 1Universitäts-Kinderklinik, Bonn
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Publication Date:
27 April 2005 (online)

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Zusammenfassung

Der enterale Eiweißverlust (EEWV) kann als Begleitsymptom bei einer Vielzahl systemischer Erkrankungen und lokaler Erkrankungen des Gastrointestinaltraktes auftreten. Leitsymptom sind die Eiweißmangelödeme, gastrointestinale Symptome sind nicht obligat. Die Diagnose wird durch den Ausschluss anderer Proteinverlustquellen und den Nachweis erhöhter Alpha-1-Antitrypsin-Konzentrationen im Stuhl (> 320 mg/L) gesichert. In der Mehrzahl der Fälle ist die Klärung der Ursache über die Diagnose einer Grundkrankheit leicht möglich. Die diagnostische Aufarbeitung muss in unklaren Fällen zunächst nach dem Pathomechanismus (Zirkulationsstörung vs. Entzündung vs. Störung der Basalmembran) differenzieren. Davon ausgehend ist die gezielte Diagnostik der Grundkrankheit möglich. Ein gestuftes Vorgehen, orientiert an Invasivität und Kosteneffektivität wird vorgestellt. Die Lokalisationsdiagnostik erfordert bildgebende Verfahren (CT-Abdomen, Sonographie, Endoskopie und Szintigraphie). Bei umschriebener Verlustquelle muss eine intraoperative Enteroskopie erwogen werden. Kann der Proteinverlust nicht kausal behandelt werden, so wird eine Substitution von Albumin und Immunglobulinen in Abständen von 1-4 Wochen erforderlich. Die Prognose wird durch eine evtl. Grundkrankheit bestimmt und ist bei Proteinverlust als einzigem Symptom relativ gut.

Abstract

The protein-loosing enteropathy (PLE) may result from a broad variety of underlying diseases. These conditions are of systemic nature or locally affecting the gastrointestinal tract. Major symptoms are oedema due to low plasma protein levels. Gastrointestinal symptoms are not necessarily present. The diagnosis is confirmed by the finding of increased faecal concentrations of Alpha-1-Antitrypsin (> 320 mg/L). In the majority of cases, in which underlying diseases are present, the etiology is obvious. In unclear cases the differentiation into inflammatory or circulatory disturbances or alterations of the architecture of the basal membrane is helpful. An economic, staged approach is presented. To localize the site of protein loss imaging is required (abdominal ultrasound, CT-scan, endoscopy and Technetium-Scan). If a circumscribed intestinal source of protein loss is suspected which may be amenable to surgery, intraoperative enteroscopy should be considered. If causal treatment is impossible; intravenous replacement of albumin and immunoglobulines in intervals from 1 to 4 weeks will be necessary. The prognosis in patients with isolated PLE is good. Otherwise it depends on the underlying disease.

Schlüsselwörter

Enteropathie - Eiweißverlust

Key words

Protein-loosing enteropathy

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Lutz Bindl

Universitäts-Klinikum

Pauwelsstr. 30

52074 Aachen

Email: lbindl@web.de