Severe ileocecal inflammatory syndrome in adult patients with cystic fibrosis

Abstract

The relevance of gastrointestinal manifestations of cystic fibrosis (CF) is increasing due to an improved life expectancy. We report on 2 adult patients with prior lung transplantation who presented with a severe inflammatory disorder of the ileocecal region. One patient underwent ileocecal resection; the second patient died after emergency surgery for intestinal perforation. Both cases did not show typical signs of CF-related distal intestinal obstruction syndrome or extensive fibrosing colonopathy. However, the clinical and histopathological findings revealed CF-induced inflammatory alterations of the intestinal mucosa. Thus, these cases illustrate a further CF-related bowel disorder, which can be especially relevant in long-term CF survivors.

Zusammenfassung

Aufgrund der gestiegenen Lebenserwartung von Patienten mit Zystischer Fibrose (CF) nimmt die Bedeutung gastrointestinaler Manifestationen der Erkrankung zu. In zwei Fallberichten stellen wir erwachsene, lungentransplantierte CF-Patienten mit ausgeprägten inflammatorischen Veränderungen der Ileozökalregion vor, welche letztlich einer operativen Versorgung zugeführt werden mussten. In beiden Fällen konnten keine eindeutigen Zeichen eines distalen intestinalen Obstruktionssyndroms oder einer zugrundeliegenden fibrosierenden Kolonopathie festgestellt werden. Dennoch sprechen sowohl das klinische Erscheinungsbild als auch die histopathologischen Befunde für das Vorliegen einer CF-induzierten entzündlichen intestinalen Erkrankung. Daher muss von einer bisher nicht ausreichend charakterisierten CF-Manifestation des Intestinums ausgegangen werden, welche besonders für erwachsene CF-Patienten von klinischer Relevanz ist.

Key words

CF - fibrosing colonopathy - immunosuppression - lung transplantation pancreatin supplementation

Schlüsselwörter

CF - fibrosierende Kolonopathie - fibrosing Immunosuppression - Lungentransplantation - Pankreatinsubstitution

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