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Wiener Medizinische Wochenschrift

, Volume 160, Issue 15–16, pp 431–436 | Cite as

Digital clubbing in primary intestinal lymphangiectasia: a case report

  • Christian J. WiedermannEmail author
  • Michael Kob
  • Stefano Benvenuti
  • Rodolfo Carella
  • Lucio Lucchin
  • Lucia Piazzi
  • Fausto Chilovi
  • Guido Mazzoleni
Case report

Summary

Primary intestinal lymphangiectasia (PIL), also known as Waldmann's disease, is a rare disorder characterized by dilated intestinal lacteals resulting in lymph leakage into the small bowel lumen and responsible for protein-losing enteropathy leading to lymphopenia, hypoalbuminemia and hypogammaglobulinemia. The symptoms usually start in early infancy. We report a case of secondary hyperparathyroidism, osteopenia, monoclonal gammopathy and digital clubbing in a 57-year-old patient with a 12-year history of discontinuous diarrhea. Malabsorption with inability to gain weight, and finally weight loss and formation of leg edema were associated with protein-losing enteropathy. A low-fat diet associated with medium-chain triglyceride supplementation was clinically effective as medical management in reducing diarrhea and leg edema, and promoting weight gain. Double-balloon enteroscopy and small bowel biopsy histopathology confirmed dilated intestinal lacteals. Digital clubbing associated with primary intestinal lymphangiectasia which may causally be related to chronic platelet excess has not been reported before.

Keywords

Intestinal lymphangiectasia Protein-losing enteropathy Hypoproteinemia Hypogammaglobulinemia Medium-chain triglycerides Platelet crit Digital clubbing 

Trommelschlägelfinger und Uhrglasnägel bei primärer intestinaler Lymphangiektasie: ein Fallbericht

Zusammenfassung

Die primäre intestinale Lymphangiektasie ist eine seltene Erkrankung, die durch erweiterte Lymphgefäße der Dünndarmschleimhaut gekennzeichnet ist und zum Verlust von Lymphe in das Darmlumen führt. Die so entstehende Eiweiß-Verlust-Enteropathie ist labordiagnostisch durch Lymphopenie, Hypoalbuminämie und Hypogammaglobulinämie gekennzeichnet. Symptome beginnen für gewöhnlich in der frühen Kindheit. Hier berichten wir über einen Fall von Hypoalbuminämie und Hypogammaglobulinämie bei einem 57-jährigen Patienten mit 12-jähriger Anamnese von Diarrhoe, welche zu fehlender Gewichtszunahme, sekundärem Hyperparathyreoidismus mit Osteopenie, Beinödemen, monoklonaler Gammopathie, Trommelschlegelfingern und Uhrglasnägeln und zuletzt Gewichtsverlust geführt hat. Eine Fett-arme Diät ergänzt durch mittelkettige Triglyzeride war als therapeutische Intervention erfolgreich mit Reduktion von Diarrhoe, Verschwinden der Beinödeme und Gewichtszunahme. Dünndarm-Schleimhautbiopsien aus einer Doppel-Ballon-Endoskopie bestätigten die Diagnose mit der Darstellung erweiterter Lymphgefäße der Zotten. Uhrglasnägel und Trommelschlegelfinger wurden bislang in der medizinischen Literatur noch nicht mit primärer intestinaler Lymphangiektasie assoziiert und könnten auch hier mit chronischem Thrombozyten-Exzess in Zusammenhang stehen, der beim Patienten zumindest über die letzten 4 Jahre verfügbarer Beobachtungszeit nachweisbar war.

Schlüsselwörter

Intestinale Lymphangiektasie Eiweiß-Verlust-Enteropathie Hypoproteinämie Hypogammaglobulinämie Mittelkettige Triglyzeride Plateletcrit Uhrglasnägel Trommelschlegelfinger 

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Christian J. Wiedermann
    • 1
    Email author
  • Michael Kob
    • 2
  • Stefano Benvenuti
    • 3
  • Rodolfo Carella
    • 4
  • Lucio Lucchin
    • 2
  • Lucia Piazzi
    • 3
  • Fausto Chilovi
    • 3
  • Guido Mazzoleni
    • 4
  1. 1.Division of Internal MedicineCentral HospitalBolzanoItaly
  2. 2.Division of Dietetics and Clinical NutritionCentral HospitalBolzanoItaly
  3. 3.Division of GastroenterologyCentral HospitalBolzanoItaly
  4. 4.Division of Anatomy and HistopathologyCentral HospitalBolzanoItaly

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