Immunodeficiency due to chylous dysplasia: Diagnostic and therapeutic considerations

Campisi Corradino, Campisi Corrado, Girino Margherita, Vidali Francesca, Corazza Gino Roberto, Bellini Carlo, Boccardo Francesco

Research output: Contribution to journalArticlepeer-review

Abstract

Among primary immunodeficiencies, common variable immunodeficiency (CVID) is defined by an impaired production of immunoglobulins, characterized by low levels of plasma immunoglobulins and altered antibody response. The case reported was initially interpreted as a CVID. A male, 20 years old, suffered from diarrhoea (2-4 times a day), weight loss (8 kilos in 5 years), and malnutrition (hypogammaglobulinemia, hypoalbuminemia, leukocytopenia with lymphocytopenia). Accurate diagnostic assessment allowed to diagnose a protein-losing enteropathy. Conventional oil contrast lymphangiography allowed to accurately assess the case and to establish a proper therapeutic approach. The operation consisted in multiple antigravitational ligatures of dilated and incompetent chylous vessels and chylous vessel-mesenteric vein microanastomoses. Parameters concerning albumin and leukocytes normalized in 1 week after operation and remained stable with time, there were no more episodes of diarrhoea and the patient recovered weight. An accurate diagnostic assessment and above all lymphangiography allow to diagnose properly difficult cases of immunodeficiency due to intestinal protido-dispersion and to plan a correct therapeutic functional approach.

Original languageEnglish
Pages (from-to)21-24
Number of pages4
JournalEuropean Journal of Lymphology and Related Problems
Volume21
Issue number59
Publication statusPublished - 2010

Keywords

  • Chylous dysplasia
  • Derivative chylous-venous shunts
  • Immunodeficiency
  • Microsurgery
  • Protein losing enteropathy

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine

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