Abernethy malformation type II with nephrotic syndrome and other multisystemic presentation: An illustrative case for understanding pathogenesis of extrahepatic complication of congenital portosystemic shunt

David F. Schaeffer, Simin Laiq, Hyun Jung Jang, Rohan John, Oyedele A. Adeyi

Research output: Contribution to journalArticlepeer-review

11 Scopus citations

Abstract

Abernethy malformation, an extrahepatic congenital portosystemic shunt, is more often diagnosed based on associated cardiac or pulmonary malformation. Although predominately a pediatric diagnosis, "late diagnoses" in adulthood have been reported especially in type II malformations that involve only a partial shunt of portal circulation directly into the inferior vena cava. Aside from the cardiac-related presentation, Abernethy malformation is also associated with multiple liver nodules, either benign or malignant, and pulmonary hypertension. In this report, we present immunoglobulin A glomerulonephritis with nephrotic syndrome as a hitherto unrecognized manifestation of this malformation outside the pediatric population, in a patient who also had pulmonary hypertension and multiple liver tumors. We also propose a pathogenetic basis for this multisystemic presentation that includes release into the systemic circulation of unfiltered bacteria, vasoactive substances, and immunoglobulin A-antigen complexes.

Original languageEnglish (US)
Pages (from-to)432-437
Number of pages6
JournalHuman pathology
Volume44
Issue number3
DOIs
StatePublished - Mar 1 2013

Keywords

  • Abernethy malformation type II
  • Congenital porto-systemic shunt
  • IgA glomerulonephritis
  • Nephrotic syndrome
  • Pathophysiology

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