Case Report: Membranoproliferative Glomerulonephritis, a Rare Clinical Manifestation of Abernethy Malformation Type

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Xue He1, Yueling Zhu2, Haidong Fu1

  • 1Department of Nephrology, National Clinical Research Center for Child Health, National Children's Regional Medical Center, The Children's Hospital, Zhejiang University School of Medicine, Hangzhou, China.

Frontiers in Pediatrics
|April 5, 2021
PubMed

Insights

This case report details a rare instance of Abernethy malformation type II in an 8-year-old boy, presenting with glomerulonephritis and unique neurological symptoms. The findings highlight a potential link between congenital vascular anomalies and renal disease.

Area of Science:

  • Pediatric Nephrology
  • Vascular Malformations
  • Neurology

Background:

  • Abernethy malformation is a rare congenital portosystemic shunt.
  • It is classified into type I (end-to-side) and type II (side-to-side) shunts.
  • Glomerulonephritis associated with Abernethy malformation is extremely rare.

Observation:

  • An 8-year-old male presented with systemic edema, heavy proteinuria, hypoproteinemia, and hypocomplementemia.
  • Imaging revealed arachnoid cysts and focal nodular hyperplasia.
  • Renal biopsy confirmed immune complex-mediated membranoproliferative glomerulonephritis with "full-house" deposits.

Findings:

  • The patient was diagnosed with Abernethy malformation type II.
  • Immune complex-mediated glomerulonephritis was confirmed via renal biopsy.
  • This case may be the first to report arachnoid cysts as a symptom of Abernethy malformation.

Implications:

  • This case underscores the association between congenital portosystemic shunts and renal pathology.
  • It highlights the potential for neurological manifestations, such as arachnoid cysts, in Abernethy malformation.
  • Further research is needed to determine optimal management strategies, including potential surgical intervention.
Keywords:
Abernethy malformationarachnoid cystcongenital extrahepatic portosystemic shuntfocal nodular hyperplasiamembranoproliferative glomerulonephritis

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