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Abstract: TH-PO529

Updates on Renal Amyloidosis in Children and Adolescents

Session Information

  • Pediatric Nephrology - I
    November 02, 2023 | Location: Exhibit Hall, Pennsylvania Convention Center
    Abstract Time: 10:00 AM - 12:00 PM

Category: Pediatric Nephrology

  • 1900 Pediatric Nephrology


  • Shaoba, Asma B S, SUNY Downstate Health Sciences University, New York City, New York, United States
  • Bamgbola, Oluwatoyin F., SUNY Downstate Health Sciences University, New York City, New York, United States

Amyloidosis affects individuals with eastern Mediterranean ethnicity; however, worldwide manifestation is frequent because of worldwide human migration. Although renal amyloidosis (RA) is a potentially fatal disease, it is relatively rare in children and adolescents. Its early manifestation as nephrotic syndrome may be mistaken for a minimal change disease. Familiarity with its geographical spread and pattern of presentation may enhance diagnostic awareness. For this purpose, we embarked on a PubMed search for articles on pediatric RA.


We searched the PubMed and Embase databases from 1960 to 2022 for relevant original articles including case reports and case series on RA in children and adolescents.


RA occurred (n = 679) commonly among a population affected by familial Mediterranean fever (FMF) (n = 2812) who are mostly residents of Turkey (n = 1907) and Armenia (n = 836). The second most common cause of RA was idiopathic juvenile arthritis (JIA) (n = 139). Other reported causes were tuberculosis (TB) (n = 10) and epidermolysis bullosa (EB) (n = 13). In general, there was often renal remission following early diagnosis and treatment of FMF, JIA, and TB. The poor response to treatment in EB frequently resulted in end-stage kidney disease and fatal outcomes. There were only 6 cases of hereditary RA, three of which were due to ALys amyloid deposition. There was a single report of ALECT2 disease.


Most cases of pediatric RA were due to auto-inflammatory syndrome. Acquired causes were mostly due to JIA. Early treatment frequently resulted in renal remission. Finally, resources for modern genetic diagnosis most likely influenced the prevalence and geographical spread of the disease.

Picken MM. Current Understanding of Systemic Amyloidosis and Underlying Disease Mechanisms. Am J Cardiol. 2022;185: S2-S10.