A rare cause of AA amyloidosis and end-stage kidney failure: Answers

Taşdemir, Mehmet; Yılmaz, Sezen; Baba, Zeliha Füsun; Bilge, İlmay

doi:10.1007/s00467-018-4153-6

Cited by 1 publication

(1 citation statement)

References 12 publications

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“…Although there are previous reports claiming regression of proteinuria by biologic treatments, there is no evidence of ceased progression of amyloidosis at the tissue level. 1,5,[15][16][17] The decrease in proteinuria may evoke regression of amyloid deposition. However, both Topaloglu et al 16 and Yıldırım et al 18 could not demonstrate a significant change in extent of amyloid accumulation in control renal biopsy even though there was complete remission of proteinuria, just like in our patient.…”

Section: Discussionmentioning

confidence: 99%

AA amyloidosis presenting with acute kidney injury, curable or not?

Uysal¹,

Vuruşkan²

2022

Turk J Pediatr

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Background. Amyloidosis is a group of disorders with extracellular accumulation of autologous fibrillary insoluble proteins in various tissues and organs such as the kidneys, liver, spleen, heart and gastrointestinal tract leading to impairment of normal organ function. Childhood amyloidosis is an exceedingly rare entity mainly caused by familial Mediterranean fever (FMF) and the other autoinflammatory diseases such as mevalonate kinase deficiency (MKD). Case. A 16-year-old male was referred to pediatric nephrology for coincidentally discovered proteinuria. He had no significant findings on physical examination except for urochromic color. He had nephrotic range proteinuria with 109 mg/m2/h and serum creatinine was 1.35 mg/dl. Kidney biopsy was performed because of nephrotic range proteinuria with acute kidney injury. In hematoxylin-eosin-stained tissue sections, amyloid was suggested as extracellular amorphous material that is lightly eosinophilic in the glomeruli. Diagnosis was confirmed by Congo red positivity, with apple-green birefringence under polarized light. MEFV gene mutation was negative and a compound heterozygote mutation found in mevalonate kinase gene. A 6-monthtrial of colchicine, enalapril, and losartan combination was not successful; Canakinumab was started thereafter. Proteinuria and creatinine decreased to 7 mg/m2/h and 0.6 mg/dl respectively 4 years after treatment. Conclusions. Amyloidosis should be considered especially in children presenting with proteinuria and with a history of recurrent fever. This report also emphasizes the efficacy of canakinumab to prevent or decelerate chronic renal failure in these patients although it does not reduce tissue deposition in long-term use.

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