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  1. Fulltext Renal-limited AL amyloidosis - a diagnostic and management dilemma
    Fuah KW, Lim CTS
    BMC Nephrol, 2018 11 06;19(1):307.
    PMID: 30400895 DOI: 10.1186/s12882-018-1118-8
    BACKGROUND: Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils which may result in a wide spectrum of symptoms depending upon their types, sites and amount of deposition. Amyloidosis can be divided into either systemic or localized disease.

    CASE PRESENTATION: We present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function. Repeated renal biopsies showed the presence of renal-limited AL amyloidosis. Systemic amyloidosis workup was unremarkable apart from a slightly raised band of IgG lambda level with no associated immunoparesis. The nephrotic syndrome and renal histology did not improve over a 3-year period despite being given two courses of chemotherapies.

    CONCLUSION: We hope that early recognition of this unusual localised presentation of renal- limited AL Amyloidosis and its poor response to conventional treatment can alert the nephrologist to the potential existence of this rare condition.

    Matched MeSH terms: Amyloidosis/therapy
  2. Fulltext Aerodigestive amyloidosis presenting as acute asthma
    Sreetharan SS, Prepageran N, Razak A, Valuyeetham KA
    Med J Malaysia, 2003 Jun;58(2):290-3.
    PMID: 14569753
    Aerodigestive amyloidosis is a rare disorder characterized by fibrillar protein deposition in the aerodigestive tree. We present a case of a 19-year-old Chinese gentleman whose diagnosis was initially missed as he presented with features suggestive of severe bronchial asthma and was intubated and ventilated. He subsequently presented 2 years later with severe stridor and required emergency tracheostomy. Current literature is reviewed for the histopathology, common clinical features, radiological findings and treatment options for aerodigestive amyloidosis.
    Matched MeSH terms: Amyloidosis/therapy
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