Renal amyloidosis overview

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Renal amyloidosis Microchapters

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Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Renal amyloidosis from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

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CT

MRI

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Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

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Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

Historical Perspective

  • In 1969, Finnish-type familial amyloidosis (FAF) was first described as one of the causes of renal amyloidosis.[3]

Classification

Renal amyloidosis may be classified according to site of amyloid deposition into 3 subtypes:[4]

  • Glomerular amyloid deposition (more common and have a poorer prognosis)
  • Vascular amyloid deposition
  • Tubular amyloid deposition

Renal amyloidosis may be classified according to type of amyloidogenic protein into 3 subtypes:[5]

Amyloidosis type Amyloidogenic protein
Common types AL/AHL/AH

(Primary amyloidosis)

Ig light chains (AL)
Fragments of Ig heavy chains and light chains (AHL)
Fragments of heavy chains only (AH)
AA

(Secondary amyloidosis)

Serum amyloid A
Rare types AFib Fibrinogen A α chain
AApo AI/AII/AIV Apo AI, Apo AII, or Apo AIV
ATTR Transthyretin
ALys Lysozyme
AGel Gelsolin
ALECT2 Leukocyte chemotactic factor 2

Pathophysiology

Causes

Differentiating Renal amyloidosis from Other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications, and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X-ray

Echocardiography and Ultrasound

CT scan

MRI

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Interventions

Surgery

Primary Prevention

Secondary Prevention

References

  1. Kyle RA (June 2011). "Amyloidosis: a brief history". Amyloid. 18 Suppl 1: 6–7. doi:10.3109/13506129.2011.574354001. PMID 21838413.
  2. Sipe JD, Cohen AS (June 2000). "Review: history of the amyloid fibril". J. Struct. Biol. 130 (2–3): 88–98. doi:10.1006/jsbi.2000.4221. PMID 10940217.
  3. Yamanaka S, Miyazaki Y, Kasai K, Ikeda S, Kiuru-Enari S, Hosoya T (April 2013). "Hereditary renal amyloidosis caused by a heterozygous G654A gelsolin mutation: a report of two cases". Clin Kidney J. 6 (2): 189–93. doi:10.1093/ckj/sft007. PMC 4432447. PMID 26019848.
  4. Bilginer Y, Akpolat T, Ozen S (August 2011). "Renal amyloidosis in children". Pediatr. Nephrol. 26 (8): 1215–27. doi:10.1007/s00467-011-1797-x. PMC 3119800. PMID 21360109.
  5. Said SM, Sethi S, Valeri AM, Leung N, Cornell LD, Fidler ME, Herrera Hernandez L, Vrana JA, Theis JD, Quint PS, Dogan A, Nasr SH (September 2013). "Renal amyloidosis: origin and clinicopathologic correlations of 474 recent cases". Clin J Am Soc Nephrol. 8 (9): 1515–23. doi:10.2215/CJN.10491012. PMC 3805078. PMID 23704299.


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