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Chapters

  • 00:00:15
    The advice for the nephrologist to diagnose AL amyloidosis
  • 00:00:19
    earlier is severe nephrotic syndrome or massive proteinuria,
  • 00:00:27
    again, in the absence of diabetes, hypertension, rheumatologic conditions,
  • 00:00:34
    specifically if nephrotic syndrome is associated with massive albuminuria
  • 00:00:40
    of greater than 5 grams, greater than 10 grams.
  • 00:00:44
    Patients also have other organ system involvement with congestive heart failure,
  • 00:00:49
    autonomic neuropathy leading to postural hypotension, or low blood pressures.
  • 00:00:55
    Again, this constellation of these syndromes and these clinical features
  • 00:00:59
    should lead a nephrologist to suspect a systemic disease
  • 00:01:05
    and maybe a renal biopsy should be performed sooner
  • 00:01:09
    rather than later to diagnose AL amyloidosis.
  • 00:01:14
    Furthermore, 10% of patients with renal AL amyloidosis may not have
  • 00:01:20
    nephrotic syndrome, may not have albuminuria, or albumin spillage in the urine.
  • 00:01:26
    They could present just with worsening of renal function.
  • 00:01:30
    And in those cases, as well, renal biopsy would make the diagnosis
  • 00:01:35
    of AL amyloidosis with amyloid deposits
  • 00:01:38
    in the tubulointerstitial region rather than the glomeruli.
  • 00:01:48
    A lot, you know, cardiologists and nephrologists,
  • 00:01:50
    along with hematologists and neurologists
  • 00:01:52
    work very closely together, especially at the
  • 00:01:56
    Boston University Amyloidosis Center,
  • 00:01:58
    because the key to diagnosing AL cardiac amyloidosis is
  • 00:02:03
    is to recognize that these organs are being involved simultaneously.
  • 00:02:07
    First of all, realize that this is somebody who has worsening heart failure
  • 00:02:11
    with thick heart walls, who also has protein in their urine,
  • 00:02:15
    and all at once the differential shifts from
  • 00:02:19
    run-of-the-mill heart failure to something that is more,
  • 00:02:23
    that is rarer, like a systemic AL amyloidosis.
  • 00:02:27
    And so that happens all the time, and it involves a collaboration
  • 00:02:30
    between the nephrologist, the kidney specialist,
  • 00:02:33
    and the heart specialist to put their heads together and realize that
  • 00:02:36
    systemic AL amyloidosis is the common theme here.