By Higgins, Dr Rob
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Extra resources for VITAL CKD KEEPING KIDNEYS HEALTHY
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It is an inherited condition. Adults have multiple cysts in both kidneys and sometimes in the liver ■ Very small cysts are present at birth and they gradually enlarge. With the imaging available 25 years ago, it was said that there was no value in scanning children at risk of APCKD. However, modern imaging may detect some small cysts in the kidneys of children, or even in utero, meaning that the diagnosis can be made much earlier ■ APCKD classically has an autosomal dominant inheritance. In other words, each child of an affected parent has a one in two chance of having APCKD, and there are no hidden carriers.
In this case, the patient should keep themselves well-hydrated, and treatment of the underlying myeloma will be a priority for the nephrology and haematology teams 5 4 | V I TAL C K D Vital CKD Final 4/12/08 10:30 Page 55 A M YLO I DO SI S ■ Amyloid is protein deposited in tissues in a particular fashion (‘beta-pleated sheets’). The amyloid sits in tissues and interferes with organ function ■ In the kidney, amyloid can cause severe nephrotic syndrome and/or kidney failure, and is diagnosed on renal biopsy ■ The commonest cause of amyloid is a monoclonal gammopathy.
The reason for this is probably an effect on intra-renal blood flow over and above the effect on systemic blood pressure, further reducing the strain on glomeruli ■ However, they may also cause a fall in eGFR/rise in blood creatinine level or hyperkalaemia ■ The level of eGFR by itself is not a contraindication to the initiation of ACEI/ARB therapy. Known or suspected renal artery stenosis (eg if the patient is a known arteriopath and smoker) is a contraindication to the initiation of these agents, though some patients with renal artery stenosis may tolerate the agents and nephrologists will sometimes continue them in selected patients.