Dense deposit disease

What causes dense deposit disease?

Dense deposit disease (DDD) can have genetic or non-genetic causes. When the condition is genetic, it may be associated with changes in several genes. Mutations that cause the condition have been identified in the C3 and CFH genes, but they account for only a small percentage of all cases. There are also variants of some genes ( C3, CFH and CFHR5 ) that do not directly cause the condition, but increase the likelihood of developing the condition. Most people with these variants do not develop the condition.

Most people with DDD do not have disease-causing mutations in the C3, CFH, or CFHR5 genes. The condition may develop due to a combination of genetic and environmental risk factors ("triggers"), most of which are unknown. The condition can also be caused by the presence of specific proteins called autoantibodies that block the activity of proteins needed for the body's immune response.

Last updated on 05-01-20

Is dense deposit disease inherited?

Most cases of dense deposit disease (DDD) are not inherited, but occur sporadically (in people with no history of the condition in the family). Only a few reported families have had more than one affected family member. The genetics of DDD is complex, and in many cases, its inheritance it is not completely understood. For these reasons, the exact recurrence risk for family members of most affected people is not known but is likely very low.

In people with DDD that are known to have disease-causing mutations in the CFH gene, the inheritance pattern is autosomal recessive. The children of a person with autosomal recessive DDD will definitely be carriers of the condition. Carriers of autosomal recessive conditions typically do not have any signs or symptoms. Genetic carrier testing for at-risk family members is possible if the two CFH mutations have been identified in the affected family member.

DDD has been seen in families in which other relatives have autoimmune disorders, such as celiac disease and type 1 diabetes mellitus.

Individuals who have questions or concerns about the risk to themselves or family members should speak with a genetics professional.

Last updated on 05-01-20

How might dense deposit disease be treated?

There is currently no specific treatment for dense deposit disease (DDD), but therapies are available to help slow the progression of the condition through aggressive blood pressure control and reduction of proteinuria. Both angiotensin-converting enzyme (ACE) inhibitors and angiotensin II type-1 receptor blockers (ARBs) can be used to decrease the amount of protein in the urine and improve kidney function. These drugs may also limit the infiltration of white blood cells into the kidney. If hyperlipidemia (increased lipid in the blood stream) is present, lipid-lowering drugs may be used to reduce long-term risks of atherosclerosis. These drugs may also delay progression of kidney disease. Steroid therapy was widely used in the past, but more recent studies have shown that it is probably not effective for DDD. Steroid therapy is effective in a form of glomerulonephritis called juvenile acute non-proliferative glomerulonephritis (JANG), which is sometimes confused with DDD.

People with DDD who develop end-stage renal disease typically need peritoneal dialysis or hemodialysis. Kidney transplant may be an option for some individuals; however, DDD will still develop in virtually all transplanted kidneys and about half of transplants will ultimately fail. There is some evidence that the likelihood of transplant failure due to recurrent DDD decreases with time.

Last updated on 05-01-20

Where To Start

NephCure Kidney International

The NephCure Foundation provides information and resources for Dense deposit disease

Last updated on 04-27-20

Name: National Kidney Foundation 30 East 33rd Street
New York, NY, 10016, United States
Phone: 212-889-2210 Toll Free: 800-622-9010 Fax : 212-689-9261 Email: info@kidney.org Url: https://www.kidney.org/
Name: American Association of Kidney Patients 3505 E. Frontage Rd., Suite 315
Tampa, FL, 33607-1796, United States
Phone: 813-636-8100 Toll Free: 800-749-2257 Fax : 813-636-8122 Email: info@aakp.org Url: http://www.aakp.org
Name: The Kidney Foundation of Canada 700-15 Gervais Drive Toronto Ontario M3C 1Y8
Canada
Phone: 416-445-0373 Toll Free: 800-387-4474 Fax : 416-445-7440 Email: kidney@kidney.on.ca Url: http://www.kidney.on.ca
Name: American Kidney Fund, Inc. 6110 Executive Boulevard Suite 1010
Rockville, MD, 20852, United States
Phone: 301-881-3052 Toll Free: 866-300-2900 Email: helpline@kidneyfund.org Url: http://www.kidneyfund.org
Name: NephCure Kidney International 150 S. Warner Road Suite 402
King of Prussia, PA, 19406,
Phone: 1-866-NephCure (637-4287) Email: info@nephcure.org Url: https://nephcure.org/
Name: National Kidney Federation NKF The Point Shireoaks
Coach Road Worksop Notts S81 8BW
United Kingdom
Phone: 0845 601 02 09 Url: http://www.kidney.org.uk/
Name: Kidneeds Email: kidneedsMPGN@yahoo.com Url: https://www.healthcare.uiowa.edu/kidneeds/

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The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

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