The Atypical HUS Foundation

aHUS Complications: Potential Impact for Multiple Organs

 Atypical HUS - It's Not Just About Kidneys

      The topic of aHUS complications has surfaced in multiple commentary, including aHUS community discussions that have referred to potential effects of aHUS damage that impact other vital organs and patient health.  Multi-organ involvement is referenced by multiple researchers, and patients/families/medical personnel may access such information via links here as a starting point to further explore the topic.  Provided via aHUS Source, a website provided by Alexion Pharmaceuticals (makers of Soliris, or eculizumab), you'll find "aHUS Clinical Complications" which notes among its information that  48% of aHUS patients experience neurological symptoms, 43% of aHUS patients experience problems with their cardiovascular systems, and about 30% of patients experience complications associated with the digestive system such as an inflamed colon, abdominal pain, or other issues involving the digestive tract.  This information is accompanied by solid citations from specific research studies and also from articles in medical journals by noted aHUS clinicians and investigators. (Source)  

 

     Patients with atypical HUS may experience damage throughout the body, not just the kidneys, with possible damage to such vital organs as the heart, bowel, or brain.  A visual representation that kidney issues are just 'the tip of the iceberg' regarding multi-organ involvement is noted by by a graphic at aHUS Source .  Visitors to Alexion’s aHUS Source site are encouraged to view the compilation of sources cited by Alexion as they note, " ....see the references used in outlining early symptoms, please view our reference list. " where they've made available in a comprehensive list of international references specific to aHUS research. 

 

     Those interested in further details regarding this particular atypical HUS info, Soliris (eculizumab), or Alexion's website (including Alexion's other site www.soliris.net) may contact 1-888-SOLIRIS for further information.  The Foundation for Atypical HUS (as well as this website) is an independent organization that strongly suggests that atypical HUS patients and their families connect with their own physicians and treatment teams to determine what is in the patient's best interests given each individual's status regarding disease diagnosis, treatments, and situation.

Notes

On June 17, 2012 Alexion Pharmaceuticals released a Business Wire titled : "New Data Show Majority of Patients with aHUS Experienced Systemic Multi-Organ Complications Prior to Treatment" viewable on their corporate website at http://alxn.newshq.businesswire.com/press-release/product-news/new-...

 

ASH Conference 2013

Abstract 2184: “Biomarkers of Complement and Endothelial Activation, Inflammation, Thrombosis, and Renal Injury in Patients with Atypical Hemolytic Uremic Syndrome (aHUS) Treated with Eculizumab,” Cofiell, et al.

Accessible at: https://ash.confex.com/ash/2013/webprogram/Paper63810.html

 

Research Article:

Extra-renal manifestations of complement-mediated thrombotic microangiopathies

Hofer et al., Frontiers in Pediatrics (Sept. 2014) reviews published data on aHUS manifestations in organs other than the kidney, and suggests screening.  Thanks to Grace for providing this information to the aHUS community.

 

 

 

 

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I found the symptom checker on the Alexion website to take to our next appointment. It highlights the low level symptoms my son has been experiencing including a puffy face and hopefully the Drs will understand my concerns that he is experiencing low level compliment activity.
Thanks for the info Linda

I just added a recently published paper (open access, Hofer et al., Frontiers in Pediatrics 2014) to the research forum which reviews published data on aHUS manifestations in organs other than the kidney, and suggests screening. Organ involvement can be subtle or challenging to diagnose, and possibly under-diagnosed and under-reported. So we need more awareness and better biomarkers, but at least we seem to moving toward those goals!

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Did you know...

CFH (Serum Complement Factor H) is a regulatory protein. The secreted protein product of CFH consists of 20 repetitive units named "short consensus repeats" or SCRs (each approximately 60 amino acids). In patients with aHUS the last 5 "pearls" in the twenty pearl strand protein, SCR16 - SCR20, should bind to protect cells but do not- they are defective in one or more of the last 5 SCR locations. If they cannot bind or stick to the kidney to protect that tissue, the platelets clump into clots that affect the glomeruli of the kidney -potentially causing acute renal failure.
  
• • • • • • • • • • • •
  
It is estimated that there are about 2 cases of aHUS in the U.S. per 1,000,000 of population, and about 60% of aHUS patients are diagnosed as children. The condition is potentially life threatening, and either can be chronic or can recur at intervals.
  
more factoids...

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