Measurement of the C1 esterase inhibitor (the first component of the complement) is used to diagnose hereditary angioedema and to monitor levels of the inhibitor during treatment.
What is hereditary angioedema?
Hereditary angioedema is a disorder characterized by recurrent episodes of severe swelling (= angioedema). The most common areas of the body to develop swelling are the limbs, face, intestinal tract, and airway. Minor trauma or stress may trigger an attack, but swelling often occurs without a known trigger. Episodes involving the intestinal tract cause severe abdominal pain, nausea, and vomiting. Swelling in the airway can restrict breathing and lead to life-threatening obstruction of the airway. About one-third of people with this condition develop a non-itchy rash called erythema marginatum during an attack.
Symptoms of hereditary angioedema typically begin in childhood and worsen during puberty. On average, untreated individuals have an attack every 1 to 2 weeks, and most episodes last for about 3 to 4 days. The frequency and duration of attacks vary greatly among people with hereditary angioedema, even among people in the same family.
There are three types of hereditary angioedema, called types I, II, and III, which can be distinguished by their underlying causes and levels of a protein called C1 inhibitor in the blood. The different types have similar signs and symptoms. Type III was originally thought to occur only in women, but families with affected males have been identified.
The most common form (85% of cases) of hereditary angioedema is due to an absolute decrease in the amount of C1 esterase inhibitor. A less common form (15% of cases) is due to the functional abnormality of the inhibitor where levels may be normal. Both abnormalities need to be tested because of the potentially life-threatening nature of the disease.
In addition to the decreased serum esterase C1 inhibitor in patients with hereditary angioedema, there is still a unique quinine polypeptide at elevated concentration during the edema crisis. Patients with episodes of hereditary angioedema also have a low total complement and C4 and C2 components of the complement. Hereditary angioedema is transmitted in an autosomal dominant manner. Heterozygotes also have reduced levels of the C1 esterase inhibitor. During acute attacks of the disease, complement components of C4 and C2 may be significantly reduced, but C1 and C3 components are normal. The factors that promote the onset of angioedema attacks are often unknown.
Notes:
- Angioedema can also occur as an acquired disease. The acquired form includes non-hereditary C1 esterase deficiency and may be caused by medications, allergies, idiopathic forms, angioedema associated with autoimmune disease especially with systemic lupus erythematosus and hypereosinophilia, angioedema occasionally associated with malignancy, and angioedema caused by natural stimuli.
- Abnormally low results are consistent with a heterozygous C1 esterase inhibitor deficiency and hereditary angioedema.
- Fifteen percent of hereditary angioedema patients have a normal or elevated level but nonfunctional C1 esterase inhibitor protein. Detection of these patients requires a functional measurement of C1 esterase inhibitor; FC1EQ / C1 Esterase Inhibitor, Functional Assay, Serum.
- Measurement of C1q antigen levels; C1Q / Complement C1q, Serum, is key to the differential diagnoses of acquired or hereditary angioedema. Those patients with the hereditary form of the disease will have normal levels of C1q, while those with the acquired form of the disease will have low levels.
- Studies in children show that adult levels of C1 inhibitor are reached by 6 months of age.
References:
Atkinson JP, Du Clos TW, Mold C, Kulkarni H, Hourcade D, Wu X. The human complement system: basic concepts and clinical relevance. In: Rich RR, Fleisher TA, Shearer WT, Schroeder HW, Frew AJ, Weyand CM, eds. Clinical Immunology: Principles and Practice. 5th ed. Philadelphia, PA: Elsevier; 2019:chap 21.
Cicardi M, Aberer W, Banerji A, et al. Classification, diagnosis, and approach to treatment for angioedema: consensus report from the Hereditary Angioedema International Working Group. Allergy. 2014;69(5):602-616. PMID: 24673465 www.ncbi.nlm.nih.gov/pubmed/24673465/.
Leslie TA, Greaves MW. Hereditary angioedema. In: Lebwohl MG, Heymann WR, Berth-Jones J, Coulson IH, eds. Treatment of Skin Disease: Comprehensive Therapeutic Strategies. 5th ed. Philadelphia, PA: Elsevier; 2018:chap 101.
Zanichelli A, Azin GM, Wu MA, et al. Diagnosis, course, and management of angioedema in patients with acquired C1-inhibitor deficiency. J Allergy Clin Immunol Pract. 2017;5(5):1307-1313. PMID: 28284781 www.ncbi.nlm.nih.gov/pubmed/28284781/.
Frank MM: Complement deficiencies. Pediatr Clin North Am. 2000;47(6):1339-1354
Gelfand JA, Boss GR, Conley CL, et al: Acquired C1 esterase inhibitor deficiency and angioedema: a review. Medicine. 1979;58(4):321-328
Rosen FS, Alper CA, Pensky J, et al: Genetically determined heterogeneity of the C1 esterase inhibitor in patients with hereditary angioneurotic edema. J Clin Invest. 1971;50(10):2143-2149
Frigas E: Angioedema with acquired deficiency of the C1 inhibitor: a constellation of syndromes. Mayo Clin Proc. 1989;64:1269-1275
Soldin SJ, Hicks JM, Bailey J, et al: Pediatric reference ranges for estradiol and C1 esterase inhibitor. Clin Chem. 1998;44(6s):A17
C1 esterase inhibitor (C1-INH) is a protein found in the fluid part of your blood. It controls a protein called C1, which is part of the complement system.
The complement system is a group of nearly 60 proteins in blood plasma or on the surface of some cells. The complement proteins work with your immune system to protect the body from infections. They also help remove dead cells and foreign material. There are nine major complement proteins. They are labeled C1 through C9. Rarely, people may inherit deficiency of some complement proteins. These people are prone to certain infections or autoimmune disorders.
Import lab results from multiple providers, track changes over time, customize your reference ranges, and get clear explanations for each result. Everything is stored securely, exportable in one organized file, and shareable with your doctor—or anyone you choose.
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Low levels of C1-INH may cause certain types of angioedema. Angioedema results in sudden swelling of the tissues of the face, upper throat and tongue. It may also cause difficulty breathing. Swelling in the intestine and abdominal pain may also occur. There are two types of angioedema that result from decreased levels of C1-INH. Hereditary angioedema affects children and young adults under age 20. Acquired angioedema is seen in adults older than age 40. Adults with acquired angioedema are much more likely to also have other conditions, such as such as cancer or autoimmune disease.
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Easily upload lab results from any provider, whether it's a hospital, independent lab, or home testing service. We support PDFs, scanned documents, and JPEGs from patient portals. No need to log in to multiple platforms — everything is centralized in one secure space.
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Whether it's a blood test, GI panel, urinalysis, or something else, lab reports measure numerous biomarkers that reveal what's happening inside your body. Our extensive database covers over 10,000 biomarkers, providing clear, simple explanations of what each result means and how you can take action—no matter your membership level. Say goodbye to confusion and get the insights you need to better understand your health.
Our database covers over 10,000 biomarkers with clear, simple explanations—so you can finally understand your results and what to do next.
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Enter Your Lab Reports — Always Free
If you'd like to handle inputting your health data into your account, you can do so with our easy-to-use data entry forms. Our user-friendly form is designed to guide you through the quick and easy submission process, making it simple to keep track of your health metrics. This is available to both Complete plan and Unlimited plan members.
Our user-friendly form guides you through a quick, simple submission process, making it easy to enter your health metrics.
Add your results anytime with our easy entry form. It's quick, guided, and helps you stay organized — free for all members.
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View your lab data through easy-to-read graphs and tables. Quickly spot patterns, track changes, and compare results across different dates — all without digging through multiple reports. You can also select and compare graphs of specific biomarkers side-by-side to better understand how they relate and change over time.
See your lab reports in clear graphs and tables. Spot patterns, track changes, and compare results over time — all in one place.
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Export Your Complete Lab History
After collecting lab results from different providers, you can download your entire history combined into a single file. Choose from PDF, Excel, or CSV formats to easily review, share, or get a second opinion—no more juggling multiple reports.
After collecting lab results from different providers, you can download your entire history combined into a single file.
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Data Entry Service for Your Reports
Our inclusive service handles the data entry for your lab reports. Just submit your information and we'll take care of the rest. Complete plan members receive one report entered for free, then $15 per report after that. Unlimited plan members receive ten report entries for free, then $15 per report after that.
Our inclusive service handles the data entry for your lab reports. Just submit your information and we'll take care of the rest.
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Whether you're working with a doctor, nutritionist, caregiver, or wellness coach, you can securely share your complete lab history by sending an invite link to anyone you trust. You have full control over who sees your information and for how long, ensuring your privacy and peace of mind at every step.
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I have been using Healthmatters.io since 2021. I travel all over the world and use different doctors and health facilities. This site has allowed me to consolidate all my various test results over 14 years in one place. And every doctor that I show this to has been impressed. Because with any health professional I talk to, I can pull up historical results in seconds. It is invaluable. Even going back to the same doctor, they usually do not have the historical results from their facility in a graph format. That has been very helpful.
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As a PRO member and medical practitioner, Healthmatters.io has been an invaluable tool for tracking my clients' data. The layout is intuitive, making it easy to monitor trends and spot patterns over time. The ability to customize reports and charts helps me present information clearly to my clients, improving communication and outcomes. It's streamlined my workflow, saving me time and providing insights at a glance. Highly recommended for any practitioner looking for a comprehensive and user-friendly solution to track patient labs!
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Healthmatters is a personal health dashboard that helps you organize and understand your lab results. It collects and displays your medical test data from any lab in one secure, easy-to-use platform.
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Professionals can also analyze client data more efficiently and save time managing lab reports.
Healthmatters.io personal account provides in-depth research on 4000+ biomarkers, including information and suggestions for test panels such as, but not limited to:
You can combine all test reports inside your Healthmatters account and keep them in one place. It gives you an excellent overview of all your health data. Once you retest, you can add new results and compare them.
If you are still determining whether Healthmatters support your lab results, the rule is that if you can test it, you can upload it to Healthmatters.
While we work with many popular labs, we welcome reports from lots of other places too.
It's as simple as this: if you can get a test done, you can upload it to Healthmatters and we can interpret results from any lab out there. If laboratories can analyze it, we can interpret it.
If you're on the hunt for a specific biomarker, contact us and we'll add it to our database. Anything from blood, urine, saliva, or stool can be uploaded, understood, and tracked with your Healthmatters account.
The Complete Plan ($15/month) is perfect for individuals who want ongoing access to their health data. It includes unlimited lab imports, visual tracking, custom ranges, result explanations, full account exports, and secure sharing — all with a simple monthly subscription. You can cancel anytime and restart your plan whenever you're ready — your data will still be there waiting for you. You can also upgrade to the Unlimited Plan at any time, with the cost prorated based on what you've already paid.
The Unlimited Plan ($250 one-time) is also designed for individuals but offers lifetime access with no ongoing subscription. You'll get all the same features as the Complete Plan, plus a larger initial data entry allowance (10 reports), making it a great choice if you prefer a one-time payment and long-term use without monthly fees.
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There are two ways to add your test reports to your Healthmatters account. One option is to input the data using the data entry forms. The other method is to utilize our "Data entry service."
Our data entry forms offer an easy, fast, and free way for you to input the reports yourself. Self-entry allows you to add an unlimited number of reports at no cost. We make the self-entry process user-friendly, providing dozens of templates that pre-populate the most popular laboratory panels and offering instant feedback on entered values.
For those who prefer assistance, we offer a "Data entry service" to help you input your data. Simply attach an image or file of your lab test results, and a qualified team member from our data entry team will add the results for you.
We support various file types, including PDFs, JPGs, or Excel. This service is particularly useful if you have many reports to upload or if you're too busy to handle the data entry yourself.
Our Data Entry Service is for when you don't want to manually type in your lab results yourself. You simply upload your report (PDF, image, or screenshot), and our trained team enters the information into your Healthmatters account for you — accurately and neatly organized, ready to view in graphs, tables, and timelines.
The $15 per report covers the time and care it takes for a real person to review your file, make sure each result is entered correctly, and double-check for accuracy. This ensures your health data is precise and easy to work with — without you having to spend the time doing it yourself.
Prefer to do it yourself? You can always use our free self-entry tool to add results manually — it just takes a bit more time and attention.
For users on the Complete monthly plan, the first report is entered free of charge, and each additional report incurs a fee of $15.
Unlimited account holders enjoy the entry of ten reports without charge. Subsequent reports are subject to a $15 fee per report.
Additionally, users on the Complete plan can upgrade to a yearly subscription from the account settings. The annual subscription includes a data entry service for five reports.
All professional accounts allow you to import and onboard an unlimited number of clients and their lab results. The distinction between professional plans lies solely in the data entry service.
The Pro Monthly Plus plan is priced at $75 per month and includes a data entry service for five reports each month. Additional reports can be self-entered at no extra cost or, if preferred, you can use our data entry service for an additional fee of $15 per report.
The Pro Monthly plan is priced at $45 per month and does not include a data entry service. Self-entry is free for an unlimited number of reports, and you can opt for the data entry service at a fee of $15 per report.
You also have the option to upgrade to higher monthly or to annual plans, which come with substantial discounts. All upgrades can be done directly from your account.
Simply log in and navigate to your account settings to cancel your subscription. Scroll down to locate the 'Cancel' button at the bottom of the page. Ensure you cancel at least one day before the renewal date to prevent any charges. Once cancellation is requested, the subscription remains active until the conclusion of the current billing cycle.
Our goal has been to make your Healthmatters account as intuitive as possible.
We've crafted multiple ways for you to navigate your data, whether you're glancing at a single report or delving into your historical test reports.
1. Graph View:Dive into a visual journey with our biomarker graphs, showcasing over 40 data points. Combining years of results unveils trends, empowering you to make informed decisions. Our visualization tools make it a breeze to compare and understand changes over time, even if your results are from different labs. A search function and filters simplify the exploration of extensive data, allowing you to focus on what needs attention.
2. All Tests ViewExplore neatly organized reports on a timeline, highlighting crucial details like dates, critical results, and lab/panel names. Each report opens up to reveal in-depth descriptions and additional recommendations for each biomarker. The history of previous results is just a click away, and you can download a comprehensive report for deeper insights. Color-coded and user-friendly, it's designed for easy reading, understanding, and navigation.
3. Table View:For a holistic view of all biomarkers side by side, our table view is your go-to. Results are neatly displayed in a categorized and dated table, ideal for those with an extensive test history. Utilize sorting, filters, and color-coding to enhance your analysis and gain extra insights.
Yes, you can download your information anytime. We offer two easy ways to export your lab data:
This makes it simple to save, back up, or share your health data whenever you need.
Yes, you can print your report. To do so, navigate to "All tests" and open the report you wish to print. You'll find a print button in the right corner of the report. Click on it, and your browser's print window will open. If you prefer to print in a bigger typeface, adjust the scale using the print window settings.
Yes, you can! We highly recommend activating Two-Factor Authentication (2FA) for your account. To do so, please navigate to the "Profile and Security" section of your account, where you will find instructions for activating 2FA.
Yes, you can. When entering values for the biomarker, you will see an "Edit Range" button. Click this button, and you'll have the option to enter a custom range.
A personal account is all about keeping your own lab test results in check. It's just for you and your personal use.
The professional account is designed for health professionals who wish to track and organize their clients' laboratory results.
To learn more about Healthmatters Pro, please refer to the professional page.
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Optimal Result:
21 - 39 mg/dL.