What is Congenital Sucrase-Isomaltase Deficiency (CSID)?

by Valerie Polley | Jan 15, 2019 | 10 comments

I attended a three day GI conference at the University of Michigan in September. It was an amazing conference packed full of presentations from some of the best GI doctors and dietitians. One presentation was on a topic I had never heard of, so I was intrigued and ready to learn more about it.

​Congenital Sucrase-Isomaltase Deficiency (CSID) is a rare disorder that causes individuals the inability to digest certain sugars due to an absence or low levels of two digestive enzymes, sucrase and isomaltase. Both of these enzymes are involved in the digestion of sugar and starch. Table sugar is broken down into glucose and fructose with the help of the sucrase enzyme. Isomaltase is one of the several enzymes that help digest starches.

Both sucrose (found in fruits, and known as table sugar) and maltose (sugar found in grains) are disaccharides, which means they are made of two simple sugars. During digestion, intestinal enzymes break these down into simple sugars called monosaccharides. Sucrose=glucose + fructose and maltose=glucose + glucose. An individual with CSID has difficulty breaking down the disaccharides into monosaccharides that the body uses for fuel. These disaccharides are too large to be absorbed in the intestines by the microvilli (known as the brush border).

Starch can be a simple or complex carbohydrate. Simple carbohydrates have one or two sugar molecules, and complex carbohydrates have three or more sugars linked together.

Multiple enzymes are needed to properly digest starch. Digestion begins in the mouth with an enzyme in the saliva called amylase. Digestion continues in the stomach by further breaking down the food. In the small intestine, starch is processed by an enzyme called pancreatic amylase and converted into maltose and sucrose. The brush border contains numerous enzymes (sucrase, lactase, maltase) to continue the breakdown of digested food into smaller particles that can be better absorbed. The isomaltase enzyme breaks the bonds linking sugars into glucose, which can't be broken down by amylase or maltase. This is one of the final steps in the digestion of starch to glucose.

To sum it up, those with CSID lack the sucrase and isomaltase enzymes to properly break down the food to a simpler form, so the body can absorb it.

Without the proper enzymes, digestion can't take place correctly and individuals can have symptoms, such as:
-Chronic Diarrhea
-Abdominal Pain
-Abdominal Distention
-Excess Gas
-Weight Loss
-Constipation
-Acid Reflux
-Burping

So, what do you do if you suspect you have CSID?
Visit sucroseintolerance.com to take their quiz. On that site there is also an "ask your doctor" form to take to your physician. A sucrose breath test can help with the diagnosis. This can be ordered by your doctor or I can order a complimentary sucrose breath test to be sent to your home.

What if you have a positive breath test?
You will do a two-week elimination diet of sucrose and starch, followed by a gradual reintroduction of both. The reintroduction will help identify how much of these foods you can tolerate. Many like the guidance of a dietitian familiar with this deficiency to help them navigate through the elimination and reintroduction phases. Others also do the diet along with a test of the sucrase enzyme, Sucraid® (sacrosidase) Oral Solution. The sucrase enzyme can be purchased through One Patient Services, a subsidiary of QOL, Medical, LLC, the manufacturer of Sucraid® Oral Solution, at SucraidASSIST.com.

Please consult with your doctor regarding your symptoms and for a final diagnosis.

If you have any questions regarding Congenital Sucrase-Isomaltase Deficiency, please feel free to call or text me at 410-963-4782 or email at valerie@bluetreenutrition.com.

"Processed food" has become a catch-all term for foods we're told to avoid, but food processing isn't automatically a bad thing.

Technically, many foods you probably enjoy regularly are processed. Frozen vegetables, canned beans, yogurt, nut butter, and whole-grain bread have all been changed from their original state in some way.

So when research links processed foods to certain health concerns, it's important to understand what kind of processing we're talking about.

Much of the recent research has focused specifically on ultra-processed foods (UPFs). Diets containing large amounts of these foods have been linked to a range of negative health outcomes, including digestive and metabolic health issues, inflammation, and heart disease.

This article will review what we know so far and give you tips for including more whole foods in your diet.

Processed vs. Ultra-Processed Foods: Understanding the Difference

In food and nutrition research, a system called the NOVA classification is often used to group foods according to the type and extent of processing they undergo.

Minimally processed foods include fruits, vegetables, eggs, milk, and plain grains that may have been washed, frozen, pasteurized, dried, or otherwise prepared without significantly changing them from their original state.

Processed foods are generally made by adding ingredients such as salt, sugar, or oil. Examples include canned vegetables, cheeses, or freshly made breads.

Finally, ultra-processed foods are industrial formulations containing ingredients or additives not typically used in home cooking. Examples include many sodas, packaged snacks, instant noodles, candies, and some ready-to-eat meals.

This distinction is important because simply avoiding all processed foods would mean missing out on many convenient and nutritious options (such as canned beans and vegetables).

It's also important to recognize that there's considerable variation among foods categorized as UPFs. For example, a packaged whole-grain bread and a soda may both meet the criteria for ultra-processing under the NOVA classification, despite having very different nutritional profiles. 

Instead of deciding not to eat a food simply because it's processed, it can be more useful to look at the bigger picture and consider what nutrients the food provides and how it fits into your overall diet.

How Can Ultra-Processed Foods Affect Digestion?

While we're still uncovering exactly how UPFs affect the digestive system, one possibility is that they lack fiber.

Some diets high in UPFs are lower in naturally fiber-rich foods like fruits, vegetables, whole grains, beans, lentils, nuts, and seeds. You probably know fiber is important for staying regular, but certain types of fiber (known as prebiotics) act as food for the beneficial bacteria living in your gut. A diet lower in fiber may therefore affect both digestion and the health of your gut microbiome. 

There's also research currently examining the effects of common additives found in UPFs, such as certain emulsifiers and artificial sweeteners. However, most of the research linking these additives to negative effects on the gut microbiome and intestinal barrier has been conducted in vitro (test tube) or animal studies, so more research is needed before we can confirm whether these additives are harmful in humans.

Finally, some studies have found links between UPF intake and certain gut conditions. For example, a large systematic review and meta-analysis found that higher UPF consumption was associated with an increased risk of developing Crohn's disease.

However, there's still a lot we don't know about how UPFs impact gut health, and more research in humans is needed to understand which food components or other dietary factors may be responsible.

UPFs and Inflammation

Chronic, low-grade inflammation has been linked to the development of several chronic diseases, prompting researchers to investigate whether diets high in UPFs could contribute to inflammation.

Here are a few possible explanations:

  • A diet high in UPFs may contain fewer foods rich in fiber, vitamins, minerals, and phytochemicals, which are associated with better overall diet quality.
  • A diet high in UPFs can also contribute significant amounts of refined carbohydrates, saturated and trans fats, sodium, or added sugars.
  • Certain ingredients in UPFs may alter the gut microbiome and promote gut inflammation.

However, much of the evidence connecting UPF intake with inflammation and chronic disease is observational. Studies like these can identify patterns, but they can't always tell us whether UPFs specifically are responsible or whether other differences in diet and lifestyle contribute to the association.

UPFs and Overall Health

The research on UPFs becomes more conclusive when we zoom out to consider overall health.

A 2024 umbrella review published in The BMJ found that higher exposure to UPFs was linked with increased risk across several health outcomes, with some of the strongest evidence involving heart disease, type 2 diabetes, mental health outcomes, and overall death rates.

However, it's important to note that the quality of the studies included in the review varied considerably. Only four of the 45 studies they reviewed received a moderate-quality rating; most were rated low or very low quality.

That said, there is some experimental evidence to consider. A small but well-controlled study of 20 adults found that those eating UPFs consumed an average of 500 additional calories per day and gained about 2 pounds. They lost a similar amount of weight when eating the minimally processed diet. The study showed that something about the UPF dietary pattern encouraged greater calorie intake, but it couldn't identify exactly which UPF component was responsible.

Do You Need to Stop Eating UPFs?

UPFs can provide valuable nutrients, and all foods classified as UPFs are not nutritionally identical. Instead of trying to eliminate all foods that qualify as UPFs, consider your overall dietary pattern. For example:

  • Are you including fruits and vegetables throughout the day (fresh, frozen, or canned)
  • Are you choosing whole grains and other fiber-rich foods regularly?
  • Do you include beans, lentils, nuts, and seeds in your diet?
  • Do you compare packaged foods based on what nutrients they actually provide rather than assuming that processing alone determines their nutritional value?
  • Do you use convenience foods like canned beans and vegetables to make balanced meals easier, rather than feeling overwhelmed trying to prepare everything from scratch?

If UPFs currently make up a large portion of your diet, you don't need to overhaul everything overnight. Instead, try adding more minimally processed, nutrient-dense foods alongside the foods you already enjoy.

The Bottom Line

Food processing exists on a spectrum, and processed doesn't automatically mean unhealthy.

Research suggests that diets high in UPFs are associated with several health concerns, but researchers are still working to determine why these associations exist and whether all foods classified as UPFs carry the same risks.

Rather than fearing individual foods, focus on your overall dietary pattern. A diet built around a variety of fiber-rich plant foods, adequate protein, and nutrient-dense choices can still leave room for the occasional UPFs.

If you need help incorporating more whole foods into your diet, a dietitian can help. Click here to book an appointment with us at Blue Tree Nutrition.

 

10 Comments

  1. Edy Trujillo

    Valerie,
    I have suffered with an unhealthy gut for years and much likely due to my bad eating habits, I’m slowing improving. Y food choices but get discouraged easily when I can’t pinpoint the cause of my problems. Reading your article makes me think I have CSID. Can I do the complimentary hydrogen test? Thank you

    Reply
    • Valerie Polley

      Edy,
      There can be many reasons for gastrointestinal issues. I will email you at Remeeedy@gmail.com so we can talk more about it.
      Valerie

      Reply
  2. Robin

    I had an endoscopy last Sept. It said CSID. I haven’t really received much more help to live with this beast. I’m on the toilet more than I’m not. I weigh 92 lbs and sad at my body appearance. Saw Rochester Mayo Dr and he is making me take the breath test AND a fructose test too whenever they get in touch with me, because he thinks the biopsy was wrong last Sept. I’m a true mess. I know I do have Sucraid and it seems to help some, if I remember to take it.

    Reply
    • Valerie Polley

      I am sorry you are dealing with this. I see clients with varying degrees of SID. Some have SID with other things on top, such as IBS. Once you get the results of the breath tests, then you can go from there. There are dietitians that can help and I am happy to help as well depending on what state you live in. I would try to use the Sucraid now to see if it gets control of some of your symptoms. You are welcome to reach out to me at valerie@bluetreenutrition.com or 410-963-4782.

      Reply
  3. Vanessa

    Does anyone know of any CSID cookbooks?

    Reply
    • Valerie Polley

      I have never been asked this question, so I did some searching. I found one on Amazon called A Place to Start Without Sugar and Starch. It is for those with CSID. That is the only cookbook I found. Pinterest has some ideas as well. This looks like something that is lacking, but could be very helpful as more individuals are diagnosed with it.

      Reply
  4. Andrea

    My 13 year old daughter was diagnosed last July with CSID and lactase deficiency. She is on sucraid and lactaid. She lost 20 pounds the first few weeks when cutting many sugars and saw improvement. Now she’s back to cramping, bloating. Where can I find directions on how to do the 2 week elimination diet? Any help will.be appreciated. She is very frustrated and sad about her condition.

    Reply
    • Valerie Polley

      I am sorry your daughter is dealing with this. I know it can’t be easy for any of you. I am not sure what you mean by the 2 week elimination diet. Is that for SID or something else? Some individuals with SID also deal with other issues, such as IBS. I would need to evaluate what she is eating now and go from there. I am happy to help out or find another dietitian closer to you. You can reach out to me at valerie@bluetreenutrition.com or 410-963-4782.

      Reply
  5. Christin Firestine

    I live in western nc do you see clients with CSID tele- health or can you recommend someone near me that went to that conference about CSID. Not many are educated in that field.

    Reply
    • Valerie Polley

      I am sorry I am just seeing this now. I have no idea why it didn’t alert me. Every state has different licensure laws and NC is one of those states, so I can’t legally see someone from NC.If you have not found someone there is a great dietitian in your state that can help. Her name is Erin Skinner and I am including a link her website.
      https://www.empowerednutrition.health

      Reply

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Hello, I am

Valerie

My name is Valerie Polley. I am a Indianapolis-based registered dietitian and owner of Blue Tree Nutrition. I consult with clients both local and far away.
I have a bachelor’s degree in nutrition from Purdue University and I have been practicing for 20 years.
I thoroughly enjoy helping clients through their gut health journey. I see a range of GI issues including, but not limited to celiac disease, IBS and SIBO. I also specialize in the FODMAP elimination diet.