Bile Acidosis Syndrome: Causes, Symptoms & Treatment

Bile acidosis syndrome develops from a physiologically significant loss of bile acids in the intestine. It manifests as persistent diarrhea that cannot be treated even with standard medications. Whether curative or merely symptomatic therapies are possible depends on the particular cause of bile acid wasting syndrome.

What is bile acidosis syndrome?

In bile acidosis syndrome, as the name implies, there is a significant loss of bile acids in the intestine. Bile acids are necessary for emulsification of dietary fats so that they can then be absorbed in the small intestine. At the same time that fats and fatty acids are absorbed there, fat-soluble vitamins are also absorbed. Bile acids pass from the liver via the bile ducts into the duodenum (small intestine), perform their functions there and are usually reabsorbed in the ileum (lower section of the small intestine). The reabsorbed bile acid is now returned to the liver via the enterohepatic circulation. Only a small portion of the bile (about five percent) is broken down in the intestine. This process produces, among other things, the degradation product bilirubin, which gives the feces their brown color. The bile acids recycled via the enterohepatic circulation are in turn used for the same process. Their minor loss through degradation in the intestine is compensated for in the liver by new synthesis from cholesterol. However, if there is a significant loss of bile acids due to certain processes, they must be increasingly re-synthesized in order to maintain normal fat digestion and vitamin supply to the organism. Loss of bile acids is caused by them entering the large intestine and being broken down there. This leads to a compensated or, in more severe cases, a decompensated bile acid loss syndrome. In compensated bile acid loss syndrome, the capacity of bile acid regeneration is sufficient to ensure normal fat digestion, whereas in the decompensated form, malabsorption of fats and fat-soluble vitamins may occur with symptoms of malnutrition.

Causes

Causes of bile acid wasting syndrome include both disruption of bile acid reabsorption in the lower part of the small intestine (ileum) and its increased degradation in the small intestine due to bacterial miscolonization. Bile acid reabsorption is omitted, among other things, when the ileum is removed during intestinal surgery for Crohn’s disease. The reabsorption of bile acids is also disturbed in Crohn’s disease itself. An increased breakdown of bile acids in the small intestine occurs in the so-called blind sac syndrome. Blind sac syndrome is characterized by a sac-like protrusion on the small intestine. The main cause of this syndrome is surgery on the small intestine. Remains of intestinal contents and bile acids accumulate in this protuberance, which are degraded under intensive bacterial growth. Thus, a considerable loss of bile occurs here, which can lead to a massive disturbance of fat digestion, vitamin B12 deficiency and severe anemia. In the past, it was assumed that mainly these two causes were responsible for a bile acid loss syndrome. However, according to more recent findings, a significant proportion of irritable bowel patients also suffer from this syndrome. Here, no identifiable causes for the resorption disorders have yet been identified. Nevertheless, this so-called idiopathic form of bile acidosis syndrome is now estimated to affect about 33 percent.

Symptoms, complaints, and signs

Bile acidosis syndrome is manifested by persistent chologenic diarrhea. These diarrheas result from the osmotic effect of bile acids. They are associated with severe abdominal cramps. The diarrhea may cause weight loss and dehydration. Eczema often forms in the anal region due to irritant effects of the bile acids. The symptoms improve with abstinence from food. In decompensated cases of bile acidosis syndrome, fatty stools (steatorrhea) appear, which are noticeable by regular fatty films on the water. Since the bile acid loss is very large, the bile acid concentration in the gallbladder also decreases. As a result, gallstones and also urinary stones can form. Gallstones are salt-like precipitates of bile acids, while urinary stones consist of oxalates, since oxalic acid is increasingly absorbed in the intestine.Permanent disruption of fatty acid absorption results in a deficiency of vitamins B12, A, D, and F. Vitamin B12 deficiency can lead to pernicious anemia in the long term.

Diagnosis

There are several investigative options for detecting bile acidosis syndrome. One option is stool testing for bile acids. More informative is the 15SeHCAT test. Another test, the 4C-glycocholate breath test, is rarely performed. The administration of the exchange resin colestyramine has proved most effective. In this case, if symptoms improve, the diagnosis of bile acidosis syndrome is confirmed.

Complications

In most cases, bile acidosis syndrome causes discomfort in the stomach and abdomen. Affected individuals suffer from severe diarrhea and pain in these regions. Dehydration also occurs due to the diarrhea if affected individuals do not take enough fluids uu. Weight loss also occurs, which is generally a very unhealthy condition for the body. Eczema and further inflammation of the stomach are also common. As the disease progresses, gallstone and urinary stone disease may occur, which usually lead to severe pain. Due to the discomfort, it is not uncommon for psychological stress to occur. The patient is no longer able to perform physical exertion or sports. The quality of life is therefore severely limited by the bile acidosis syndrome. Treatment of bile acidosis syndrome is usually causal and is based on the underlying disease. Complications rarely occur. In many cases, surgical intervention is necessary, after which, however, the symptoms and complaints disappear. No further complications occur and the patient’s life expectancy remains unchanged.

When should one go to the doctor?

In case of repeated and persistent diarrhea symptoms, a doctor should be consulted. If the diarrhea cannot be stopped despite all efforts and a change of diet, the affected person needs medical assistance. If there is general weakness, lassitude, loss of the usual level of performance and lack of strength, a doctor is needed. If it is no longer possible to cope with everyday life due to the symptoms, a visit to the doctor is necessary. If deficiency symptoms of the body occur, there is cause for concern and a doctor should be consulted. The patient is threatened with an undersupply of the organs and thus with functional disorders, which are often painful. Help is needed for dizziness, unusual bowel sounds, an inner restlessness, irritability, and skin changes on the anus. The patient should undergo comprehensive examination and treatment to prevent further diseases from developing. Persistent stress, noticeable changes in behavior or changes in personality should be discussed with a physician. If there is a feeling of dryness in the organism, a doctor should be consulted. There is a threat of dehydration, which is a medical emergency. If there is severe weight loss, there may be malnutrition that needs medical attention to prevent further complications. If there are cramps in the stomach or intestines, an elevated body temperature, or blood in the stool, a visit to the doctor is necessary.

Treatment and therapy

The best therapy for bile acidosis syndrome is treatment of the underlying condition. Often, however, this is no longer possible. For example, if the lower portion of the small intestine (ileum) had to be removed as part of bowel surgery, only symptomatic treatments can be given to improve symptoms. In this sense, the exchange resin cholestyramine has proved most effective against diarrhea. It is ingested with food and absorbs the bile acids. Thus, they can no longer exert their osmotic effect against the intestinal walls and the diarrhea stops. However, the resin should not be used in cases of fatty stools, because here the deficiency symptoms would be aggravated. Vitamin deficiency symptoms must be treated by vitamin substitution.

Outlook and prognosis

The prognosis and course of the disease depend on the curability of the diagnosed underlying disease. In many cases, freedom from symptoms is not possible because the causative disease is irreparable.If parts of the intestine had to be removed, the bile acidosis syndrome patient can no longer fully recover. The focus of treatment in these patients is on alleviating the existing symptoms and improving the current quality of life. If malnutrition is present, a change in diet and adequate vitamin intake can significantly improve health. Relapses are documented as soon as the nutritional intake is not optimal in the further course of life and adapted to the needs of the organism. If the vitamin deficiency can be permanently and stably compensated, the patient normally achieves freedom from symptoms. Even in the case of irreparable underlying diseases, a balanced and healthy diet can alleviate the symptoms. The well-being is strengthened, although no healing occurs. In these cases, it is easier for the patient to cope with the disease. In addition, for a good prognosis of the bile acidosis syndrome, the strengthening of the psyche and an optimistic attitude towards life are elementary. As a result, an improvement in the general state of health is possible and dealing with the symptoms is easier for patients to endure.

Prevention

Because bile acidosis syndrome occurs mainly after intestinal surgery for severe bowel disease or is caused by unclear causes, a specific recommendation for prevention cannot be made. However, affected individuals can contribute to the improvement of symptoms by eating a healthy, varied and low-fat diet. Dietary supplements containing medium-chain fatty acids and fat-soluble vitamins are recommended.

Follow-up

In most cases, patients with bile acidosis syndrome have no particular options for follow-up care. The patient himself or herself is primarily dependent on medical treatment of this complaint so as not to reduce life expectancy. However, early detection and treatment of this syndrome has a positive effect on the further course and can prevent further damage. Sometimes bile acidosis syndrome reduces life expectancy. In most cases, bile acidosis syndrome is treated by intestinal surgery. In any case, the patient should rest and take care of his body after such an operation. Effort or other stressful activities should therefore be avoided. Likewise, only light and low-fat food should be consumed in order not to overload the intestines and stomach. Supplements are used to treat deficiencies in vitamins and other nutrients, so the patient must ensure that they are taken regularly and in the correct dosage. Only after successful treatment of the bile acidosis syndrome should the patient switch to a regular diet. Likewise, in this syndrome, the support and care of one’s family has a positive effect on the course of the disease.

This is what you can do yourself

It is not possible to treat bile acidosis syndrome directly by means of self-help. In this case, the affected persons are always dependent on medical treatment, which is usually carried out with a surgical intervention, in order to completely combat the disease. By means of self-help, only the individual symptoms can be weakened. Since bile acidosis syndrome often causes diarrhea, those affected should pay attention to an increased intake of fluids in order to avoid dehydration. Similarly, supplements can be used here to counteract deficiency symptoms. However, the use of these agents should always be discussed with a doctor. He or she can also determine which deficiencies are currently occurring by means of a blood test. Vitamins in particular often have to be substituted. Furthermore, a low-fat and varied diet can have a positive effect on the course of bile acidosis syndrome and alleviate the symptoms. The diet should be healthy and be part of a healthy lifestyle. Likewise, taking cholestyramine can have a good effect on the course of the disease. This also alleviates the symptoms.