What is Cronkhite Canada Syndrome?

The Cronkhite-Canada syndrome is a hamartöses polyps syndrome of the gastrointestinal tract, which leads into malnutrition and hair loss. Many of the patients develop carcinoma of the stomach or intestines in the course of the syndrome, so that the prognosis is considered to be rather unfavorable. Treatment of the disease has so far only been symptomatic, as the causes have not been conclusively clarified.

What is Cronkhite Canada Syndrome?

Polyps are protuberances of the mucous membrane that can be both benign and malignant. All polyp diseases within hollow organs are summarized as polyposis. Polyposis gastrointestinalis is a sub-form of the disease group. The technical term includes polyp diseases of the gastrointestinal tract, which are often associated with several hundred polyps.

In the gastrointestinal tract, polyps are associated with bleeding, digestive problems such as diarrhea and constipation, and colic. Cronkhite Canada Syndrome (CCS) is a gastrointestinal polyposis syndrome. It is a non-hereditary disease of the gastrointestinal region, which, in addition to gastrointestinal complaints, is characterized by skin changes.

The disease was discovered by the American internist Cronkhite Jr. and Canada described for the first time. It is a rather rare disease that has so far been described in around 500 cases and usually only occurs in late adulthood. The disease is now classified as hamartous polyposis syndrome.

Causes

The pathogenesis, including the exact mechanisms by which it develops, has not yet been conclusively clarified for Cronkhite-Canada syndrome. Since no familial accumulation was observed in the cases documented so far, a genetic cause can be almost ruled out. The vast majority of cases have been observed in patients of Japanese nationality or descent. Only a third of those affected came from North America.

Among the Japanese cases, men were more likely to be affected than women, with a ratio of 2: 1. There does not seem to be any gender bias in the rest of the world. An altered intestinal function with regard to digestion, motility, absorption and secretion as well as increased bacterial colonization may play a causal role in the outbreak of the disease.

The primary cause for these factors involved is so far completely in the dark. Due to the geographical accumulation, the involvement of external factors is possible. Since the syndrome tends to occur at an advanced age, age-specific changes are also conceivable.

Symptoms, ailments & signs

In most cases, patients with Cronkhite Canada syndrome do not develop manifest symptoms until they are 50 years old. The early symptoms usually consist of watery diarrhea with absorption disorders and malnutrition symptoms, the partial or holistic loss of taste and appetite as well as weight losses of up to ten kilograms.

General weakness arises as a result of malnutrition. Almost all patients suffer from phenomena such as hypoproteinemia or hypoalbuminemia. As a result of the persistent diarrhea, in addition to hair loss, nail dystrophy often occurs, sometimes with complete nail loss. In addition, many patients suffer from hyperpigmentation, which manifests itself in diffuse light to dark brown spots.

As the disease progresses, some patients develop neurological disorders such as numbness or tingling. Also convulsions can occur. Apart from that, autoimmune disorders such as hypothyroidism or membranous glomerulonephritis often occur. Polyps in the gastrointestinal tract are usually generalized in the disease.

In addition to gastric polyps, colon polyps are the most common. The small intestine is also usually involved. The esophagus can also contain polyps. In the late stages of Cronkhite-Canada Syndrome, carcinomas of the intestine or stomach often occur.

Diagnosis & course

The clinically and endoscopically characteristic findings lead to the diagnosis of Cronkhite-Canada syndrome. A biopsy is taken of the polyps. The tumors impress with a broadly seated base, often show enlarged edematous lamina propria and are mostly characterized by cystic glands.

As part of diagnostics, differential diagnoses are made for generalized juvenile polyposis, hyperplastic polyposis, cap polyposis and Peutz-Jeghers syndrome. In addition, CCS must be differentiated from lipomatous polyposis, nodular lymphoid hyperplasia, inflammatory polyposis and lymphomatous polyposis.

The prognosis for patients with CCS is rather unfavorable, which is due to an increased risk of carcinoma of the gastrointestinal tract and malnutrition. Remissions are common. Despite the risk of mortality, long-term survivors have been described.

Complications

In most cases, Cronkhite Canada Syndrome leads to severe hair loss, which can occur in different parts of the body. The syndrome also leads to malnutrition and thus to underweight. In any case, underweight is a very unhealthy condition for the human body. Most of those affected suffer from watery diarrhea.

This can severely restrict the patient’s everyday life. The malnutrition cannot be compensated for by overeating, as the patient suffers from an absorption disorder and cannot absorb the nutrients. Often there are also taste disorders in the mouth and the affected person has no appetite.

There is also a general feeling of weakness and a feeling of illness. It is not uncommon for patients to lose consciousness. Sensory disturbances can also occur in different parts of the body, which can lead to numbness, for example. Medicines are typically used to treat Cronkhite Canada Syndrome.

However, effectiveness cannot be guaranteed. Intensive therapy is necessary in the case of severe bleeding or anemia. Cronkhite Canada Syndrome usually reduces life expectancy. However, the further complications depend on the patient’s health conditions.

When should you go to the doctor?

If you have watery diarrhea, stomach pain, loss of appetite, and general weakness, you may have Cronkhite Canada Syndrome. A visit to a doctor is advisable if the symptoms have not subsided after a few days, if there is a sharp decrease in well-being, or if further symptoms appear as the disease progresses. If you have sensory disturbances, cramps and hair loss, you should see a doctor quickly. If deficiency symptoms or signs of dehydration are noticed, it is best to contact the emergency medical service or to take the person to the nearest hospital.

A medical evaluation and treatment is necessary in any case for the complaints mentioned – regardless of whether it is CCS or another disease. Cronkhite Canada Syndrome primarily affects people over the age of 50. The disease has been observed particularly frequently in male patients of Japanese nationality or descent. Whoever these risk factors apply to should speak to a general practitioner if they have symptoms mentioned. Other contact persons are the gastroenterologist or the medical emergency service.

Treatment & Therapy

A causal treatment for patients with Cronkhite-Canada syndrome is not yet available because the etiology has not been conclusively clarified. Therapy is symptomatic and focuses on balancing the fluid balance, electrolyte balance and protein loss.

Treatment types such as dietary supplementation are just as possible as conservative drug treatments with antibiotics, corticosteroids, anabolic steroids, acid inhibitors or cromolyn. In addition, the administration of zinc was described, which was able to improve prognostic negative factors such as Helicobacter pylori in individual cases. A consistent effectiveness could not be established for any of the symptomatic therapies.

Invasive procedures are available to treat complications. A regular follow-up of the disease is absolutely necessary in order to recognize and treat malignant changes early enough. In the event of complications such as anemia, bleeding, cardiac insufficiency or infections, intensive therapy is carried out. If there are neurological symptoms such as abdominal pain, supportive symptomatic treatment with pain relievers is given.

Outlook & forecast

In most cases, Cronkhite-Canada syndrome does not develop positively. Since the causes of the disease are not fully understood either, only symptomatic treatment is possible.

The life expectancy of those affected is significantly reduced by the syndrome. Without treatment, the patients suffer from very severe pain and cramps in the stomach and abdomen, which also significantly reduces the quality of life. The treatment itself is based on the pain and the balance of the electrolyte balance.

However, the complaints can only be partially limited. Certain insufficiencies or deficiency symptoms must then be treated by taking supplements. Often the Cronkhite Canada Syndrome also leads to psychological complaints, which, however, can occur not only in the patient, but also in the relatives and parents.

However, if Cronkhite Canada Syndrome is recognized early on, the chances of a higher life expectancy increase. For this reason, sufferers should take part in regular checkups in order to diagnose and treat the disease early on. Some symptoms of the disease can also be limited by a special nutrition plan.

Prevention

In the context of the Cronkhite-Canada syndrome, there is no prophylaxis as the causes of the phenomenon are largely unknown. At most, preventive measures against apparent risk factors such as increased bacterial colonization can be pursued. General health prophylaxis for the gastrointestinal tract can at least not cause any damage. However, whether these preventive measures can serve a specific purpose in the context of Cronkhite Canada Syndrome is not clear.

Aftercare

With Cronkhite Canada Syndrome, there are usually few or no aftercare measures available to those affected. This disease always has to be treated by a doctor, because it cannot heal itself and in the worst case, if left untreated, the person concerned can die. The earlier the Cronkhite Canada syndrome is recognized, the better the further course of the disease is usually.

In most cases, however, the patient’s life expectancy is reduced by the disease. Most of the symptoms of the syndrome can be relieved relatively well by taking medication. The person affected should always ensure that they are taken regularly and that the dosage is correct. Consult a doctor first if anything is unclear or if you have any questions.

A doctor should also be contacted if there are any interactions or side effects. Since the Cronkhite Canada Syndrome causes discomfort in the intestines, the person affected should avoid fatty or very sweet food. In general, a healthy lifestyle with a healthy diet has a positive effect on this disease. It cannot generally be predicted whether this will result in a reduced life expectancy.

You can do that yourself

Cronkhite Canada Syndrome is a very serious disease that must not be treated by yourself, even if the symptoms in the early stages are very similar to those of a harmless digestive disorder. However, many sufferers develop malignant tumors in the gastrointestinal tract in the course of the disease.

The earlier these tumors are discovered and adequately treated, the higher the patient’s chances of survival. An important step towards self-help is therefore not simply to classify diffuse digestive problems, the causes of which are unknown, as harmless and to accept them, but instead to consult a doctor early on.

Even if carcinomas are not detected after the syndrome is diagnosed, they can develop spontaneously. It is therefore imperative that patients undergo all preventive medical check-ups suggested by the attending physician.

Some of the symptoms, particularly hair loss or changes in the skin and nails due to malnutrition, can be counteracted by taking nutritional supplements. Over-the-counter supplements should only be consumed after consulting a doctor.

Those affected can prevent weight loss caused by anorexia by having a nutrition plan drawn up and consistently implementing it, even if they are neither hungry nor hungry. The calorie intake in liquid form is often easier. Patients should therefore seek advice from a nutritionist about special products if necessary.

Cronkhite Canada Syndrome

A (a) - B (b) - C (c) - D (d) - E (e) - F (f) - G (g) - H (h) - I (i) - J (j) - K (k) - L (l) - M (m) - N (n) - O (o) - P (p) - Q (q) - R (r) - S (s) - T (t) - U (u) - V (v) - W (w) - X (x) - Y (y) - Z (z)