About cronkhite-canada syndrome

What is cronkhite-canada syndrome?

Cronkhite-Canada syndrome (CCS) is a very rare disease with symptoms that include loss of taste, intestinal polyps, hair loss, and nail growth problems. It is difficult to treat because of malabsorption that accompanies the polyps. CCS occurs primarily in older people (the average age is 59) and it is not believed to have a genetic component. There have been fewer than 400 cases reported in the past 50 years, primarily in Japan but also in the U.S. and other countries.

What are the symptoms for cronkhite-canada syndrome?

The symptoms of Cronkhite-Canada syndrome occur because of multiple polyps occurring in the stomach, small intestine, colon and, less frequently, the esophagus. These include chronic or recurring watery Diarrhea, cramps, and abdominal discomfort. These people may also have abnormally low levels of protein in the blood (protein-losing enteropathy), causing a feeling of general ill health (cachexia), malnutrition, Nausea and Vomiting.

The earliest symptoms reported are changes in taste and loss of smell. Patients can even experience a profound loss of appetite, sometimes to the point of malnutrition, weight-loss and/or excess fluid accumulation in the arms and legs (peripheral edema). An imbalance of certain essential minerals (electrolytes) may occur because of chronic Diarrhea. Some people with CCS may also have large skin bruises (ecchymotic plaques) and/or impaired lung function. Other symptoms may include loss of hair (alopecia), large areas of dark spots on the skin (hyperpigmentation) and degenerative changes and, eventually, loss of the fingernails (onychodystrophy).

Patients with CCS can also have coexisting autoimmune disorders, where the body develops antibodies against an organ, thereby attacking itself, e.g. hypothyroidism, rheumatoid arthritis, scleroderma, systemic lupus erythematous, etc.

What are the causes for cronkhite-canada syndrome?

The exact cause of Cronkhite-Canada syndrome is unknown. It seems to occur for no known reason (sporadically) and is not thought to be hereditary.

What are the treatments for cronkhite-canada syndrome?

Treatment is based on controlling symptoms and providing support. The primary goal of treatment is to correct fluid, electrolyte and protein loss by nutritional supplementation or a nutritionally balanced liquid diet. Corticosteroids (i.e., prednisone) may be given occasionally to help reduce intestinal inflammation. Bacterial overgrowth in the intestines, which can cause malabsorption, may be treated with antibiotics. In rare cases, symptoms have resolved for no apparent reason (spontaneous remission). Surgical removal of polyps may help to relieve some of the symptoms of Cronkhite-Canada syndrome. However, they may recur or be too numerous to remove individually. If necessary, severely affected portions of the colon may be removed. Case reports have suggested the use of immunosuppressive treatment, including aziathioprin and ciclosporin, if other treatments are not effective.

What are the risk factors for cronkhite-canada syndrome?

The exceedingly rare disease Cronkhite-Canada syndrome (CCS) is characterized by a variety of intestinal polyps, taste loss, hair loss, and issues with nail growth. Because the polyps are accompanied by malabsorption, it is challenging to treat. CCS is more common in older people (average age 59) and mostly affects males. It is seen as an acquired disease rather than a hereditary one.

  • The cause for this is still not known, and it is believed to occur sporadically. It is very risky and has a cancer tendency with a high mortality rate. It can also lead to bleeding from the rectum.
  • CCS tends to be malignant and might be aggravated by a variety of illnesses. Gastric cancer and other gastrointestinal cancers affect 10–20 percent of persons with CCS. Colon cancer and large duodenal papillary adenocarcinoma are two intestinal malignancies connected to CCS.
  • Malabsorption, hypocalcemia, and vitamin D deficiency are caused by intestinal mucosal alterations in CCS patients, which might ultimately result in rib fracture and a flail chest.
  • There have also been reports of CCS coupled with pulmonary embolism, arteriovenous thrombosis, and vestibular impairment. Peripheral neuropathy, such as mononeuritis multiplex, which disrupts sensory and motor transmission, might potentially exacerbate CCS. The underlying cause is still a mystery.


Conditions
Intestinal polyps,Hair loss,Nail growth problems
Drugs
Prednisone,Salazosulfapyridine,Cyclosporine
Symptoms
Diarrohea,Abdominal pain,Skin pigmentation,Alopecia

Is there a cure/medications for cronkhite-canada syndrome?

There is no universally accepted standard of care regarding how to treat Cronkhite-Canada syndrome (CCS).

  • Prednisone is the main medication, and hormone therapy is the main therapy. More than 85% of patients reacted to a dosage of more than 30 mg/d.
  • Anti-TNF therapy: Patients with CCS have high levels of TNF- expressed in their small intestine mucosa. According to research, infliximab had a positive therapeutic impact on CCS.
  • Immunosuppressive medications such as cyclosporine, azathioprine, and sirolimus were used to treat certain patients with hormone treatment failure or hormone resistance, and these treatments were also successful.
  • Additionally, non-steroidal anti-inflammatory medications like salazosulfapyridine were given. Sulindac was used in research to treat colorectal adenomas and CCS polyps, and remission was reported.
  • For the treatment of CCS, it was discovered that the combination of hormones and mesalazine produced long-lasting relief. Prednisone and mesalazine were employed in the situation, and the symptoms were effectively managed. The hair and nails progressively improved, and there was no return of diarrhea.
  • Large polyps or those with the potential to become cancerous, as determined by endoscopic appearance and pathology, should be removed as soon as possible. Malignant lesions need to be promptly removed.
  • If existing treatments are ineffective or inappropriate for patients, tranexamic acid is an additional option.


Conditions
Intestinal polyps,Hair loss,Nail growth problems
Drugs
Prednisone,Salazosulfapyridine,Cyclosporine
Symptoms
Diarrohea,Abdominal pain,Skin pigmentation,Alopecia

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