What Is Empty Sella Syndrome?

Cronkhite-Canada syndrome (Cronkhite-Canada's syndrome), also known as polyposispigmentation-alopecia-onycholrophia syndrome, is extremely rare clinically. The etiology is not clear. The clinical features of multiple polyps in the gastrointestinal tract with skin pigmentation, hair loss, and finger (toe) nail atrophy are the main characteristics. Currently, there is no specific treatment method, mainly symptomatic treatment, nutritional support and hormone therapy.

Cronkhite-Canada syndrome

Cronkhite-Canada's syndrome (Cronkhite-Canada's syndrome)

Cronkhite and Canada first reported two cases in 1955. In 1966, Jarnum and Jensen named the trait as Cronkhite-Canada syndrome.

According to Ishifuji statistics in 1985, Japan had the highest incidence, followed by North America and Europe. Its geographical distribution is directly proportional to population density. The age of onset was 26 to 85 years, with an average of 61 years. About 80% of patients were over 50 years old. Europe and the United States reported no difference in male and female incidence, but Japan reported a male to female ratio of 2.3: 1.

The cause is unknown. Most patients have causes, such as mental stimulation, overwork, long-term medication or surgery, etc., which are similar to the causes of stomach and duodenal ulcers. Total gastrointestinal polyposis may be related to inflammation. Due to the proliferation of bacteria in the small intestine and the lack of a delayed immune response, the plasma immunoglobulin IgM value is reduced, while the immunoglobulin IgA value is normal. Immunofluorescence showed that small intestine production of IgA was reduced, while IgG-producing cells were increased, which may be related to bacterial or viral infections. Due to the lack of small intestinal disaccharidase, bacteria can change carbohydrates that stay in the intestine for a long time to short-chain fatty acids and cause diarrhea due to their osmotic pressure.

Intrinsic

Japanese scholar Ishito divides it into 4 types according to the pathogenesis of the symptoms.

Type I: Diarrhea is the first symptom;

Type : Taste abnormality before all symptoms appear;

Type III: The first symptoms are hair loss and atrophy of claw nails;

Type : First, loss of appetite, general burnout, followed by atrophy of claw nails, hair loss, and abnormal taste, but no diarrhea.

A diagnosis can be made based on clinical manifestations and the above examinations. The main points are:

1. The incidence is mostly middle-aged and elderly;

2. The main symptoms are diarrhea, abnormal claw nails, hair loss, pigmentation, and abnormal taste;

3 It is characterized by gastrointestinal polyposis, mainly of the digestive tract;

4 Pathological biopsy showed that the polyp was covered with epithelial cells, glandular hyperplasia showed cystic expansion, interstitial edema and inflammatory cell infiltration was visible;

5. The protein leakage test is abnormal, with hypoproteinemia.

Cronkhite-Canada syndrome medical treatment

Generally, symptomatic therapy and nutritional therapy are used. Antibiotics, glucocorticoids, anabolic hormones, and antifibrinolytic enzymes are used. Plasma products can also be used. In recent years, some people have used sulfasalazine for anti-inflammatory treatment. Japanese scholars have adopted high-energy therapies to achieve certain effects.

Cronkhite-Canada syndrome surgical treatment

Rectal sigmoid polyps can be resected or coagulated by fractional ligation of the anus. If you find cancerous or systemic consumption or intussusception, you can do colon and rectum resection.