Cronkhite–Canada syndrome

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Cronkhite–Canada syndrome (CCS) is a rare, non-inherited gastrointestinal disorder characterized by diffuse polyposis of the digestive tract, along with ectodermal abnormalities such as alopecia, skin hyperpigmentation, and onychodystrophy. The syndrome was first described by Dr. Leonard W. Cronkhite Jr. and Dr. Wilma Jeanne Canada in 1955.

Clinical Features

Patients with Cronkhite–Canada syndrome typically present with symptoms such as diarrhea, weight loss, abdominal pain, and anorexia. The polyps found in CCS are usually hamartomatous and can occur throughout the gastrointestinal tract, including the stomach, small intestine, colon, and rectum.

Pathophysiology

The exact cause of Cronkhite–Canada syndrome remains unknown. However, it is believed to involve an abnormal immune response leading to inflammation and subsequent polyp formation. The syndrome is not inherited, and no specific genetic mutations have been consistently associated with it.

Diagnosis

Diagnosis of Cronkhite–Canada syndrome is primarily based on clinical presentation and endoscopic findings. Endoscopy and colonoscopy are used to visualize the polyps, and biopsy samples are taken to rule out other conditions. Additional tests may include CT scans, MRI, and laboratory tests to assess nutritional deficiencies and inflammatory markers.

Treatment

There is no specific cure for Cronkhite–Canada syndrome. Treatment focuses on managing symptoms and nutritional support. This may include corticosteroids, immunosuppressive drugs, and nutritional supplements. In severe cases, total parenteral nutrition (TPN) may be necessary. Regular monitoring and follow-up are essential to manage complications and assess the effectiveness of treatment.

Prognosis

The prognosis for patients with Cronkhite–Canada syndrome varies. Some patients respond well to treatment and experience symptom relief, while others may have a more chronic and relapsing course. Complications such as malnutrition, sepsis, and gastrointestinal bleeding can significantly impact the prognosis.

Related Pages

• Gastrointestinal tract
• Polyposis
• Alopecia
• Skin hyperpigmentation
• Onychodystrophy
• Endoscopy
• Colonoscopy
• Biopsy
• Corticosteroids
• Immunosuppressive drugs
• Total parenteral nutrition