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Is Cronkhite-Canada Syndrome necessarily a late-onset disease?
Piero Vernia1, Adriana Marcheggiano, Vanessa Marinaro
1Gastroenterology Unit, Department of Clinical Science, University of Rome La Sapienza, Rome, Italy. vernia@uniroma1.it
European Journal of Gastroenterology & Hepatology
|September 9, 2005
Summary
Cronkhite-Canada Syndrome, a rare gastrointestinal disorder, can manifest in adolescents, challenging the notion of its late-onset nature. Early diagnosis and treatment of co-occurring conditions are crucial for managing this hamartomatous polyp disease.
Area of Science:
- Gastroenterology
- Internal Medicine
- Pediatric Gastroenterology
Background:
- Cronkhite-Canada Syndrome (CCS) is a rare, non-inherited disorder characterized by hamartomatous gastrointestinal polyps.
- It typically presents with late onset, but this case highlights potential earlier manifestations.
- Co-occurring conditions like type I diabetes and thalassaemia minor can complicate diagnosis and management.
Observation:
- A 17-year-old male with type I diabetes and thalassaemia minor was diagnosed with Cronkhite-Canada Syndrome.
- The patient presented with severe symptomatic intestinal candidiasis.
- Ectodermal abnormalities were noted, alongside proteinuria from membranous glomerulopathy.
Findings:
- Successful treatment of intestinal candidiasis and correction of protein/electrolyte imbalances led to the resolution of ectodermal abnormalities.
- The patient remained asymptomatic during a 7-year follow-up period.
- Despite proteinuria, the gastrointestinal polyps did not cause further symptoms.
Implications:
- The typical late-onset presentation of Cronkhite-Canada Syndrome may need reconsideration.
- Asymptomatic cases might go undiagnosed for extended periods, suggesting a need for broader diagnostic considerations.
- This case underscores the importance of managing comorbidities in CCS patients.