Gardner's syndrome in several family members.
This article describes chronologically a case of a fatal hereditary disease, which manifests itself in multiple polyps in the large intestine, accompanied by the appearance of numerous mesenchymal tumors on and under the skin and in the bones. In the Internal Department of the General Hospital Murska Sobota, we diagnosed the disease in one member of the family; in another, we ascertained the illness was retrograde. We confirmed the two cases in our dispensary; one of them remains under the constant surveillance of a gastroenterologist. The disease was in an advanced stage in the two family members at the time of discovery, so the treatment was radical. Nevertheless it terminated fatally. We expect to undertake future radical surgical measures on the other member in whom the disease was diagnosed. In this article, we illustrate the course of Gardner's Syndrome (GS), where in spite of early diagnoses, which now begin with molecular genetics and continues to endoscope examinations, and radical operative intervention, the quality of life of the patient is not improved to any great degree. The patient represents an exceptionally high risk group for the development of other mesenchymal tumors growths and colorectal cancer. The open question is also what is surgical treatment: total colectomy or proctocolectomy and what to do with the ileum when it is full of polyps.
Seruga
M
M
General Hospital M. Sobota, Department of Internal Medicine, Slovenia.
Hauzer-Petrovic
A
A
D000328:Adult; D005260:Female; D005736:Gardner Syndrome / Q000453:epidemiology / Q000235:genetics* / Q000628:therapy; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D011379:Prognosis; D012307:Risk Factors; D017524:Slovenia / Q000453:epidemiology