ABSTRACT
We have examined 15 members of a family with Gardner's syndrome. In the first and second generations, 4 (possibly 5) of 6 members died of cancer of the colon/rectum. Two of them were known to have plyposis coli. Three members of the third generation had the complete triad of Gardner's syndrome with polyposis coli, soft tissue and hard tissue tumours. Three of 5 children in the fourth generation, aged between 7 and 11 years, probably have polyposis coli, and 2 of them have stigmata of Gardner's syndrome. All members of any family with Gardner's syndrome should be examined regularly at 2--3 year intervals from an age of about 10 years until the diagnosis is established, or if polyposis does not develop, up to the age of 40 years. Proctocolectomy is recommended at the age of 18--20 years or later as soon as the diagnosis is determined.
Subject(s)
Bone Neoplasms/genetics , Fibroma/genetics , Intestinal Polyps/genetics , Neoplasms, Multiple Primary/genetics , Osteoma/genetics , Soft Tissue Neoplasms/genetics , Adult , Aged , Bone Neoplasms/diagnosis , Bone Neoplasms/surgery , Child , Child, Preschool , Female , Fibroma/diagnosis , Fibroma/surgery , Humans , Intestinal Polyps/diagnosis , Intestinal Polyps/surgery , Male , Neoplasms, Multiple Primary/diagnosis , Neoplasms, Multiple Primary/surgery , Osteoma/diagnosis , Osteoma/surgery , Pedigree , Soft Tissue Neoplasms/diagnosis , Soft Tissue Neoplasms/surgery , SyndromeABSTRACT
Fourteen young females with acute pains in the right hypochondrium were admitted to the Surgical Department. The history and clinical findings were typical of acute perihepatitis (Fitz-Hugh-Curtis syndrome), and the diagnosis was confirmed by laparoscopy. The gynecological symptoms and signs were negligible, and Neisseria gonorrhoeae were cultured from the endocervix in two of the patients. Pathogenes in the suprahepatic space were not demonstrated. Eight patients used intrauterine contraceptive device (IUD) of the Copper-T-type. Acute perihepatitis is an important differential diagnosis in young females presenting with acute pains in the right hypochondrium.