著明な改善を認めたCronkhite-Canada症候群の1例

Cronkhite-Canada Syndrome with a Remarkable Improvement, Report of a Case

要旨　著明な改善を認めたCronkhite-Canada症候群の1例を報告する．患者は64歳，男性．下腿浮腫の主訴にて当科受診．四肢の爪の萎縮があり，直腸脱も認めた．検査所見では著明な蛋白尿と低蛋白血症，糖質脂質の吸収不良があった．¹²⁵I-PVPテストは陰性．消化管X線検査にて胃および大腸に多数のポリープが認められた．大腸ポリープには組織学的に同症候群に一致するポリープのほかに，腺腫と過形成性ポリープとが混在していた．直腸脱の組織はsolitary ulcer (mucosal prolapse) syndromeに相当するものであった．腎生検にて膜性糸球体腎炎と診断しmethylprednisoloneによるパルス療法を実施．約8か月後に蛋白尿の改善と胃・大腸ポリープの著明な減少を認めた．

The patient, a 64 year-old man, went to a hospital with complaints of anal discomfort and edema of the lower extremitles and was found to have a polyposis and giant rugae in the stomach. He was admitted to our hospital for further survey and treatment, No particular family histories were found. He had slight degree of hair loss, atrophy of the nails, edema of the lower extremities, and prolapsed anus with hypertrophied mucosa. Laboratory investigation revealed marked proteinuria, hypoprotinemia, anemia, and malabsorption of the saccharides and lipids. ¹²⁵I-PVP test was normal So hypoproteinemia was thought to be due to the proteinuria. Radiographic examination of the stomach showed a polyposis with giant rugae. Barium enema x-ray study revealed many sessile round polyps. Histologically cystic dilatation of the glands and hyperplasia were recognized in the gastric polyps and the colonic polyps. In addition, in the colonic polyps adenomatous changes were seen. Histological examination of the prolapsed anus also revealed hyperplastic changes. In the kidney membranous changes of the glomerulus were histologically recognized. He received methylprednisolone pulse therapy and showed a dramatic improvement of proteinuria, polyposis of the stomach and the colon, and of atrophy of the nails after eight months of the therapy.