家族集積なく散発性に発症をみた小児の若年性胃腸管ポリポーシスの1例

Non-familial Generalized Juvenile Polyposis in an Infant Manifesting Clinical Symptoms Sporadically, Report of a Case

要旨　若年性胃腸管ポリポーシスはまれな疾患であるが，症例の集積と共に癌化の危険性も指摘され，治療上慎重な対応が求められている．本症例は，生後10か月に胃部分切除で胃若年性ポリープと診断され，その後大腸の若年性ポリポーシスが指摘された．2歳6か月ごろから大腸ポリポーシスに伴う貧血，低蛋白血症が出現し補充療法を受けていたが，徐々に成長障害もみられたため，5歳9か月時当科を紹介された．内視鏡的に大腸ポリポーシス切除を繰り返し行った結果，治療開始後から貧血，低蛋白血症の症状は改善し，成長速度も上昇した．その臨床所見，病理学的所見，治療経過を供覧すると共に，本邦報告例の文献的考察を行った．

　A 5-year-9-month-old girl suffering from anemia and hypoproteinemia was admitted to the department of pediatrics in our hospital. She had undergone partial gastrectomy because of bleeding from gastric juvenile polyps at 10 months of age. She was diagnosed as having juvenile polyposis coli by colonoscopic examination at 1 year 1 month of age. Since then, she had been suffering from hypoproteinemia and anemia derived from diarrhea and anal bleeding. Intermittent transfusion of blood and albumin improved her general status. These supplemental therapies were, however, not enough to give her normal development. On admission she was 99 cm (-2.8 SD) in height and 15.4 kg (-1.3 SD) in weight. She was referred to our department for treatment to cure the juvenile polyposis coli. Considering her age, we chose endoscopic polypectomy instead of surgical treatment against polyposis. A total of 180 polyps was removed. After polypectomy her serum protein and hemoglobin improved to the normal range. Thereafter, she showed normal growth without any supplemental therapies, although the treatment by polypectomy was sometimes required against the gradual increase in polyps (removal of 150 polyps at 6 years 9 months,100 polyps at 8 years 9 months, and 90 polyps at 9 year 9 months of age).

　Endoscopic polypectomy is an option in the treatment of generalized juvenile polyposis. However, a careful follow-up should be required since the risk of cancer formation in this disease is indicated in the literature.