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要旨 動悸と浮腫を主訴とした29歳女性で,その後28か月間経過をみているMénétrier病の1例を報告した.検尿正常,便潜血反応陽性で,低蛋白血症,低Ca血症,鉄欠乏性貧血,無酸症を伴っていた.胃X線,内視鏡検査で穹窿部から体下部にかけて巨大皺襞を,幽門前庭部には萎縮性変化を認めた.胃生検で腺窩上皮の過形成と強い細胞浸潤像を認めた.蛋白漏出試験では高値を示した.以上の所見より低蛋白血症を伴うMénétrier病と診断した.治療にはトラネキサム酸は全く無効で,臭化プロパンテリン療法でTP量の上昇を認め,更に臭化プロパンテリンとシメチジンとの併用療法でTP量は上昇を示した.その後シメチジン単独療法を行ったが,TP量は8か月間にわたり漸減した後,著明な上昇を示し,現在は安定した値であり,肥厚していた胃壁および胃の大きさの正常化,巨大皺襞の改善,皺襞ならびに皺襞間表面の顆粒状変化は消失した.現在患者はシメチジン療法により正常な日常生活を送っている.筆者らは低蛋白血症を伴うMénétrier病では,外科的治療の前に,臭化プロパンテリン,シメチジンによる単独あるいは併用療法を試みるべきであると考えている.
A 29 year-old woman was admitted to our hospital in May 1981, complaining of palpitations and edema of the face and lower extremities. Laboratory studies revealed hypoproteinemia with a level of 4.2 g/dl, iron deficiency anemia (RBC 339×104/mm3, Hb 4.7 g/dl, Ht 16.6%, serum iron 23 μg/dl), hypocalcemia, a normal urinalysis, and stools which were positive for occult blood. X-ray and gastroscopic examinations of the stomach showed giant rugae with granular surface from the lower part of the corpus to the fundus, and mucosal atrophic change in the antrum. A mucosal biopsy of tissue from the fundic giant folds revealed hyperplasia of the foveolar epithelium and infiltration of plasma cells and neutrophils. 131I-HSA losing % was 6.4.
From these findings, we diagnosed this case as Ménétrier's disease with hypoproteinemia.
Initially, antifibrinolytic therapy with oral tranexamic acid was performed, but it was totally ineffective. Therefore, an administration of 75 mg/day of propantheline bromide was given. During the two-month period of propantheline treatment, the total serum protein value increased significantly (p<0.005) from 4.8±0.26 to 5.5±0.21 g/dl. Following this twomonth period, 75 mg/day of propantheline was combined with 600 mg/day of cimetidine. During the period of combination therapy, the total serum protein value rose to 6.2±0.31 g/dl, which was statistically significant (p<0.01), as compared to the value achieved during the propantheline treatment period.
As the patient had improved both subjectively and objectively, only 800 mg/day of cimetidine was administered from May 1982. And, though the total-serum protein value decreased gradually for eight months, it has showed a remarkable increase from January 1983, and now keeps completely stable value. The findings of x-ray and gastroscopic examinations of the stomach in August 1983 showed remarkable changes The thickened gastric wall and the size of the stomach have become normalized, the giant rugae and the convexo-concave pattern on the surface of the folds and the valley between folds have disappeared.
At the present time, the patient leads a completely normal life with administration of 800 mg/day of cimetidine alone. Our experience with this patient leads us to advocate propantheline and cimetidine therapy for the patient of Ménétrier's disease with hypoproteinemia before resorting to gastrectomy.
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