ito tetsuya

A patient 71-year-old female came to the emergency room of our hospital with the complaint of vomiting and upper abdominal pain. Blood study revealed WBC of 11,100/μl, CRP of 0.5 mg/dl and serum amylase of 1472 IU/L and a diagnosis of acute mild pancreatitis was made and she was treated with pancreatic enzyme inhibitors, antibiotics and intravenous fluid administration. The value of serum amylase came down to normal on the 2^nd day of treatment, but the abdominal pain worsened along with elevation of WBC and CRP value. Abdominal CT revealed a vague tumor shadow of 3×1.5 cm size on the midline of upper abdomen directly beneath the abdominal wall. On the 9^th day of admission this tumor grew to the size of 7×4 cm with low consistency stripe pattern inside. Necrosis of ligamentum teres hepatis was suspected and laparotomy was performed which revealed abscess formation of the inflamed ligament. The culture of the specimen revealed Enterobacter cloacae and histological study revealed necrotic panniculitis. Abscess of ligamentum teres hepatis is classified as acute abdomen, but it is very rarely reported and therefore we report its surgical significance here.

女児.兄が高チロシン血症I型であり,本例も新生児期の血中アミノ酸分析により同症と診断された.前医でフェニルアラニン・チロシン除去粉乳により管理されていたが,皮疹,下痢が出現し,全身状態不良となったため,生後1ヵ月半時に当科に紹介された.低タンパク血症や凝固系異常を認め,高カロリー輸液,アミノ酸製剤の投与,エリスロポエチン投与などを併用したが症状は改善しなかった.NTBC(2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione)を投与したところ症状は劇的に改善した.以後NTBC投与を続け良好な状態を保っていたが,家族から生体肝移植の強い希望があったため1歳9ヵ月時に施行した.移植後,血中チロシン値は正常域まで低下し,NTBCから離脱できた