Hyperammonemia in a carbamoyl-phosphate synthetase 1 deficiency recipient after living-donor liver transplantation from a carrier donor: a case report

Carbamoyl-phosphate synthetase 1 (CPS1) deficiency is an autosomal recessive congenital urea cycle disorder (UCD) characterized by hyperammonemia. The recipients of liver transplantation (LT) for UCD are often children, and the potential donors are often the parents. Hereditary congenital diseases involving UCD entail the possibility of both parents being genetically heterozygous. Herein, we describe the case of a 12-year-old girl with CPS1 deficiency receiving a liver transplant (soon after birth) from her father, who had a heterozygous CPS1 mutation. She was referred to our hospital with respiratory distress after contracting two infections (respiratory syncytial virus and human metapneumovirus) within a short period, both of which presented with hyperammonemia. Medication for hyperammonemia quickly lowered the ammonia levels. The hyperammonemia was thought to be caused by the heterozygous mutation in the donor liver; moreover, it is likely that the low enzyme activity in the patient’s liver was increased due to the infections. This is the first study to report hyperammonemia in a CPS1 deficiency patient due to an infection after LT. Thus, patients with CPS1 deficiency should be aware of the development of hyperammonemia after LT

スイタイブ ヒシンジュンセイ スイカンナイ ニュウトウセンガン ニ タイシ ヒ オンゾン スイタイ ビブ セツジョジュツ オ シコウ シタ 1レイ

We report a case of non-invasive intraductal papillary-mucinous carcinoma that was performed spleen-preserving distal pancreatectomy. A 74-year-old man was admitted to our hospital because he was pointed out a cystic tumor of the pancreatic body by near doctor. Abdominal ultrasonography and endoscopic ultrasonography showed a cystic tumor with papillary elevated lesion. But there were no evidence of infiltration to another organs. Based on these various examinations a diagnosis non-invasive intraductal papillary-mucinous carcinoma was determined. Then we determined to perform a minimal invasive operation, and underwent spleen-preserving distal pancreatectomy. After the operation there were no major complications and he discharged on 45th post operative days. To determine the surgical procedure of non-invasive intraductal papillary-mucinous carcinoma, because of the high incidence of postoperative infections after splenectomy, we should try to preserve the organ function. It was considered that this procedure was a useful method for non-invasive intraductal papillary-mucinous carcinoma of the pancreas body

Protective effect of FK506 and Thromboxane synthase inhibitor on ischemia-reperfusion injury in non-heart-beating donor in rat orthotopic liver transplantation

The study investigated the possibility of pharmacologically modulating hepatic allograft function from non-heart-beating donors (NHBDs)using male Lewisrats. The donors were divided into 4 groups :Group 1 in which the vehicle was administered, Group 2 in which FK506 (tacrolimus a powerful immunosuppressive agent)was administered,Group3 in which OKY046 (a specific thromboxane synthetase inhibitor) was administered and Group 4 in which FK506 and OKY046 were administered. The recipients received orthotopic liver transplantation. The survival rates differed significantly between the recipients that had received liver transplantation from Groups1 and 4. The serum liver enzyme and inflammatory cytokine concentrations of the recipients which had received liver transplantation from Groups 2, 3 and 4 were significantly lower than those of the recipients that had received liver transplantation from Group1. Although there was no significant difference, all parameters were better in the recipients that had received transplantation from Group 4 than those that had received transplantation from Groups 2 and 3. The action mechanisms of FK506 and OKY046 are completely different. Therefore, concomitant use of FK506 and OKY046 might have additive effects on liver transplantation from NHBDs. In conclusion, we demonstrated that pretreatment of NHBDs using FK506 and OKY 046 ameliorated graft viability

スイカン ヒユゴウ ニ ガッペイ シタ スイカンナイ ニュウトウ シュヨウ ノ イチセツジョレイ

We report a case of partial pancreas divisum with Intraductal Papillary Mucinous Tumor (IPMT) that was performed pancreatic segmentectomy. A 68-year-old woman was admitted to our hospital because she was pointed out a cystic tumor of the pancreatic body by near doctor. Abdominal ultrasonography and endoscopic retrograde pancreaticography showed a partial pancreas divisum and cystic tumor with small elevated lesion. Based on these various examinations a diagnosis partial pancreas divisum with IPMT was determined. Then we performed a minimal invasive operation, and underwent pancreatic segmentectomy. After the operation there were no major complications and she discharged on 34th post operative days. In a review of the Japanese literature, only three such cases including our case have been reported so far

Arterial infusion chemotherapy for the patient of unresectable pancreatic carcinoma with multiple liver metastases : a case report

Introduction : Despite various treatment trials for unresectable pancreatic carcinoma with liver metastases, the outcome has not been satisfactory. This paper reports a case of pancreatic carcinoma with multiple liver metastases that responded well to arterial infusion chemotherapy. Case report : A 65-year-old male was diagnosed with multiple liver tumors, and needle biopsy revealed adenocarcinoma. With endoscopic ultrasonography, a tumor in the pancreatic head was detected, and pancreatic carcinoma with multiple liver metastases was diagnosed. He received arterial infusion chemotherapy : cisplatin at a dose of 10mg/body/day and 500mg/body/day of 5-fluorouracil. After 14 days administration, liver metastases had decreased in number and size, but thereafter, because of hepatic arterial occlusion, the same dose of drugs was administered intravenously. The patient was discharged from the hospital and was given chemotherapy 3 days a week on an outpatient basis. Although the chemotherapy was effective, it was stopped because of severe general fatigue 5months after discharge. His general status continued to gradually worsen,and he died 12 months after diagnosis. Conclusions : Prognosis of pancreatic carcinoma with liver metastases is poor however, transarterial infusion chemotherapy may be effective to improve the prognosis and quality of life of the patients

ヒダリガワ タンノウショウ ニ タイシ フククウキョウカ タンノウ テキシュツジュツ オ シコウ シタ 3レイ ノ ケントウ

We experienced a series of 370 cases of laparoscopic cholecystectomy (Lap-C) between January 1992 and December 2001, of which three cases (0.8%) were left-sided gallbladder. When we perform Lap-C to left-sided gallbladder, we have to recognize the anatomical specificity of this disease, and to avoid the complication like injury of arteries or bile ducts. In this study we performed Lap-C to three cases of left-sided gallbladder. In all cases, the gallbladder bed were located at the left side of the hepatic round ligament, and the cystic duct were connected to normal position of the common bile duct. And in all cases, there were no anomalies of the intrahepatic portal vein. One of these cases, falling of the hepatic round ligament was seen, then we tried to insert a trocar at the left side of the ligament and to pick up it by silk. Then we could get a good view and easily performed Lap-C. In all cases we could underwent Lap-C without complication. We considered that Lap-C was to be a standard operation method for malformation cases like a left-sided gallbladder

スイトウブ スイカンナイ ニュウトウ シュヨウ ニ タイシ スイトウ ジュウニシチョウ ダイ2ブ セツジョジュツ オ オコナッタ 1レイ

We report a case of IPMT (intraductal papillary-mucinous tumor) that was performed pancreatic head resection with segmental duodenectomy. A 42-year-old man was admitted to our hospital because he was pointed out a cystic tumor of the pancreas head by near doctor. Abdominal ultrasonography and intra ductal ultrasonography showed a multiple cystic tumor with hypertrophied septum. But there were no elevated tumors in the cystic mass. MRCP showed a racemose multiple cystic tumor. ERCP showed a big orifice of papilla Vater and mucinous discharge. Based on these various examinations a diagnosis IPMT was determined. Because of no elevated tumor in the cystic mass, we suspected it was adenoma or hyperplasia. Then we determined to perform a minimal invasive operation, and underwent pancreatic head resection with segmental duodenectomy. After the operation there were no stasis of stomach and no weight loss. To determine the surgical procedure of benign IPMT, we should try to preserve the organ function. It was considered that this procedure was a useful method for benign IPMT of the pancreas head