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Yasushi Imamura

Publications and source records attributed to Yasushi Imamura.

6 recordsLinked to original sources

Effectiveness of carbohydrate-restricted diet and arginine granules therapy for adult-onset type II citrullinemia: a case report of siblings showing homozygous SLC25A13 mutation with and without the disease.

Case 1 is a 37-year-old Japanese man who was admitted to this hospital with a disturbance of consciousness. A diagnosis of adult-onset type II citrullinemia was made by DNA analysis of SLC25A13 (851del4/851del4) and measurement of hepatic ASS activity (2% of control value). After regaining consciousness, Case 1 was started on a dietary therapy, revealing that hypertriglyceridemia and ketogenesis impairment deteriorated on a low-protein diet and ameliorated on a carbohydrate-restricted (high-protein) diet. Case 1 could tolerate 70 g/day of protein while using arginine granules and developed hyperammonemia only after discontinuation of the administration. Case 2 (an elder brother of Case 1) is also homozygote for the same SLC25A13 mutation. The hepatic activity of argininosuccinate synthetase was about 20% of the control value. However, Case 2 exhibited neither hyperammonemia nor lipid metabolism abnormalities. These results suggest that, although adult-onset type II citrullinemia is caused by a deficiency of citrin, which plays key roles in carbohydrates, amino acids and even lipid metabolism, some other environmental or genetic factors are required for the onset of the disease, and from the authors' clinical experience, a carbohydrate-restricted (relatively high-protein) diet is advocated as a benefit to the patients, and that arginine granules are indispensable to this new dietary therapy.

Journal Article↗

Branched-chain amino acids supplements in the late evening decrease the frequency of muscle cramps with advanced hepatic cirrhosis.

Eight outpatients with advanced hepatic cirrhosis were tested over 3 months for branched-chain amino acids (BCAA) supplements in the late evening. Serum albumin level (2.8+/-0.3 vs. 3.1+/-0.2 g/dl, P<0.002), serum cholin esterase activity (54+/-13 vs. 67+/-17 IU/l, P<0.02), and plasma Fischer's ratio (1.3+/-0.9 vs. 1.4+/-0.9, P<0.005) increased over the 3 months. The frequency of muscle cramps decreased (7.4+/-2.0 vs. 0.3+/-0.5 times/week, P<0.0001) dramatically. These data suggest that BCAA supplements in the late evening are of benefit to malnourished patients with hepatic cirrhosis. Relief of muscle cramping is an important outcome of BCAA supplements in the late evening.

Journal Article↗

Prognostic performance of the new classification of primary liver cancer of Japan (4th edition) for patients with hepatocellular carcinoma: a validation analysis.

The Japanese staging system that is generally used for hepatocellular carcinoma (HCC) (3rd edition) was considerably revised recently, especially T category. No study, however, has revealed how well the new classification (4th edition) works to stratify HCC patients at a pre-intervention stage. The purpose of this study is to assess the discriminatory value and predictive power of the 4th edition, and to compare its utility with the clinical utilities of the 3rd edition and the cancer of the liver Italian program (CLIP) score, as determined from 662 Japanese patients. We performed a retrospective analysis of the HCC diagnoses at four Japanese institutions from 1990 and 1998. Overall survival was the only end-point used in the analysis. The discriminatory ability and homogeneity of the 4th edition were compared to those of the 3rd edition and the CLIP score. As of January 1999, 440 patients (66.4%) had died. The overall median survival was 37.7 months. Liver function and tumor variables, which are already accounted for by the different scoring systems, were significantly associated with survival. Compared with the 3rd edition, the 4th editions' discriminatory ability (tested by the linear trend test) and homogeneity of survival within each category (tested by the likelihood ratio test) were enhanced in both the overall group of patients and the subgroups of patients receiving transcatheter arterial chemoembolizations and percutaneous ethanol injections. In patients receiving surgery, however, the 4th edition's abilities were the lowest among the indices. Multivariate analysis revealed that the CLIP score that includes liver function had additional explanatory power above that of the 4th edition. These findings indicate that the 4th edition has a higher stratification value than the 3rd edition. However, this benefit is due to the non-surgical patients, rather than to the surgical patients. If the 4th edition had an additional scoring system based on its original tumor staging and liver damage, it might be highly beneficial, although relative risk ratios of those should be analyzed.

Journal Article↗

[A patient with hepatocellular carcinoma with intraperitonial lymph node metastases in whom oral UFT treatment was markedly effective].

We report a patient with hepatocellular carcinoma (HCC) with intraperitoneal lymph node metastases in whom UFT (uracil + tegafur) was markedly effective. The patient was a 70-year-old woman with chronic hepatitis C, who developed HCC mainly infiltrating the medial segment of the liver. Arterial infusion chemotherapy and embolization were performed, and radiofrequency ablation was also conducted. Despite these interventions, the serum alpha-fetoprotein level continued to increase, and reached a level as high as 208,000 ng/ml by the second month of treatment. Abdominal computed tomography (CT) revealed no recurrence in the liver, but multiple metastases to intraperitoneal lymph nodes were identified. UFT-E treatment was initiated at the dose of 400 mg/day. A subsequent abdominal CT revealed complete disappearance of the intraperitoneal lymph node metastases 2 months after the start of UFT treatment. The serum alpha-fetoprotein level returned to normal 4 months after the start of UFT treatment. We consider that the patient described here is a good example to illustrate the remarkable effectiveness of UFT in the treatment of metastatic HCC.

Administration, Oral↗