Late-onset type ornitine transcarbamirase deficiency (OTCD) with fulminant onset following a fatal prognosis]

A 16-year-old man was admitted to our hospital with nausea, general fatigue, and consciousness disturbance along with extreme hyperammoniemia eight days after the onset of symptoms. Familial history and the high concentration of orotic acid in urine lead us to a diagnosis of OTCD. We immediately initiated intensive treatment such as continuous hemodiafiltration and sodium benzoate administration; however, the patient died twelve days after admission. Since OTCD is not so rare and can be found in all ages, it should be considered fundamental for evaluation of hyperammoniemia. This case suggested that for a better prognosis of OTCD patients it is very important to prevent such an onset, and to make an as early as possible diagnosis and start to treatment.     

Big mitogen-activated protein kinase 1 (BMK1)/extracellular signal regulated kinase 5 (ERK5) is involved in platelet-derived growth factor (PDGF)-induced vascular smooth muscle cell migration

Big mitogen-activated protein kinase 1 (BMK1), also known as extracellular signal-regulated kinase 5 (ERK5), is a newly identified member of the mitogen-activated protein (MAP) kinase family. Recently, several studies have suggested that BMK1 plays an important role in the pathogenesis of cardiovascular disease. To clarify the pathophysiological significance of BMK1 in the process of vascular remodeling, we explored the molecular mechanisms of BMK1 activation in vascular smooth muscle cells (VSMCs). From the results of co-immunoprecipitation and immunoblotting analyses, it was found that platelet-derived growth factor (PDGF), a known potent mitogen, activated BMK1 and triggered the Gab1-SHP-2 interaction in rat aortic smooth muscle cells (RASMCs). The abrogation of SHP-2 phosphatase activity by transfection of the SHP-2-C/S mutant suppressed PDGF-stimulated BMK1 activation. Infection with an adenoviral vector expressing dominant-negative MEK5alpha, which can suppress PDGF-stimulated BMK1 activation to the control level, inhibited PDGF-induced RASMC migration. Moreover, we observed an increase of BMK1 activation in injured mouse femoral arteries. From these findings, it is suggested that BMK1 activation leads to VSMC migration induced by PDGF via Gab1-SHP-2 interaction, and that BMK1-mediated VSMC migration may play a role in the pathogenesis of vascular remodeling.     

A case of intraductal papillary mucinous neoplasm with internal pancreatic fistula causing left ureteral obstruction]

A 75-year-old man had been admitted to another hospital because of left abdominal pain, and was given a diagnosis of left hydronephrosis and acute pancreatitis. After a JJ stent insertion and medication, he was transferred to our hospital for further examinations. US and EUS revealed a chronic pancreatitis-like pattern and multicystic lesion in the pancreas head and body. At that time enhanced CT findings showed an extrapancreatic low density area to be inflammatory change, extending from the pancreas body to the left crus of the diaphragm and posteriorly the spreading from the left crus of the diaphragm via the left urinary duct into the left iliopsoas muscle, in which MRI revealed partial high intensity. ERCP and MRCP showed focal irregular narrowing of the pancreatic duct of unknown cause, and we decided that an internal pancreatic fistula due to pancreatitis had induced left ureteral obstruction, caused by a protein plug or alcohol. Follow-up 6 months later showed that extrapancreatic spreading of the low density area had markedly regressed without any change in the ureteral obstruction.     

The effect of external gamma-irradiation on 59Fe release in vitro from alveolar macrophages previously having ingested 59Fe-iron hydroxide colloid

The effect of external gamma-irradiation on the digestive function of rat alveolar macrophages (AM) was studied by using the in vitro assay system, where the 59Fe release from AM engulfing 59Fe-iron hydroxide colloid was measured as an indicator of their digestive capability. Graded doses of gamma-irradiation up to 100 Gy had no effect on the extracellular release of 59Fe in AM cultured at 4 and 37 degrees C for 8-72 hrs postirradiation. When 10 mM Ca-DTPA was added to the culture medium, the 59Fe release was slightly depressed by irradiation at a dose of 30 Gy. It is apparent from these results that gamma-irradiation had no effect on the 59Fe transfer between cell and medium, and little effect on the catabolism of 59Fe-colloid in rat AM.     

Study of para-aortic lymph node metastasis of gastric cancer subjected to superextensive lymph node dissection

Para-aortic lymph nodes (n4), were dissected out to the technical extreme (superextensive lymph node dissection) from 129 gastric cancer cases, and were subjected to the histological study for metastasis. Following observations led us to the conclusions in reference to the significance of n4 node dissections on curability of surgery. 1) Among 25 cases with n4(+) metastasis n3 was free [n3(-)] in 11 cases (44.0%). 2) ps(+) cancer presented high rate of n4(+) (31.5%). 3) n4(+) occurred irrespective of the location of the cancer, with particularly high rate of occurrence among CMA and cancers. 4) The rate of the metastasized lymph nodes to the total number of the n4 lymph nodes, was found low (34.9%) in cases with n3(-), and high (90.1%) with n3(+). 5) The cumulative survival rate of the cases with n4(+) was found significantly high with n3(-), as compared to n3(+) cases. The lymphatic drainage from the stomach seems more direct and/or more abundant to the n4 than to the n3 nodes. Such observation coincides with our experience that the n4 nodes are involved in metastasis in earlier timing and in higher incidence than n3 nodes. These results warn the present evaluation of curability in which the n4 node dissection is not performed. It is our opinion that the thorough dissection extended to the n4 nodes (superextensive lymph node dissection) is warranted, particularly in order to improve the curability of n3(-)-n4(+) cases.     

Carcinoma of the main hepatic duct junction: indications, operative morbidity and mortality, and long-term survival

Carcinoma of the main hepatic duct junction tends to spread extensively along the hepatic ducts into the liver parenchyma. Therefore extensive resection of the bile ducts combined with hepatic resection is the procedure of choice. Between January 1973 and April 1989, 25 of 50 patients with this type of carcinoma underwent resection, a resectability rate of 50%. One patient died of staphylococcal sepsis on the postoperative day 42 after right trisegmentectomy and resection of the bile ducts, a hospital death rate of 4%. Twenty-four patients were discharged from the hospital. The 5-year actuarial survival rate calculated by the Kaplan-Meier method was 19%. Four patients lived longer than 5 years after surgery; the longest survival was 9 years after right trisegmentectomy and resection of the bile ducts. These four patients had clear margins at the resected bile ducts. This article was designed to clarify the point at issue by presenting our results in terms of indications, operative morbidity and mortality, and long-term survival.