Comparison of the pathologic and pathogenic features in six different regions of postmortem brains of three patients with fatal familial insomnia.

Fatal familial insomnia (FFI) is an autosomal dominant prion disease clinically characterized by rapidly progressive insomnia, prominent autonomic alterations and behavioral disturbance. The D178N mutation of the prion protein gene (PRNP) on chromosome 20 in conjunction with methionine at codon 129 is a molecular feature. Although the neuropathological characteristics of FFI are well documented, the neuropathologic and pathogenic features of FFI patients remain poorly understood. Six brain regions of postmortem brains from 3 FFI patients were examined using immunohistochemistry, western blot analyses and quantitative real-time PCR. In all 3 brain specimens, reactive astrogliosis was found to be more severe in the thalamus than in the cortex regions. Western blot analyses showed that all three brains expressed PrP, but only 2 were associated with significantly weak proteinase K (PK) resistance. However, the conformational stabilities of PrPSc in the 3 FFI brains were significantly weaker than those presented in a G114V genetic Creutzfeldt-Jakob disease (gCJD) case. Immunohistochemistry and western blot analyses showed comparable amounts of neuron-specific enolase (NSE)-positive stained cells and NSE protein among the different regions in the three brains. In addition, the transcriptional levels of glial fibrillary acidic protein (GFAP) and NSE-specific mRNAs were coincident with the expression of these proteins. In conclusion, in the present study, we described the detailed regional neuropathology of FFI cases.

[Multi-modal serial therapy for primary liver cancer in senile patients: a report of 153 cases].

OBJECTIVE: To study the feasibility and validity of multi-modal serial therapy for primary liver cancer in senile patients. METHODS: 153 senile primary liver cancer patients (>or= 60 years) were given multi-modal serial therapy from June 1993 to December 2000. Hepatectomy was performed in 37, deep cryosurgery in 32 and non-operative therapy in 84 (intervention as chief therapy in 81, combined local and intervention therapy in 3). The multi-course intervention therapy was given postoperatively in hepatectomy and cryosurgery groups, while bioimmunotherapy and traditional Chinese medicine were used in all groups. RESULTS: The 1-, 3- and 5-year survival rates in the hepatectomy group were 78.4%, 46.4% and 35.7%, without operative mortality. The 1- and 3- and 5-year year survival rates in the cryosurgery group were 64.5%, 40.9% and 25.0% with mortality of 3.1%. Among patients with non-operative therapy, the 1- and 3- and 5-year year survival rates in intervention group were 47.5%, 23.5% and 4.3%. The operative mortality was 1.2%. The 3 patients who received combined local and intervention therapy have survived for 2.5, 3.8 and 7.1 years. CONCLUSION: Multi-modal serial therapy with surgical treatment as the chief means, being precise in the effect and good in safety, is feasible and valid for primary liver cancer in senile patients.

Serial therapies oriented by surgery for large primary liver carcinoma.

OBJECTIVE: To discuss the methods and effects of serial therapies oriented by surgery in the treatment of primary large liver cancers. METHODS: From January 1993 to June 1999, 191 patients with large liver carcinoma were treated surgically. The size of tumors varied from 5.2 to 19.7 cm (mean 9.4 cm). Several types of liver resections were made in 121 patients and as a supplement, cryosurgery was carried out for the remaining 70 patients. Importable drug delivery system was instituted intraoperatively. Transcatheter arterial chemo-embolization (THP 30-60 mg, E-ADM 20-40 mg, CDDP 40-80 mg, MMC 10-20 mg, iodin oil 5-30 ml), percutaneous ethanol injection, bioimmunotherapy and traditional Chinese medicine were used pre- and post-operatively. CT angiography and CT during arterial portography were used to find satellite nodules. Early stage recurrences were predicted by AFPmRNA in peripheral blood. Child-Pugh's classification plus branch chain amino acid/aromatic amino acid ratio (BCAA/AAA) was adopted in evaluating pre-operative liver functions. RESULTS: Marked results were observed after serial treatments oriented by surgery. The 1-, 3- and 5-year survival rates in resection group were 75.8%, 45.6% and 30.4%, respectively. The 1- and 3-year survival rates in cryosurgery group were 63.2% and 37.0%. The operative mortality was 1.57%. Recurrence rates were 69.2% in AFPmRNA positive group and 33.3% in AFPmRNA negative group (P<0.05). The BCAA/AAA ratio was lower than 1.5 in two patients who died of hepatic failure after resection. CONCLUSIONS: Serial treatments with surgery as the chief modality gives satisfactory results in patients with large primary liver carcinoma. This regimen should be regarded as a main strategy to deal with large liver carcinoma. AFPmRNA in the peripheral blood, signifying a recurrence, may become a new clinical parameter. The BCAA/AAA ratio plus Child-Pugh's classification is able to evaluate more accurately liver function reserve before surgery.