Normal plasmic cleavage of the gamma-chain variant of "fibrinogen Saga" with an Arg-275 to His substitution.

We have identified a gamma-Arg-275 to His substitution in an abnormal fibrinogen designated as "fibrinogen Saga" characterized by impaired fibrin monomer polymerization. By chromatofocusing chromatography, we isolated normal and abnormal fragment D1 populations separately from the plasmic-calcium digests of fibrinogen derived from the propositus, a heterozygote for the abnormality. We found that both normal and abnormal fragment D1's were similarly protected from digestion by plasmin in the presence of calcium ions and further degraded to fragments D2 and D3 due to cleavage of the gamma-chain remnant when calcium ions were replaced by chelating agents. Abnormal fragment D1 failed to inhibit both thrombin-clotting of normal fibrinogen and polymerization of normal fibrin monomer, while normal D1 exhibited marked inhibitory activities. In an aberrant peptide comprising residues gamma-274-302 isolated by HPLC from the lysyl endopeptidase-digests of abnormal fragment D1, we identified a His substituting for an Arg at position 2, which corresponds to position 275 of the mutant gamma-chain.

Differences in survival based on the type of follow-up for the detection of hepatocellular carcinoma: an analysis of 547 patients.

The aim of this study was to identify any significant variables in the prognosis of 547 cases with hepatocellular carcinoma (HCC), and simultaneously confirm the survival among the different surveillance modalities for the initial detection of HCC in a closely followed-up group (regular periodic follow-up with monthly alpha-fetoprotein (AFP) and ultrasonography at least every 4 months), a not closely followed-up group (neither performed with AFP nor ultrasonography regularly) and an incidental group (incidentally discovered due to related symptoms). Five hundred and forty-seven consecutive patients with HCC diagnosed at the Internal Medicine Department of Saga Prefectural Hospital Koseikan from January 1989 to December 1998 were retrospectively analyzed. The 1-, 3- and 5-year survivals in all 547 cases were 69.7, 42.4 and 26.9%, respectively. The 1-, 3- and 5-year survivals in the cases found to have solitary HCC measuring 2 cm or less in diameter at the time of diagnosis were 97.3, 76.2 and 52.3%, respectively. Forty-seven point one percent of the closely followed-up group, which was the high-risk group were found to have solitary HCC measuring 2 cm or less in diameter (48 out of the 102 followed-up cases), while only 18.5 and 11.8% were found in the not closely followed-up group (46 out of 248 cases) and the incidental group (22 out of 186 cases), respectively. The 5-year survival in the closely followed-up, the not closely followed-up and the incidental groups were 42.9, 26.1 and 15.3%, respectively. The significant factors obtained in the closely followed-up group compared to those from the not closely followed-up group included AFP, tumor size, tumor number and portal thrombosis. These findings indicate the importance of a close follow-up for high-risk groups in order to identify HCC at an early stage, and thereby have a positive influence on survival.

Colour Doppler ultrasound for the evaluation of bowel wall thickening.

We performed colour Doppler ultrasound to evaluate bowel wall thickening and to determine the effectiveness of this modality. 42 patients (aged 8-83 years old, mean age 43.5 years) with bowel disease underwent both grey scale and colour Doppler ultrasound examinations. The diagnoses were classified into three categories: inflammation, vasculitis or ischaemia. The bowel wall thickness, wall echotexture and location of the involved portion were all recorded by grey scale ultrasound, while the presence of an intramural colour Doppler flow and arterial signal was evaluated by colour Doppler ultrasound. The colour Doppler flow was graded as "absent", "mild", or "abundant", and the resistive index was also calculated. Bowel wall thickening was observed in the bowel diseases demonstrating inflammation, vasculitis and ischaemia. Patients with ischaemia were significantly older than those with inflammation. The difference in bowel wall thickness was not significant among the disease categories. Differences in bowel wall echotexture, colour Doppler flow, arterial signal and resistive index among the disease categories were significant. The absence of a colour Doppler flow and of an arterial signal suggested ischaemia, while in younger patients, an abundant colour Doppler flow and a stratified echotexture suggested inflammation. The mean resistive index in the ischemic group was significantly higher than that in the inflammatory group. In conclusion, both grey scale and colour Doppler ultrasound are considered to provide useful information for evaluating and differentiating bowel wall thickening in various bowel diseases.

Visualization of an adrenal pheochromocytoma with thallium-201.

An adrenal pheochromocytoma was visualized with thallium-201 chloride. Tl-201 concentration was observed in the cellular and vascular portion of the tumor immediately after its intravenous injection and decreased with time. Tl-201 may be a useful imaging agent for localizing pheochromocytomas.

Application of ACTH stimulation to adrenal imaging with radioiodocholesterol.

ACTH-stimulation adrenal imaging (ACTH-I) was performed in 14 patients after baseline imaging (B-I) was performed. In six patients with no adrenal diseases in whom the findings of B-I were equivocal, morphologic normality of the adrenals was confirmed by ACTH-I because of increased adrenal uptake of radioiodocholesterol. In three patients with cortisol-producing tumors, visualization of contralateral glands by ACTH-I provided indirect evidence for autonomous cortisol secretion of the tumors. In three patients with pheochromocytoma or cyst, ACTH-I increased adjacent cortical radioactivity to more clearly delineate the lesions. In two patients with primary adrenocortical insufficiency, exogenous ACTH had no effect on adrenal uptake of the tracer. Correlation was observed between response of the adrenal net counts and urinary excretion of 17-OHCS and 17-KS. ACTH-I is useful when B-I does not provide sufficient diagnostic information or further information is needed due to low or absent radioiodocholesterol uptake by the adrenal gland(s).

[A case of metastatic brain tumor from prostatic cancer showing remarkable shrinkage following CDDP administration].

A patient with brain metastases from prostatic cancer received chemotherapy using CDDP alone. A dose of 80 mg of CDDP was administered twice intravenously, followed by intra-arterial injections of 70 mg and 50 mg for the primary lesion over the course of three months. The main brain tumor showed a 70% decrease in size when estimated by CT, along with improvement of clinical symptoms related to the brain metastases. Intravenous injection of CDDP may thus have potential therapeutic value against metastatic brain tumors.

[Transarterial "pile-up" infusion therapy of cisplatin and lipiodol emulsion in hepatic malignancies without TAE (transcatheter arterial embolization)].

We developed a modified transcatheter arterial infusion method using anticancer agents to treat hepatic malignancies; intermittent injections of iodized oil, lipiodol, containing adriamycin or epirubicin during the arterial infusion of cisplatin (75-200 mg/body) in order to achieve a higher concentration and longer retention of these anticancer agents in the tumor tissue. Fourteen patients with hepatocellular carcinoma (HCC) and five patients with metastatic liver cancer were treated with this "pile-up" arterial infusion therapy by anticancer agents without gelatin sponge TAE. In HCC patients, 50% or greater reduction in tumor size was obtained in 7 of 14 patients (50%). Serum AFP levels decreased by more than 75% in 6 of 7 patients in whom pretreatment serum levels of AFP were more than 200 ng/ml. The one-year and two-year survival rates were estimated at 55% and 27.5%, respectively, by the Kaplan-Meier method. Significant reduction in tumor size was not observed in 5 cases with metastatic liver cancer. Concerning the adverse effects, alimentary symptoms and fever were noted for a few days in many cases, but they were temporary and tolerable in almost all of the patients. Severe adverse changes in laboratory data were not observed. Thus this "pile-up" infusion therapy of anticancer agents without TAE may be a useful therapy for HCC.

Obstructive jaundice due to multiple hepatic abscesses.

A 63-year-old Japanese male with diabetes mellitus developed obstructive jaundice following the onset of multiple hepatic abscesses. Percutaneous transhepatic cholangiography showed intrahepatic bile duct irregularity and dilatations accompanied by a complete obstruction of the right branch of the intrahepatic bile duct. Three kinds of organisms were cultured from the blood and the drained bile. The cholangiographic changes returned to the normal after the liver abscesses subsided following biliary drainage and the administration of intravenous antibiotics.

Intra-arterial cis-platinum infusion with sodium thiosulfate protection and angiotensin II induced hypertension for treatment of hepatocellular carcinoma.

Cis-diamminedichloroplatinum II (CDDP; 52-169 mg/m2) mixed with angiotensin II (1.5-10 micrograms/min) was infused into the hepatic artery in 33 patients with hepatocellular carcinoma. Simultaneously, sodium thiosulfate (10-50 g) was administered intravenously in order to reduce the systemic toxicity of CDDP. Over 50 per cent reduction in tumor size was obtained in 18 patients (55%). Complete response was achieved in 4 patients (12%). Serum alpha-fetoprotein (AFP) levels decreased by more than 75 per cent in 10 of 18 patients in whom the previous AFP level was more than 200 ng/ml. The one year survival rate was estimated at 61 per cent by the Kaplan-Meier method. Alimentary symptoms (nausea, vomiting) were mild or non-existent in nearly 90 per cent of treatments. Peptic ulcer and abdominal pain were manifested in small numbers. Severe changes in the laboratory data were not observed. High dosage arterial infusion of CDDP and angiotensin II and intravenous injection of sodium thiosulfate was well tolerated and gave effective therapy in hepatocellular carcinoma.

[Combined treatment of invasive bladder cancer by intra-arterial infusion chemotherapy of cisplatin with or without other anticancer agents and concurrent radiotherapy--initial response and side effects].

Fourteen patients with invasive bladder cancer were treated by bilateral internal iliac artery infusion of cisplatin, with or without other anticancer agents, and concurrent radiotherapy. Angiotensin II was simultaneously infused in 10 cases. Of the 14 patients, complete response and partial response were achieved in 9 (64%) and 3, respectively. Hematuria was controlled in all 8 patients, and pain was relieved in 3 of 4 patients. Side effects were observed in 8 patients: appetite loss in 8, nausea and/or vomiting in 7, and leukocytopenia in 6 patients, in 3 of whom radiotherapy had to be intermittent because of severe leukocytopenia (less than 2000/mm3). However, restoration of leukocytopenia occurred one week later. Thus, intra-arterial anticancer agents including cisplatin together with concurrent radiation may be one of the most effective therapies for invasive bladder cancer.

[Magnetic resonance imaging of the adrenal gland].

Adrenal imaging was performed using magnetic resonance (MR) was in 100 patients who had no clinical or biochemical evidence of adrenal abnormality and in 19 patients with 24 adrenal lesions (adenoma in 5, hyperplasia in 2, metastasis in 5, (lung cancer in 1, hepatoma in 4) adrenal cancer in 1, pheochromocytoma in 3, neuroblastoma in 3). Normal adrenal glands showed intermediate intensity between muscle and liver, and were detected in over 90% of cases on T1-weighted images (T1-weighted SE, inversion recovery). Adenomas and hyperplasias had the same intensity as normal glands. Medullary masses showed extreme hyperintensity on T2-weighted images and could be differentiated from cortical masses. Neuroblastomas were detected as hyperintense tumors with intratumoral hemorrhage and necrosis on T2-weighted MR images. Metastatic adrenal tumors from lung cancer were hyperintense on T2-weighted images, while metastasis from hepatoma showed low intensity on the same pulse sequence. In diagnosing adrenal metastasis, we must compare and contrast the tumor intensity and structure with those of the primary lesions. MR is considered a useful modality in characterizing adrenal tissue.

Ruptured bronchial artery aneurysm associated with pleural telangiectasis and tortuous portal obstruction: report of a case.

A 25-year-old woman presenting with an emergent condition of massive hemothorax due to a ruptured bronchial artery aneurysm was successfully treated by transcatheter arterial embolization. She had previously undergone portosystemic shunt splenopneumopexy for hepatic portal hypertension at 6 years of age. When undergoing right thoracotomy for the removal of a clot, a prominent telangiectasis on the pleural surface was noted. The lesion appeared to be a rare systemic vascular abnormality although this could not be confirmed.

[Treatment results of small cell lung cancer: referring to the clinical value of BAI combined with radiotherapy].

Results of 65 cases of small cell lung cancer (SCLC) treated from Jan. 1981 to May 1991 were reviewed. There were 58 male and 7 female patients and their age was ranging from 37 to 86 (mean 65). There were 41 limited disease (LD) and 24 extensive disease (ED) cases. According to TNM (UICC 1987) staging system, there were 2 cases of stage I, 4 of stage II, 9 of stage III A, 28 of stage III B and 22 of stage IV. Among 65 cases, 60 cases received radiotherapy and 55 cases of them received radiotherapy for primary site. There were 29 cases received radiotherapy combined with BAI (bronchial artery infusion) and 20 cases received systemic chemotherapy. On survival, the 2-year survival rate was 26% and MST was 13 months in LD patients (n = 41). No 2-year survivors were seen in ED patients and MST was 10 months. Tumor response of primary site was as follows. In systemic chemotherapy group, CR 35%, PR 59%, NC 6% and PD 0% before radiotherapy and CR 59% after radiotherapy were obtained. In BAI group (including BAI + systemic chemotherapy), CR 6% and PR 88% before radiotherapy in BAI group. BAI did not seem to improve response rate compared to systemic chemotherapy. On survival, BAI group did not show significant better survival compared to BAI (-) group in LD cases (n = 31). In responders (evaluable LD cases, n = 24), the MSTs were 25 months in CR cases and 13 months in PR cases. No 2-year survivors were seen in PR cases.(ABSTRACT TRUNCATED AT 250 WORDS)