Clinical and hematologic characteristics in acute leukemia with 11q23 translocations

We studied the clinical, morphological, and immunologic characteristics of 11 patients with 11q translocation-associated acute leukemia. There were three patients with t(9;11)(p22;q23), one with a variant of the t(9;11), three with t(11;19)(q23;p13), two with t(1;11)(p32;q23), one with t(10;11)(p15;q22or23), and one with t(11;17) (q23;q25). The breakpoints in chromosome 11 clustered in band q23. The morphological feature was FAB-M5 in two patients, FAB-M2 in one, FAB-L1 in six, and lymphoblastic lymphoma in one. The remaining patient underwent morphological changes from FAB-L1 seen at the time of diagnosis to M5b at relapse. Immunologic marker studies in ten patients revealed that one had T cell type; another pre-B cell type; three CALLA- Ia- non-T, non-B type; two CAL-LA- Ia+ non-T, non-B type; two monocytic type (positive Fc-receptor); and the remaining one underwent phenotypic changes from CALLA+ Ia+ non-T, non-B type to monocytic type. The patients were usually young; five were under 1 year and two were 9 and 13 years. Hyperleukocytosis was observed in eight of the ten patients with acute leukemia, and two of the eight died of intracranial hemorrhage within two days of admission, associated with disseminated intravascular coagulation. These findings indicate that leukemia with the 11q23 translocation share certain characteristics in common, irrespective of the recipient chromosome, even though the latter may have some influence on the morphological and immunologic phenotype. Our data provide a hypothesis that multipotent stem cells are involved in the genesis of the 11q translocation-associated leukemia.     

Left ventricular diastolic function in apical hypertrophic cardiomyopathy]

To investigate left ventricular (LV) diastolic function in patients with apical hypertrophic cardiomyopathy (AHCM), we analyzed the LV cineangiograms (RAO 30 degrees) and pressures (tip manometer) in 11 patients with AHCM who had giant negative T waves on their electrocardiograms and "ace of spades" configurations on the LV angiograms. Ten patients with non-obstructive HCM (HNCM) and 10 normal subjects served as controls. LV volumes and instantaneous rates of LV volume changes were derived from frame-by-frame analyses of their LV angiograms. LV isovolumic relaxation was assessed according to the time constant (T) of LV pressure decay. LV diastolic distensibility was evaluated by plotting diastolic pressure-volume curves. There was no significant change in the LV systolic functions among these 3 groups. Compared with normals, LV end-diastolic pressure was equally elevated in AHCM and HNCM. The T of isovolumic pressure decay was significantly prolonged in AHCM and HNCM. LV early diastolic filling was maintained at the normal level in AHCM as assessed by the peak filling rate (PFR) during the rapid filling period and the time from end-systole to PFR. The LV diastolic pressure-volume relation shifted upwards in both AHCM and HNCM. In conclusion, impaired LV isovolumic relaxation and decreased diastolic distensibility, which are associated with HNCM, may also be present in AHCM.     

The effect of beta-blocker on intractable ascites in cirrhotic patients undergoing hepatectomy for hepatocellular carcinoma

BACKGROUND/AIMS: Intractable ascites is one of the serious complications after hepatectomy. Only little is known about their effect on postoperative ascites in patients with liver cirrhosis although beta-blockers have been used for cirrhotic complications including ascites. METHODOLOGY: Here, we report five cases of intractable ascites after hepatectomy, which were treated by propranolol (1 mg/kg/body). RESULTS: In three patients, plasma renin activity and aldosterone concentrations were markedly increased before propranolol administration, but fell to normal levels thereafter. Ascites subsided in all subjects except one, who developed cardiac dysfunction. CONCLUSIONS: Beta-blockers might be a promising drug for intractable ascites in cirrhotic patients undergoing hepatectomy.     

Angiotensin-converting enzyme insertion/deletion genotype is associated with the activities of plasma coagulation factor VII and X independent of triglyceride metabolism

BACKGROUND: The D allele of angiotensin-converting enzyme (ACE) insertion/deletion (I/D) polymorphism and coagulation activity play important roles in cardiovascular events, however, the precise association between these two risk factors remains unclear. METHODS: We identified the ACE I/D genotype and measured the plasma coagulation factor VII and X (FVII and FX) activities and serum lipids in 172 patients (110 men and 62 women, mean age 56.7+/-13.3 years) undergoing coronary angiography. RESULTS: The frequency of the D allele was significantly higher in those with a history of myocardial infarction (MI) than in those with normal coronary arteries, but there was no significant association between FVII and FX activities and the stage of coronary disease. Plasma coagulation factor VII and FX activities were significantly lower in the DD genotype (n=42) than in the II genotype (n=67, P<0.001 and P<0.001, respectively) or the ID genotype (n=63, P<0.01 and P<0.05, respectively). The association of the ACE D allele with lower activities of FVII and FX was also seen in patients with coronary artery disease (CAD). There was a significant association between serum triglyceride levels with FVII and FX, but not with the ACE I/D genotype. CONCLUSION: We concluded that the ACE I/D polymorphism may contribute more to the onset of MI than the activities of FVII and FX and that the ACE D allele might be associated with lower plasma activities of FVII and FX. The potential link between ACE I/D polymorphism and the plasma activities of FVII and FX is probably independent of triglyceride metabolism.     

Importance of abdominal X-ray to confirm the position of levonorgestrel-releasing intrauterine system: A case report

BACKGROUNDLevonorgestrel-releasing intrauterine systems (LNG-IUSs) gradually release levonorgestrel into the uterus and is effective against hypermenorrhoea and dysmenorrhea. Complications associated with the insertion include expulsion, displacement, and uterine perforation. Ultrasonic identification of copper intrauterine devices (IUDs) is possible due to echogenicity from the copper coils. However, the barium sulfate coatings of LNG-IUSs do not always provide hyperechoic images. Both barium sulfate and copper are radiopaque and clearly identifiable on X-ray. Thus, X-ray imaging is required to locate LNG-IUSs.CASE SUMMARYA 46-year-old woman with hypermenorrhoea due to submucosal myomas was treated with LNG-IUS at another hospital. Three LNG-IUS insertions had apparently been followed by spontaneous expulsion, although objective confirmation using imaging was not performed. The patient was referred to our institution for surgery. At the first visit, there appeared to be no device in the uterus, and none was observed on transvaginal ultrasound. However, two LNG-IUSs were observed in the pelvis on abdominal plain X-rays prior to surgery. Hysteroscopic myomectomy was performed, and the two LNG-IUSs were found to have perforated the myometrium. The devices were safely removed during surgery, and the submucosal myomas were also removed. The perforated section of the myometrium was minimal+ADs- therefore, a repair operation was not required.CONCLUSIONPlain abdominal X-rays facilitate the determination of whether an LNG-IUS is in the uterine cavity. Therefore, it is important to confirm a device’s location, regardless of whether spontaneous expulsion is suspected, prior to inserting another device.     

Biologically active aspidofractinine, rhazinilam, akuammiline, and vincorine alkaloids from Kopsia

Eleven new indole alkaloids, in addition to the previously reported rhazinal (1), and 14 other known alkaloids, were obtained from the Malayan Kopsia singapurensis, viz., kopsiloscines A-F (2-7), 16-epikopsinine (8), kopsilongine- N-oxide (9), 16-epiakuammiline (10), aspidophylline A (11), and vincophylline (12). The structures of these alkaloids were determined using NMR and MS analyses. Rhazinal (1), rhazinilam (17), and rhazinicine (18) showed appreciable cytotoxicity toward drug-sensitive as well as vincristine-resistant KB cells, while kopsiloscines A (2), B (3), and D (5) and aspidophylline A (11) were found to reverse drug-resistance in drug-resistant KB cells.     

Iriomoteolides-1b and -1c, 20-membered macrolides from a marine dinoflagellate Amphidinium species

Two 20-membered macrolides, iriomoteolides-1b ( 1) and -1c ( 2), have been isolated from a marine dinoflagellate Amphidinium sp. (strain HYA024), and the structures were elucidated on the basis of detailed analyses of 2D NMR data and chemical correlation.     

Radioimmunoassay of serum group I and group II pepsinogens in normal controls and patients with various disorders

A radioimmunoassay (RIA) for human group I pepsinogens (PgI) in serum was developed, using Pgl purified from gastric mucosa. The sensitivity (0.7 μg/1) and reproducibility of the assay were satisfactory for clinical use. In normal controls, total serum pepsinogen (T-Pg) level was 58.9 ± 31.7 μg/1 (mean ± SD) (Pgl, 43.6 ± 25.0 μg/1; Pgll, 15.3 ± 11.1 μg/1). Peptic ulcer cases had elevated T-Pg levels (gastric ulcer, gastroduodenal ulcer and duodenal ulcer, in increasing order of magnitude). T-Pg levels were not useful for diagnosis of peptic ulcer because of a large overlap with normal controls. T-Pg levels were low in patients with gastric polyp and in aged subjects. In these groups, the decrease of Pgl was more marked than that of PgII.     

Diagnostic accuracy of enhanced liver fibrosis test for nonalcoholic steatohepatitis-related fibrosis: Multicenter study

Aim

The enhanced liver fibrosis (ELF) test is a noninvasive method for diagnosing hepatic fibrosis in patients with nonalcoholic fatty liver disease (NAFLD). This multicenter cohort study aimed to evaluate the accuracy of the ELF test and compare it with other noninvasive tests in Japan.

Methods

We analyzed 371 Japanese patients with biopsy-proven NAFLD. We constructed area under the receiver operator characteristic curves (AUROC) to determine the diagnostic accuracies of the ELF test, the Mac-2-binding protein glycosylation isomer (M2BPGi), the Fibrosis-4 (FIB-4) index, and combinations of these indices.

Results

In patients with F0/F1/F2/F3/F4 fibrosis, the median values of the ELF test were 8.98/9.56/10.39/10.92/11.41, respectively. The AUROCs of the ELF test for patients with F0 versus F1–4, F0–1 versus F2–4, F0–2 versus F3–4, and F0–3 versus F4 fibrosis were 0.825/0.817/0.802/0.812, respectively. The AUROCs of the ELF test were greater than those of the FIB-4 index and M2BPGi at each fibrosis stage. Respective low and high cut-off values yielded sensitivities and specificities for predicting advanced fibrosis (≥F3) of 91.1% and 50.8%, and 38.5% and 92.8%, respectively. For F3 or F4 fibrosis, the combined values from the ELF test and FIB-4 index showed a sensitivity of 98.5%, and the combined values from the ELF test and M2BPGi assay showed a specificity of 97.5%.

Conclusions

In Japan, the ELF test predicts NAFLD-related fibrosis from its early stages. The diagnostic ability of the ELF test was not inferior to that of other indices, and the combined values of ELF plus other indices were more accurate.