Two Independent Clones in Myelodysplastic Syndrome Following Treatment of Acute Myeloid Leukemia

We describe a 55-year-old Japanese woman with therapy-related myelodysplastic syndrome (t-MDS) with 2 independent clones, t(1;2)(p36;p21) and t(11;12)(pl5;ql3). She was diagnosed with acute myeloid leukemia (AML) with cytological features of the bone marrow and peripheral blood. Cytogenetic evaluation revealed a 46,XX karyotype. She received chemotherapy and achieved complete remission (CR). Despite maintenance chemotherapy, she suffered a relapse. Chromosomal analysis showed t(1;2)(p36;p21) in 2 of 20 metaphases. At second CR, this clone transiently disappeared. Nine months later, t(1;2) (p36;p21) was detected again in 3 of 20 metaphases while the patient remained in CR. Six months later, bone marrow examination disclosed trilineage dysplasia without an excess of blasts, suggesting MDS. t(1;2)(p36;p21) was observed in 16 of 20 metaphases. The clinical course and serial cytogenetic findings were diagnostic of t-MDS. The duration of t-MDS was 6 years. During this period, persistent t(1;2)(p36;p21) and transient t(11;12)(p15;q13) were found. When t-MDS evolved toAML, cytogenetic evaluation revealed 46,XX,t(1;2)(p36;p21),del(7)(q22),add(19)(p13).     

Assessment of coronary function in children with a history of Kawasaki disease using (15)O-water positron emission tomography

BACKGROUND: Coronary abnormalities after Kawasaki disease (KD) may be associated with endothelial dysfunction due to intimal hypertrophy. The purpose of this study was to evaluate myocardial flow reserve (MFR) and endothelial function in regressed aneurysmal regions after KD. Methods and Results- Subjects were 12 patients aged 16.0+/-2.6 years who suffered from KD at 1.7+/-1.5 years and 12 normal subjects aged 26.5+/-3.4 years. MFR and endothelial function were estimated, respectively, by changes in myocardial blood flow (MBF) during ATP infusion and by that during cold pressor test using (15)O-water positron emission tomography. Data from 24 regressed aneurysmal regions were compared with those from the corresponding regions (n=36) in the control group. Although the MBF at rest in the regressed aneurysmal regions was similar to that in controls, the MBF at a hyperemic state induced by ATP infusion in the regressed aneurysmal regions was significantly lower than that in the control regions. Therefore, the MFR in regressed aneurysmal regions was significantly lower than that in controls (3.53+/-0.95 versus 4.60+/-1.14; P<0.05). MBF at rest and during the cold pressor test did not change in the control regions, but it was significantly reduced in regressed aneurysmal regions. The ratio of MBF during the cold pressor test to MBF at rest was significantly lower in regressed aneurysmal regions than in control regions (0.67+/-0.15 versus 1.00+/-0.15; P<0.05). CONCLUSIONS: MFR and endothelial function are often impaired in regressed aneurysmal regions after KD, and tomography enables the noninvasive evaluation of coronary function.     

Failure of disopyramide to improve right ventricular outflow tract obstruction after living-donor lobar lung transplantation.

Right ventricular (RV) outflow tract obstruction (RVOTO) is an uncommon complication of lung transplantation in patients with pulmonary hypertension (PH) and both medical management and surgical intervention are required. A 28-year-old female with primary PH was referred and because she did not respond to medical treatment, living-donor lobar lung transplantation was performed. The operation was successful, but dyspnea and exercise intolerance developed during rehabilitation and transthoracic echocardiography revealed RVOTO. Intravenous disopyramide during cardiac catheterization reduced the pressure gradient from 35 mmHg to 16 mmHg without decreasing RV systolic pressure. However, electrical and hemodynamic parameters were adversely affected by disopyramide and thus, after cardiac catheterization, administration of fluid and a low dose of atenolol was started, and her symptoms improved. Transthoracic echocardiography showed improvement in the RVOTO. This case suggests that disopyramide should be avoided for patients with RVOTO following lung transplantation and that other negative inotropic agents, such as beta-blockers, are more effective for relief of RVOTO.     

In vitro antimicrobial activity of rokitamycin, a macrolide antibacterial agent, against clinically isolated strains of Campylobacter and other enteritis-causing bacteria]

We determined the minimum inhibitory concentrations (MICs) of rokitamycin (TMS-19-Q, RKM), a macrolide antimicrobial agent, against strains of various bacterial species isolated from enteritis patients, and compared them with those of josamycin (JM), erythromycin (EM) and ofloxacin (OFLX). MIC90 of RKM against 147 strains of Campylobacter jejuni, and each 25 strains of Shigella spp., Salmonella spp. and diarrheagenic Escherichia coli were 1.56, 200, 800 and 200 micrograms/ml, respectively. There was only one RKM resistant (MIC greater than 100 micrograms/ml) C. jejuni strain, while most of the strains of the other species were resistant to RKM. MIC values of the other drugs were all similar to those of RKM. MIC90 of OFLX against 147 strains of C. jejuni was 0.78 micrograms/ml, lower than other drugs.     

Screening and diagnosis of acute and chronic bird-related hypersensitivity pneumonitis by serum IgG and IgA antibodies to bird antigens with ImmunoCAP®

BackgroundBird antigens are some of the most relevant antigens in hypersensitivity pneumonitis (HP). Possible sources of bird antigens are bird breeding, feather products and fertilizer with fowl droppings. For the screening and diagnosis of HP, the measurement of bird-specific antibodies should be standardized. The aim of this study was to clarify the utility of serum IgG (sIgG) and IgA (sIgA) antibodies to bird antigens in screening and diagnosing acute/chronic bird-related HP with ImmunoCAP® in multi-centre clinical research.MethodsWe executed a clinical performance test by conducting a multi-institutional study to measure the levels of sIgG/sIgA against pigeon, parrot and budgerigar antigens by the ImmunoCAP® system in 29 acute and 46 chronic bird-related HP patients.ResultsThe levels of sIgG/sIgA against the bird antigens of the three species were significantly higher in subjects with acute bird-related HP and chronic bird-related HP with acute episodes (recurrent type) than in the control subjects. For sIgG, the optimal cutoff values by receiver operating characteristic (ROC) analysis were 24.6 mgA/L for pigeon, 14.0 mgA/L for parrot, and 8.7 mgA/L for budgerigar. By measuring multiple bird antigens and combining sIgG values of two species, the sensitivity and specificity for acute and recurrent-type chronic bird-related HP patients were 85–91% and 73–80%, respectively. For recurrent and insidious types of chronic bird-related HP, the sensitivity and specificity were 48–61% and 73–80%, respectively.ConclusionsMeasurement of the levels of sIgG/sIgA against pigeon, budgerigar and parrot antigens by ImmunoCAP® was useful for screening and diagnosis in bird-related HP.     

High-frequency potentials developed in wavelet-transformed electrocardiogram as a novel indicator for detecting Brugada syndrome

BACKGROUND: A reliable alternative method for detecting Brugada syndrome is desirable because the diagnosis of Brugada syndrome using 12-lead ECG is not optimal. OBJECTIVES: The purpose of this study was to assess the usefulness of the wavelet-transformed ECG in detecting Brugada syndrome. METHODS: The study consisted of 15 patients with Brugada syndrome and 15 healthy subjects (control group). The parameters on the signal-averaged ECG and the frequency components recorded from the wavelet-transformed ECG were compared between the two groups. Measurements were repeated after pilsicainide infusion in the two groups of patients, after an isoproterenol infusion following pilsicainide injection, and after administration of cilostazol in the group of patients with Brugada syndrome. RESULTS: The positive rate of late potentials was 80% in the Brugada syndrome group and 0% in the control group (P <.01). The high-frequency components (80-150 Hz) were developed in the Brugada syndrome group to a greater extent than in the control group, but the low-frequency components (10-50 Hz) did not differ (mean peak power at 80 Hz; 713 +/- 36 vs 488 +/- 60, P <.001). After pilsicainide injection, high-frequency components significantly increased in both groups. However, after isoproterenol and cilostazol administration, high-frequency components significantly decreased but remained higher than in the control group (80 Hz; 655 +/- 40 vs 488 +/- 60, P <.001). The sensitivity of the development of high-frequency components in detecting Brugada syndrome was higher than that of signal-averaged ECG (100% vs 80%), but specificity remained high and similar (100% for both methods). CONCLUSION: Abnormally high-frequency components recorded from the wavelet-transformed ECG might be a novel factor in detecting Brugada syndrome.