Clinical and hematologic manifestations in patients with Diamond Blackfan anemia in Korea.

BACKGROUND: Diamond Blackfan anemia (DBA), characterized by impaired red cell production, is a rare condition that is usually symptomatic in early infancy. The purpose of this study was to assess nationwide experiences of DBA encountered over a period of 20 years. METHODS: The medical records of 56 patients diagnosed with DBA were retrospectively reviewed from November 1984 to July 2010. Fifteen institutions, including 13 university hospitals, participated in this study. RESULTS: The male-to-female ratio of patients with DBA was 1.67:1. The median age of diagnosis was 4 months, and 74.1% were diagnosed before 1 year of age. From 2000 to 2009, annual incidence was 6.6 cases per million. Excluding growth retardation, 38.2% showed congenital defects: thumb deformities, ptosis, coarctation of aorta, ventricular septal defect, strabismus, etc. The mean hemoglobin concentration was 5.1±1.9 g/dL, mean corpuscular volume was 93.4±11.6 fL, and mean number of reticulocytes was 19,700/mm(3). The mean cellularity of bone marrow was 75%, with myeloid:erythroid ratio of 20.4:1. After remission, 48.9% of patients did not need further steroids. Five patients with DBA who received hematopoietic transplantation have survived. Cancer developed in 2 cases (3.6%). CONCLUSION: The incidence of DBA is similar to data already published, but our study had a male predilection. Although all patients responded to initial treatment with steroids, about half needed further steroids after remission. It is necessary to collect further data, including information regarding management pathways, from nationwide DBA registries, along with data on molecular analyses.

Early changes in SOFA score as a prognostic factor in pediatric oncology patients requiring mechanical ventilatory support.

PURPOSE: To evaluate whether changes in outcome prediction scores during the first 72 hours after admission to a pediatric intensive care unit (PICU) are more predictive of outcome than single assessments at admission in pediatric oncology patients requiring mechanical ventilatory support for more than 3 days. PATIENTS AND METHODS: The medical records of 54 consecutive pediatric oncology patients requiring mechanical ventilation over 72 hours in the PICU of the Asan Medical Center, Seoul, Korea, between January 2006 and December 2008, were retrospectively reviewed. RESULTS: Although both initial Sequential Organ Failure Assessment (SOFA) score and change in SOFA score (Δ-SOFA) correlated well with mortality, Δ-SOFA score showed a significantly stronger correlation (P<0.001) and a larger area under the receiver operating characteristic curve than did initial SOFA score. Patients with positive and negative Δ-SOFA scores showed statistically significant differences in mortality (18.5% vs. 88.2%, P<0.001). In addition, early changes in respiratory parameters, such as PaO2/FiO2 (P/F) ratio, oxygenation index (OI), and ventilation index (VI), evaluated serially during the first 3 days, also correlated with mortality. Patients showing improvement in these respiratory parameters displayed significantly lower mortality than did patients with worsening of these parameters (P<0.01). CONCLUSIONS: Serial evaluation of SOFA score during the first few days after PICU admission was a good predictor of prognosis in pediatric oncology patients mechanically ventilated over 3 days. Independent of initial SOFA score, Δ-SOFA score during the first 72 hours closely correlated with outcome. Early changes in respiratory parameters, such as P/F ratio, OI, and VI, may also provide valuable prognostic information in such patients.

Bubble velocity, diameter, and void fraction measurements in a multiphase flow using fiber optic reflectometer.

A fiber optic reflectometer (FOR) technique featuring a single fiber probe is investigated for its feasibility of measuring the bubble velocity, diameter, and void fraction in a multiphase flow. The method is based on the interference of the scattered signal from the bubble surface with the Fresnel reflection signal from the tip of the optical fiber. Void fraction is obtained with a high accuracy if an appropriate correction is applied to compensate the underestimated measurement value. Velocity information is accurately obtained from the reflected signals before the fiber tip touches the bubble surface so that several factors affecting the traditional dual-tip probes such as blinding, crawling, and drifting effects due to the interaction between the probe and bubbles can be prevented. The coherent signals reflected from both the front and rear ends of a bubble can provide velocity information. Deceleration of rising bubbles and particles due to the presence of the fiber probe is observed when they are very close to the fiber tip. With the residence time obtained, the bubble chord length can be determined by analyzing the coherent signal for velocity determination before the deceleration starts. The bubble diameters are directly obtained from analyzing the signals of the bubbles that contain velocity information. The chord lengths of these bubbles measured by FOR represent the bubble diameters when the bubble shape is spherical or represent the minor axes when the bubble shape is ellipsoidal. The velocity and size of bubbles obtained from the FOR measurements are compared with those obtained simultaneously using a high speed camera.