Tumor biopsy is rarely performed in diffuse intrinsic pontine glioma (DIPG) due to the presumed risk of surgical complications, although data on the surgery related morbidity of DIPG biopsy is sparse. We performed a retrospective review on 22 consecutive cases of DIPG diagnosed from 2002 to 2012 at Children's Hospital of Michigan, 15 of which underwent biopsy. Transient new or worsening neurological deficits were observed in three of 15 cases following surgery. No surgery related mortality or permanent deficit was observed, and the mean overall survival was 10.4 ± 3.8 months. Undergoing biopsy did not adversely affect the outcome.
The β thalassemia syndromes constitute the most frequent inherited anemia managed with chronic red cell transfusions around the world. 1,2 The prevalence of thalassemia in the United States was underestimated in past surveys that were limited only to the major specialty centers. 3 Recent data show that the aggregate number of patients followed at smaller centers and community practices surpasses those at the major centers. 4 A precise estimate of the total number of individuals with thalassemia in the U.S. is unavailable due to the lack of either a state or national database. Surveys have shown that 10 large thalassemia centers in the country collectively follow about 1100 patients, while additional 1500 patients are estimated to receive care at other hospitals. 4,5 The Thalassemia Western Consortium (TWC) study showed that of the 717 patients, 44% of patients had β thalassemia syndromes (including 15% with HbE β thalassemia) and 55% had various α thalassemia disorders. Transfusion-dependent patients comprised 35% of the population, 9% had received 1 or more life-time transfusions, and 56% had never been transfused. Thus,
1182 Introduction: ABO hemolytic disease of the newborn occurs almost exclusively in infants of blood group A or B who are born to group O mothers. Although ABO incompatibility is common, its related hemolytic disease has been reported to be low. In this study, we aimed to investigate the rate of direct anti-globulin test (DAT) positivity and clinical events, such as hyperbilirubinemia or anemia in infants born to group O mothers. Methods: Using the charge code for cord blood evaluation, we were able to identify all cord blood evaluations from January 1, 2006 - December 31, 2007, and then select out the ABO incompatible births from group O mothers. We then reviewed the electronic medical records for demographic, clinical and laboratory information. Clinical events (anemia, jaundice, hemolytic disease) were investigated only in babies born at 37 weeks or higher gestation. Chi-square tests were used to cross-tabulate clinical events, demographic parameters (gender, ethnicity), and laboratory parameters (AO versus BO incompatibility, DAT-positivity). Results: There were 10,891 live births during the two-year period and 1519 (14%) of these were ABO incompatible. ‘Black’ ethnicity was registered in 80% of these babies. AO and BO incompatibility comprised 57.8% and 42.2% of the cases, respectively. 5.3% of the cases had concomitant Rh incompatibility. DAT was positive in 16.7% of the cases: 13.8% weakly or 1+ positive, and 2.9% 2+ or 3+ positive. DAT was more commonly positive among BO-incompatible cases compared to AO-incompatible cases (21.7% versus 13.1%). Among blacks, DAT-positivity in BO incompatibility was more common (24.9% among blacks compared to 7.8% among non-blacks, p<0.001). Concomitant Rh incompatibility did not affect DAT positivity rate. Among AO-incompatible babies, DAT-positivity was more frequent among females (15.5% in females vs 10.8% in males, p=0.045). 1299 babies were born at term (3 37 weeks gestation).of these infants, hyperbilirubinemia (defined as indirect bilirubin 3 8 mg/dL) was detected in 17.3% of babies. This was significantly associated with DAT positivity (40.6% in DAT-positive cases vs 12.3% in DAT-negative cases, p<0.001) and BO incompatibility (p=0.001). Hemolytic anemia (defined as hematocrit £ 45% and reticulocyte count 3 250,000/mm3 in the first week of life) was noted in 3.4% of cases, and was significantly associated with DAT positivity (13.2% in DAT-positive cases vs 1.1% in DAT-negative cases, p<0.001); BO incompatibility (p=0.001); and black ethnicity (p=0.001). Discussion: Our study indicated that cord blood DAT was positive in 16.7% of ABO incompatible pregnancies. BO-incompatible cases were more likely to be DAT-positive in blacks. AO-incompatibility was more common among girls, consistent with earlier studies that had shown a stronger A antigen expression among female newborns. DAT-positive cases were more likely to develop hyperbilirubinemia or hemolytic anemia. In addition, black ethnicity and BO incompatibility conferred significantly increased risk of hemolytic anemia in our study. Despite this strong association, the sensitivity of the positive DAT was 41.3% for hyperbilirubinemia and 70.5% for hemolytic anemia in ABO incompatibility. Disclosures: No relevant conflicts of interest to declare.
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