[HLA-mismatched bone marrow transplantation with post-transplant cyclophosphamide for pediatric patients with aplastic anemia].

Post-transplant cyclophosphamide (PTCy) has improved the efficacy of HLA-mismatched hematopoietic cell transplantation (HCT) by decreasing the risk of graft-versus-host disease (GVHD) and nonrelapse mortality. If an HLA-matched donor is not available, GVHD prophylaxis with PTCy can also be used for HLA-mismatched HCT in patients with pediatric aplastic anemia (AA). We report two cases of pediatric AA that were treated with HLA-mismatched HCT with reduced-intensity conditioning and PTCy. We administered 50 mg/kg/day Cy for GVHD prophylaxis on days 3 and 4, and tacrolimus and mycophenolate mofetil (or methotrexate) were initiated from day 5. In both the cases, the time to engraftment was favorable and GVHD and infection were controllable. PTCy probably allows us to expand donor candidates in pediatric AA when an HLA-matched donor is not available; however, further studies regarding optimal conditioning regimens and late complications are required.

Large difference between Enghoff and Bohr dead space in ventilated infants with hypoxemic respiratory failure.

BACKGROUND: Ventilated neonates with hypoxemic respiratory failure (HRF) may show a ventilation-perfusion (V/Q) mismatch. OBJECTIVE: To evaluate the difference between the Bohr (Vd, Bohr ) and Enghoff (Vd, Enghoff ) dead spaces in infants by using volumetric capnography based on ventilator graphics and capnograms. METHODS: This study enrolled 46 ventilated infants (mean birth weight, 2239 ± 640 g; mean gestational age, 35.5 ± 3.3 weeks). We performed volumetric capnography and calculated Vd, Bohr and Vd, Enghoff when arterial blood sampling was necessary for treatment. According to the oxygenation index (OI) based on the Montreux definition of neonatal acute respiratory distress syndrome, each measurement was classified into the HRF (OI ≥ 4) or control (OI < 4) group. Then, a regression analysis was performed to evaluate the correlation between the OI and the difference between Vd, Enghoff and Vd, Bohr . RESULTS: The median Vd, Enghoff /tidal volume (VT ) was significantly higher in the HRF group (0.55 [interquartile range, 0.47-0.68]) than in the control group (0.46 [0.37-0.57]). The HRF group showed a larger difference between Vd, Enghoff /VT and Vd, Bohr /VT than the control group (median, 0.22 [0.15-0.29] vs. 0.10 [0.06-0.14], respectively). Moreover, the regression analysis of the relationship between OI and Vd, Enghoff /VT - Vd, Bohr /VT showed a positive correlation (r = .60, p < .001). CONCLUSION: Ventilated neonates with hypoxemic respiratory failure showed a large difference between Vd, Enghoff and Vd, Bohr , possibly reflecting a low V/Q mismatch and right-to-left shunting.