Efficacy of autologous peripheral blood stem cell (PBSC) harvest and engraftment after ablative chemotherapy in pediatric patients.

Thirty-five pediatric patients, 1-16 years of age (median 6.3 years), with neoplastic solid tumors (n=32) or acute leukemia (n=3) underwent peripheral blood stem cell (PBSC) harvest and transplantation at Children's Memorial Hospital between September 1992 and April 1997. A median of four phereses were performed on each patient. Blood samples from 34 of the 35 patients were harvested through existing double-lumen central catheters, using either a Fenwal CS-3000 or COBE Spectra pheresis machine. The pheresis procedures were well tolerated overall. A median of 3.7 x 10(6)/kg CD34+ cells were infused (range, 0.2-15.5 x 10(6)/kg), and all patients engrafted. The median time to an absolute neutrophil count >500/microL was 13 days (range, 9-44 days) and to a platelet count >20,000/microL was 21 days (range, 9-210 days). Two patients died from transplant-related complications. Patients were discharged from the hospital after a median of 22 days (range, 15-64 days). Twenty of the 35 patients are alive, 17 of whom remain disease-free with a median follow-up of 1144 days. According to this study, PBSCs can be successfully harvested and re-infused for marrow reconstitution after myeloablative therapy in children for a variety of pediatric malignancies with low morbidity and mortality.

Red cell depletion of umbilical cord blood (UCB): comparison between unmanipulated and red cell-depleted UCB by Ficoll-Paque density gradient separation.

Stem cells from umbilical cord blood (UCB) represent an alternative source of cells for clinical transplantation. Many issues remain unsolved with regard to their collection, manipulation, and storage. In this study, we attempted to compare the effect of red cell depletion by Ficoll-Paque density gradient separation versus unmanipulated cord blood stem cells postthawing. We found no statistical difference between the two technologies when comparing viability, 98.6 +/- 0.3% versus 99.1 +/- 0.42% (p = < 0.16); CD34+/CD38+, 1.6 +/- 0.13% versus 1.2 +/- 0.17% (p = < 0.13); HLA DR+/CD34+, 1.8 +/- 0.15% versus 1.9 +/- 0.21% (p = < 0.6); blast colonies, 8.0 +/- 1.5 versus 12.2 +/- 2.1 (p = < 0.15); CFU-GEMM colonies, 143.7 +/- 27.9 versus 80.7 (p = < 0.10); CFU-GM colonies, 101.2 +/- 23 versus 173 +/- 23.2 (p = < 0.07). There was a statistical difference in the content of CD34+/CD38+, 1.6 +/- 0.14% versus 1.2 +/- 0.14% (p = < 0.05), and the BFU-E colonies, 96 +/- 29.8 versus 165 +/- 23.9 (p = < 0.03). We conclude that red cell depletion using Ficoll-Paque gradient separation preserves viability of progenitor cells, as evidenced by immunophenotyping and colony assays.

Mixed germ cell tumor of the ovary with a prominent polyembryoma component.

A mixed germ cell tumor of the ovary containing polyembryoma and abundant vascular tissue occurred in a 17-year-old woman. She underwent surgical resection of the tumor. Both 1 and 3 years after the original surgery, metastatic tumor deposits composed of mature and immature teratoma were excised from the abdominal cavity. Eleven years after her original surgery, she is living and well.

Congenital absence of the pulmonary valve.

Congenital absence of the pulmonary valve (CAPV) is a rare anomaly usually associated with ventricular septal defect or Fallot's tetralogy. CAPV may be rapidly fatal in infancy because of severe disturbance of pulmonary ventilation resulting from bronchial compression by massively dilated pulmonary arteries. Other cases may be relatively benign. Our group of five cases with CAPV ranged from the severe to one case in which there was documented closure of a patent ductus arteriosus and a ventricular septal defect, leaving the CAPV as an apparently isolated, well-tolerated lesion. Plain chest radiographs play a major part in the diagnosis of CAPV since they almost invariably give some indication od the aneurysmal dilatation of the pulmonary arteries. On angiography, dilated main pulmonary arteries are shown giving rise to branches of normal or even diminished caliber.

Status of patients 5 or more years after correction of coarctation of the aorta over age 1 year.

In this retrospective study, we reviewed the records of patients who had coarctectomies at the University of Virginia Hospital after 1 year of age. Follow-up data for 5 years or more after surgery were available for 52 patients. Data from 23 similar patients from the Medical College of Virginia brought the total postoperative sample size to 75. The blood pressure of this group of patients did not differ significantly from that of the population at large. We conclude that successful repair of coarctation of the aorta in childhood or early adolescence does not lead to a higher-than-expected incidence of resting hypertension in childhood.

Attitudinal versus ecological approaches to the characterization of institutional treatment environments.

The relational and discriminative properties of attitudinal and ecological measures of environments were examined within the context of a state hospital for mentally retarted clients. Three hypotheses were advanced, and the results gave substantial support for one of these and partial support for a second: (a) that the correlations between attitudinal and ecological measures should not differ significantly from zero, and (b) that differences among hospital treatment programs and living units should be reflected in ecological but not in attitudinal measures. However, the results did not provide clear confirmation for a third hypothesis: that differences among staff demographic characteristics and employment history should be reflected in attitudinal but not in ecological measures. It was suggested that these background variables may not be appropriate measures of personality-linked attributes.

Single coronary artery with a fistula to the main pulmonary artery in a patient with tetralogy of Fallot.

The presence of a single coronary artery arising in the anterior cusp and terminating in a large fistula to the main pulmonary artery was noted during the preoperative evaluation of a patient with tetralogy of Fallot. Recognition of this rare association dictated the use of a valved conduit to avoid injury to the left anterior descending coronary as it crossed the right ventricular outflow tract and permitted abolition of intracardiac shunting by ligation of the fistula. It is postulated that the increasing arterial saturation noted in this patient prior to intracardiac repair may have been related in part to progressive augmentation in flow through the coronary fistula to the main pulmonary artery.

Fluidotherapy: evaluation of a new heat modality.

The development of a new heat therapy modality (fluidotherapy) consisting of a bed of solid particles suspended in air is reported. Measurements of time integrated heat absorption by a hand immersed in the fluidotherapy unit at 120F (48.9C), a whirlpool at 105.2F, and a paraffin bath at 126F show the average heat absorption to be 17.2, 5.6 and 4.8 BTU respectively during a 15-minute treatment.

Factor structure of the SREB Attendant Opinion Scale.

A factor analysis of the SREB (Southern Regional Education Board) Attendant Opinion Scale was performed, based on the responses of 105 subjects. The results were very similar to those reported by the authors of the scale. Four factors were interpreted as relating to job dissatisfaction, maintenance of the rights of residents, belief in making residents conform to moral standards, and authoritarianism. The scale appears to be a promising instrument for evaluating attendants' attitudes.

Comparison of two sets of Piagetian scales with severely and profoundly retarded children.

Corman and Excalona's (1969) scales for Object Permanence and Spatial Relationships and the corresponding scales of Uzgiris and Hunt (1975) were administered to 64 severely and prfoundly retarded children. Most of the findings applied equally to both sets of scales: the scoring reliability was very high; the total scores did not vary as a function of subject characteristics (except IQ) or testing conditions; differences in the difficulty of the items did not correspond closely to those reported for nonretarded infants, and the scalability of the items was much lower. Less time was required to administer the two scales constructed by Uzgiris and Hunt, but their Object Permanence scale showed a pronounced ceiling effect and the internal consistency of their Spatial Relationships scale was unacceptably low.