Perinatal factors associated with retinopathy of prematurity

The etiology of retinopathy of prematurity appears to be multifactorial. Introduction of new treatments in neonatal care may add new risk factors. We have analyzed thc relationship between 42 perinatal factors and the development of retinopathy of prematurity in 78 infants with a birth weight < 1501 g and/or gestational age < 33 weeks. We have also applied a chronological analysis of the maximum and minimum pO₂ and pCO₂ values. Retinopathy of prematurity was seen in 37 of 78 infants (47.4%)). Nineteen factors were found to be related to the development of retinopathy of prematurity. However, when step-wise logistic regression analysis was used, only birth weight, number of days of oxygen therapy and use of beta-blocking agents by the mother before birth were found to be associated with the development of retinopathy or prematurity. The results suggest that medication with beta blockers immediately before birth should be used cautiously.     

Perinatal factors associated with retinopathy of prematurity

The etiology of retinopathy of prematurity appears to be multifactorial. Introduction of new treatments in neonatal care may add new risk factors. We have analyzed thc relationship between 42 perinatal factors and the development of retinopathy of prematurity in 78 infants with a birth weight < 1501 g and/or gestational age < 33 weeks. We have also applied a chronological analysis of the maximum and minimum pO₂ and pCO₂ values. Retinopathy of prematurity was seen in 37 of 78 infants (47.4%). Nineteen factors were found to be related to the development of retinopathy of prematurity. However, when step-wise logistic regression analysis was used, only birth weight, number of days of oxygen therapy and use of beta-blocking agents by the mother before birth were found to be associated with the development of retinopathy or prematurity. The results suggest that medication with beta blockers immediately before birth should be used cautiously.     

Reporting the outcomes of care: effecting the transition from caring to comparing

Accountability in health care has taken on new dimensions with the drive to base contracting and provider of care selection upon data driven "report cards." The measurement and development of reportable outcomes are driving providers to move their organizations from a focus not only on "caring" but to one highly cognizant of "comparing" in order to maintain market position and meet regulatory requirements. This article defines the areas of organizational transition required for reporting, and profiles the actions taken by three health care providers moving to an organizational style ready for "comparative" competition.     

10 years' experience with regular haemodialysis and renal transplantation

75 children aged under 15 years have entered the regular haemodialysis/renal transplant programme at Guy's Hospital in the 10 years since its inception; 13 children have subsequently died. A combination of hospital and home haemodialysis and renal transplantation was used. 64 children received 80 renal allografts; 37 1st grafts were from live, related donors and 27 were from cadaver donors. The 5-year acturial patient survival for the whole group and for those who had transplants was 76%. Live donor graft survival was 65% at 3 years, and 55% at 5 years; 1st cadaver graft survival was 42% at 3 years. Results obtained during the last 6 years of the 10-year period showed an improvement with a live related donor graft survival rate of 71%, and a 1st cadaver graft survival rate of 47% at 3 years. Particular experience was gained with home haemodialysis and live, related donor transplantation. Despite growth, and psychosocial and rehabilitation problems, the overall results were encouraging, particularly for the 46 children who had successful transplants. These children grew better, had fewer psychosocial difficulties, and were rehabilitated more successfully into normal life than those on long-term dialysis. We conclude that dialysis and transplantation should be offered to all suitable children with terminal renal failure.     

Mutation analysis in 46 British and Irish patients with Gaucher's disease

DNA from 46 unrelated patients with Gaucher's disease was analysed for 10 known mutations: 84GG(c84 G 85ins), N370S (c1226G), L444P (c1448C), R463C (c1504T), R496H (c1604A), IVS2+1, D409H (c1342C), RecNcil (c1448C-1498C), RecTL (c1342C-1498C), and c1263del (c1264-1318del). Fifty four mutations (90%) were identified in 30 patients with type I disease. These included a previously undescribed recombinant mutation RecA456P (c1448C-1484C). Thirteen (54%) of 24 type II alleles were identified, including one new point mutation N462K (c1503G) and one new 55bp deletion also incorporating the RecTL mutations c1263del+RecTL (c1264del-1498C). All four type III patients were homozygous for the L444P point mutation. Generally, patients with one copy of the N370S mutation had mild adult onset disease, regardless of the nature of their second mutation. Three exceptions had childhood onset disease and genotypes N370S/R463C, N370S/RecA456P, and N370S/?. The L444P/L444P genotype was thought to be associated with neurological disease. Two type I patients with this genotype who exhibited no central nervous system disease were identified, however. The R463C and c1263del mutations were found to be present at a higher frequency than reported in other populations and they should be included in any mutation screen of this population. The recombinant mutations RecA456P and c1263del+RecTL have not been previously described and are the fourth and fifth recombinant mutations identified in the glucocerebrosidase gene.     

The role of Epstein-Barr virus in Hodgkin's disease from different geographical areas

Recent studies have suggested that Epstein-Barr virus (EBV) may play a role in the aetiology of Hodgkin's disease. To determine the role of EBV in childhood Hodgkin's disease in different geographical areas, immunohistochemical staining and in situ hybridisation were used to analyse latent membrane protein 1 (LMP 1) and small nuclear non-transcribed RNAs (EBER-1) respectively. Testing for EBV within the Reed-Sternberg and Hodgkin's cells was carried out in childhood Hodgkin's disease from 10 different countries. The proportion of LMP 1 positive cases varied significantly, being 50% of cases from the United Kingdom (38/75), South Africa (9/18), Egypt (7/14), and Jordan (8/16), 60% from the United Arab Emirates (6/10), 70% from Australia (11/16), 81% from Costa Rica (34/42), 88% from Iran (7/8), 90% from Greece (20/22), and 100% of the 56 cases from Kenya. A sensitive polymerase chain reaction based EBV strain typing technique was established using archival tissues. EBV strain type 1 was shown to be predominant in childhood Hodgkin's disease from the United Kingdom, South Africa, Australia, and Greece. Type 2 was predominant in Egypt. EBV strain types 1 and 2 were both detected in some cases of childhood Hodgkin's disease in the United Kingdom, Costa Rica, and Kenya. The high incidence of EBV and the presence especially in developing countries of dual infection with both strain types 1 and 2 may reflect socioeconomic conditions leading to malnutrition induced immunological impairment. The possibility of HIV infection also needs to be explored.     

Focus-forming ability and surface markers of hamster-human malignant lymphoma hybrids

We have examined the properties of hybrid cells formed by polyethylene glycol-mediated fusion of the GRC+L-73 line of Chinese hamster ovary (CHO) cells with peripheral blood cells from patients with chronic lymphocytic leukemia (CLL) or with bone marrow cells from patients with malignant lymphoma. The results indicate that hybrid cells can be detected by their ability to form "foci" of characteristic morphology in the presence of a monolayer of parental CHO cells and that clones isolated from such foci express aspects of the differentiation status, as detected by immunologic markers, of the human parental cells.     

Distribution of complement receptors on human normal and malignant mononuclear cells

Mononuclear cells from normal human subjects and patients with chronic lymphocytic leukemia (CLL), chronic lymphosarcoma cell leukemia (LCL), and hairy cell leukemia (HCL) were labeled with fluoresceinated, purified human C3b (FI-C3b) and analyzed using the fluorescence- activated cell sorter (FACS). FI-C3b labeled 17.6% +/- 6.0% of peripheral blood mononuclear cells (PBM) from 20 normal subjects, which, when separated by the FACS, consisted of B lymphocytes and approximately 5% monocytes. Analyses in which either monocytes or B lymphcoytes were excluded from consideration demonstrated that both these cell types were labeled by the FI-C3b with a heterogeneous distribution of fluorescence intensity, indicating either heterogeneity of CR density or variable avidity of individual CR for the FI-C3b. FACS profiles of PBM ( < 5% monocytes) from 14 of 15 patients with CLL showed a homogeneous distribution of very low fluorescence intensity, with > 60% of the cells being slightly more fluorescent than unlabeled controls. This low, homogeneous distribution of fluorescence is strikingly similar to profiles of CLL cells labeled with anti-Ig reagents and suggests homogeneity of low CR density and/or avidity. Similarly, CR+ mononuclear cells from five patients with HCL and three patients with LCL displayed more homogeneous FI-C3b labeling than normal CR+ PBM. Homogeneity of FI-C3b binding to CLL, LCL, and HCL cells further supports the concept for a clonal origin for these disorders.