Prospective research on infants with mild encephalopathy: the PRIME study.

OBJECTIVE: To determine short-term outcomes of infants with evidence of hypoxia-ischemia at birth and classified as mild neonatal encephalopathy (NE) at <6 h of age. STUDY DESIGN: Prospective multicenter study. Mild NE was defined as ⩾1 abnormal category in modified Sarnat score. Primary outcome was any abnormality on early amplitude integrated electroencephalogram (aEEG) or seizures, abnormal brain magnetic resonance imaging (MRI) or neurological exam at discharge. RESULTS: A total of 54/63 (86%) of enrolled infants had data on components of the primary outcome, which was abnormal in 28/54 (52%): discontinuous aEEG (n=4), MRI (n=9) and discharge exam (n=22). Abnormal tone and/or incomplete Moro were the most common findings. MRI abnormalities were confined to cerebral cortex but two infants had basal ganglia and/or thalamus involvement. The 18 to 24 months follow-up is ongoing. CONCLUSIONS: A larger than expected proportion of mild NE infants with abnormal outcomes was observed. Future research should evaluate safety and efficacy of neuroprotection for mild NE.

Children born at 32 to 35 weeks with birth asphyxia and later cerebral palsy are different from those born after 35 weeks.

OBJECTIVE: The objectives of this study were to (1) establish the proportion of cerebral palsy (CP) that occurs with a history suggestive of birth asphyxia in children born at 32 to 35 weeks and (2) evaluate their characteristics in comparison with children with CP born at ⩾36 weeks with such a history. STUDY DESIGN: Using the Canadian CP Registry, children born at 32 to 35 weeks of gestation with CP with a history suggestive of birth asphyxia were compared with corresponding ⩾36 weeks of gestation children. RESULTS: Of the 163 children with CP born at 32 to 35 weeks and 738 born at ⩾36 weeks, 26 (16%) and 105 (14%) had a history suggestive of birth asphyxia, respectively. The children born at 32 to 35 weeks had more frequent abruptio placenta (35% vs 12%; odds ratio (OR) 4.1, 95% confidence interval (CI) 1.5 to 11.2), less frequent neonatal seizures (35% vs 72%; OR 0.20, 95% CI 0.08 to 0.52), more frequent white matter injury (47% vs 17%; OR 4.3, 95% CI 1.3 to 14.0), more frequent intraventricular hemorrhage (IVH) (40% vs 6%; OR 11.2, 95% CI 3.4 to 37.4) and more frequent spastic diplegia (24% vs 8%; OR 1.8, 95% CI 1.2 to 12.2) than the corresponding ⩾36 weeks of gestation children. CONCLUSIONS: Approximately 1 in 7 children with CP born at 32 to 35 weeks had a history suggestive of birth asphyxia. They had different magnetic resonance imaging patterns of injury from those born at ⩾36 weeks and a higher frequency of IVH. Importantly, when considering hypothermia in preterm neonates with suspected birth asphyxia, prospective surveillance for IVH will be essential.

Clinical profile of children with cerebral palsy born term compared with late- and post-term: a retrospective cohort study.

OBJECTIVE: To determine whether cerebral palsy (CP) risks factors, neurological subtype, severity and co-morbidities differ between early/full-term-born children with CP compared with those born late/post-term. DESIGN: Retrospective cohort study. SETTING: Children with CP born between 1998 and 2014, residing in Canada, and registered in the Canadian Cerebral Palsy Registry (CCPR) (n = 1691), a database with information from 15 participating centres across six Canadian provinces. POPULATION: Children with CP from the CCPR born at 37 weeks of gestation and later (n = 802). METHODS: The clinical profile of children with CP born at 37-40 weeks of gestation was compared with those born at 41 weeks and later using the Pearson chi-square test (or Fisher's exact test) for univariate analyses of categorical data. A P value <0.05 was considered significant a priori. MAIN OUTCOME MEASURES: CP neurological subtype, Gross Motor Function Classification System (GMFCS) severity, risk factors and co-morbidities. RESULTS: Neonatal encephalopathy was found in 23.9% of children with CP born early/full-term and in 33.6% of those born late/post-term (P = 0.026). Neonatal hyperbilirubinaemia was found in 10.2% of children born in the earlier period and in 2.6% of those born in the later period (P = 0.008). Apgar score at 5 minutes, but not 10 minutes, was significantly higher in the early/full-term group (9) compared with its late/post-term counterpart (7; P = 0.046). Rates of CP subtype, severity (GMFCS) and co-morbidities did not differ significantly between the two gestational periods. CONCLUSIONS: In children with CP, neonatal encephalopathy was significantly less frequent and neonatal hyperbilirubinaemia was significantly more frequent in those born early/full-term compared with their later-born counterparts. However, clinical outcomes of CP were not significantly different between these two gestational epochs. TWEETABLE ABSTRACT: Children with cerebral palsy born early/full-term have similar outcomes to those born late/post-term.

Mutations in the gene encoding c-Abl-binding protein SH3BP2 cause cherubism.

Cherubism (MIM 118400) is an autosomal dominant inherited syndrome characterized by excessive bone degradation of the upper and lower jaws followed by development of fibrous tissue masses, which causes a characteristic facial swelling. Here we describe seven mutations in the SH3-binding protein SH3BP2 (MIM 602104) on chromosome 4p16.3 that cause cherubism.

Representations of self and other: a semantic space model.

This article describes a semantic space model of personality. According to the model, representations of facets of the self (e.g., actual self, ideal self) and of others are arrayed in a semantic space, with proximities among representations predicted to be associated with mood, self-evaluation, and broad personality dimensions. The relation of proximities among pairs of representations to personality and mood measures was found to be mediated by the spatial proximity of other representations, indicating that the representations constitute dynamic systems. Proximities among representations were shown to predict self-esteem and self-evaluative moods even after controlling for the contribution of the personality dimensions of Neuroticism, Extraversion, and Agreeableness. Generally, the semantic space model led to better predictions than did several related models concerning representations of self and other.

Trends in the use of drug therapies in patients with acute myocardial infarction: 1988 to 1992.

OBJECTIVES: In this study, we investigated the use of thrombolytic agents and other cardiac drugs in a national cohort of patients with acute myocardial infarction and assessed the influence of large clinical studies on types of thrombolytic therapy prescribed. BACKGROUND: Information about usage patterns for these drugs is unavailable, and little is known about the impact of large clinical trials on their use. METHODS: We conducted a retrospective cohort study of 65,011 patients who were treated for acute myocardial infarction during fiscal years 1988 to 1992 (October 1, 1987 to September 30, 1992) in hospitals participating in the SMS Corporation's on-line data pool. RESULTS: The overall thrombolysis rate for patients with acute myocardial infarction increased from 11% in fiscal year 1988 to 18% in fiscal year 1990 and has remained approximately at that level since then. In mid-1989, tissue plasminogen activator was used in 90% of the patients receiving thrombolysis, whereas streptokinase was used in only 10%. Since 1991, tissue plasminogen activator has been used in 60% of patients and streptokinase in almost 30%. Much of this change came after presentation and publication of results of the Second Gruppo Italiano per lo Studio Della Sopravvivenza nell'Infarto Miocardico (GISSI-2) and the Third International Study of Infarct Survival (ISIS-3) trials. Over these 5 years, use of beta-adrenergic blocking agents increased steadily, and use of calcium-channel blocking agents declined steadily. CONCLUSIONS: Current usage rates of thrombolytic therapy are lower than expected, but trends in usage rates for beta-blockers and calcium channel blockers reflect their increasing and decreasing approval, respectively. Presentation and publication of results from the Third International Study of Infarct Survival and the Second Gruppo Italiano per lo Studio Della Sopravvivenza nell'Infarto Miocardico trials appear to have influenced the type of thrombolytic agent prescribed.

The relation between resource use and in-hospital mortality for patients with acquired immunodeficiency syndrome-related Pneumocystis carinii pneumonia.

A central issue in health policy with regard to the acquired immunodeficiency syndrome (AIDS) is whether quality of care and patient outcomes are affected by resource constraints. In an earlier study of 15 California hospitals between October 1986 and October 1987, we observed a markedly lower in-hospital mortality rate for Pneumocystis carinii pneumonia in the group of patients treated in hospitals that had a high level of experience with AIDS relative to the group treated in hospitals with low experience. We present the patterns of resource use at hospitals with high and low AIDS familiarity. Average charges and resource use did not differ between the two groups of hospitalized patients; however, there were marked variations in how the resources were used. Among survivors, patients who received care at hospitals with high AIDS familiarity stayed in the hospital longer, underwent a bronchoscopy more often, stayed in an intensive care unit longer, and accrued higher average total charges than patients at hospitals with low AIDS familiarity. Conversely, among nonsurvivors, a greater intensity of care was received at the hospitals with low AIDS familiarity. These results suggest that, in these 15 hospitals, the markedly higher rate of in-hospital death at hospitals with low AIDS familiarity was not related to the quantity of resources that were used; rather it was related to differences in how the resources were used. Our results show that additional resources significantly improved the chances of in-hospital survival for patients at hospitals with high AIDS familiarity, but did not affect the chances of survival in hospitals with low AIDS familiarity. Our findings suggest that physicians in those hospitals in which the care of patients with AIDS is relatively infrequent might improve the chances of in-hospital survival of patients with AIDS by more timely and efficient use of resources.

The relation between hospital experience and in-hospital mortality for patients with AIDS-related PCP.

There is marked debate by physicians and policymakers regarding the creation of regionalized acquired immunodeficiency syndrome (AIDS) centers. A central issue is whether outcomes of care, particularly mortality, differ as a function of hospital experience with patients with AIDS. We evaluated the experience of 257 patients with AIDS and Pneumocystis carinii pneumonia treated at 15 California hospitals between October 1986 and October 1987. An overall 15.2% in-hospital mortality rate was observed. However, a markedly lower in-hospital mortality rate was observed in the group of patients treated at hospitals that had a high level of experience with patients with AIDS (greater than or equal to 30 human immunodeficiency virus-related discharges per 10,000 hospital discharges) relative to the group treated at hospitals with less experience (less than 30 human immunodeficiency virus-related discharges per 10,000 hospital discharges): 12% vs 33%. Other factors significantly associated with in-hospital mortality included intensive care unit use, admission from an emergency department or through an interhospital transfer, and a history of hospitalizations. A logistic regression model indicated that, after controlling for severity indicators, AIDS experience remained significantly related to mortality. Our findings suggest that policymakers should consider three options: creating regional AIDS centers, implementing policies that promote a rapid but carefully monitored increase in experience of low-volume hospitals with human immunodeficiency virus-infected individuals, or providing highly focused educational efforts at low-AIDS-experience facilities. Without such policy initiatives, differences in mortality rates like those we have found might persist as cases of AIDS begin to occur in every area of the country.

Cryptosporidial and cytomegaloviral hepatitis and cholecystitis.

We describe an immunosuppressed patient with enteric cryptosporidiosis who developed combined cryptosporidial and cytomegaloviral hepatitis and cholecystitis as well as Enterobacter cloacae cholecystitis. To our knowledge, the presence of Cryptosporidium in a liver biopsy specimen has not previously been reported.

Detection and quantitation of Newcastle disease virus proteins in infected chicken embryo cells.

A technique was analyzed by which Newcastle disease virus (NDV) proteins could be quantitatively detected in the presence of chicken embryo cellular proteins in NDV-infected cells. The technique involved removal of electropho-proteins from a sodium dodecyl sulfate-polyacrylamide-agarose gel matrix by chemical cleavage of the acrylamide gel cross-linker. The proteins were subsequently transferred and covalently bound to diazobenzyloxymethyl paper. By incubating the paper with unlabeled antisera and 125I-labeled Staphylococcus aureus protein A, the specificity of the antisera and the sensitivity of this method of quantitative antigen detection were tested. The results demonstrated that as little as 1 ng of an individual NDV protein could be detected. Furthermore, this technique can simultaneously quantitate the synthesis of multiple NDV proteins under experimental conditions in which immunofluorescence, hemadsorption, and plaque assays failed to show virus protein synthesis or the formation of virus progeny.

Infants with birth weight under 1500 g: physical, neurological, and developmental outcome.

One hundred and seventy-eight infants with birth weights less than or equal to 1500 g born in 1973-1975 were followed for a period of 1-3 years, and the physical, neurological, and developmental outcome evaluated. Although there was a high incidence of maternal problems, these did not correlate with outcome. Asphyxia at birth followed by neonatal complications leading to ventilatory assistance was significantly correlated with poor outcome. Over 50% of infants less than 100 g birth weight required assisted ventilation, but the outcome in small ventilated infants was comparable to that of ventilated infants of 1001-1500 g birth weight. Infants with neurological abnormality showed a high incidence of associated abnormalities in growth, vision, hearing, and development. Spastic quadriplegia emerged as the most common neurological diagnosis. Despite the many perinatal problems, 82% of the group were normal neurologically and 66% developmentally. The overall outcome was generally favorable in these infants even for those requiring ventilation.