Differences in mortality characteristics in neonates with Down's syndrome.

OBJECTIVE: Neonates with Down's syndrome (nDS) may have multiple medical issues that place them at increased risk for mortality during the newborn period. Goal of this study was to determine if there are differences in baseline characteristics, medical complications or procedures performed during hospitalization between nDS who survived versus those who died during initial hospitalization. STUDY DESIGN: Data from 2000 to 2014 were reviewed using the Pediatric Health Information Systems (PHIS) database on all DS patients admitted to the hospital <30 days postnatal life. Baseline demographics, medical complications, procedures performed and mortality were recorded. Patients were divided into nDS patients who were discharged alive (nDS-a) versus nDS patients who died (nDS-d). Multivariate logistic analysis with odds ratios was performed to determine significant predictors of death. A P<0.05 was considered significant. RESULTS: A total of 5737 nDS were evaluated. Overall mortality was 7.5% (431/5737). nDS-d were more likely than nDS-a to have a lower birth weight (1.0 (0.9 to 1.0)), presence of a diaphragmatic hernia (6.9 (1.9 to 25.1), or a cardiac diagnosis of a pulmonary venous abnormality (6.8 (1.9 to 24.4)), Ebstein's anomaly (3.2 (1.2 to 8.5)) or left-sided obstructive lesion (2.0 (1.3 to 3.0). nDS-d were more likely to develop hydrops (5.7 (3.5 to 9.5)) and necrotizing enterocolitis (1.7 (1.2 to 2.6)). In addition, nDS-d had significantly higher odds of requiring mechanical ventilation (20.7 (9.9 to 43.1)) or extracorporeal membrane oxygenation (8.7 (4.7 to 16.1)). CONCLUSIONS: A number of characteristics, specifically certain cardiac diagnosis, place nDS at increased risk for mortality. Furthermore, development of specific medical complications or need for particular procedures increases the odds for mortality in nDS. Caregivers should be cognizant that they are taking care of a high-risk population nDS with an increased risk for mortality if these variables are present.

Right ventricular performance using myocardial deformation imaging in infants with bronchopulmonary dysplasia.

OBJECTIVE: Right ventricular (RV) performance among infants with bronchopulmonary dysplasia (BPD) remains poorly understood. We tested the hypothesis that myocardial deformation imaging (MDI) strain and strain rate would allow for differentiation between infants with severe and milder forms of BPD, independent of tissue Doppler imaging (TDI) and superior to conventional echocardiographic measurements. STUDY DESIGN: Infants with various severities of BPD (11 with none or mild, 13 with moderate and 10 with severe) underwent conventional echocardiography, TDI and MDI assessments at >36 weeks of corrected gestational age. BPD severity grading was determined according to the National Institutes of Child Health and Disease workshop rating scale by physicians blinded to the echocardiogram results. Group data were compared with one-way analysis of variance or Kruskal-Wallis tests, with post hoc multiple comparisons. RESULTS: No differences in traditional echocardiographic parameters or TDI among the three BPD severity groups were observed; none of the infants had evidence of pulmonary hypertension. Using MDI, infants with severe BPD had lower peak global systolic strain than did infants with moderate BPD (P<0.01) or mild/none BPD (P<0.01). Early and late diastolic strain rate measurements were similar across the three groups. CONCLUSIONS: Among infants with severe forms of BPD, evidence of abnormal RV systolic function was detected with MDI, but not traditional echocardiographic or TDI measurements. Infants with severe forms of BPD may represent a particularly high-risk subgroup for decreased RV performance warranting cardiac surveillance. MDI should be considered as a method to quantitate RV function in this population.

Assessment of the severity of Ebola virus disease in Sierra Leone in 2014-2015.

The current Ebola virus disease (EVD) epidemic in West Africa is unprecedented in scale, and Sierra Leone is the most severely affected country. The case fatality risk (CFR) and hospitalization fatality risk (HFR) were used to characterize the severity of infections in confirmed and probable EVD cases in Sierra Leone. Proportional hazards regression models were used to investigate factors associated with the risk of death in EVD cases. In total, there were 17 318 EVD cases reported in Sierra Leone from 23 May 2014 to 31 January 2015. Of the probable and confirmed EVD cases with a reported final outcome, a total of 2536 deaths and 886 recoveries were reported. CFR and HFR estimates were 74·2% [95% credibility interval (CrI) 72·6-75·5] and 68·9% (95% CrI 66·2-71·6), respectively. Risks of death were higher in the youngest (0-4 years) and oldest (⩾60 years) age groups, and in the calendar month of October 2014. Sex and occupational status did not significantly affect the mortality of EVD. The CFR and HFR estimates of EVD were very high in Sierra Leone.

Placental transfusion strategies in extremely preterm infants: the next piece of the puzzle.

Evidence is growing on the potential value of enhancing placental-fetal transfusion at birth, with recent endorsement of the practice by the World Health Organization and American College of Gynecologists. However, these recommendations provide clinicians with little guidance on the optimal practice among infants born extremely premature (<28 weeks gestation) and those requiring immediate resuscitation. The goals of this review are to: 1) provide rationale for better outcomes among extremely preterm infants following delayed cord clamping or umbilical cord "milking" than with immediate cord clamping; 2) describe clinical situations that warrant immediate cord clamping following delivery and explore the controversy regarding optimal cord clamping practice among extremely premature infants, including those requiring immediate resuscitation; 3) discuss the quality of evidence in this subgroup of infants; 4) consider areas for future research, with a focus on characterizing if placental-fetal transfusion affects the magnitude or timing of variables associated with physiological transition. The review provided herein suggests that delayed cord clamping or umbilical cord milking can be applied safely to infants born prior to 28 weeks gestation, but the lack of evidence on the best practice among infants born severely depressed and requiring immediate resuscitation, who comprise a greater proportion of infant deliveries at the lowest gestational ages, is recognized. Future studies using well-defined physiologic outcome measures are needed to understand the role of placental transfusion in premature infants' adaptations to extrauterine life.

Relationships among vitamin D, disease activity, pain and disability in rheumatoid arthritis.

BACKGROUND AND OBJECTIVES: Vitamin D is a steroid hormone with important skeletal and non-skeletal biologic functions. Vitamin D deficiency is common and manifests with musculoskeletal symptoms. In rheumatoid arthritis (RA), vitamin D deficiency may be associated with increased disease activity and disability. We aimed to estimate the relationship between Vitamin D level and disease activity, pain, and disability in RA. METHODS: Data were drawn from 62 RA patients seen in an academic arthritis clinic. 25(OH)D levels were evaluated along with markers of RA disease activity, physician and patient global assessments, pain (VAS) and HAQ. DAS-28 was calculated. Vitamin D deficiency was defined as 25(OH)D levels<30ng/ml. RESULTS: Sixty-one percent of RA patients were classified as vitamin D deficient. In patients with active RA (DAS 28 score≥2.6), 25(OH)D was moderately and inversely associated with DAS 28 (-0.38), pain (-0.49) and HAQ (-0.54) (p<0.01). However, no significant associations were found between 25(OH)D and these variables in patients in remission (DAS 28<2.6). Vitamin D deficient patients with active RA had six times the odds (OR=6.0, 95% CI 1.2-31.2) of being moderately or severely disabled (HAQ≥1.25). CONCLUSIONS: Vitamin D deficiency was common in this RA group. In patients with moderate to high disease activity, vitamin D deficiency was associated with higher DAS scores, pain and disability. Clinicians in northern climates may wish to monitor vitamin D status in their RA patients.

Central nervous system vasculitis as a complication of refractory dermatomyositis.

We describe a 47-year-old woman with refractory dermatomyositis (DM) who developed progressive cognitive dysfunction. Magnetic resonance imaging showed a large cerebral infarction, and the diagnosis of central nervous system (CNS) vasculitis was confirmed by both angiogram and brain biopsy. Her DM and CNS vasculitis responded promptly to the institution of daily cyclophosphamide, and her previously refractory disease entered remission.

Colon stricture and volvulus in a patients with scleroderma.

Gastrointestinal involvement in scleroderma is almost universal. We describe a patient with a benign stricture and volvulus of transverse colon, a life threatening but treatable complication of scleroderma bowel disease. We review the literature on colon volvulus in scleroderma and discuss the importance of recognizing this rare complication.

Takayasu's arteritis involving proximal pulmonary arteries and mimicking thromboembolic disease.

The pulmonary manifestations of Takayasu's arteritis (TA) are frequently overshadowed by the systemic circulation involvement. We describe a patient who presented with life threatening complications of unrecognized proximal pulmonary arterial disease that mimicked thromboembolic disease. We review the literature on pulmonary involvement in TA, and discuss the use of imaging studies in this disease.

Heterogeneity in cytosolic calcium responses to hypoxia in carotid body cells.

Previous investigators have reported that intracellular pH responds to hypoxia with a heterogenous pattern in individual glomus cells of the carotid body. The aim of the present study was to examine whether hypoxia had similar effects on cytosolic calcium ([Ca2+]i) in glomus cells, and if so, whether a heterogenous response pattern is also seen in other cell types. Experiments were performed on glomus cells from adult rat carotid bodies, rat pheochromocytoma (PC12) and vascular smooth muscle (A7r5) cells. Changes in [Ca2+]i in individual cells were determined by fluorescence imaging using Fura-2. Glomus cells were identified by catecholamine fluorescence. [Ca2+]i in glomus cells increased in response to hypoxia (pO2 = 35 +/- 8 mmHg; 5 min), whereas hypoxia induced decreases in [Ca2+]i were not seen. Increases in [Ca2+]i were observed in 20% of the isolated cells and strings of cells, but clustered glomus cells never responded. The magnitude of the calcium change in responding cells was proportional to the hypoxic stimulus. Under a given hypoxic challenge, there were marked variations in the response pattern between glomus cells. The response pattern characteristic of any given cell was reproducible. At comparable levels of hypoxia, PC12 cells also responded with an increase in [Ca2+]i with a heterogenous response pattern similar to that seen in glomus cells. In contrast, increases in [Ca2+]i in A7r5 cells could be seen only with sustained hypoxia (approximately 20 min), and little heterogeneity in the response patterns was evident. These results demonstrate that: (a) hypoxia increases cytosolic calcium in glomus cells; (b) response patterns were heterogeneous in individual cells; and (c) the pattern of the hypoxia-induced changes in [Ca2+]i is cell specific. These results suggest that hypoxia-induced increases in [Ca2+]i are faster in secretory than in non-secretory cells.