Tricuspid regurgitation velocity and other biomarkers of mortality in children,adolescents and young adults with sickle cell disease in the United States: The PUSH study

In the US, mortality in sickle cell disease (SCD) increases after age 18-20 years. Biomarkers of mortality risk can identify patients who need intensive follow-up and early or novel interventions. We prospectively enrolled 510 SCD patients aged 3-20 years into an observational study in 2006-2010 and followed 497 patients for a median of 88 months (range 1-105). We hypothesized that elevated pulmonary artery systolic pressure as reflected in tricuspid regurgitation velocity (TRV) would be associated with mortality. Estimated survival to 18 years was 99% and to 25 years, 94%. Causes of death were known in seven of 10 patients: stroke in four (hemorrhagic two, infarctive one, unspecified one), multiorgan failure one, parvovirus B19 infection one, sudden death one. Baseline TRV ≥2.7 m/second (>2 SD above the mean in age-matched and gender-matched non-SCD controls) was observed in 20.0% of patients who died vs 4.6% of those who survived (P = .012 by the log rank test for equality of survival). The baseline variable most strongly associated with an elevated TRV was a high hemolytic rate. Additional biomarkers associated with mortality were ferritin ≥2000 μg/L (observed in 60% of patients who died vs 7.8% of survivors, P < .001), forced expiratory volume in 1 minute to forced vital capacity ratio (FEV1/FVC) <0.80 (71.4% of patients who died vs 18.8% of survivors, P < .001), and neutrophil count ≥10x10⁹/L (30.0% of patients who died vs 7.9% of survivors, P = .018). In SCD children, adolescents and young adults, steady-state elevations of TRV, ferritin and neutrophils and a low FEV1/FVC ratio may be biomarkers associated with increased risk of death.     

An unusual case of cardiac dysfunction after left ventricular reconstruction

This report describes an unusual cause of low cardiac output after coronary artery bypass grafting and left ventricular remodeling. It details left ventricular remodeling techniques and discusses the most recent advances and outcomes. As well, significant attention is paid to the issues surrounding failure to separate from cardiopulmonary bypass.     

Antenatal determinants of oro-facial clefts in Southern Nigeria

Objectives

Cleft lip with or without cleft palate, is the most common serious congenital anomaly that affects the orofacial regions. The management and care of the cleft patient constitutes a substantial proportion of the workload of the Nigerian maxillofacial surgeon and allied specialties. Yet, there are no specific programmes targeted at this group. We believe that the findings of this study is capable of identifying useful interventions for designing programs that will lead to a reduction in the burden of orofacial cleft in Nigeria.

Methods

It was a transverse cross-sectional study that was undertaken at the Maxillofacial Units of the University of Benin Teaching Hospital and the Central Hospital, Benin City respectively. The prevalence and antenatal determinants of cleft lip and palate were determined.

Results

Cleft lip and palate were often encountered in clinical practice in Benin City with a prevalence of 1.35%. The results showed that orofacial clefts were commoner in females and that the combined unilateral cleft lip and palate was the commonest entity encountered amongst the cases. The following risk factors were associated with the risk of development of cleft lip and palate: Paternal age >40 years, maternal age >35 years, genetic/family history, low socio-economic status, alcohol consumption and indulgence in the intake of herbal medications in pregnancy.

Conclusion

Public health education programmes and advocacy activities geared towards raising awareness of the identified risk factors for the development of cleft lip and or cleft palate would go a long way to obviate the occurrence and reduce the burden.Key Words: Prevalence, Antenatal determinants, orofacial clefts, Southern Nigeria     

Ventricular hypertrophy increases NT-proBNP in subjects with and without hypertension

BACKGROUND: It has been published that hypertension (HT) must be taken into account when using NT-proBNP, but left ventricular (LV) hypertrophy without HT could be a cause of NT-proBNP elevation. In a population study we compared NT-proBNP in subjects with hypertrophy, with and without diagnosis of HT. METHODS: We studied 215 subjects from a random sample of 432 people who had declared to suffer from dyspnea. These 432 subjects were referred to their hospital where blood samples were taken, an echo-Doppler study was performed and a specific questionnaire was completed. We got a positive answer from 215, and 52 (24%) have LV hypertrophy. RESULTS: When we compared NT-proBNP in non-hypertrophic population, 148 +/- 286 pg/ml, with NT-proBNP in LV hypertrophic population, 202 +/- 209 pg/ml, we found P < 0.001. In the hypertrophic group, when we compared NT-proBNP (199 +/- 201 pg/ml) in normotensive subjects (LV mass index 170 +/- 70 g/m2, Vp 50 +/- 18 cm/s, LVEF 62 +/- 8) with NT-proBNP (205 +/- 220 pg/ml) in subjects with diagnosis of HT (LV mass index 169 +/- 37 g/m2, Vp 55 +/- 20 cm/s, LVEF 64 +/- 10), we found NS. CONCLUSIONS: This population study shows that NT-proBNP is elevated in patients with LV hypertrophy with or without HT. In LV hypertrophy the presence of HT does not influence the peptide levels significantly.     

Altered renal function in acute sodium nitrite intoxication experiment

In experiments on non-inbred albino rats, intoxication was reproduced with 1% aqueous sodium nitrite solution, which was rated by the degree of hemic hypoxia (HH). The findings suggest that changes in renal functions (osmo-regulating, sodium-regulating) occur due to the development of mild HH, by increasing as hypoxia deteriorates. Decreased glomerular filtration and proteinuria, which are concomitantly detected, point to their mixed glomerulotubular pattern of renal lesion in nitrite intoxication.