ABSTRACT
BACKGROUND/AIM: Cardiovascular pathologies are ubiquitous in sickle cell disease (SCD). A targeted literature review was conducted to compare the overall epidemiology of selected vasculopathies seen in SCD (SCDVs) compared to the general population. Since many SCDV may originate in childhood, the study also focused on the retrospective investigation of SCDVs in a pediatric cohort at the Harbor-UCLA Medical Center. PATIENTS AND METHODS: SCDVs were studied along patient age, ß-globin genotypes, and fetal hemoglobin (HbF). Urine microalbumin/creatinine ratios (UM/Cr), trans-cranial doppler (TCD) and tricuspid regurgitant jet velocities (TRJV) were analyzed as well. Retinographies and overt vasculopathies were presented descriptively. RESULTS: Among 20 females and 20 males [average 8.3 years (2.3-19 years)], 70% had HbSS/Sß0, 22.5% HbSC and 7.5%-HbSß+. The mean(±SD) HbF% was 17.4±12.7% (30% higher in <10 vs. ≥10 y/o, and 3 times higher in SS/Sß0). Twenty-six patients received hydroxyurea and 13/26, L-glutamine. Thirty-six patients had TCDs within 1.4±0.9 years and all laboratory values were obtained within the last 12 months. TCDs showed low-normal velocities, but 2 were higher for HbSS/Sß0 vs. HbSC/Sß+ (MCA-96 vs. 86 cm/s, p=0.03; and PCA-50 vs. 41, p<0.001). Nineteen of 28 patients with echocardiograms had measurable TRJV (2.46±0.19 m/s); 9 had TRJV ≥2.5-2.8 m/s, but BNP ≤80 pg/ml. SS/Sß0 was associated with higher UM/Cr. There were 2 cases with silent infarcts, 1-Moyamoya, 2-persistent macroalbuminuria, and 1-hematuria/renal papillary necrosis. Most ≥9 y/o patients had retinographies without SCD-related changes. There was no correlation among TCD (MCA), TRJV, and UM/Cr (n=17); thus, in this subpopulation, pathologies of cerebral, cardiopulmonary, and renal vasculatures evolved independently. Patients with higher TRJV and/or overt vasculopathy (n=14) were older than ones without (12.5±4.7 vs. 6.1±3.1 y/o, p<0.001), and had lower HbF (11.4±7.6 vs. 20.6±13.8%, p=0.026). CONCLUSION: While overt SCDVs are less frequent in children, age-dependent trends/surrogate markers suggest their early origination in youth, justifying intense screening to prevent their progression with disease-modifying measures.
Subject(s)
Anemia, Sickle Cell , Humans , Anemia, Sickle Cell/complications , Anemia, Sickle Cell/epidemiology , Female , Male , Child , Adolescent , Child, Preschool , Young Adult , Vascular Diseases/epidemiology , Vascular Diseases/etiology , Vascular Diseases/diagnosis , Retrospective StudiesABSTRACT
The purpose of the study is to examine (1) nationally representative incidence rates of Emergency Department (ED) visits due to sudden cardiac arrest (SCA) in pediatric and young adult populations, (2) basic characteristics of the ED visits with SCA, and (3) patient and hospital factors associated with survival after SCA. We used the Nationwide Emergency Department Sample from 2006 to 2013. ICD-9-CM diagnostic codes identified ED visits due to SCA for patients ≤ 30 years old. Outcomes included yearly incidence of ED visits for SCA, and survival to hospital discharge. Predictors of interest were age groups, sex, and SCA case volume. A logistic regression model adjusted by patient- and hospital-level variables was used. Stratified analyses of age by (< 12 and ≥ 12 years old) were performed to explore the effect of pubertal development on SCA. With 71,881 ED visits due to SCA, the total incidence rate was 6.9 per 100,000 population, with a mortality rate of 89.6% and male/female ratio of 1.7. With the adjusted regression models, there were no differences in survival rate by sex; however, when stratified at 12 years old, males were less likely to survive than females above 12 years old (odds ratio [OR] 0.71, P < 0.01), but not under 12 years old. No statistically significant differences in survival rates between low- and high-SCA volume EDs were detected (OR 1.03, P = 0.77). Data showed no benefit of regionalized care for post-SCA in ≤ 30-year-old populations. With further examination of the differences between sexes, new management strategies for SCA cases can be developed.
