Sepsis is one of the major causes of death in the hospital worldwide. The pathology of sepsis is tightly associated with dysregulation of innate immune responses. The contribution of macrophages, neutrophils, and dendritic cells to sepsis is well documented, whereas the role of natural killer (NK) cells, which are critical innate lymphoid lineage cells, remains unclear. In some studies, the activation of NK cells has been reported as a risk factor leading to severe organ damage or death. In sharp contrast, some other studies revealed that triggering NK cell activity contributes to alleviating sepsis. In all, although there are several reports on NK cells in sepsis, whether they exert detrimental or protective effects remains unclear. Here, we will review the available experimental and clinical studies about the opposing roles of NK cells in sepsis, and we will discuss the prospects for NK cell-based immunotherapeutic strategies for sepsis.
Background
Spinal Muscular Atrophy (SMA) is a progressive neuromuscular disease affecting multiple organs and systems, including nutritional and metabolic status. There is still a lack of relevant large sample size research. We aimed to investigate the nutritional status, serum lipid profile, and body composition characteristics in children with SMA.
Methods
A hospital-based, case-control study was conducted on 91 children with SMA (17.6% of type I, 48.4% of type II, 34.1% of type III) and 91 age- and gender-matched healthy children in a single hospitalized center in China. Anthropometric information, serum lipids parameters and body composition (in subgroup of patients older than 3 years) were collected. According to the anthropometric Z scores, nutritional status was categorized into normal and malnutrition (including under- and over-nutrition) groups.
Results
The prevalence of malnutrition and dyslipidemia in children with SMA was higher than that of controls (49.45% vs. 23.08% and 56.04% vs. 34.07%, respectively and p<0.01). Particularly, the prevalence of lower-than-normal levels of high density lipoprotein (HDL) and apolipoprotein A1 (Apo A1) were significantly higher than that in healthy controls in children with SMA (26.37% vs. 7.69% and 41.76% vs. 6.59%, respectively and p<0.01). In terms of the body composition characteristics in children with SMA II & III, on average, the total body fat mass percentage (FM%) was 43.38% (36.99%, 48.20%), the fat mass index (FMI) was 7.30 (5.07, 8.72), the lean body mass index (LBMI) was 7.70 (6.96, 9.02) and the bone mineral density-z score (BMD-z) was -1.40 (-2.80, -0.60), generally different from the reference profiles in published literature.
Conclusion
Children with SMA in China have obvious nutritional imbalances, abnormal levels of serum lipid and unbalanced body composition. Further research is warranted to explore the potential mechanisms and health management strategies on nutritional and metabolic disorders of SMA.
BackgroundPatients with spinal muscular atrophy (SMA) may suffer from multisystem injury, including an impaired cardiovascular system. However, M-mode echocardiography, the current dominant echocardiographic modality, is limited in the detection of myocardial injury. We considered the use of left ventricular strain imaging in detecting myocardial injury and explored the serum lipid profile related to cardiovascular disease in later-onset SMA children.MethodsA case-control study involving 80 patients with later-onset SMA and 80 age-, gender-, and body surface area-matched control children was conducted in a single tertiary pediatric hospital in China. Data on the left ventricular strain measured using two-dimensional speckle tracking echocardiography, left ventricular function parameters assessed by M-mode echocardiography, and serum lipid profile of these two groups were retrospectively collected for differential analysis.ResultsThe mean age of the 80 SMA patients were (6.87 ± 2.87) years, of which 46 were type 2 and 34 were type 3 patients. The global longitudinal strain (GLS) of the SMA group (−18.7 ± 2.9%, p < 0.001) was lower than that of the control group; the time to peak longitudinal strain (TTPLS) of the SMA group (22.9 ± 13.6 ms, p < 0.001) was higher than that of the control group, while left ventricular ejection fraction (LVEF) and left ventricular fractional shortening (LVFS), measured by the Teichholz method of M-mode echocardiography, showed no significant differences between the two groups. In addition, independent indicators for cardiovascular risk, including total cholesterol (TC)/HDL, low-density lipoprotein (LDL)/HDL, and Apo B/Apo A1 levels, were higher in SMA children than in the control group.ConclusionCompared with healthy controls, later-onset SMA children presented with reduced GLS and prolonged TTPLS while the LVEF and LVFS values were within normal range. In particular, whether a reduced GLS or prolonged TTPLS in later-onset SMA compared to the control group can predict the risk of future cardiomyopathy remains to be investigated.
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