Plasma sphingolipids in patients with sickle cell disease: Multiple-site vaso-occlusive crises could be associated with lower sphingolipid levels.

Although sickle cell disease (SCD) and its manifestations have been associated with various lipid alterations, there are a few studies exploring the impact of sphingolipids in SCD. In this study, we determined plasma ceramide (Cer) and sphingomyelin (CerPCho) species and investigated their association with the crisis in SCD. SCD patients (N = 27) suffering from vaso-occlusive crisis (VOC) or acute chest syndrome (ACS) were involved in this study. Blood samples were drawn at crisis and later at steady state periods. Clinical history, white blood cell count (WBC), C-reactive protein and lactate dehydrogenase (LDH) levels were recorded. 16:0, 18:0, 20:0, 22:0 Cer and 16:0, 18:0, 24:0 CerPCho were measured via LC-MS/MS. All measured Cer and CerPCho levels of SCD patients at crisis and steady-state were found to be similar. Inflammation-related parameters were significantly higher in patients with ACS compared to single-site VOC. Patients with multiple-site VOC were found to have significantly lower sphingolipid levels compared with those with single-site VOC, at crisis (16, 18, 24 CerPCho and 18, 22 Cer) and at steady-state (24:0 CerPCho and 18 Cer). Our results show that sphingolipid levels in SCD patients are similar during crisis and at steady state. However, lower sphingolipid levels appear to be associated with the development of multiple-site VOC. Since the differences were observed at both crisis and steady-state, sphingolipid level could be an underlying factor associated with crisis characteristics in patients with SCD.

Is Selective Head Cooling Combined with Whole-Body Cooling the Most Effective Hypothermia Method for Neonatal Hypoxic-Ischemic Encephalopathy?

This study aimed to compare combined hypothermia (CH) to the 2 classical therapeutic hypothermia (TH) methods selective head cooling (SHC) and whole-body cooling (WBC). This retrospective cohort study included neonates who underwent CH, SHC, and WBC between 2012 and 2020. Mean rectal temperature was maintained at 33.5 ± 0.5°C by cooling the head and the body in the CH group, at 34.5 ± 0.5°C by cooling the head in the SHC group, and at 33.5 ± 0.5°C by cooling the body in the WBC group. The groups were compared in terms of side effects, magnetic resonance imaging (MRI) scores, and status at discharge. The study included 60 neonates in the CH group, 112 in the WBC group, and 27 in the SHC group. There was no significant difference in side effects between the groups (p > 0.05). There was no significant difference in brain MRI scores between the groups (p > 0.05); however, gray matter, white matter, and total MRI scores in the CH group were lower than in the WBC group. Duration of hospitalization was shorter in the CH group than in the other two groups (p = 0.022). CH was not associated with more side effects than the two classical TH methods. In addition, some of these findings suggest that CH might result in better clinical outcome than the two classical TH methods.

Evaluation of the relationship between intravascular hemolysis and clinical manifestations in sickle cell disease: decreased hemopexin during vaso-occlusive crises and increased inflammation in acute chest syndrome.

The aims of this study were to determine the possible relationships between the levels of hemin, hemopexin, acid sphingomyelinase, nitrite/nitrate (NOx), and other parameters in patients with SCD and to assess whether they were associated with vaso-occlusive crises (VOCs) or acute chest syndrome (ACS). Patients with SCD (homozygous or sickle beta-thalassemia) who were confirmed to have VOC or ACS were included. Blood samples were obtained at admission, on the third day of hospitalization, and at steady state. Demographic characteristics, pain (visual analog scale), complication history, complete blood count, lactate dehydrogenase, and C-reactive protein levels were recorded. Hemin, hemopexin, acid sphingomyelinase, and NOx were measured via ELISA. A total of 31 patients (22 VOC, 9 ACS) were included. Mean age was 16.4 ± 4.7 years. Admission white blood cell count and C-reactive protein levels were significantly higher in the ACS group. Patients with ACS also demonstrated a significant decreasing trend of LDH and an increasing trend of NOx values from admission to steady state. Notably, hemopexin levels were significantly lower on the third day of hospitalization compared to steady-state levels. Despite limited patient count in the ACS group, these patients appear to have strikingly greater inflammatory activation at admission, and the progression of ACS may be associated with LDH and NOx levels. Lower hemopexin levels during hospitalization versus steady state appear to support a role for the administration of hemopexin therapy during crises.