Serum Free Carnitine Levels in Children with Kawasaki Disease

2021 ◽  
Author(s):  
Taichiro Muto ◽  
Nami Nakamura ◽  
Yu Masuda ◽  
Shingo Numoto ◽  
Shunsuke Kodama ◽  
...  
2020 ◽  
Author(s):  
Taichiro Muto ◽  
Nami Nakamura ◽  
Yu Masuda ◽  
Shingo Numoto ◽  
Shunsuke Kodama ◽  
...  

Abstract Background: Carnitine plays an essential role in transfer of long-chain fatty acids to mitochondria for subsequent β-oxidation. No studies to date have characterized carnitine in children with KD. The objective of this study is to investigate the characteristics of serum free carnitine (FC) in hospitalized pediatric patients with Kawasaki disease (KD). Methods: In total, 45 patients with KD measured the levels of serum FC from October 2018 to December 2019 were analyzed retrospectively. We analyzed the clinical and laboratory parameters just before the Intravenous immunoglobulin (IVIG) including serum levels of serum FC with respect to the IVIG response. We also analyzed the relationship between serum FC and liver deviation enzymes or the duration of fever at diagnosis. Results: The median age was 33 months. IVIG was effective in 33 children (responders) and was ineffective in 12 (non-responders). The serum FC levels were higher in non-responders than in responders [(35.3 mmol/L (range, 26.8-118.4 mmol/L) vs. 31.4 mmol/L (range, 20.9-81.2 mmol/L), p value = 0.0496]. The FC levels before intravenous immunoglobulin (IVIG) in four-fifths of responders were below the normal range. The levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin, and FC were higher in non-responders than in responders. There were no different in patient characteristics and laboratory data according to fever duration at diagnosis. There was a correlation between FC and AST (R2=0.364, P=0.0015) and between FC and ALT (R2=0.423, P<0.001) levels. Conclusion: FC levels were upregulated in patients with KD who were refractory to IVIG. Additionally, FC levels in children with KD correlated with AST and ALT levels. The pathogenesis resulting in the elevation of FC levels remains elusive. Further studies are necessary to understand more precisely carnitine properties in patients with KD.


2020 ◽  
Author(s):  
Taichiro Muto ◽  
Nami Nakamura ◽  
Yu Masuda ◽  
Shingo Numoto ◽  
Shunsuke Kodama ◽  
...  

Abstract Background Carnitine plays an essential role in transfer of long-chain fatty acids to mitochondria for subsequent β-oxidation. No studies to date have characterized carnitine in children with KD. The objective of this study is to investigate the characteristics of serum free carnitine (FC) in hospitalized pediatric patients with Kawasaki disease (KD). Methods In total, 45 patients with KD measured the levels of serum FC from October 2018 to December 2019 were analyzed retrospectively. We analyzed the clinical and laboratory parameters just before the Intravenous immunoglobulin (IVIG) including serum levels of serum FC with respect to the IVIG response. Results The median age was 33 months. IVIG was effective in 33 children (responders) and was ineffective in 12 (non-responders). The serum FC levels were higher in non-responders than in responders [(35.3 µmol/L (range, 26.8-118.4 µmol/L) vs. 31.4 µmol/L (range, 20.9–81.2 µmol/L), p value = 0.0496]. The FC levels before intravenous immunoglobulin (IVIG) in four-fifths of responders were below the normal range.The levels of aspartate aminotransferase (AST), alanine aminotransferase (ALT), total bilirubin, and FC were higher in non-responders than in responders. There was a correlation between FC and AST (R2 = 0.364, P = 0.0015) and between FC and ALT (R2 = 0.423, P < 0.001) levels. Conclusion FC levels were upregulated in patients with KD who were refractory to IVIG. Additionally, FC levels in children with KD correlated with AST and ALT levels. The pathogenesis resulting in the elevation of FC levels remains elusive. Further studies are necessary to understand more precisely carnitine properties in patients with KD.


1991 ◽  
Vol 11 (3) ◽  
pp. 196-197
Author(s):  
F.N. Bruscato ◽  
D.R. Avery ◽  
G. Roberts ◽  
S. Lane ◽  
W.S. Flournoy

2020 ◽  
Vol 41 (07) ◽  
pp. 443-449
Author(s):  
Tomoka Matsuda ◽  
Tamaki Furuhata ◽  
Hazuki Ogata ◽  
Kayoko Kamemoto ◽  
Mizuki Yamada ◽  
...  

AbstractThis study aimed to investigate the effect of the menstrual cycle on serum carnitine and the endurance performance of healthy women. Fifteen eumenorrheic women underwent cycle ergometer exercise at 60% maximal oxygen uptake (V̇ O2max) for 45 min, followed by exercise at an intensity that was increased to 80% V̇ O 2max until exhaustion, during two menstrual cycle phases, including the early follicular phase (FP) and the midluteal phase (LP). The blood levels of estradiol, progesterone, total carnitine, free carnitine, and acylcarnitine were assessed. Compared with the FP, the LP had significantly lower serum total carnitine (p<0.05) and free carnitine (p<0.01). Moreover, the group with decreased endurance performance in the LP than in the FP showed a significantly higher change in serum free carnitine compared with the group that showed improved endurance performance in the LP than in the FP (p<0.05). The results of this study suggested that the changes in serum free carnitine during the menstrual cycle might influence endurance performance.


2019 ◽  
Vol 47 (Suppl. 2) ◽  
pp. 38-44 ◽  
Author(s):  
Yoshinari Hatanaka ◽  
Terumi Higuchi ◽  
Yurie Akiya ◽  
Tomomi Horikami ◽  
Ritsukou Tei ◽  
...  

Background: Patients on hemodialysis (HD) are known to be at risk of carnitine deficiency. The aims of this study were to investigate the prevalence of carnitine deficiency in patients on dialysis and to compare the likelihood of a reduction in the serum carnitine level on HD with that on hemodiafiltration (HDF). Methods: The prevalence of carnitine deficiency, defined as a serum free carnitine level < 20 μmol/L, and that of carnitine insufficiency, defined as an acyl/free carnitine ratio > 0.4, was investigated in 150 patients on dialysis. The reduction rate of serum carnitine was then compared between HD and HDF. Results: The prevalence of carnitine deficiency and that of carnitine insufficiency was 25.3 and 86.7%, respectively. Patients at high risk of carnitine deficiency accounted for 64.7%. Multivariate regression identified an association of duration of dialysis with the free serum carnitine level. The reduction rates of serum free carnitine in HD and HDF were 64 ± 4 and 75 ± 7%, respectively (p < 0.0001). Conclusion: The prevalence rates of carnitine deficiency and carnitine insufficiency were high in patients on dialysis. The serum carnitine reduction rate was greater with HDF than with HD.


1999 ◽  
Vol 97 (4) ◽  
pp. 493-501 ◽  
Author(s):  
Tomoki NAKAMURA ◽  
Hiroki SUGIHARA ◽  
Noriyuki KINOSHITA ◽  
Kazuki ITO ◽  
Yoshihiko ADACHI ◽  
...  

We evaluated the clinical significance of serum carnitine concentrations in determining the severity of impaired myocardial fatty acid metabolism in idiopathic hypertrophic cardiomyopathy (HCM). We studied 56 asymptomatic or mildly symptomatic patients with HCM. Serum levels of free carnitine and acylcarnitine were measured by the enzymic cycling method. Myocardial scintigraphy with 123I-labelled 15-(p-iodophenyl)-3-R,S-methylpentadecanoic acid (BMIPP) was performed, and the images were analysed quantitatively and semi-quantitatively. Serum free carnitine levels were significantly higher in HCM patients than in normal subjects (52.5±9.5 and 42.3±5.5 nmol/ml respectively; P< 0.0001). On the other hand, serum acylcarnitine levels and acyl/free carnitine ratios were lower in HCM patients than in normal subjects (10.2±4.0 nmol/ml and 0.19±0.08, compared with 13.2±3.9 nmol/ml and 0.32±0.11 respectively; P< 0.0001). Clinical characteristics were not significantly different between the patients showing high and normal free carnitine levels, although female patients with high free carnitine levels were few (P = 0.02). Both quantitative and semi-quantitative analyses revealed that the severity of decreased myocardial BMIPP uptake was significantly correlated with serum free carnitine levels (quantitative analysis: r = -0.422, P< 0.0012; semi-quantitative analysis: r = 0.633, P< 0.0001). In the presence of reduced carnitine uptake into the myocardium in HCM, there may also be reduced transport of acylcarnitines out of the myocardium into the plasma. Although inborn errors of fatty acid metabolism and carnitine deficiencies are reported to provoke secondary HCM and are associated with low serum carnitine concentrations, this study has revealed that the levels of carnitine are, in contrast, increased in idiopathic HCM. Moreover, serum carnitine concentrations are a sensitive indicator of the severity of impaired myocardial fatty acid metabolism even in asymptomatic patients with HCM.


2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Yoshimasa Tokuchi ◽  
Goki Suda ◽  
Megumi Kimura ◽  
Osamu Maehara ◽  
Takashi Kitagataya ◽  
...  

AbstractWe aimed to evaluate factors associated with changes in skeletal muscle mass in hepatitis C virus (HCV)-infected patients after treatment with direct-acting antivirals (DAAs). Consecutive HCV-infected patients after treatment with DAA were recruited into the study. Patients who achieved sustained virological response (SVR); and had complete clinical information, preserved serum samples at baseline and SVR48, and skeletal muscle mass evaluations based on the psoas muscle mass index (PMI) on computed tomography at baseline and ≥ 12 months were included. Altogether, 70.7% of patients (41/58) showed increased PMI after DAA therapy, and mean relative PMI was significantly higher after DAA therapy than at baseline. There were no significant associations between baseline clinical factors routinely examined in clinical practice and increased PMI. Among factors reported to be associated with skeletal muscle loss in patients with chronic liver disease, serum zinc levels and total and free carnitine levels increased significantly after DAA therapy and only changes in serum free carnitine levels were significantly associated with an increased PMI (r = 0305, P = 0.020). In conclusion, increased skeletal muscle mass after successful HCV eradication by DAAs was significantly associated with increased serum-free carnitine levels. l-carnitine supplementation may be beneficial in patients with low skeletal muscle mass after DAA.


2019 ◽  
Vol 446 ◽  
pp. 116208
Author(s):  
Mo Wang ◽  
Ruiyue Yang ◽  
Hongna Mu ◽  
Jie Zeng ◽  
Tianjiao Zhang ◽  
...  

Nutrients ◽  
2019 ◽  
Vol 11 (11) ◽  
pp. 2645 ◽  
Author(s):  
Satoshi Shimizu ◽  
Hiroyuki Takashima ◽  
Ritsukou Tei ◽  
Tetsuya Furukawa ◽  
Makiyo Okamura ◽  
...  

Background: Carnitine deficiency is common in patients on dialysis. Serum free carnitine concentration is significantly lower in patients on hemodialysis (HD) than in healthy individuals. However, there are few reports on serum free carnitine concentration in patients on peritoneal dialysis (PD). Methods: We examined serum concentrations of total, free, and acylcarnitine and the acylcarnitine/free carnitine ratio in 34 PD and 34 age-, sex-, and dialysis duration-matched HD patients. We investigated the prevalence of carnitine deficiency and clinical factors associated with carnitine deficiency in the PD group. Results: Prevalence of carnitine deficiency was 8.8% in the PD group and 17.7% in the HD group (p = 0.283). High risk of carnitine deficiency was found in 73.5% of the PD group and 76.4% of the HD group (p = 0.604). Carnitine insufficiency was found in 82.3% of the PD group and 88.2% of HD group (p = 0.733). Multivariate analysis revealed that duration of dialysis and age were independent predictors of serum free carnitine level in the PD group. Conclusions: The prevalence of carnitine deficiency, high risk of carnitine deficiency, and carnitine insufficiency in PD patients was 8.8%, 73.5%, and 82.3%, respectively. These rates were comparable to those in patients on HD.


Sign in / Sign up

Export Citation Format

Share Document