Prevalence of Carnitine Deficiency and Decreased Carnitine Levels in Patients on Hemodialysis

2019 ◽  
Vol 47 (Suppl. 2) ◽  
pp. 38-44 ◽  
Author(s):  
Yoshinari Hatanaka ◽  
Terumi Higuchi ◽  
Yurie Akiya ◽  
Tomomi Horikami ◽  
Ritsukou Tei ◽  
...  
Keyword(s):  
At Risk ◽  
High Risk ◽  
Multivariate Regression ◽  
Reduction Rate ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Prevalence Rates ◽  
Carnitine Level ◽  
Serum Free ◽  
Free Serum

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.

scholarly journals Prevalence of Carnitine Deficiency and Decreased Carnitine Levels in Patients on Peritoneal Dialysis

Nutrients ◽  
2019 ◽  
Vol 11 (11) ◽  
pp. 2645 ◽  
Author(s):  
Satoshi Shimizu ◽  
Hiroyuki Takashima ◽  
Ritsukou Tei ◽  
Tetsuya Furukawa ◽  
Makiyo Okamura ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
High Risk ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Serum Concentrations ◽  
Clinical Factors ◽  
Carnitine Level ◽  
Serum Free ◽  
Factors Associated ◽  
Carnitine Concentration

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.

scholarly journals Prevalence of Carnitine Deficiency and Decreased Carnitine Levels in Patients on Peritoneal Dialysis

2019 ◽  
Author(s):  
Satoshi Shimizu ◽  
Hiroyuki Takashima ◽  
Ritsukou Tei ◽  
Tetsuya Furukawa ◽  
Makiyo Okamura ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
High Risk ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Serum Concentrations ◽  
Clinical Factors ◽  
Carnitine Level ◽  
Serum Free ◽  
Factors Associated ◽  
Carnitine Concentration

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 14.7% in the HD group (P = 0.45). High risk of carnitine deficiency was found in 79.4% of the PD group and 85.3% of the HD group (P = 0.52). Carnitine insufficiency was found in 82.3% of the PD group and 88.2% of HD group (P = 0.49). 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%, 79.4%, and 82.3%, respectively. These rates were comparable to those in patients on HD.

Carnitine Deficiency in Epilepsy: Risk Factors and Treatment

1995 ◽  
Vol 10 (2_suppl) ◽  
pp. 2S32-2S39 ◽  
Author(s):  
David L. Coulter
Keyword(s):  
Risk Factors ◽  
High Risk ◽  
Preliminary Data ◽  
Metabolic Diseases ◽  
Drug Effects ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Specific Drug ◽  
Asymptomatic Patients ◽  
The Relationship

Numerous studies have shown that plasma carnitine levels are significantly lower in patients taking valproate than in controls. Free carnitine deficiency is not uncommon in these patients and also occurs in newborns with seizures and in patients taking other anticonvulsant drugs. Carnitine deficiency in epilepsy results from a variety of etiologic factors including underlying metabolic diseases, nutritional inadequacy, and specific drug effects. The relationship between carnitine deficiency and valproate-induced hepatotoxicity is unclear. Carnitine treatment does not always prevent the emergence of serious hepatotoxicity, but it does alleviate valproate-induced hyperammonemia. These studies suggest that specific risk factors for carnitine deficiency can be identified. Preliminary data suggest that carnitine treatment may benefit high-risk, symptomatic patients and those with free carnitine deficiency. Carnitine treatment is not likely to benefit low-risk, asymptomatic patients and those with normal carnitine levels. (J Child Neurol 1995;10(Suppl):2S32-2S39).

Carnitine insufficiency in children with inborn errors of metabolism: prevalence and treatment efficacy

2015 ◽  
Vol 28 (11-12) ◽  
Author(s):  
Ilgar Mamedov ◽  
Irina Zolkina ◽  
Ekaterina Nikolaeva ◽  
Pavel Glagovsky ◽  
Vladimir Sukhorukov
Keyword(s):  
Fatty Acids ◽  
Plasma Level ◽  
Psychomotor Retardation ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Hereditary Diseases ◽  
Inborn Errors ◽  
Carnitine Level ◽  
Subsequent Investigation ◽  
Systemic Carnitine Deficiency

AbstractCarnitine is necessary for the transfer of long-chain fatty acids from the cytosol into mitochondria for subsequent β-oxidation. A carnitine deficiency results in impaired energy production from fatty acids.We reviewed the plasma level of total carnitine, free carnitine, and acylcarnitines in 1270 children with psychomotor retardation, low growth, and weight parameters. Tandem mass spectrometry (MS/MS) was applied. Low free carnitine level in plasma was used as a marker of carnitine deficiency.A total of 102 (8%) children had free carnitine level <19 μmol/L (norm 19–60 μmol/L). As a result of the subsequent investigation, hereditary diseases were diagnosed in 76 (¾) children and out of that 19 had very low free carnitine plasma level (<10 μmol/L). Fanconi syndrome, fat oxidation defects, primary systemic carnitine deficiency, mitochondrial encephalomyopathy, and Noonan syndrome were revealed in these 19 children.Efficacy of levocarnitine treatment in children with very low free carnitine level differs according to pathogenesis of diseases. The highest efficacy was observed in primary systemic carnitine deficiency.

Association of Carnitine Deficiency in Indian Continuous Ambulatory Peritoneal Dialysis Patients with Anemia, Erythropoietin Use, Residual Renal Function, and Diabetes Mellitus

2007 ◽  
Vol 27 (2_suppl) ◽  
pp. 235-238
Author(s):  
S. Ramalakshmi ◽  
Bjoe Baben ◽  
Ben S. Ashok ◽  
V. Jayanthi ◽  
Nancy Leslie ◽  
...  
Keyword(s):  
Renal Function ◽  
Peritoneal Dialysis ◽  
Residual Renal Function ◽  
Carnitine Deficiency ◽  
Free Carnitine ◽  
Dialysis Patients ◽  
Carnitine Level ◽  
Daily Urine ◽  
The Mean ◽  
Daily Urine Output

♦ In the present study, we aimed to determine levels of free carnitine in hemodialysis (HD) and peritoneal dialysis (PD) patients in India and to correlate carnitine deficiency with various clinical parameters. ♦ Patients on HD and PD at two tertiary care centers were selected for the study. Baseline data were obtained, and a free carnitine analysis was performed. Carnitine deficiency was defined as a free carnitine level of less than 40 μmol/L. ♦ The total number of study patients was 96 (77 on HD, 19 on PD). In the PD group, the mean age was 56 years, with 26.3% of the patients being vegan, 47.4% having diabetes, and 57.9% having a daily urine output of <500 mL. The mean carnitine level in that group was 38.9 μmol/L, and 68.4% of the patients had a carnitine deficiency. A Pearson correlation test failed to show any association of carnitine level with parameters such as anemia, use of erythropoietin, non-vegetarian diet, diabetes, and hypertension. In the HD group, the mean age was 45 years, with 22% of the patients being vegan, 23% having diabetes, and 45.5% having a daily urine output of <500 mL. The mean carnitine level in the group was 38.2 μmol/L, and 64.3% of the patients had a carnitine deficiency. Residual renal function and duration of dialysis were different in HD patients with and without carnitine deficiency. Carnitine levels in the HD group correlated positively and statistically significantly with the presence of diabetes and hypertension. ♦ Conclusion This study is the first demonstration that Indian dialysis patients have carnitine deficiency.

scholarly journals An Examination of Serum Acylcarnitine and Amino Acid Profiles at Different Time Point of Ketogenic Diet Therapy and Their Association of Ketogenic Diet Effectiveness

Nutrients ◽  
2020 ◽  
Vol 13 (1) ◽  
pp. 21
Author(s):  
Pi-Lien Hung ◽  
Ju-Li Lin ◽  
Chien Chen ◽  
Kai-Yin Hung ◽  
Tzu-Yun Hsieh ◽  
...  
Keyword(s):  
Ketogenic Diet ◽  
Retention Rate ◽  
Reduction Rate ◽  
Diet Therapy ◽  
Free Carnitine ◽  
Carnitine Level ◽  
Seizure Reduction ◽  
Amino Acid Profiles ◽  
Drug Resistant Epilepsy ◽  
Glycine Level

Background: This study aimed to identify metabolic parameters at different time points of ketogenic diet therapy (KDT) and investigate their association with response to KDT in pediatric drug-resistant epilepsy (DRE). Methods: Prospectively, twenty-nine patients (0.67~20 years old) with DRE received classic ketogenic diet with non-fasting, gradual KD initiation protocol (GRAD-KD) for 1 year were enrolled. A total of 22 patients remaining in study received blood examinations at baseline, 3rd, 6th, 9th, and 12th months of KDT. β-hydroxybutyrate, free carnitine, acylcarnitines, and amino acids were compared between responders (seizure reduction rate ≥ 50%) and non-responders (seizure reduction rate < 50%) to identify the effectiveness of KDT. Results: The 12-month retention rate was 76%. The responders after 12 months of KDT were 59% (13/22). The free carnitine level decreased significantly at 9th months (p < 0.001) but increased toward baseline without symptoms. Propionyl carnitine (C3), Isovaleryl carnitine (C5), 3-Hydroxyisovalerylcarnitine (C5:OH) and methylmalonyl carnitine (C4-DC) decreased but 3-hydroxybutyrylcarnitine (C4:OH) increased significantly at 12th months of KDT. The glycine level was persistently higher than baseline after KDT. KDT responders had lower baseline C3 and long-chain acylcarnitines, C14 and C18, as well as lower C5, C18, and leucine/isoleucine. Conclusions: KDT should be avoided in patients with non-ketotic hyperglycemia. Routine carnitine supplementation is not recommended because hypocarnitinemia was transient and asymptomatic during KDT. Better mitochondrial βoxidation function associates with greater KDT response.

Serum Free Carnitine Levels in Children with Kawasaki Disease

2021 ◽  
Author(s):  
Taichiro Muto ◽  
Nami Nakamura ◽  
Yu Masuda ◽  
Shingo Numoto ◽  
Shunsuke Kodama ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Free Carnitine ◽  
Serum Free

scholarly journals FRI0155 Α MULTICENTER “AT-RISK” COHORT FOR THE DISCOVERY OF ENVIRONMENTAL, CLINICAL AND MOLECULAR PREDICTORS FOR THE TRANSITION INTO SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 661.1-661
Author(s):  
C. Adamichou ◽  
D. Nikolopoulos ◽  
M. Nikoloudaki ◽  
Z. Rahme ◽  
M. Fredi ◽  
...  
Keyword(s):  
At Risk ◽  
High Risk ◽  
Mediterranean Diet ◽  
Lupus Erythematosus ◽  
Autoimmune Disorder ◽  
Classification Criteria ◽  
Renal Disorder ◽  
Clinical Criteria ◽  
Hematologic Disorder ◽  
Physical Activity Adherence

Background:SLE onset is preceded by a preclinical phase evidenced by the presence of anti-nuclear and other autoantibodies (autoAbs), which however, have low predictive value for development of clinical SLE.Objectives:To define the subgroup of autoAbs-positive individuals who are at high risk for progression into SLE by integrating environmental, clinical/serological, genetic and transcriptome data.Methods:A multicenter, across five European countries, inception cohort of autoAbs-positive individuals or first-degree relatives (FDRs) of SLE patients who are monitored prospectively over five years for possible transition to SLE according to the classification criteria. Structured data collection on demographics, family and medical history, clinical (criteria and selected non-criteria manifestations) and serological parameters, use of medications, hydroxyvitamin D levels and lifestyle (tobacco, alcohol use, physical activity, adherence to Mediterranean diet). Blood samples are stored for RNA-sequencing and genotyping.Results:A total 254 at-risk individuals (93% women, 99% Caucasians, aged [mean ± standard deviation] 36 ± 12 years) have been included and enrolment/monitoring is still ongoing. Forty individuals (16%) have FDR with SLE and 88 individuals (35%) have FDR with another autoimmune disorder. The frequency of active and past use of tobacco was 28% and 20%, respectively. Sedentary lifestyle (moving only for necessary chores or outdoor activity 1-2 times/week) was reported by 54% and adherence to the Mediterranean diet was low (3.4 ± 2.3, maximum score: 9). At enrolment, individuals had 1.9 ± 1.1 ACR-1997 classification criteria, with anti-nuclear antibodies (ANA) being the most frequent (88%), followed by synovitis (39%), photosensitivity (33%) and immunologic disorder (30%) (Table 1). During follow-up of 15.2 ± 7.2 months, a total 15 individuals (5.9%) have progressed into classified SLE, including cases with severe hematological and neurological disease.Table 1.Baseline characteristics of the at-risk for SLE cohortN (%) or mean ± SDACR 1997 classification criteria1.9 ± 1.1 Malar rash68 (27%) Discoid rash29 (11%) Photosensitivity83 (33%) Mucosal ulcers49 (19%) Synovitis100 (39%) Serositis30 (12%) Renal disorder28 (11%) Neurologic disorder31 (12%) Hematologic disorder58 (23%) Immunologic disorder77 (30%) ANA222 (88%)SLICC 2012 classification criteria Clinical criteria1.0 ± 0.9 Immunological criteria1.3 ± 0.9Conclusion:Among individuals with positive autoAbs or FDRs with SLE, the short-term risk for transition into clinical SLE is low. Following the study completion, clinical and lifestyle data will be combined with blood transcriptome to define a high-risk subgroup of individuals for progression into SLE.Acknowledgments:The study is supported by the Foundation for Research in Rheumatology (FOREUM; preclin016)Disclosure of Interests:Christina Adamichou: None declared, Dionysis Nikolopoulos: None declared, Myrto Nikoloudaki: None declared, Zahra Rahme: None declared, Micaela Fredi: None declared, Antigoni Pieta: None declared, ARGYRO REPA: None declared, Alice Parma: None declared, Eleni Kalogiannaki: None declared, Nestor Avgustidis: None declared, Nikolaos Kougkas: None declared, Aggelos Banos: None declared, Anastasios Eskitzis: None declared, Alessandra Bortoluzzi: None declared, Søren Jacobsen: None declared, Prodromos Sidiropoulos: None declared, Emmanouil Dermitzakis: None declared, Marta Mosca: None declared, Luís Inês: None declared, Laura Andreoli: None declared, Angela Tincani: None declared, Antonis Fanouriakis Paid instructor for: Paid instructor for Enorasis, Amgen, Speakers bureau: Paid speaker for Roche, Genesis Pharma, Mylan, George Bertsias Grant/research support from: GSK, Consultant of: Novartis

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