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Adult acquired carnitine deficiency

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https://www.readbyqxmd.com/read/22258985/interventions-for-fatigue-and-weight-loss-in-adults-with-advanced-progressive-illness
#1
REVIEW
Cathy Payne, Philip J Wiffen, Suzanne Martin
BACKGROUND: Fatigue and unintentional weight loss are two of the commonest symptoms experienced by people with advanced progressive illness. Appropriate interventions may bring considerable improvements in function and quality of life to seriously ill people and their families, reducing physical, psychological and spiritual distress. OBJECTIVES: To conduct an overview of the evidence available on the efficacy of interventions used in the management of fatigue and/or unintentional weight loss in adults with advanced progressive illness by reviewing the evidence contained within Cochrane reviews...
January 18, 2012: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/22258360/acquired-encephalopathy-associated-with-carnitine-deficiency-after-cefditoren-pivoxil-administration
#2
Heyun Kim, Kon Chu, Keun-Hwa Jung, Soon-Tae Lee, Jeong-Min Kim, Sang Kun Lee
We describe a 47-year-old woman who presented with palinopsia and subacute altered mental change after cefditoren pivoxil administration. The patient showed characteristic clinical manifestations of hypocarnitinemia, which affected her state of consciousness and she had radiologic findings that revealed metabolic encephalopathy with cytotoxic edema in the right occipital area and intracranial hemorrhages in right occipital and left frontal areas. Follow-up imaging after oral carnitine supplementation demonstrated complete resolution of the bilateral frontal subcortical T2 high-intensity lesions...
December 2012: Neurological Sciences
https://www.readbyqxmd.com/read/18045196/aplidine-a-paradigm-of-how-to-handle-the-activity-and-toxicity-of-a-novel-marine-anticancer-poison
#3
REVIEW
C Le Tourneau, E Raymond, S Faivre
The marine ecosystem that has contributed to the discovery of cytarabine and its fluorinated derivative gemcitabine is now considered the most productive toll to acquire new natural derived anticancer entities. Few marine anticancer agents have entered clinical development, including bryostatin-1, dolastatin 10, LU103793, ET-743, kahalalide F, didemnin B and aplidine. The marine plitidepsin aplidine derived from the mediterranean tunicate Aplidium albicans is a synthetically produced anticancer agent that is structurally related to didemnins...
2007: Current Pharmaceutical Design
https://www.readbyqxmd.com/read/16687135/effects-of-resistance-exercise-and-growth-hormone-administration-at-low-doses-on-lipid-metabolism-in-middle-aged-female-rats
#4
Ji-Yeoun Yang, Ji-Hyun Nam, Hyon Park, Youn-Soo Cha
hGH (human growth hormone) is a key factor for metabolism as well as for growth. Lack of hGH usually increases LDL (low-density lipoprotein) while impairing exercise capacity and cardiac function [Amato G., Carella C., Fazio S., La Montagna G., Cittadini A., Sabatini D., Marciano-Mone C., Sacca L., and Bellastella A., 1993. Body composition, bone metabolism, and heart structure and function in growth hormone (GH)-deficient adults before and after GH replacement therapy at low doses. J. Clin. Endocrinol. Metab...
June 6, 2006: European Journal of Pharmacology
https://www.readbyqxmd.com/read/15591002/carnitine-deficiency-disorders-in-children
#5
REVIEW
Charles A Stanley
Mitochondrial oxidation of long-chain fatty acids provides an important source of energy for the heart as well as for skeletal muscle during prolonged aerobic work and for hepatic ketogenesis during long-term fasting. The carnitine shuttle is responsible for transferring long-chain fatty acids across the barrier of the inner mitochondrial membrane to gain access to the enzymes of beta-oxidation. The shuttle consists of three enzymes (carnitine palmitoyltransferase 1, carnitine acylcarnitine translocase, carnitine palmitoyl-transferase 2) and a small, soluble molecule, carnitine, to transport fatty acids as their long-chain fatty acylcarnitine esters...
November 2004: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/10331466/primary-and-secondary-alterations-of-neonatal-carnitine-metabolism
#6
REVIEW
F Scaglia, N Longo
Carnitine plays an essential role in the transfer of long-chain fatty acids across the inner mitochondrial membrane, in the detoxification of acyl moieties, and in maintaining normal levels of free coenzyme A. Although carnitine can be synthesized in liver and kidney, normal adults obtain the majority of carnitine from the diet. Preterm newborns have a reduced capacity to synthesize carnitine. Total parenteral nutrition lacks carnitine and exposes very low birth weight infants to carnitine deficiency, with decreased production of ketones from long-chain fatty acids...
April 1999: Seminars in Perinatology
https://www.readbyqxmd.com/read/9030365/acetyl-carnitine-deficiency-in-aids-patients-with-neurotoxicity-on-treatment-with-antiretroviral-nucleoside-analogues
#7
G Famularo, S Moretti, S Marcellini, V Trinchieri, S Tzantzoglou, G Santini, A Longo, C De Simone
OBJECTIVE: A severe dose limiting axonal peripheral neuropathy may develop in subjects on treatment with the nucleoside analogues didanosine (ddl), zalcitabine (ddC), and stavudine (d4T). The impairment of mitrochondrial DNA synthesis is crucial to the pathogenesis of this disorder although other mechanisms have not been ruled out. The depletion of acetyl-carnitine, which regulates the metabolism and function of peripheral nerves could contribute to the neurotoxicity of these compounds...
February 1997: AIDS
https://www.readbyqxmd.com/read/8450178/high-dose-l-carnitine-improves-immunologic-and-metabolic-parameters-in-aids-patients
#8
RANDOMIZED CONTROLLED TRIAL
C De Simone, S Tzantzoglou, G Famularo, S Moretti, F Paoletti, V Vullo, S Delia
Several reports indicate that systemic carnitine deficiency could occur in acquired immunodeficiency disease syndrome (AIDS), and that primary and secondary carnitine deficiency leads to critical metabolic dysfunctions. L-carnitine supplementation to peripheral blood mononuclear cells (PBMCs) of AIDS patients resulted in significant enhancement of the phytohemagglutinin (PHA)-driven proliferative response. High dose L-carnitine administration (6 gr per day for two weeks) to AIDS patients treated with zidovudine also led to increased PBMCs proliferation and reduced blood levels of triglycerides...
January 1993: Immunopharmacology and Immunotoxicology
https://www.readbyqxmd.com/read/8353366/carnitine-metabolism-and-human-carnitine-deficiency
#9
REVIEW
V Tanphaichitr, P Leelahagul
Carnitine in the human body is derived from the intake of preformed dietary carnitine and biosynthesized carnitine, stemming from the metabolism of lysine and methionine. Carnitine is synthesized in liver and kidney, stored in skeletal muscle, and excreted mainly in urine. Carnitine has two main functions, i.e., transporting long-chain fatty acids into the mitochondrial matrix for beta-oxidation to provide cellular energy and modulating the rise in intramitochondrial acyl-CoA/CoA ratio, which relieves the inhibition of many intramitochondrial enzymes involving glucose and amino acid catabolism...
May 1993: Nutrition
https://www.readbyqxmd.com/read/8154877/zidovudine-induced-mitochondrial-myopathy-is-associated-with-muscle-carnitine-deficiency-and-lipid-storage
#10
M C Dalakas, M E Leon-Monzon, I Bernardini, W A Gahl, C A Jay
The use of zidovudine (AZT) for the treatment of acquired immunodeficiency syndrome (AIDS) induces a DNA-depleting mitochondrial myopathy, which is histologically characterized by the presence of muscle fibers with "ragged-red"-like features, red-rimmed or empty cracks, granular degeneration, and rods (AZT fibers). Because dysfunctioning muscle mitochondria may lead to defects of beta-oxidation of fatty acids, we examined the degree of neutral fat accumulation and muscle carnitine levels in the muscle biopsy specimens from 21 patients with AZT-induced myopathic symptoms of varying severity...
April 1994: Annals of Neurology
https://www.readbyqxmd.com/read/7914733/carnitine-depletion-in-peripheral-blood-mononuclear-cells-from-patients-with-aids-effect-of-oral-l-carnitine
#11
RANDOMIZED CONTROLLED TRIAL
C De Simone, G Famularo, S Tzantzoglou, V Trinchieri, S Moretti, F Sorice
OBJECTIVE: Reduced levels of serum carnitines (3-hydroxy-4-N-trimethyl-ammonio-butanoate) are found in most patients treated with zidovudine. However, since serum carnitines do not strictly reflect cellular concentrations we examined whether a carnitine depletion could be found in peripheral blood mononuclear cells (PBMC) from AIDS patients with normal serum carnitine levels. In addition, we explored whether it was possible to relate the host's immunoreactivity to the content of carnitine in PBMC and whether carnitine levels can be corrected by oral supplementation of L-carnitine...
May 1994: AIDS
https://www.readbyqxmd.com/read/3922885/-carnitine-biochemistry-and-clinical-aspects
#12
H Böhles
Carnitine was discovered in 1905. The human organism is able to synthetize carnitine from lysine and methionine. However the most important source of carnitine in human nutrition is meat. Carnitine plays a central role in the oxidation of long chain fatty acids, mediating their transport across the inner mitochondrial membrane. By means of the intramitochondrial reaction of acetyl-CoA with carnitine it favours an improved availability of CoA. This mechanism seems to be of major importance when pathological short chain fatty acids are accumulated within the mitochondrial matrix...
April 1985: Infusionstherapie und Klinische Ernährung
https://www.readbyqxmd.com/read/3552320/clinical-varieties-of-carnitine-and-carnitine-palmitoyltransferase-deficiency
#13
REVIEW
C Angelini, C Trevisan, G Isaya, G Pegolo, L Vergani
Several clinical entities are associated with disorders of fatty acid oxidation or transfer across the inner mitochondrial membrane. Over 40 cases of the primary carnitine deficiency syndrome have been reported to date and various subtypes have been characterized. This represents a large clinical spectrum. The deficiency of carnitine in muscle is at the basis of a syndrome characterized by muscle weakness and lipid storage myopathy. The systemic form of carnitine deficiency is more generalized and includes recurrent episodes of hepatic encephalopathy as well as lipid storage in muscle, liver and heart...
February 1987: Clinical Biochemistry
https://www.readbyqxmd.com/read/3524622/carnitine-metabolism-and-function-in-humans
#14
REVIEW
C J Rebouche, D J Paulson
It is apparent from the foregoing discussion that carnitine plays an essential role in human intermediary metabolism. The question of a dietary requirement for carnitine, particularly for the human infant, is of significant theoretical and practical interest. Aberrant carnitine metabolism resulting from abnormal genetic or acquired conditions may have serious consequences for the affected individual. At present many of the treatment modalities for carnitine deficiency are empirical. Further clarification of the mechanisms by which carnitine depletion is manifest in these conditions is essential for designing treatment programs...
1986: Annual Review of Nutrition
https://www.readbyqxmd.com/read/3079943/plasma-carnitine-levels-in-patients-receiving-home-parenteral-nutrition
#15
B A Bowyer, C R Fleming, D Ilstrup, J Nelson, S Reek, J Burnes
Patients on long-term home parenteral nutrition (HPN) are known to frequently develop hepatic steatosis or steatohepatitis. The etiology of this steatosis or steatohepatitis is unknown, but carnitine deficiency has been one of the postulated mechanisms. The importance of L-carnitine in hepatic fatty acid oxidation and the steatosis observed in primary and acquired carnitine deficiencies prompted us to determine plasma carnitine levels in 37 patients receiving long-term HPN. Thirteen patients (35%) had low total and free plasma carnitine levels...
January 1986: American Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/2742580/age-dependent-decrease-of-carnitine-content-in-muscle-of-mice-and-humans
#16
M Costell, J E O'Connor, S Grisolía
Carnitine deficiency associated with impairment of muscle function has been described in infants and young adults as a result of inherited or acquired disease but there are no reports on carnitine levels in healthy aging people. In this paper we show a marked reduction of carnitine and its derivatives in muscle, and of long-chain acyl carnitine in heart of 6-month-old mice, when compared to animals of 6 weeks of age. Analysis of muscle samples of healthy humans of different ages showed a drastic reduction of carnitine and acetyl carnitine in the older subjects with a strong reverse correlation between age and carnitine levels...
June 30, 1989: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/2143048/secondary-carnitine-deficiency-in-handicapped-patients-receiving-valproic-acid-and-or-elemental-diet
#17
N Igarashi, T Sato, S Kyouya
We examined serum-free carnitine (SFC) concentrations and serum acylcarnitine (SAC)/SFC ratios in 40 severely handicapped patients, aged 2 to 36 years, and 69 age-matched control subjects. SFC levels in the patients treated with valproic acid (VPA) and/or receiving carnitine-deficient elemental diets (ED) were significantly lower, and their SAC/SFC ratios were significantly higher than in the other patients or in control subjects. There were 6 patients whose SFC levels were less than the -2SD level (15.8 +/- 6...
April 1990: Acta Paediatrica Japonica; Overseas Edition
https://www.readbyqxmd.com/read/1558717/l-carnitine-deficiency-in-aids-patients
#18
C De Simone, S Tzantzoglou, E Jirillo, A Marzo, V Vullo, E A Martelli
OBJECTIVE: To evaluate carnitine (3-hydroxy-4-N-trimethyl-ammoniobutanoate) deficiency in AIDS patients by measuring serum total, free and short-chain carnitine concentrations. DESIGN: We conducted an open study. SETTING: All patients were seen at the Infectious Diseases Clinic, Università 'La Sapienza', Rome, Italy. PATIENTS, PARTICIPANTS: Twenty-nine AIDS patients, aged 27-41 years, with a previous history of drug use; and 14 healthy age- and sex-matched controls were studied...
February 1992: AIDS
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