Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for &lt;i&gt;L&lt;/i&gt;-Carnitine Treatment?

El‐Beshlawy, Amal; Raouf, E Abd El; Mostafa, Fatma; Talaat, Mohamed; Isma’eel, Hussain; Aoun, Elie; Av, Hoffbrand; Taher, Ali T.

doi:10.1159/000089472

Cited by 16 publications

(17 citation statements)

References 66 publications

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“…In the present study, LVEDD decreased in group B. This is in agreement with the aforementioned study in patients with sickle cell anemia that found that cardiac diastolic function was improved after 6-month treatment with l-carnitine (28).…”

Section: Discussionsupporting

confidence: 93%

“…l-Carnitine could therefore be effective in alleviating pulmonary hypertension in patients with thalassemia intermedia possibly through reduction in hemolysis and anemia. This is in agreement with the finding observed in the study in patients with sickle cell anemia that l-carnitine administration resulted in improvement of pulmonary artery pressure (28). We noted a significant increase in PAT in group-C patients, who were receiving magnesium chloride and hydroxyurea simultaneously.…”

Section: Discussionsupporting

confidence: 93%

“…It is a safe, well-tolerated compound with antioxidant properties. It alleviates pulmonary hypertension, the most prominent cardiac complication of thalassemia intermedia (20)(21)(22)(23)(24)(25)(26)(27)(28).…”

mentioning

confidence: 99%

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Effect of combination therapy of hydroxyurea with l‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia

Karimi

Mohammadi

Behmanesh

et al. 2009

European J of Haematology

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Section: Discussionsupporting

confidence: 93%

Section: Discussionsupporting

confidence: 93%

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Effect of combination therapy of hydroxyurea with l‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia

Karimi

Mohammadi

Behmanesh

et al. 2009

European J of Haematology

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“…SCD is a hypercoagulable state. Studies show that SCD patients have elevated steady-state plasma levels of circulating tissue factor [529] and PAI-1, both of which further increase during sickle vaso-occlusive crises [530, 531]. Elevated PAI-1 has been implicated in primary PHT [509, 510].…”

Section: Pulmonary Hypertensionmentioning

confidence: 99%

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

Ballas

Kesen

Goldberg

et al. 2012

The Scientific World Journal

158

144

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The sickle hemoglobin is an abnormal hemoglobin due to point mutation (GAG → GTG) in exon 1 of the β globin gene resulting in the substitution of glutamic acid by valine at position 6 of the β globin polypeptide chain. Although the molecular lesion is a single-point mutation, the sickle gene is pleiotropic in nature causing multiple phenotypic expressions that constitute the various complications of sickle cell disease in general and sickle cell anemia in particular. The disease itself is chronic in nature but many of its complications are acute such as the recurrent acute painful crises (its hallmark), acute chest syndrome, and priapism. These complications vary considerably among patients, in the same patient with time, among countries and with age and sex. To date, there is no well-established consensus among providers on the management of the complications of sickle cell disease due in part to lack of evidence and in part to differences in the experience of providers. It is the aim of this paper to review available current approaches to manage the major complications of sickle cell disease. We hope that this will establish another preliminary forum among providers that may eventually lead the way to better outcomes.

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“…In addition propionyl-L-carnitine increased eNOS protein expression in the same animal model (38). Lastly, two recent small studies on children with sickle cell disease and β-thalassemia associated PAH, suggested a benefit of L-carnitine therapy in decreasing pulmonary artery systolic pressure (39, 40). …”

Section: Discussionmentioning

confidence: 99%

L-Carnitine preserves endothelial function in a lamb model of increased pulmonary blood flow

et al. 2013

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BackgroundIn our model of congenital heart disease (CHD) with increased pulmonary blood flow (Shunt), we have recently shown a disruption in carnitine homeostasis, associated with mitochondrial dysfunction and decreased eNOS/Hsp90 interactions that contribute to eNOS uncoupling, increased superoxide levels, and decreased bioavailable NO. Thus, we undertook this study to test the hypothesis that L-carnitine therapy would maintain mitochondrial function, and NO signaling.MethodsThirteen fetal lambs underwent in utero placement of an aortopulmonary graft. Immediately following delivery, lambs received daily treatment with oral L-carnitine or its vehicle.ResultsL-carnitine-treated lambs had decreased levels of acyl carnitine, and a reduced acyl carnitine: free carnitine ratio compared to vehicle treated Shunt lambs. These changes correlated with increased carnitine acetyl transferase (CrAT) protein and enzyme activity and decreased levels of nitrated CrAT. The lactate: pyruvate ratio was also decreased in L-carnitine-treated lambs. Hsp70 protein levels were significantly decreased and this correlated with increases in eNOS/Hsp90 interactions, NOS activity, NOx levels, and a significant decrease in eNOS-derived superoxide. Further, acetylcholine significantly decreased left pulmonary vascular resistance (PVR) only in L-carnitine-treated lambs.ConclusionL-carnitine therapy may improve the endothelial dysfunction noted in children with CHD, and has important clinical implications that warrant further investigation.

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Diastolic Dysfunction and Pulmonary Hypertension in Sickle Cell Anemia: Is There a Role for <i>L</i>-Carnitine Treatment?

Cited by 16 publications

References 66 publications

Effect of combination therapy of hydroxyurea with l‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia

Effect of combination therapy of hydroxyurea with l‐carnitine and magnesium chloride on hematologic parameters and cardiac function of patients with β‐thalassemia intermedia

Beyond the Definitions of the Phenotypic Complications of Sickle Cell Disease: An Update on Management

L-Carnitine preserves endothelial function in a lamb model of increased pulmonary blood flow

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