##article.abstract##
Background: Angiotensin converting enzyme inhibitors (ACEIs) have been reported to be useful in sickle cell disease (SCD), reducing bone pain crises and proteinuria in humans as well as attenuate pulmonary hypertension (PHT) in lower animals. Since PHT results later in SCD, sufferers may survive childhood and succumb eventually to its complications. With the knowledge that ACEIs attenuate PHT apart from reducing painful crises and proteinuria, we felt that giving ACEIs to sickle cell disease patients will also reduce PHT and consequently morbi-mortality arising from its cardio-pulmonary sequelae.
Methods: SCD patients on a cross-sectional study of Right Ventricular function assessment were availed of ACEI intervention with low dose Ramipril and followed up for 3 months with outcome measures as proteinuria (using Combi 9 Urinalysis strips) and measures of pulmonary artery pressure, peak tricuspid regurgitant velocity (TRV max) and pulmonary artery pressure (PAP) determined by echocardiography
Results: There were no changes in proportion of cases with proteinuria before and after treatment. TRVmax dropped significantly with treatment from 1.17 + 0.38 m/s to 1.06 + 0.24 m/sec (p=0.044), while PAP dropped from 23.87 + 11.76 mmHg to 18.91 + 8.50 mmHg (p=0.006). Using standard cut-offs that define PHT; with TRVmax the proportion of those with PHT dropped significantly with treatment (Chi square = 4.999; p = 0.025) and for PAP it was Chi square 6.909 (p = 0.0005.)
Conclusion: ACEIs were well tolerated in this small cohort of SCD patients. Low doses that do not result in hypotension reduced pulmonary hypertension significantly in the short term; with potential of ameliorating morbi-mortality consequences of chronic haemolysis and its attendant PHT
Keywords: Sickle Cell disease; Angiotensin Converting Enzyme Inhibitors; Benefit; Pulmonary hypertension
Résumé
Contexte: Les inhibiteurs de l’enzyme de conversion de l’angiotensine (IECA) se sont révélés utiles dans la drépanocytose (SCD), réduisant les crises de douleur des os et la protéinurie chez les humains ainsi que l’atténuation de l’hypertension pulmonaire (PHT) chez les animaux inférieurs. Étant donné que la PHT entraîne plus tard une SCD, les personnes atteintes peuvent survivre à l’enfance et succomber éventuellement à ses complications. Sachant que les ACEI atténuent les PHT en plus de réduire les crises douloureuses et la protéinurie, nous avons estimé que l’administration d’ACEI aux patients drépanocytaires réduirait également la PHT et, par conséquent, la morbi-mortalité résultant de ses séquelles cardiopulmonaires.
Méthodes: Les patients atteints de drépanocytose dans une étude transversale de l’évaluation de la fonction ventriculaire droite ont bénéficié d’une intervention ACEI avec du ramipril à faible dose et ont été suivis pendant 3 mois avec des mesures de résultats telles que la protéinurie (à l’aide de bandelettes d’analyse d’urine Combi 9) et des mesures de la pression artérielle pulmonaire, pic vitesse de régurgitation tricuspide (TRV max) et pression artérielle pulmonaire (PAP) déterminées par échocardiographie
Résultats: Il n’y a pas eu de changement dans la proportion de cas de protéinurie avant et après le traitement. TRVmax a chuté de manière significative avec le traitement de 1,17 + 0,38 m / s à 1,06 + 0,24 m / s (p = 0,044), tandis que PAP a chuté de 23,87 + 11,76 mmHg à 18,91 + 8,50 mmHg (p = 0,006). Utilisation de seuils standard qui définissent PHT; avec TRVmax, la proportion de personnes atteintes de PHT a chuté de manière significative avec le traitement (Chi carré = 4,999; p = 0,025) et pour PAP, elle était de Chi carré 6,909 (p = 0,0005.)
Conclusion: les IECA ont été bien tolérés dans cette petite cohorte de patients SCD. De faibles doses qui n’entraînent pas d’hypotension réduisent significativement l’hypertension pulmonaire à court terme; avec un potentiel d’amélioration des conséquences de la morbi-mortalité de l’hémolyse chronique et de son PHT associé
Mots clés: drépanocytose; Inhibiteurs de l’enzyme de conversion de l’angiotensine; Avantage; Hypertension pulmonaire
Correspondence: Prof. Basil N Okeahialam, Department of Medicine, Jos University Teaching Hospital, Jos, Nigeria. Email: basokeam@yahoo.com
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