Echocardiographic Left Ventricular Hypertrophy and Geometric Patterns in Patients with Sickle Cell Anaemia.

BACKGROUND: Left ventricular hypertrophy (LVH) is a common complication in patients with sickle cell anaemia (SCA), and it has been associated with systolic and diastolic dysfunction, and sudden death. There is a wide variation in the reported prevalence of LVH in patients with SCA, partly due to the varying criteria applied, and the impact of small weight and body surface area (BSA) in SCA patients. We used four different criteria to determine echocardiographic LVH and geometric patterns in patients with steady-state SCA. Left ventricular hypertrophy was defined by LVM, LVM indexed to BSA, LVM indexed to height and LVM indexed to height2.7 using gender-specific reference values. Left ventricular geometry was determined using LVH and relative wall thickness. RESULTS: Eighty-two patients with steady-state SCA, aged 18years and above were studied from January 2018 to April 2018. The median [IQR] age of the patients was 23 [10] years. Forty-seven (57.3%) were females. The prevalence of LVH was highest when LVM was indexed to BSA (80.5%), followed by LVM indexed to height (73.2%). Comparable prevalences of 68.3% and 69.5% were observed using LVM and LVM indexed to height2.7, respectively. The prevalence of LVH was similar in males and females for all the criteria. CONCLUSION: The prevalence of LVH is high among patients with steady-state SCA irrespective of the criteria applied. The most prevalent geometric pattern was eccentric LVH. Indexing to BSA might result in over-estimation of LVH given the relatively small BSA in patients with SCA. Indexing to height 2.7 might give a more accurate estimate of LVH.

CONTEXTE: L'hypertrophie ventriculaire gauche (HVG) est une complication fréquente chez les patients atteints d'anémie falciforme (ACS), et elle a été associée à un dysfonctionnement systolique et diastolique, ainsi qu'à une mort subite. La prévalence de l'HVG chez les patients atteints d'anémie falciforme varie considérablement, en partie à cause des différents critères appliqués et de l'impact du petit poids et de la surface corporelle (BSA) des patients atteints d'anémie falciforme. Nous avons utilisé quatre critères différents pour déterminer l'HVG échocardiographique et les schémas géométriques chez les patients atteints d'ACS à l'état stable. L'hypertrophie ventriculaire gauche a été définie par la MVL, la MVL indexée sur la surface corporelle, la MVL indexée sur la taille et la MVL indexée sur la taille2,7 en utilisant des valeurs de référence spécifiques au sexe. La géométrie du ventricule gauche a été déterminée en utilisant l'HVG et l'épaisseur relative de la paroi. RÉSULTATS: Quatre-vingts deux patients atteints d'ACS à l'état stable, âgés de 18 ans et plus ont été étudiés de janvier 2018 à avril 2018. L'âge médian [IQR] des patients était de 23 [10] ans. Quarantesept (57,3 %) étaient des femmes. La prévalence de l'HVG était la plus élevée lorsque la MVL était indexée sur la BSA (80,5 %), suivie de la MVL indexée sur la taille (73,2 %). Une prévalence comparable de 68,3 % et 69,5 % a été observée en utilisant la MVL et la MVL indexée sur la taille2,7, respectivement. La prévalence de l'HVG est similaire chez les hommes et les femmes pour tous les critères. CONCLUSION: La prévalence de l'HVG est élevée chez les patients atteints d'ACS à l'état stable, quel que soit le critère appliqué. Le modèle géométrique le plus répandu est l'HVG excentrique. L'indexation à la BSA pourrait entraîner une surestimation de l'HVG étant donné la BSA relativement faible chez les patients atteints d'ACS. L'indexation à la taille 2,7 pourrait donner une estimation plus précise de l'HVG. Mots clés: Hypertrophie ventriculaire gauche; Géométrie ventriculaire gauche; Drépanocytose ; Échocardiographie.

Chronic Kidney Disease amongst Sickle Cell Anaemia Patients at the University of Maiduguri Teaching Hospital, Northeastern Nigeria: A Study of Prevalence and Risk Factors.

BACKGROUND: Involvement of the kidneys in patients with sickle cell anaemia is a well recognised chronic complication. This study seeks to determine the prevalence of chronic kidney disease in patients with homozygous sickle cell disease (HbSS) and to identify risk factors associated with its development. METHODOLOGY: The subjects consisted of adolescents and adults with HbSS recruited sequentially from the adult haematology outpatient clinic and Daycare ward of the unit. Clinical variables including age at diagnosis of SCA, the frequency of vaso-occlusive crisis and transfusion therapy, as well as laboratory data including haematological profile and renal function tests were obtained. The glomerular filtration rate was estimated (eGFR) using the 'modification of diet in renal disease' (MDRD) formula. RESULTS: Two hundred and eighty-four HbSS patients were recruited. The prevalence of CKD amongst them was 38.9%. Further stratification of the patients based on eGFR showed that sixty-nine (26.8%) had hyperfiltration; 35 (13.6%) stage 1 CKD; 53 (20.6%) stage 2 CKD; 33 (12.8%) stage 3a CKD; 28 (10.9%) stage 3b CKD; 30 (11.7%) stage 4 CKD and 9 (3.5%) had end stage renal disease. There was significant association between eGFR and clinical parameters such as age (r -0.353, p=0.000), SBP (r -0.148, p= 0.021), DBP (r -0.213, p=0.001) and total number of blood received (r -0.276, p=0.000); and laboratory parameters such as PCV (r 0.371, p=0.000); urea ( r 0.527, p=000); creatinine (r 0.625, p=0.000) and uric acid ( r -0.419, p=0.000). CONCLUSIONS: The present study has revealed a high prevalence of CKD amongst patients with SCA in our region. Clinical and laboratory predictors of CKD using eGFR were identified to include age, SBP, number of units of blood transfusion, PCV, urea, creatinine and uric acid levels.

Imatinib theraphy in chronic myeloid leukaemia : review of mechanisms of resistance and therapheutic options in imatinib failure

Chronic myeloid leukaemia is triphasic, clonal malignancy, arising from the haemopoeitic stem cell. It is characterized by the presence of philadelphia chromosome, which result from reciprocal translocation between chromosome 9 and 22. The resulting oncogen- brc-abl has proliferative activity and survival advantage against normal cell and this account for the clinical and laboratory manifestation of this myeloproliferative disorder. Imatinib, a tyrosine kinase inhibitor (TKIs) is currently the first line of treatment, however, one third of patient develope resistance to it, thus necessitating alternative TKIs. Many factors are associated with the development of resistance to imatinib, such as mutation in the brc-abl gene, increase production of the mutant protein and activation of alternatve pathways amongst other causes. The aim of this reveiw is to explore these factors, and also to avaluate current TKIs that are use as alternative in Imatinib resistant cases