Pharmacodynamics of rocuronium in sickle cell patients.

BACKGROUND: Sickle cell disease is the most widespread genetic disease in the world. The chronic organ damage due to this disease could lead to variability of responses to the anaesthetic drugs. We analysed the pharmacodynamics response of rocuronium to sickle cell patients. METHODS: We observed and compared the onset time and clinical duration (time to recovery first twitch) of 0.6 mg kg-1 of rocuronium using a TOFscan® monitor, as well as the time before the first incremental dose (time to recovery second twitch), in a group of 22 homozygous sickle cell patients and a group of 23 controls, all programmed for laparoscopic surgical procedures. RESULTS: The onset time of rocuronium was longer in sickle cell patients [mean ± SD (extremes)], [6.3 ± 2.1 (1.8-10) min] than in the control group [2.5 ± 0.6 (1.4-3.5) min] (P < 0.01). The clinical duration was shorter in sickle cell patients [19.2 ± 7.1 (13-41) min] when compared to the control group [28.9 ± 6.9 (21-48) min] (P < 0.01). The time before the first incremental dose was shorter in the sickle cell patients group [27.7 ± 7.9 (19-49) min] compared to the control group [39.9 ± 8.7 (30-56) min] (P < 0.01). CONCLUSION: The onset time of rocuronium was significantly longer with a shorter duration of action in patients with sickle cell disease versus the general population.

Assessment of MTR Rs1805087 SNP as Possible Modifier ofSickle Cell Disease Severity in a Nigerian Population

BACKGROUND: Sickle cell disease is the commonest geneticdisorder in Nigeria, affecting 2­3% of an estimated population of 160million people. The role of genetic mutations in folate cycle genes,and the variable phenotypic expressions constituting disease severity,needs to be critically examined.OBJECTIVE: This study was carried out to establish the pattern ofmethionine synthase gene mutations (rs1805087 SNP), and its possibleassociation with disease severity in adults with sickle cell anaemia inLagos, Nigeria.METHODOLOGY: This is a cross-sectional study of seventy (70)subjects with sickle cell disease (HbSS) matched for age and genderwith known apparently healthy haemoglobin genotype AA (HbAA)subjects, as cases and controls respectively. Structured questionnaireswere used to obtain demographic, clinical and other phenotypic dataneeded to compute disease severity. Pattern of MTR A2756G genemutation and homocysteine assay (Hcy) were assessed by PolymeraseCh ain Reaction and Enzyme- linked Immun osorbent Assayrespectively. Full blood count analysis of participants was done usingthe KX-21 Automated Analyzer (Sysmex Corporation, Japan).RESULTS: The mutant genotypes MTR 2756 AG/GG were recordedin 46.4% (n =55) of subjects with disease severity score >7. Elevatedplasma homocysteine (HHcy) was significantly associated withdisease severity among HbSS subjects (OR=17.2, CI: 3.490-86.079;p=0.0001). Conversely, no significant association was observed withthe mutant genotypes MTR 2756 AG/GG and disease severity(p>0.05).CONCLUSION: While HHcy is significantly associated withphenotypic expression of HbSS, the MTR 2756 SNPs did not appearto independently influence homocysteine level or disease severity inHbSS subjects

Management of a broken modular femoral stem following total hip arthroplasty in a patient with sickle cell disease using an endofemoral trephine reamer: A case report.

INTRODUCTION AND IMPORTANCE: Breakage of the femoral stem with intact bone is a rare complication that has only been reported once. Sickle cell disease (SCD) patients are more prone to variable complications due to the nature of their disease. We discuss how to safely remove a fixed broken stem using an intramedullary trephine reamer to achieve optimal outcome in a SCD patient. It is important to keep an intact femoral cortex during arthroplasty to achieve stable prostheses. CASE PRESENTATION: We report a 35 years old SCD male, who complains of left hip pain and decreased activity 18-months following total hip arthroplasty with no history of trauma nor infection. Lab work and radiography showed signs of aseptic loosening and breakage of the femoral stem with no signs of cortical fractures. A trephine reamer was used to extract the stem during revision arthroplasty. Two years post-operative follow up showed improved Harris hip score (HHS) and apparent clinical improvement in function and pain. DISCUSSION: Multiple femoral stem extraction techniques have been reported in the literature. However, these approaches have variable disadvantages. Using the trephine reamer intramedullary helped to extract the distal femoral stem fragment and preserve the integrity of the femoral cortex. However, caution should be taken to avoid iatrogenic injuries. CONCLUSION: Using the trephine reamer to extract a broken femoral stem is a safe technique that preserves the cortical integrity. Complications of the trephine reamer include heat necrosis and iatrogenic fractures. Intra-operative measures can be taken to limit those complications.

Haematological Changes Associated with Hepatitis B Virus Infection in Individuals with and without Sickle Cell Disease.

BACKGROUND: Hepatitis B virus infection, a major public health problem that primarily affects the liver, may cause reduction in the levels of haemoglobin, haematocrit and in the extreme, could cause aplastic anaemia. The haematological characteristics could be detected with a complete blood count which could provide invaluable information for diagnosis and management of the disease. AIM: To determine the effect of HBV infection on the blood count of individuals with sickle cell disease (SCD) and apparently normal healthy (Non-SCD). SETTING: Non-SCD participants were recruited from the community while SCD patients in steady state were recruited from SCD routine clinics. METHODS: The study was a cross - sectional study carried out on 1017 non-SCD and 1017 SCD individuals. Haematology Autoanalyzer was used to determine the complete blood count. Granulocyte-to-lymphocyte ratio (GLR), platelet to white blood cell count ratio (PWR) and platelet-to-lymphocyte ratio (PLR) were calculated. ELISA for HBsAg and HBV core antigen IgM antibodies were used to identify participants with HBV. RESULTS: The non- SCD individuals infected with HBV had significantly higher WBC (7.51 ± 5.8 X109/L)) compared to a WBC (6.1 ± 3.4 X109/L) in uninfected individuals (p =0.001). PWR for HBV negative (49.9±28.6) was higher than that for HBV positive participants (41.4±17.6) (p=0.034). Mean platelet volume (MPV) of 9.93 ± 1.1fl in SCD individuals with HBV was significantly higher than 8.30 ± 0.95fl in SCD individuals without HBV (p=.001). CONCLUSIONS: PWR and MPV may be useful as surrogate marker for detection of HBV disease progression in apparently normal healthy non - SCD and SCD populations to institute prompt appropriate ancillary investigation and treatment.

CONTEXTE: L'infection par le virus de l'hépatite B, un problème majeur de santé publique qui affecte principalement le foie, peut entraîner une réduction des taux d'hémoglobine et d'hématocrite et, dans l'extrême, peut provoquer une anémie aplastique. Les caractéristiques hématologiques peuvent être détectées par un hémogramme complet qui pourrait fournirdes informations précieuses pour le diagnostic et la et la gestion de la maladie. OBJECTIF: Déterminer l'effet de l'infection par le VHB sur sanguine complète d'individus atteints de drépanocytose (SCD) et d'individus apparemment normaux en bonne santé (non-SCD). SITE: Les participants de non-SCD ont été recrutés dans la communauté tandis que les patients drépanocytaires en état stable ont été recrutés dans les cliniques de routine de la drépanocytose. MÉTHODES: L'étude était une étude transversale menée sur 1017 personnes non-SCD et 1017 personnes SCD. sur 1017 personnes nonSCD et 1017 personnes SCD. Un autoanalyseur hématologie a été utilisé pour déterminer la formule sanguine complète. Le rapport granulocytes/ lymphocytes (GLR), le rapport plaquettes/blancs (PWR) et le rapport plaquettes/lymphocyte (PLR). ont été calculés. Les tests ELISA pour les anticorps IgM de l'Ag HBs et de l'antigène central du VHB ont été utilisés pour identifier les participants atteints du VHB. RÉSULTATS: Les individus non atteints de DSC et infectés par le VHB présentaient les caractéristiques suivantes un nombre significativement plus élevé de GB (7,51 ± 5,8 X109/L) par rapport à une WBC (6,1 ± 3,4 X109/L) chez les individus non infectés (p =0,001). Le TPM pour lesparticipants négatifs pour le VHB (49,9±28,6) était plus élevé que celuipour les participants positifs au VHB (41,4±17,6) (p=0,034). Le volume moyen des plaquettes Le volume plaquettaire moyen (VPM) de 9,93 ± 1,1fl chez les personnes atteintes de MCS avec VHB était significativement plus élevé que celui des personnes atteintes de MCS sans VHB (8,30 ± 0,95fl) (p=0,001). sans VHB (p=0,001). CONCLUSIONS: Le PWR et le MPV peuvent être utiles comme marqueurs de substitution pour la détection de la progression de la maladie VHB chez population de SCD et Non-SCD apparemment normale, en bonne santé afin d'instituer rapidement les examens complémentaires et le traitement appropriés.

Caregiver's Health Locus of Control and Medication Adherence in Sickle Cell Disease.

ACKNOWLEDGEMENTS: The authors would like to thank Dr. Morisky for giving us permission to use the Morisky Medication Adherence Scale PURPOSE: To explore caregivers' Health Locus of Control's relationship to self-reported adherence to penicillin prophylaxis or hydroxyurea in children with sickle cell disease (SCD). PROCEDURE: A questionnaire-based study was conducted of caregivers of children with SCD who visited a comprehensive sickle cell center in an inner city hospital, who were either on penicillin prophylaxis or hydroxyurea or both. Multidimensional Health Locus of Control Scale (MHLC) and the Morisky Medication Adherence Scale (MMAS-8) questionnaires were used for the study. RESULTS: Caregivers of 43 children (27 on penicillin prophylaxis, 13 on hydroxyurea, and 3 on both) completed the MHLC and the MMAS-8. There was no significant difference in adherence between the penicillin and the hydroxyurea groups. The mean Powerful Others score of caregivers of the hydroxyurea only group (25.5+5.6) was higher than that of the penicillin only group (21.2+6.1, p=0.043). Regression analysis revealed an inverse relationship of Chance Locus of Control to adherence in the entire group (Beta = -0.306, R2=0.093, F[1,40]=4.12, p=0.049). CONCLUSION: Chance Locus of control may identify caregivers of children with SCD at risk for non-adherence to treatment.