Knowledge, Attitudes and Practices of Parents in the Vaso-Occlusive Crises of the Sick Children in Brazzaville

Objectives: to evaluate the parents’ knowledge of vaso-occlusive crises, to identify their attitudes and practices. Method: a retrospective, transversal, descriptive and analytical study carried out between January and June 2016 at the HUC of Brazzaville. The parents of sickle cell children aged 2 to 17 years, followed for at least one year, were interviewed. Results: a total of 319 parents were interviewed, including 221 women (69.3%) and 98 men (30.7%) with a mean age of 40.8 ± 9 years. They were unschooled 65 cases (20.4%), had primary education 41 cases (12.9%), secondary 149 cases (46.7%) and higher level 64 cases (20.1%). They had one child sickle cell 258 cases (80.9%). They knew sickle cell disease 283 cases (88.7%), its genetic transmission 249 cases (78.1%). The source of information was a health worker 211 cases (66.1%). They had a good knowledge of the vaso-occlusive crisis 101 cases (31.7%). In a vaso-occlusive crisis, they used a health facility 207 cases (64.8%), self-medicated 66 cases (20.7%). Their home practices were good 99 cases (31%), fairly good 135 cases (42.3%) and bad 85 cases (26.5%). Parents aged 25 to 34 (OR = 1.8 95% 1 - 3), secondary (OR = 1.7 95% 1 - 2.7) and with good practices (OR = 4.5 95% 2.7 - 7.5) are those who have a good knowledge of vaso-occlusive crises. Conclusion: The importance and severity of vaso-occlusive crises in the sickle-cell child’s life experience, the crucial role played by parents in their care requires that the information, education and communication of these children be strengthened To improve their quality of life.

Tramadol use in pediatric sickle cell disease patients with vaso-occlusive crisis

AIM: To evaluate whether the addition of scheduled oral tramadol to intravenous morphine and intravenous ketorolac reduces morphine requirements.METHODS: This single-centered, Institutional Review Board-approved, retrospective study at Moses Cone Memorial Hospital included pediatric patients who were ≥ 2 years old with vaso-occlusive crisis(VOC) caused by sickle cell disease(SCD), were on morphine patientcontrolled analgesia(PCA), and had scheduled oral tramadol added to their standard pain regimen. The study population was admitted between March 2008 and March 2011. The data was collected from electronic records and included age, weight, morphine use, tramadol use, hemoglobin, pain scores, number of days on PCA, length of hospital stay, respiratory rate, and polyethylene glycol use. Thirty patients were analyzed as independent admissions and seven patients as paired admissions. RESULTS: Eighteen pediatric SCD patients with VOC received morphine PCA and intravenous ketorolac and twelve patients received morphine PCA and intravenous ketorolac and scheduled oral tramadol. Baseline characteristics were similar between both groups with the exception of the average weight, which was greater in the tramadol group than in the morphine group. The average morphine requirements in patients with and without the use of tramadol were similar, both for the independent admissions [0.58 mg/kg per day vs 0.65 mg/kg per day(P = 0.31)] and the paired admissions [0.71 mg/kg per day vs 0.77 mg/kg per day(P = 0.5)]. The daily polyethylene glycol requirement was less in the tramadol group for both the independent [0.5 g/kg per day vs 0.6 g/kg per day(P = 0.64)] and paired admissions analyses [and 0.41 g/kg per day vs 0.55 g/kg per day(P = 0.67)].CONCLUSION: The addition of scheduled tramadol in patients receiving concomitant morphine and ketorolac demonstrates a trend toward decreased morphine and polyethylene glycol use.

Conventional and non-conventional management stratagies for pregnant women with sickle cell disease

Sickle cell disease is the most common inherited hemoglobinopathy and is characterized by painful vaso-occlusive crisis.Pregnancy in women with sickle cell disease is associated with an increased incidence of maternal and perinatal complications,but notwithstanding,adequate care through preconception,antenatal,intrapartum,and postnatal stages ensures a better outcome.Despite the management of pregnant women with sickle cell disease being best accomplished using a multidisciplinary approach,few therapeutic interventions have been explored.This paper focuses on the effective conventional and non-conventional management of pregnant women with sickle cell disease with the aim of suppress the alternating effect of sickle cell disease and pregnancy for better maternal and fetal outcomes.The conscious use of these therapies integratively or alternatively can improve self-care and quality of life,decrease the occurrence of discomforts and complications,and reduce frequency of hospitalization.

Sickle Cell Disease and Pregnancy: Does Outcome Depend on Genotype or Phenotype?

Objective: Women with Sickle Cell Disease (SCD) who become pregnant are at risk for serious maternal and fetal complications. Our objective was to determine if pregnancy outcome is dependent on phenotype. Methods: Retrospective cohort study of pregnant women with SCD, including hemoglobin (Hb) SS, Hb SC, and Hb Sβ-thalassemia, between January 1999 and December 2008). Antenatal and neonatal outcomes were compared between pregnancies with painful episodes and those without. The primary outcome was preterm birth (PTB) <37 weeks. Secondary outcomes included maternal medical complications, antenatal complications, delivery outcomes, and neonatal outcomes. Results: 31 women were included (18 (58%) with painful episodes, 13 (42%) without painful episodes). The median number of painful episodes was 2.5 (1 - 19) and these women required a median of 13 total days (1 - 59) of inpatient treatment. At delivery, women who had experienced painful episodes had lower Hb levels and were more likely to be taking chronic narcotic pain medications. The overall incidence of PTB <37wks was 55% and was not significantly different between groups (11 [61%] with painful episodes versus 6 [46%] without painful episodes;p = 0.485). Secondary outcomes were also not significantly different between groups. There was one maternal death. Conclusion: Adverse obstetrical out-comes were more common among women with sickle cell disease who experienced painful crises however, in this small sample, the difference were not statistically significant.

Modeling and Simulation of the Autocatalytic Kinetics of Haemoglobin SS Polymerization: Onset of Polymerization

We report a fresh and simpler approach to the modelling of the kinetics of the polymerization of Hb SS in sickle cell patients that couples the kinetics and the hydrodynamics of blood flow in mechanistic understanding of the process. The well-known two-step autocatalytic reaction scheme was used for the polymerization reaction with the assumption of simpler first-order reaction scheme for each stage. In addition, the forces acting on a particle in motion were also introduced to account for compelling settling of the red cells that lead to vessel occlusion (vaso-occlusion). A first attempt on the prediction of vessel blockage was made using this novel model. The time for the onset of the polymerization reaction was derived from hydrodynamic considerations and kinetics while the kinetic rate constants were obtained from the autocatalytic nature of the reaction. Experimental data for model validation were obtained from recruited SS patients and in vitro data of Hofrichter. Over 100 volunteers were recruited for participation in this work but less than 40% met the inclusion criteria. Participants were of age range 13 - 43 (with a mean of 26 ± 8 years) for SCD patients and 18 - 43 (with a mean of 28 ± 7 years) for control participants. Blood indices and Transcranial Doppler (TCD) test parameters of all participants were the principal parameters used for model validation. Constant k2/k1 ratios was obtained for individual in vivo/in vitro system. This ratio is unique for any individual, independent on protein sequence and also suggests the degree of expression of the symptoms of Sickle Cell Disease (SCD) with higher values reflecting greater propensity to pain crisis. Delay time, tD, was found to have an inverse relationship with the kinetic constant for the residual reaction, k1. Therefore, long delay times calculated, offer insight on why SCD patients are not in perpetual crises because enough time is provided the cells to escape microcirculation while keeping the residual reaction at the minimum. Sensitivity analysis was carried out to obviate the limitations encountered in the course of the work. Results showed the onset of occlusion to be most sensitive to the diameter of the blood vessel.

Sequential bilateral central retinal artery occlusion as the primary manifestation of systemic lupus erythematosus

Bilateral central retinal artery occlusion （CRAO） has been rarely reported as the primary manifestation in patients with systemic lupus erythematosus （SLE）. The severe retinal vaso-occlusive diseases usually cause devastating and permanent damage to visual function in spite of vigorous treatment. A 42-year-old Chinese woman presented with abrupt bilateral vision loss. The diagnosis of bilateral CRAO was suggested by the ocular presentation and fluorescein angiography. Laboratory studies showed positive results of antinuclear antibody, anti-Ro/SSA anti-La/SSB; decreased levels of C3, C4 complement and normal levels of antiphospholipides antibodies （APAs）. Her visual acuity deteriorated despite systemic steroid and immunosuppressant treatment. Severe vaso-occlusive retinopathy may be an earlier manifestation of SLE without elevated level of APAs.

Triterpenoid inducers of Nrf2 signaling as potential therapeutic agents in sickle cell disease： a review

Sickle cell disease （SCD） is an inherited disorder of hemoglobin in which the abnormal hemoglobin S polymerizes when deoxygenated. This polymerization of hemoglobin S not only results in hemolysis and vaso- occlusion but also precipitates inflammation, oxidative stress and chronic organ dysfunction. Oxidative stress is increasingly recognized as an important intermediate in these pathophysiological processes and is therefore an important target for therapeutic intervention. The transcription factor nuclear erythroid derived- 2 related factor 2 （Nrf2） controls the expression of anti-oxidant enzymes and is emerging as a protein whose function can be exploited with therapeutic intent. This review article is focused on triterpenoids that activate Nrf2, and their potential for reducing oxidative stress in SCD as an approach to prevent organ dysfunction associated with this disease. A brief overview of oxidative stress in the clinical context of SCD is accompanied by a discussion of several pathophysiological mechanisms contributing to oxidative stress. Finally, these mechanisms are then related to current management strategies in SCD that are either utilized currently or under evaluation. The article concludes with a perspective on the potential of the various therapeutic interventions to reduce oxidative stress and morbidity associated with SCD.