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...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.展开更多
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, retr...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.展开更多
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 a...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.展开更多
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: Ret...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.展开更多
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...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.展开更多
Sickle cell disease is an autosomal recessive genetic disease. Vaso occlusive crisis (VOC) is frequently seen in such patients. Painful VOC is usually recurrent, of variable severity due to many factors and its manage...Sickle cell disease is an autosomal recessive genetic disease. Vaso occlusive crisis (VOC) is frequently seen in such patients. Painful VOC is usually recurrent, of variable severity due to many factors and its management poses important challenge in the clinical practice. Few patients do not respond to standard therapies and continue to suffer severe pain for prolonged period or land to serious life threatening situation. The red cell exchange by aphaeresis is presumed to be one efficient alternative in this situation which can reduce the level of HbS below 40% - 50%. However, it is costly and not available everywhere. Both circumstances are common in our state where incidence of sickle cell disease is quite high. In such situations simple red cell exchange i.e. removing 1 unit (350 ml) of blood manually (by phlebotomy) and replacement with one unit normal red cell is effective. All of our four cases of SCA with severe acute VOC, are not responding to standard therapy but responded efficiently to this simple red cell exchange transfusion. Our present observation may pave the way of one simple, affordable, and effective measure to reduce the pain of severe acute VOC not responding to standard therapy. Moderate reduction of HbS by 8% - 14% by simple red cell exchange transfusion was associated with relief of pain of acute VOC;a new observation was reported in all our 4 cases which need to be validated by larger controlled studies.展开更多
文摘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.
文摘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.
文摘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.
文摘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.
文摘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.
文摘Sickle cell disease is an autosomal recessive genetic disease. Vaso occlusive crisis (VOC) is frequently seen in such patients. Painful VOC is usually recurrent, of variable severity due to many factors and its management poses important challenge in the clinical practice. Few patients do not respond to standard therapies and continue to suffer severe pain for prolonged period or land to serious life threatening situation. The red cell exchange by aphaeresis is presumed to be one efficient alternative in this situation which can reduce the level of HbS below 40% - 50%. However, it is costly and not available everywhere. Both circumstances are common in our state where incidence of sickle cell disease is quite high. In such situations simple red cell exchange i.e. removing 1 unit (350 ml) of blood manually (by phlebotomy) and replacement with one unit normal red cell is effective. All of our four cases of SCA with severe acute VOC, are not responding to standard therapy but responded efficiently to this simple red cell exchange transfusion. Our present observation may pave the way of one simple, affordable, and effective measure to reduce the pain of severe acute VOC not responding to standard therapy. Moderate reduction of HbS by 8% - 14% by simple red cell exchange transfusion was associated with relief of pain of acute VOC;a new observation was reported in all our 4 cases which need to be validated by larger controlled studies.
