scholarly journals Outcomes of Splenectomy in Children with Sickle Cell Disease

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 1382-1382
Author(s):  
Peter B. Soh ◽  
Abdul H. Siddiqui
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Doppler Ultrasonography ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Operative Complications ◽  
The Mean ◽  
Penicillin Prophylaxis

Abstract Background Children with splenic sequestration, related to sickle cell disease, are managed acutely with blood transfusions followed by an elective splenectomy to prevent recurrent episodes. The objective of this study was to review the outcomes of splenectomy in children with sickle cell disease as a single center experience. Methods A retrospective chart review of children with sickle cell disease who had splenectomy between 1999 and 2014 at the University of South Alabama was performed. Data on demographics, sequestration episodes, post-operative complications, bacteremia, transcranial doppler ultrasonography and death were collected. Results A total of 52 patients (36 with Hemoglobin SS, 7 with Hemoglobin SC, 5 with S-Beta Thalassemia Plus, and 4 with S-Beta Thalassemia Zero) received splenectomy during the study period. Mean age at first splenic sequestration event was 39 months. The mean age of splenectomy was 5 years (Minimum: 18 months; Maximum: 18 years). There were 24 males and 28 females. Over 95 percent of patients were on penicillin prophylaxis. In only 73 percent of patients, proof of completed vaccination including pneumococcal polysaccharide, pneumococcal conjugate and meningococcal conjugate vaccines, could be found. Average post-splenectomy follow-up was 7.4 years. The post-operative complications included fever in 4 patients, acute chest syndrome in 4 patients, lobar pneumonia in 2 patients, pleural effusion in 1 patient, atelectasis on chest radiograph in 2 patients and surgical wound abscess in 1 patient. One patient had an intra-abdominal bleed which required reoperation. The average number of hospitalizations for vaso-occlusive pain crises was 3.3 per year prior to splenectomy and 2.2 per year during the 2 years following splenectomy (p=0.04). Mean platelet count before splenectomy was 267/m3 compared to 533/m3 at 1 year after splenectomy (p<0.05). Differences in mean white blood cell and reticulocyte counts before and after splenectomy were not statistically significant. Only 2 of the patients had culture proven bacteremia, but both of them occurred prior to their splenectomy. One of the patients grew Staphylococcus hominis and the other grew Staphylococcus lugdunensis. No true bacteremia were reported in patients after splenectomy. None of the patients developed stroke while four (~8%) patients developed critical transcranial doppler ultrasonography velocities (≥200 cm/s) and were started on chronic blood transfusions. The mean time-average maximum velocity before splenectomy was 127 cm/sec and increased to 151 cm/sec at 2 years after splenectomy (p=0.002). Among the splenectomized patients, 18 (35%) of them had been started on Hydroxyurea. Discussion Our results indicate that with proper vaccination and penicillin prophylaxis, the risk of infection after splenectomy can be controlled. The mean hemoglobin levels did not change after splenectomy but our patients had fewer hospitalizations for pain crises after splenectomy. The cerebral blood flow velocity increased after splenectomy. This might imply that more patients will require chronic blood transfusions for stroke prevention after splenectomy. We conclude that splenectomy is a safe and effective modality for management of life threatening splenic sequestrations in children with sickle cell disease. Disclosures No relevant conflicts of interest to declare.

scholarly journals Clinical events in the first decade in a cohort of infants with sickle cell disease. Cooperative Study of Sickle Cell Disease [see comments]

Blood ◽  
1995 ◽  
Vol 86 (2) ◽  
pp. 776-783 ◽  
Author(s):  
FM Gill ◽  
LA Sleeper ◽  
SJ Weiner ◽  
AK Brown ◽  
R Bellevue ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
The United States ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cooperative Study ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Clinical Events

Within the Cooperative Study of Sickle Cell Disease, 694 infants with confirmed sickle cell disease were enrolled at less than 6 months of age. Information about the nature and frequency of complications was collected prospectively over a 10-year period. Painful crises and acute chest syndrome were the most common sickle cell-related events in homozygous sickle cell anemia (SS), hemoglobin SC disease (SC), and S beta thalassemia patients (overall incidence in SS patients of 32.4 and 24.5 cases per 100 person-years, respectively). Bacteremia occurred most frequently in SS children under 4 years of age and in SC patients less than 2 years of age. The mortality rate was low in this cohort compared with that found in previous reports. Twenty children, all with Hb SS, died (1.1 deaths per 100 person-years among SS patients). Infection, most commonly with Streptococcus pneumoniae and Hemophilus influenzae, caused 11 deaths. Two children died of splenic sequestration, 1 of cerebrovascular accident, and 6 of unclear causes. Two patients underwent cholecystectomies, and 17 underwent splenectomies after one or more splenic sequestration crises. The experience of this cohort should reflect closely the true clinical course of those children with Hb SS and Hb SC disease who are observed in sickle cell centers in the United States.

scholarly journals Comparison of Clinical Evolution of Children with Sickle Cell Disease before and after Treatment with Hydroxyurea at Saint Damien Hospital, Tabarre-Haiti,2013-2018

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 2306-2306
Author(s):  
Nora St Victor Dély ◽  
Ofelia A. Alvarez ◽  
Vanessa J Dor ◽  
Emmeline Lerebours
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Clinical Course ◽  
Acute Chest Syndrome ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Clinical Evolution ◽  
Before And After ◽  
The Mean ◽  
Painful Crisis

INTRODUCTION Sickle cell disease (SCD), an autosomal recessive hemoglobinopathy, is associated with a high morbidity and mortality rate, especially in low income countries. In Africa, 5% of deaths among children under five are attributable to SCD [59th World Health Assembly, WHO 2006]. This chronic disease greatly alters the quality of life of affected children. However, according to several published studies, SCD clinical course can be improved with the administration of hydroxyurea, an antimetabolite drug. [Nkashama, Pan African Medical Journal,2015] Saint-Damien, a pediatric hospital in Haiti, has a current cohort of 1248 sickle cell children. Forty of them (3 %) benefit from hydroxyurea administration since November 2015. In this hospital, data on how hydroxyurea modifies SCD clinical course are lacking, despite the advantage of this drug described in literature [Charache,New England Journal of Medicine,1995]. This study aims to compare the evolution of children treated at Saint Damien Hospital, before and after receiving hydroxyurea. METHODS A retrospective analytic study was conducted from November 2013 to June 2018 in the Sickle Cell Clinic at Saint-Damien Hospital. We included 40 children aged 2 to 15 years old treated with hydroxyurea. All of them benefit of the same treatment protocol: Initial dose of 10 mg per kg per day increase to maintenance dose of 25 mg per kg per day. Any child whose treatment has been permanently discontinued regardless of the cause was excluded. Epidemiological and clinical data were collected using Excel 2010. We compared children clinical evolution two years before and two years after hydroxyurea administration using these parameters: frequency and duration of hospitalizations, hospitalization frequency for specific complications (pain crisis, stroke and acute chest syndrome), and frequency of blood transfusions. We calculated frequencies, ratios and means using Epi Info. We realized statistical analysis to compare quantitative variables with a p value significant when less than 5%. RESULTS Gender ratio was 1:1. The mean age of children at enrollment on hydroxyurea was 8 years. Thirty-eight children of 40 (95 %) experienced at least one hospitalization before receiving the drug, compared with 17 (42.5%) after, p=0.025. The mean duration of hospitalization was 9 days before and 6 days after, p=0.0319. The average number of hospitalizations per child was decreased by 30 %. Seventy percent of children were hospitalized at least once due to painful crisis 2 years before receiving hydroxyurea, compare to 22.5 % after. Thirty-one children (77.5%) were transfused at least once before receiving the drug and 9 (22.5%) after receiving it. There was no cases of acute chest syndrome or stroke reported after hydroxyurea, unlike before the introduction of the drug. (Table 1) CONCLUSION The percentage of hospitalized children and the average length of hospitalization stay decreased significantly with hydroxyurea intake; as well as the frequency of painful crisis and blood transfusions. Hydroxyurea acts directly on the two main causes of hospitalization in the sickle cell, reducing the morbidity related to this pathology; and demonstrating the direct benefit of this drug at Saint Damien Hospital. Since our cohort is young, we have not been able to follow his evolution over a longer period of time. We plan to continue to observe this cohort. But these first results already allow us to recommend a broader use of hydroxyurea for pediatric patients with SCD in Haiti. Disclosures Alvarez: Forma Therapeutics: Consultancy; Novartis: Consultancy.

scholarly journals Risk of Stroke in Children with Sickle Cell Disease after Splenectomy

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4930-4930
Author(s):  
Peter B. Soh ◽  
Abdul H. Siddiqui
Keyword(s):  
Blood Flow ◽  
Cerebral Blood Flow ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Cerebral Blood Flow Velocity ◽  
T Test ◽  
Control Group ◽  
Blood Transfusions ◽  
Cell Disease ◽  
The Mean

Abstract Background Stroke and splenic sequestration are known complications associated with sickle cell disease in children. The cerebral blood flow velocity measured by transcranial doppler ultrasonography (TCD) is a screening tool to identify patients who are at increased risk of stroke. Children with critical time-average maximum velocity (TAMV) are started on chronic blood transfusion program to prevent stroke. Iron overload, inhibitor development and risk of infection are some of the complications associated with chronic blood transfusions. The objective of this study was to assess the changes in cerebral blood flow and the need for chronic blood transfusions after splenectomy performed in children with splenic sequestration. Methods A retrospective chart review of sickle cell disease children splenectomized between 1999 and 2014 at the University of South Alabama was performed. In addition to demographic data, results of routine screening TCDs for up to 15 years were obtained and compared to pre-splenectomy TCDs by using the paired t-test. We also compared the TAMV and blood counts in splenectomized and non-splenectomized patients of the same sickle cell disease variant that were age- and sex-matched. The cases were also compared to a non-splenectomized cohort control group comprising of children with sickle cell disease using the independent t-test. Results A total of 40 patients (36 with Hemoglobin SS disease and 4 with S-Beta Thalassemia Zero) received splenectomy during the study period at a mean age of 2.5 years (1.5 to 18 years). The mean TAMV before splenectomy was 129 cm/sec, which increased to 157 cm/sec and decreased back to 137 cm/sec at two and five years post-splenectomy, respectively. When comparing these changes using the paired t-test, the difference was not statistically significant. Four (10%) of these patients needed to be started on chronic blood transfusions due to critical TCDs. Another patient had a critical TCD before splenectomy and was continued on chronic transfusions post-splenectomy. The mean TAMV in our sickle cell patients without splenectomy at age 14 years was 123 cm/sec (n=107). Five (4.7%) of these patients were receiving chronic blood transfusions for critical TCDs. The odds ratio of having a critical TCD in splenectomized patients versus non-splenectomized patients was 2.3:1. The TAMV in patients at 2 years post-splenectomy was 157 cm/sec compared to 139 cm/sec in age- and sex-matched non-splenectomized controls (p = 0.035). The mean platelet count prior to splenectomy was 301/m3. It increased to 536/m3 one year post-splenectomy and remained elevated at 510/m3 four years post-splenectomy. The differences in platelet counts were statistically significant. The mean platelet count in non-splenectomized patients at 14 years of age was 387/m3. Prevalence of treatment with Hydroxyurea was 45% (18 patients) in splenectomized patients and 18% (19 patients) in the non-splenectomized control group. Conclusion We conclude that the mean cerebral blood flow velocity was increased after splenectomy. When comparing cases to matched controls, the biggest difference in TAMVs was found at two years post-splenectomy. Splenectomy is effective in preventing life threatening sequestration events. However, in our patient cohort, the risk of requiring chronic blood transfusions for a critical TCD (≥200 cm/s) doubled after splenectomy. Disclosures No relevant conflicts of interest to declare.

scholarly journals Sickle cell disease and the adherence to guidelines for the use of blood transfusions in Duhok, Kurdistan, Iraq

2021 ◽  
Vol 25 (1) ◽  
pp. 486-492
Author(s):  
Fahad Jameel ◽  
Adnan Sadeeq
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Sickle Cell ◽  
Cross Sectional Study ◽  
Beta Thalassemia ◽  
Blood Transfusions ◽  
Cell Disease ◽  
Cross Sectional ◽  
Medical Visits ◽  
Transfusion Guidelines

Background and objective: One of the important health problems in Duhok city, Iraqi Kurdistan Region, is sickle cell disease. Blood transfusion remains a significant therapeutic intervention in patients with sickle cell disease that reduces complications related to vaso-occlusions. This study aimed to assess compliance to guidelines for the use of blood transfusionsin Duhok, Kurdistan, Iraq. Methods: This is a cross-sectional study that included 135 patients with sickle cell disease registered at Jeen center of pediatric hematological diseases in Duhok, Kurdistan, Iraq. Between April 1st and July 31st, 2019, 205 medical visits of sickle cell disease were registered. Every patient was evaluated to record the clinical setting and explanations behind visiting and indications for transfusion. Results: Of 135 patients,65.9% had sickle cell anemia (HbSS disease), 33.3% had sickle beta thalassemia, and one patient (0.7%) had Sickle/D disease. A total of 205 medical visits of sickle cell disease were registered with 84 blood transfusion decisions. The most common indicated guideline reasons for transfusion were symptomatic anemia and acute hemolytic crisis with a drop of hemoglobin >2 g/dl below steady state hemoglobin and severe painful crisis only accounted for 38.1%. Conclusion: In this study of patients with sickle cell disease, most blood transfusions were not indicated according to the transfusion guidelines (British Committee for Standards in Haematology – BCSH, 2017). Keywords: Transfusion guidelines for sickle cell disease (BCSH 2017); Sickle cell disease; Iraq.

scholarly journals Prevalence and Predisposing Factors of Atrial Fibrillation in a Multi-Ethnic Society: The Impact of Racial Differences in Bahrain

2011 ◽  
Vol 4 ◽  
pp. OJCS.S8032 ◽  
Author(s):  
Taysir Garadah ◽  
Saleh Gabani ◽  
Mohamed Al Alawi ◽  
Ahmed Abu-Taleb
Keyword(s):  
Atrial Fibrillation ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Middle Eastern ◽  
Predisposing Factors ◽  
Cell Disease ◽  
Clinical Events ◽  
History Of ◽  
The Mean ◽  
One Year

Background The prevalence and epidemiological data of atrial fibrillation (AF) among multi-ethnic populations is less well studied worldwide. Aim Evaluation of the prevalence and predisposing factors of AF in patients who were admitted to acute medical emergencies (ER) in Bahrain over the period of one year. Methods Two hundred and fifty three patients with onset of AF were studied. The mean difference of biochemical data and clinical characteristics between Middle Eastern (ME) and sub continental (SC) patients was evaluated. The odds ratio of different predisposing factors for the development of clinical events in AF patients was assessed using multiple logistic regression analysis. Results Out of 7,450 patients that were admitted to ER over one year, 253 had AF based on twelve leads Electrocardiogram (ECG), with prevalence of 3.4%. In the whole study, the mean age was 59.45 ± 18.27 years, with 164 (65%) male. There were 150 ME patients (59%), and 107 (41%) SC, 55 (22%) were Indian (IND) and 48 (19%) were South Asian (SA). In the whole study clinical presentation was of 48% for palpitation, pulmonary edema was of 14%, angina pectoris on rest of 12%, 10% had embolic phenomena, 6% had dizziness, and 7% were asymptomatic. The odds ratio of different variables for occurrence of clinical events in the study was positive of 2.2 for history of hypertension, 1.8 for sickle cell disease, 1.2 for high body mass index (BMI) >30, 1.1 for mitral valve disease. The ME patients, compared with SC, were older, had significantly higher body mass index, higher history of rheumatic valve disease, sickle cell disease with high level of uric acid and lower hemoglobin. The history of hypertension, DM and smoking was higher among the SC patients. The rate of thyroid disease was equal in both groups. Conclusion The prevalence of atrial fibrillation was 3.4% with male predominance of 65%. Patients of sub continental origin were younger with a significantly high history of hypertension and ischemic heart disease. The patients of Middle Eastern origin had significantly high rate of rheumatic heart disease, and sickle cell disease. The history of hypertension was the most important independent clinical predictor of adverse events in patients presented with AF.

scholarly journals A case of severe multi-system disease in a sickle cell SC patient caused by parvovirus B19 infection

2021 ◽  
Vol 2 (5) ◽  
Author(s):  
Mohamed Almuqamam ◽  
◽  
Swetha Madhavarapu ◽  
Nataly Apollonsky ◽  
◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Parvovirus B19 ◽  
Acute Infection ◽  
Organ Injury ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Splenic Sequestration ◽  
Increased Risk ◽  
Parvovirus B19 Infection

Sickle Cell Disease (SCD) is an inherited hemoglobinopathy, which results in production of abnormal hemoglobin S. HbSC disease is a variant of SCD, which shares a similar clinical complication profile to HbSS disease, but often thought to be a milder condition. In patients with SCD, Hb S in deoxygenated state undergoes polymerization, leading to hemolysis, vaso-occlusive events, and eventually end-organ damage. Among other complications in patients with SCD is increased risk of complications caused by parvovirus B19. We present a case of a 14-year-old female with HbSC disease who presented to the emergency room with complaint of abdominal pain and found to have splenic sequestration. Splenic sequestration progressed rapidly, Hemoglobin (hb) dropped to 4.6 g/dl and acute chest syndrome (ACS) developed. She was treated following the ACS protocol, received 4 units of Packed Red Blood Cells (PRBC) and subsequently underwent a single volume PRBC exchange transfusion. Considering her unusual presentation, with severe ARDS from alveolar hemorrhage requiring mechanical ventilation and multi-organ injury, several autoimmune and infectious conditions with a cytokine storm component including COVID-19 disease, were considered. Results of viral testing revealed parvovirus B19 IgM antibodies signifying an acute infection. She fully recovered with supportive care and was discharged home. Multisystem involvement simulating connective tissue disorders or malignancies with acute parvovirus B19 infection has been reported and is considered extremely rare. To our knowledge, there were no reports of pediatric patients with SC disease presenting with splenic sequestration and ACS in the setting of parvovirus B19 multisystem disease. Keywords: sickle cell disease; acute respiratory distress syndrome; acute chest syndrome; parvovirus B19.

scholarly journals Ischemic Stroke in Children and Young Adults with Sickle Cell Disease (SCD) in the Post-STOP Era

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 68-68 ◽  
Author(s):  
Janet L. Kwiatkowski ◽  
Julie Kanter ◽  
Heather J. Fullerton ◽  
Jenifer Voeks ◽  
Ellen Debenham ◽  
...  
Keyword(s):  
Ischemic Stroke ◽  
High Risk ◽  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Stroke Prevention ◽  
Cell Disease ◽  
Clinical Series ◽  
The Mean

Abstract Background: The Stroke Prevention Trial in Sickle Cell Anemia (STOP) and Optimizing Primary Stroke Prevention in Sickle Cell Anemia (STOP 2) established routine transcranial Doppler ultrasound (TCD) screening with indefinite chronic red cell transfusions (CRCT) for children with abnormal TCD as standard of care. To identify children at high-risk of stroke, annual TCD screening is recommended from ages 2 to 16 years, with more frequent monitoring if the result is not normal. A reduction in stroke incidence in children with SCD has been reported in several clinical series and analyses utilizing large hospital databases when comparing rates before and after the publication of the STOP study in 1998. We sought to determine the rate of first ischemic stroke in a multicenter cohort of children who had previously participated in the STOP and/or STOP 2 trials and to determine whether these strokes were screening or treatment failures. Subjects and Methods: Between 1995 and 2005, STOP and STOP 2 (STOP/2) were conducted at 26 sites in the US and Canada. These studies included 3,835 children, ages 2 to 16 y with SCD type SS or S-beta-0-thalassemia. Participation in STOP/2 ranged from a single screening TCD to randomization. STOP 2 also had an observational arm for children on CRCT for abnormal TCD whose TCD had not reverted to normal. The Post-STOP study was designed to follow-up the outcomes of children who participated in one or both trials. 19 of the 26 original study sites participated in Post-STOP, contributing a total of 3,539 (92%) of the STOP/2 subjects. After exit from STOP/2, these children received TCD screening and treatment according to local practices. Data abstractors visited each clinical site and obtained retrospective data from STOP/2 study exit to 2012-2014 (depending on site) including follow-up TCD and brain imaging results, clinical information, and laboratory results. Two vascular neurologists, blinded to STOP/2 status and prior TCD and neuroimaging results, reviewed source records to confirm all ischemic strokes, defined as a symptomatic cerebral infarction; discordant opinions were resolved through discussion. For the first Post-STOP ischemic stroke, prior TCD result and treatment history subsequently were analyzed. Results: Of the 3,539 subjects, follow-up data were available for 2,850 (81%). Twelve children who had a stroke during STOP or STOP2 were excluded from these analyses resulting in data on 2,838 subjects. The mean age at the start of Post-STOP was 10.5 y and mean duration of follow-up after exiting STOP/2 was 9.1 y. A total of 69 first ischemic strokes occurred in the Post-STOP observation period (incidence 0.27 per 100 pt years). The mean age at time of stroke was 14.4±6.2 (median 13.8, range 3.5-28.9) y. Twenty-five of the 69 patients (36%) had documented abnormal TCD (STOP/2 or Post-STOP) prior to the stroke; 15 (60%) were receiving CRCT and 9 (36%) were not (treatment data not available for 1 subject). Among the 44 subjects without documented abnormal TCD, 29 (66%) had not had TCD re-screen in the Post-STOP period prior to the event; 7 of these 29 (24%) were 16 y or older at the start of Post-STOP, which is beyond the recommended screening age. Four of the 44 (9%) patients had inadequate TCD in Post-STOP (1 to 10.7 y prior to event). Six (14%) had normal TCD more than a year before the event (1.2 - 4 y); all but one of these children were younger than 16 y at the time of that TCD. Only 5 (11%) had a documented normal TCD less than 1 year prior to the event. Conclusions: In the Post-STOP era, the rate of first ischemic stroke was substantially lower than that reported in the Cooperative Study of Sickle Cell Disease, prior to implementation of TCD screening. Many (39%) of the Post-STOP ischemic strokes were associated with a failure to re-screen according to current guidelines, while only 11% occurred in children who had had recent low-risk TCD. Among those known to be at high risk prior to stroke, treatment refusal or inadequate treatment may have contributed. While TCD screening and treatment are effective at reducing ischemic stroke in clinical practice, significant gaps in screening and treatment, even at sites experienced in the STOP protocol, remain to be addressed. Closing these gaps should provide yet further reduction of ischemic stroke in SCD. Disclosures No relevant conflicts of interest to declare.

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