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  • 1
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 4935-4935
    Abstract: Introduction: Genetic counseling in sickle cell disease (SCD) allows to inform people about the genetic condition and to make informed decisions about screening and treatment opportunities. With improvement of care and follow-up of SCD and in order to provide adequate genetic counseling, the current study compared the clinical presentation of patients with hemoglobin SC disease (SC) and sickle cell anemia (SS) or SBeta°-thalassemia (Sβ°). Methods: The Belgian SCD Registry currently includes patients followed in 8 centers. Data was retrieved retrospectively from their medical charts until 2008. From 2008 till December 2012, all data (known and new cases) was prospectively entered. Data was collected either from birth, for patients diagnosed by means of the neonatal screening program (NSP) or from diagnosis following the first contact in a center and consistently until the last follow-up (FU) visit, or death. Data included genotype, demography, method and date of diagnosis, SCD-related complications, biological parameters, radiological results, treatment and hospitalizations. Results: Among the 469 patients recorded, 36 (8%) were SC. Their demographic and outcome data are given in Table 1, subgroups of SC patients detected at birth or not are given in Table 2. FU was 292 and 4749 patient-years (PY) for SC and SS/Sβ° groups respectively. Median age at diagnosis for SC and SS/Sβ° subgroups not detected at birth was 6.7 and 2.6 y. The first vaso-occlusive crisis (VOC) in SC patients occurred earlier in neonatal screened patients (5y vs 17y; P=0.004). Osteonecrosis was observed only in 6 patients diagnosed at birth but was significantly more frequent in SC individuals (P=0.02). Among the 6 SC patients treated with hydroxyurea (HU), indications for treatment were recurrent VOC (n=4), proteinuria (n=1) and osteonecrosis (n=1). Hospitalization days per 100 PY were 123 for the SC NSP cohort and 373 for the SS/Sβ° NSP cohort, with total hospitalization days significantly lower in SC patients (160 vs. 4059 days ; P=0.03). Discussion: SC patients represent 8 % of the whole cohort but asymptomatic and undiagnosed patients probably exist. Overall they are much less represented than in other Western series. They are significantly less affected by VOC, acute chest syndrome (ACS), severe anemia than SS/Sβ° patients but did not differ for occurrence of retinopathy or osteonecrosis. Several published studies confirmed these data but showed also others complications such as severe infection, stroke and death. Our data doesn’t support any impact of NSP on occurrence of major complications for SC disease. Retinopathy incidence that increases with age is as expected lower in the NSP group (younger patients at last FU when compared to no NSP group). Older age at first VOC in the no NSP group results probably from previously undiagnosed mild disease. Nevertheless several SC patients were more severely affected requiring HU treatment. Limitations of our work are linked to the small size of our SC cohort, the nature of the partial retrospective study and the unknown number of SC patients not requiring care in a specific program. In conclusion, SC patients have a lower incidence of clinical events than SS/Sβ°, excepted for osteonecrosis and retinopathy equally observed in both groups. Our data support the need of early ophthalmological FU and special awareness to detect early osteonecrosis. These Belgian data may support a dedicated genetic counseling for SC patients and affected families. Table 1. SC and SS/S β 0 patients: demographic and outcome SC SS/Sβ0 P Number of patients 36 (8%) 423 (90,2%) Male 14 (39%) 201 (48%) NS NSP 19 (53%) 142 (34%) Median age at diagnosis years (range) 0 (0-35) 1 (0-29) NS Median age at last FU years (range) 9,9 (1,4-44) 13 (1-53) NS Severe anemia 〈 60g/L 3 (8%) 208 (49%) 〈 0,01 ACS 1 (2,8%) 119 (28%) 〈 0,01 VOC 11 (31%) 253 (60%) 〈 0,05 Stroke 0 17 (4%) NS Severe infection 0 35 (8%) NS Osteonecrosis 4 (11%) 36 (8,5%) NS Retinopathy 4 (11%) 25 (6%) NS HU treatment 6 (17%) 179 (42%) 〈 0,05 Deaths 0 13 (3%) NS Table 2. SC patients from NSP vs no NSP: demographic and outcome NSP no NSP P Number of patients 19 (53%) 17 (47%) Male 10 (53%) 4 (24%) NS Median age at last FU years (range) 6 (1-14) 14 (4-44) NS Severe anemia 〈 60g/L 2 (10,5%) 1 (6%) NS ACS 0 1 (6%) NS VOC 5 (26%) 6 (35%) NS Osteonecrosis 3 (16%) 1 (6%) NS Retinopathy 0 4 (23,5%) 0,04 Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
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  • 2
    In: Blood, American Society of Hematology, Vol. 122, No. 21 ( 2013-11-15), p. 2231-2231
    Abstract: In Western countries, mortality among patients with sickle cell disease (SCD) has decreased in the last decades by means of neonatal screening (NS), infectious prophylaxis and care improvements. The major causes of death in children include acute chest syndrome, sepsis, splenic sequestration, stroke, aplastic crisis while in adults end-stage organ failure contributes also to premature death. Hydroxyurea (HU) and stem cell transplantation (SCT) are used in Belgium for more than 20 years but their possible influences on survival have not been yet analyzed. The Belgian SCD Registry was created in 2008 including patients of 8 centres. All available data in 2008 were retrospectively encoded in the database. After 2008 and until 2012, all data were recorded prospectively for already registered patients as well as newly diagnosed subjects. Data were registered from NS or from diagnosis (first contact) until last follow up (FU) visit, SCT or death. Data included diagnosis, demography and outcome data. After SCT, only vital status and cause of death were recorded. Up to date, data from 470 pts are recorded (224 males), 412 are HbSS, 14 HbSβ0, 7 HbSβ+ and 37 HbSC. The median age at diagnosis and at last FU was respectively 0.7 year (y) and 9.9 y. The FU for the whole cohort was 3810 patient-years (PY) and with 136 patients aged over 18y, their FU during adulthood accounted for 520 PY. Thirteen patients died (2.8%). The mortality per 100-PY was 0.34 and the median age at death was 14.5 y (range, 1.5-23.7 y). All deaths occurred in HbSS patients, 5 after SCT and 8 due to an acute event. Complete data set is missing for 3 of the 8 patients. For the 5 well documented SCD related deaths, causes were: hemorrhagic stroke (2), sepsis due to S. pneumoniae (1), aplastic crisis (1) and infection during stay in homeland (1). At last FU, 91 patients were transplanted, 182 were on HU, 7 on HU + chronic transfusion (CT), 19 on CT (4 after HU treatment). The remaining 171 patients never had disease modifying therapy (DMT). Compared with the latter, mortality rate for those on HU was significantly reduced (0.1 vs 0.5/100-PY) while patients on HU have longer FU and are older at last FU (Table 1). Among 91 patients transplanted at a median age 6.9 y, 5 died: 3 from acute transplant related toxicity, 1 from secondary acute myeloblastic leukemia after cGVHD, and 1 is unexplained more than 7 years post SCT. The data issued from the most recent NS cohorts report a low death rate during childhood ranging from 0.13 to 0.52. Even if our Belgian cohort is not exclusively issued from neonatal diagnosis, the observed death rate is low (0.34/100-PY). Several methodological biases are present in this partially retrospective study (incomplete or unavailable data, lost of FU, no information if death occurred before the first contact in a center, …). Nevertheless our low mortality is not underestimated since 1) most patients were followed since infancy and during a long period (3810 PY); 2) the FU during adulthood (period of increased mortality) accounted for 520 PY; 3) our cohort represents a very large part of the Belgian SCD population since a national inquiry performed in 2007 estimated the whole SCD population to 500. The effect of HU on mortality has been reported in adults and more recently in children. Despite longer FU and older age at last FU, our data confirms those previously results With only one case, death by infection is rare while SCT complications contributed to about 40 % of deaths. Even if SCT is the only curative option for SCD, it encompasses a risk of mortality. As life expectancy of SCD patients has been extended which is confirmed by our results (especially for patients on HU), SCT should be reserved for clinically severe cases. Population-based prospective studies evaluating the survival in transplanted and non transplanted patients are needed. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2013
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  • 3
    In: The Lancet, Elsevier BV, Vol. 398, No. 10304 ( 2021-09), p. 957-980
    Type of Medium: Online Resource
    ISSN: 0140-6736
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    Language: English
    Publisher: Elsevier BV
    Publication Date: 2021
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  • 4
    In: The Lancet, Elsevier BV, Vol. 390, No. 10113 ( 2017-12), p. 2627-2642
    Type of Medium: Online Resource
    ISSN: 0140-6736
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    Language: English
    Publisher: Elsevier BV
    Publication Date: 2017
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  • 5
    In: Blood, American Society of Hematology, Vol. 120, No. 21 ( 2012-11-16), p. 2107-2107
    Abstract: Abstract 2107 The 6-minute walk test (6MWT) evaluates the sub-maximal functional exercise capacity and can be used together with the tricuspid regurgitant jet velocity (TRV) and pro-BNP to screen pulmonary hypertension in adults with sickle cell disease (SCD). A reduced 6-minute walk distance (6MWD) is observed in adults with SCD with chronic pain, hip avascular necrosis and osteopenia. In children with SCD, baseline elevated TRV is associated with a decline in age-standardized 6MWD. The aim of our study is to explore the submaximal exercise capacity of children with SCD followed at the Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium and to analyze the factors affecting the 6MWT and the 6MWD. Since September 2011, all patients with SCD above 6 years of age were screened with the 6MWT as part of their follow-up in order to test if their functional capacity was altered. The age-standardized predicted value of the 6MWD was established as reported by Geiger. The 6MWT was considered as normal if the 6MWD was more than 80% of the age-standardized predicted value, moderately decreased between 60–80%, and severely altered less than 60%. Baseline hematological values, clinical events, cerebro-vascular disease, cardio-pulmonary parameters and disease-modifying treatment (DMT) were compared between those with normal and abnormal 6MWT and according to the 6MWD. Forty-six patients (20 boys and 26 girls) with a median age of 12 yrs were investigated. Forty-three were HbSS or HbSβ°, 2 HbSC and 1 HbSβ+. Thirty-two patients had a normal 6MWT and 14 an abnormal 6MWT. Only one patient had a severely altered test. These 2 groups were similar for age, sex, genotype and history of vaso-occlusive crisis or acute chest syndrome (ACS) as well as for the number of patients receiving DMT (either hydroxyurea (HU) or chronic transfusion). The proportion of patients with normal, conditional or abnormal transcranial doppler was also similar in both groups. Silent infarct (SI) on routine cerebral magnetic resonance imaging was found in 42.9% in the group with abnormal 6MWT versus only 19.4% in the group with normal 6MWT (p= 0.087). Pulmonary functional test, blood pressure, heart rate, systolic function and TRV were identical in both groups and only one patient had TRV 〉 2.5m/sec. Baseline pulse oxymetry was slightly but significantly decreased in patients with abnormal 6MWT (98 versus 100%; p=0.022). Biological parameters were not statistically different between both groups. The 6MWD was not modified according to Hb, MCV, HbF, LDH and reticulocytes count or previous history of clinical event, except for the presence of SI (Table 1). Patients with or without SI were similar for age, sex, previous ACS or painful crisis as well as for hemolytic parameters (LDH: 945 versus 825 UI/l, p=0.832; reticulocytes: 273 versus 329 × 103/μl, p=0.548) and basal Hb (9.7 versus 8.8 g/dl, p=0.06). However patients without SI had significantly higher HbF and MCV values, and lower PMN count reflecting that most of them were treated with HU. In this cross-sectional study, the majority of children with SCD have a normal 6MWT. Abnormal 6MWT was not predicted by any clinical or biological features despite a trend to more SI in the group of children with abnormal test. In this series with only one high TRV patient, the sole factor which influences the 6MWD is the presence of SI. The lower exercise capacity of children with SCD with silent stroke may reflect some subclinical motor or sensitive impairment. Our data suggest also that HU might prevent SI which needs to be confirmed by larger prospective studies. Table 1. 6-minute walk distance (6MWD) in 46 SCD children according to their biological values and clinical complications Mean 6MWD in meters (SD) p value Mean Age in years (SD) p value Hemoglobin (g/dl) · ≥ 9 (N = 24) 531.5 (95.4) 0.173 11.2 (2.8) 〈 0.001 · 〈 9 (N = 22) 569.8 (92.2) 14.5 (2.7) MCV (fL) · ≥ 90 (N = 24) 536.1 (100.7) 0.251 12.6 (3.5) 0.518 · 〈 90 (N = 22) 568.3 (86.8) 13.2 (2.8) HbF (%)* · ≥ 10% (N = 30) 544.0 (101.7) 0.360 13.2 (3.5) 0.352 · 〈 10% (N = 15) 570.1 (81.8) 12.6 (2.4) LDH (UI/l) · ≥ 1000 (N = 14) 526.8 (86.5) 0.229 11.8 (3.0) 0.105 · 〈 1000 (N = 32) 562.3 (97.4) 13.4 (3.1) Previous ACS* · Yes (N = 38) 548.6 (98.3) 0.625 13.5 (3.1) 0.453 · No (N = 7) 566.9 (85.5) 12.5 (4.2) Silent Infarct · Yes (N = 12) 502.5 (113.9) 0.035 12.1 (2.2) 0.374 · No (N = 34) 568.9 (82.0) 13.2 (3.4) * Missed information for 1 patient. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2012
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  • 6
    In: Blood, American Society of Hematology, Vol. 124, No. 21 ( 2014-12-06), p. 4091-4091
    Abstract: Chronic transfusion in sickle cell disease (SCD) remains the gold standard therapy for primary and secondary stroke prevention and is indicated for patients with recurrent severe vaso occlusive crisis (VOC) or acute chest syndrome (ACS). Automatized apheresis (AA) has several advantages compared to manual exchange transfusion (MET): shorter procedure, continuous control of fluid balance, etc. The aim of our study was to assess the safety and efficacy of AA in SCD patients previously treated with MET at Hôpital Universitaire des Enfants Reine Fabiola, Brussels, Belgium and to evaluate the change of the costs related to transfusion and chelation overtime. From January 2012, the AA program for SCD patients started in our institution. Patients on chronic transfusion program (CTP) and previously treated with MET were eligible to switch to AA if sufficient peripheral venous access to allow AA without the use of central venous line and if weight ≥ 30kg. On CTP, target HbS was 〈 30% in case of stroke risk and 〈 50% for other indications. Data on biological values, duration of the procedure, intervals between procedures as well as adverse events were recorded for the last 6 months on MET and compared to the data on AA. The overall costs of last year on MET, 1st year and 2nd year on AA were analysed. The cost of packed red blood cell (RBC), one-day care facility, apheresis kit and chelation were recorded. For patients on AA for less than 2 years, costs for the 2nd year were extrapolated taking into account the data from the 6 previous months. Data were collected for this analysis until June 2014. Friedman test was used to compare treatment across years and Dunn's Multiple Comparison Test to compare each year of treatment among them. Ten patients switched from MET to AA at a median age of 11.8 years (range, 9.6-16.8y). The median duration of MET before start of AA was 1.9 years (range 0.5-4.4y). The median duration of AA was 1.7 years (range 1-2.4y). Four patients are on AA for 〉 2 years, 4 ≥ 20 months and 2 〉 than 12 months. Indications for CTP were overt stroke (2), pulmonary hypertension (2), recurrent VOC/ACS (5) and poorly tolerated severe anemia (1). All patients remained stable without any SCD related event, except one child who presented seizures without evidence of new stroke and for whom anticonvulsivant therapy was resumed. HbS level remained in the target values for all patients despite a slightly but significantly higher value on AA. The ferritin level and the duration of the procedure decreased significantly (Table 1). The 2 patients on iron chelation stopped it after 10 and 1 AA procedures. Interval between 2 AA was significantly longer than on MET (P 〈 0.0001). On 181 procedures, 9 adverse events (4.9%) required medical intervention: transient hypotension (1), symptomatic hypocalcemia (2), transient headache (2), fever (1), nausea-vomiting (1) and abdominal pain (2). On AA, the requirement of packed RBC was significantly higher than on MET. During the 1st year, costs of AA were significantly higher than MET (132937€ vs.107560€; P=0.01). Nevertheless, during the 2nd year of treatment, the costs of AA were not significantly different from those on MET (102965€ vs. 107560€). Indeed chelation could be stopped in patients previously treated. AA is useful and safe for SCD patients requiring exchange transfusion program. It is less time consuming for nurses and patients, improves iron overload and interval between 2 procedures is significantly reduced. Despite higher costs related to the increase packed RBC requirement, the costs of AA and MET in the Belgian Health Care System are the same as chelation could be stopped in previously treated patients. Abstract 4091. Table 1.Changes in age, weight, biological data and procedure parameters on MET and on AA On METOn AAP value 1st year 2nd yearMedianRangeMedianRangeMedianRangeMedian age (years)11,89,7-16,812,810,8-17,713,311,8-18,6 〈 0,0001Median weight (kg)45,530,4-66,349,933,8-72,05336,1-76,0 〈 0,0001Median height (cm)153,5138-178161143-180165145-182 〈 0,0001Hb (g/L)9,958,6-10,89,958,8-10,6109,2-11,7NSHb S (%)33,525-424028,5-424631-480,0002Ferritin (µg/L)666182-151225552-8111489-622 〈 0,001Duration of procedure (min)245195-36087,375,5-1269164-1540,0002Interval between procedures (d)2821-293428-35,54228-42 〈 0,0001Packed RBC requirement (ml/kg)18,315,1-2032,227,4-36,13026,8-36 〈 0,0001Packed RBC requirement (unit)39,515-796749-12065,538-137 〈 0,0001 Disclosures No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2014
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  • 7
    In: Blood, American Society of Hematology, Vol. 116, No. 21 ( 2010-11-19), p. 4821-4821
    Abstract: Abstract 4821 Background: Red cell exchange transfusion is frequently used in the management of patients with sickle cell disease either electively or therapeutically to maintain an hemoglobin S (Hb S) level 〈 30–50%. This target is often difficult to maintain. In order to assess the effects of chronic partial exchange transfusion (CPET) a) on level of Hb and Hb S, b) on iron overload c) the need for chelation, d) on risk of long term adverse events and e) clinical outcome, we analyzed the data of sickle cell disease patients treated by long term CPET in our center. Methods/subjects: In the cohort of 163 SCD patients followed at University Children's Hospital at Brussels (Belgium), 10 benefit from CPET. Main reasons for CPET were neurologic disease (4), frequent ACS (3), previous severe hepatic cholestasis (2), leg ulcer (1) and pulmonary hypertension (1). The median age at start of treatment was 13 years (range 4 –19). These patients (6 males and 4 females) account for 248 exchanges during a median follow-up of 20 months (range 6– 36). These exchanges are until now performed manually and the volume exchanged is calculated taking into account the Hb level and the last HbS percentage. It is usually between 30 and 40 ml/kg BW. Except if severe anemia occurs, the goal of these exchanges is to keep a constant hematocrit level. All patients had a full red cell phenotype performed and received blood matched for ABO, Rhesus, Kell and Duffy antigens systems. The estimation of iron balance (iron intake- iron removed) was calculated yearly. Results: The pre-exchange Hb value was 9.5 g/dl (median; range: 7.7–10.9 g/dl) and the mean post value was 9.4/dl (range: 8.4– 11.1 g/dl). These values are not statistically different (p 〉 0.05). The majority of patients (9/10) are reached an HbS 〈 50% when measured 3–5 weeks after PET (just before the next procedure) with a median HbS value of 40% (range: 30–54). At start of CPET program, the median ferritin level was 439 ng/ml (range: 80 – 1704). Five patients had already a ferritin 〉 500 ng/mL due to numerous previous transfusions. At last evaluation, the median ferritin did not change significantly and was 531 ng/ml (range 84– 3840). The two patients with ferritin higher than 1000 ng/ml start chelation with good result for one. One The mean annual net RBC load were 1.72 ml RBC/kg/yr provided approximately 1.85 mg of iron/kg/yr. Individual data are given in table 1. CPET-treated patients exchanged showed clinical improvement with disappearance of SCD crisis and related complications. The procedure was well tolerated by most patients, and adverse effects were limited to mild hypotension (3/10). No autoimmune hemolysis or allo-immunisation was documented in this cohort. All children remained negative for HIV and hepatitis C virus infections. Conclusion: Manual CPET seems to be safe to prevent middle-term iron overload and the need of elation therapy in most of patients. CPET can therefore be recommended for SCD patients who required decreased in Hb S levels either prophylactically or therapeutically. Manual are safe, effective and easy to use when mechanized exchanges are not possible for technical reasons. Disclosures: No relevant conflicts of interest to declare.
    Type of Medium: Online Resource
    ISSN: 0006-4971 , 1528-0020
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    Publisher: American Society of Hematology
    Publication Date: 2010
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  • 8
    In: British Journal of Haematology, Wiley, Vol. 165, No. 3 ( 2014-05), p. 402-408
    Abstract: Despite improvements in medical management, sickle cell disease ( SCD ) remains associated with severe morbidity and decreased survival. Allogeneic haematopoietic stem cell transplantation ( HSCT ) remains the only curative approach. We report the outcome of 50 consecutive children with severe SCD that received HSCT in our unit between N ovember 1988 and A pril 2013. The stem cell source was bone marrow ( n  = 39), cord blood ( n  = 3), bone marrow and cord blood ( n  = 7) and peripheral blood stem cells ( n  = 1). All patients had ≥1 severe manifestation: 37 presented with recurrent vaso‐occlusive crises/acute chest syndrome, 27 cerebral vasculopathy and 1 nephropathy. The conditioning regimen consisted of busulfan + cyclophosphamide ( B u C y) before November 1991 and B u C y + rabbit antithymocyte globulin after that date. Since 1995, all patients have been treated with hydroxycarbamide ( HC ) prior to transplantation for a median duration of 2·7 years. Median age at transplantation and median follow‐up was 8·3 and 7·7 years, respectively. Acute graft‐versus‐host disease ( GVHD ) and chronic GVHD were observed in 11 and 10 patients, respectively. An excellent outcome was achieved, with 8‐year overall survival and event‐free survival ( EFS ) rates of 94·1% and 85·6%, respectively. Since HC introduction, no graft failure occurred and EFS reached 97·4%. Prior treatment with HC may have contributed to successful engraftment.
    Type of Medium: Online Resource
    ISSN: 0007-1048 , 1365-2141
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    Publisher: Wiley
    Publication Date: 2014
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  • 9
    In: The Lancet, Elsevier BV, Vol. 403, No. 10431 ( 2024-03), p. 1027-1050
    Type of Medium: Online Resource
    ISSN: 0140-6736
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    Publisher: Elsevier BV
    Publication Date: 2024
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  • 10
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    American Society of Hematology ; 2010
    In:  Blood Vol. 116, No. 21 ( 2010-11-19), p. 4807-4807
    In: Blood, American Society of Hematology, Vol. 116, No. 21 ( 2010-11-19), p. 4807-4807
    Abstract: Abstract 4807 Background: Pica defined as a compulsive and persistent intake of inedible substances or atypical food combinations at least one month. Association of pica and sickle cell disease (SCD) are poorly documented. Alerted by parents, we decided to verify systematically a) the occurrence of pica in SCD children b) to determine its duration, c) to identify the substances commonly ingested and d) to determine the characteristics among children and adolescents with SCD who reported practicing pica. Methods: SCD patients were seen in the outpatient clinic at the University Children's Hospital Queen Fabiola at Brussels between May 1, 2010 and July 30, 2010 as part of their regular follow-up. Data on sex, age, weight, height, body mass index, sickle cell phenotype, G6PD deficiency as well as biological data such as hematocrit mean corpuscular volume, fetal hemoglobin, plasma iron, zinc, copper, lead levels were recorded from their medical chart. Parents and patients were interviewed for the presence of eating disorders. Children and care givers completed questionnaires assessing symptoms of pica. Patients with acute illness, pregnancy, developmental delay, cognitive impairment or age younger than 3 years were excluded. Student t-test was used for difference in mean values groups by pica presence or absence. Fisher exact test was used to compare categorical variable. Difference in means values were considered statistically significant at p 〈 0.05. Results: Fifty-Five patients (24 males and 31 females) with a median age of 8. 9 years (6. 7– 11. 3) were evaluated during the study period. Forty-five patients (81.8%) originate from Central Africa, 10.9% (6/55) from West Africa, 1.8 %(1/55) from respectively East Africa, Italy and 3.6% (2/55) have mixed origin. Fifty –two (94.5%) patients are homozygous for Hb S (Hb SS), 3 are SC and 1 has Sβ° thalassemia. Thirty-one patients (56.4%) reported an history of pica and during the study period 29.1% (16/55) reported practicing pica regularly. The mean age of patient with pica was 7. 4 years significantly lower than non-pica patients (p 〈 0.05). Pica prevalence by substances ingested was 16 for amylophagia, 14 for paper and powder, 13 for geophagia, 7 for pencils and textiles, 5 for foam, 4 for soap, and 3 for hair. In 6 cases, there was a familial history of pica in non SS individuals. Except for the fact that the mean hematocrit was lower in pica patients compared to non-pica ones (p 〈 0.05), all the other biological variables were similar in both groups (p 〉 0.05) (Table 1). Iron status was identical. Lead overload was absent in the entire cohort. Hemolytic phenotype assessed by LDH was identical in both group and mild zinc deficiency was present in both groups. We did not observe difference due to hydroxyurée intake (p 〉 0.05) Conclusion: In our study, pica appeared to have a very high prevalence in SCD children and adolescents. However, its etiology is still unknown. Except lower age and significant lower hematocrit value in pica patients when compared to non-pica ones, no differences in iron status, zinc deficiency or hematological parameters was found. The general health status evaluated by BMI was identical in both groups and the trend to more boys in the pica group is not significant. Further studies are necessary to establish the etiology of pica and its possible consequences on SCD. Disclosures: No relevant conflicts of interest to declare.
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    ISSN: 0006-4971 , 1528-0020
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    Language: English
    Publisher: American Society of Hematology
    Publication Date: 2010
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