Sickle Cell Anaemia Group Research Articles

A Cross-Sectional Study Comparing Placental Characteristics in Pregnancy-Induced Hypertension and Sickle Cell Anaemia.

Placental health plays a critical role in pregnancy outcomes as it serves as the interface between the mother and fetus. High-risk pregnancies, such as those complicated by pregnancy-induced hypertension (PIH) and sickle cell anaemia (SCA), are associated with significant alterations in placental morphology and histopathology, potentially leading to adverse maternal and fetal outcomes. While previous studies have explored placental changes associated with either PIH or SCA, nobody has comparatively analysed these conditions to understand their unique and overlapping effects on placental pathology. This study aimed to compare and contrast the morphological and histopathological changes in the placenta associated with high-risk pregnancies likePIH and SCA. A comparative analysis was conducted using data fromstudies at two different tertiary care centres. The study populations included 100 pregnant women diagnosed with PIH and 56 pregnant women with SCA, alongside a matched control groupof 100 healthy pregnant women for the PIH group and 56 healthy pregnant women for the SCA group. Inclusion criteria were restricted to singleton pregnancies in women aged 18 to 35 years with gestational ages ranging from 28 to 40 weeks. Following delivery, placental specimens were collected, and various parameters such as weight, volume, surface area, number of cotyledons, and umbilical cord attachment were meticulously measured. Histopathological examinations were conducted to identify specific pathological features like infarcts, calcifications, syncytial knots, and fibrin deposition. Data analysis was performed using SPSS software version 25.0 (IBM Corp., Armonk, NY, USA), with a p-value of less than 0.05 considered statistically significant. The study revealed significant differences in placental parameters between the PIH and SCA groups compared to their respective controls. Placental weight, volume, and surface area were significantly reduced in both the PIH and SCA groups, with more pronounced reductions observed in PIH (p < 0.001). The umbilical cord attachment was predominantly marginal in the PIH group (75%), compared to a central attachment in the SCA group (70%), suggesting different patterns of placental development. Histopathological analysis demonstrated a higher incidence of infarcts (65% vs. 30%), calcifications (80% vs. 45%), syncytial knots (90% vs. 50%), and fibrin deposition (70% vs. 35%) in the PIH group compared to the SCA group, indicating more severe placental pathology in PIH. This study offered a comprehensive comparison of placental changes in pregnancies affected by PIH and SCA. It identified both structural abnormalities in terms of placental weight, volume, and surface area and distinctive pathological features like infarctions, calcifications, syncytial knot formation, and fibrin deposits in the placenta. When compared to control groups, these findings strongly suggest that placental dysfunction is a key contributor to the adverse outcomes associated with these high-risk pregnancies.

Academic achievement in Ugandan children with sickle cell anaemia: A cross-sectional study.

Academic achievement in school-age children is crucial for advancing learning goals. Children with sickle cell anaemia (SCA) in Sub-Saharan Africa may be at risk of disease-associated school difficulties. Limited data exist on the academic achievement of children with SCA in the region. This study aimed to assess academic achievement of children with SCA in Uganda compared to siblings without SCA. A cross-sectional study conducted at Mulago Hospital SCA Clinic in Uganda. School-going children (6-12 years) with SCA and age-matched sibling controls without SCA. Academic achievement was tested using the Wide Range Achievement Test, Fourth Edition (WRAT4). Outcome measures were spelling, mathematical computation, word reading, and sentence comprehension by age-normalized Z-scores on the WRAT4 test. Among 68 SCA and 69 control, the mean age (standard deviation) was 9.44 (2.04) and 9.42 (2.02) years and males were 55.9% and 46.4% respectively. Mean haemoglobin was 7.9 (SD 0.89)g/dL in the SCA group versus 12.8 (SD 0.89)g/dL in the controls, (p<0.001). Children with SCA scored lower in spelling, (mean difference [95% confidence interval] - 0.36 [-0.02 to -0.69], p=0.04) and mathematical computation, (mean difference [95% confidence interval] -0.51 [-0.17 to -0.85], p=0.003) than the controls. In the SCA group, lower scores in spelling correlated with age, while males performed better than females in mathematical computation. School-aged children with SCA are at risk of poor performance in spelling and mathematical computation. Our findings support the need for educational evaluation and possible support, especially in these two areas.

Neurofilament light chain: A potential biomarker for cerebrovascular disease in children with sickle cell anaemia.

Cerebrovascular injury frequently occurs in children with sickle cell anaemia (SCA). Limited access to magnetic resonance imaging and angiography (MRI-MRA) in sub-Saharan Africa impedes detection of clinically unapparent cerebrovascular injury. Blood-based brain biomarkers of cerebral infarcts have been identified in non-SCA adults. Using plasma samples from a well-characterized cross-sectional sample of Ugandan children with SCA, we explored relationships between biomarker levels and MRI-detected cerebral infarcts and transcranial Doppler (TCD) arterial velocity. Testing was performed using a 4-plex panel of brain injury biomarkers, including neurofilament light chain (NfL), a central nervous system neuron-specific protein. Mean biomarker levels from the SCA group (n = 81) were similar to those from non-SCA sibling controls (n = 54). Within the SCA group, NfL levels were significantly higher in those with MRI-detected infarcts compared to no infarcts, and higher with elevated TCD velocity versus normal velocity. Elevated NfL remained strongly associated with MRI-detected infarcts after adjusting for sex and age. All non-SCA controls and SCA participants lacking MRI-detected infarcts had low NfL levels. These data suggest potential utility of plasma-based NfL levels to identify children with SCA cerebrovascular injury. Replication and prospective studies are needed to confirm these novel findings and the clinical utility of NfL versus MRI imaging.

Micronutrient levels and haemato-biochemical status of patients with sickle cell anaemia at a tertiary hospital in Abakaliki, south-eastern Nigeria: a cross-sectional study

Introductionnutritional status is an under-studied environmental factor that can impact the phenotypic manifestations of patients with Sickle Cell Anaemia (SCA). This study aimed to define hemato-biochemical parameters and micronutrient status in patients with SCA.Methodsthis was a cross-sectional study of patients with SCA and hemoglobin genotype HBAA controls at a tertiary health facility in Abakaliki, from 2nd December 2020 to 31st March 2021. Plasma micronutrient levels, haemato-biochemical parameters were analyzed and anthropometric measurements obtained from all participants.Resultssixty participants with SCA had 58.3% females (mean age of 24.77±7.39 years) while controls had 50% females (mean age of 26.23 ± 8.44 years). The SCA group had significantly lowered calcium (2.733 ± 1.593 vs 1.846 ± 1.123 mmol/l; p=0.009) and magnesium (19.38 ± 6.37 vs 9.65 ± 1.38 mg/dl; p= < 0.001) levels but higher plasma iron (1.70 ± 0.89 vs 1.06 ± 0.53; p=0.001). Zinc and Copper did not reveal significant differences between the two groups. Chloride ion levels was significantly lower in the SCA patients (107.50 ± 17.42 vs 100.19 ± 12.92; p=0.026) while Alkaline phosphatase (ALP), bilirubin, total white blood cell (WBC) and platelets (PLT) count were higher compared with the HBAA group (255.72 ± 124.52 vs 134.56 ± 39.67; p= <0.001, 46.86 ± 25.03 vs 25.63 ± 18.80; p = 0.001, 13.21± 6.57 vs 6.10 ± 1.35; p= < 0.001 and 369.25 ± 138.11 vs 209.36 ± 47.85; p= <0.001).ConclusionCopper and zinc deficiency was not present in our population of SCA patients but, they had lower plasma calcium and magnesium levels and elevated levels of blilirubin, ALP, WBC and platelets PLT counts. These parameters can be explored in designing better management for patients with SCA

Distinct pattern and prevalence of Plasmodium falciparum dihydropteroate synthase gene mutations in children with sickle cell anaemia and haemoglobin AA in Benin City, Nigeria: the impact of HbAA.

specific mutations on the Plasmodium falciparum dihydropteroate synthase (Pfdhps) gene mediate sulphadoxine/pyrimethamine (SP) resistance and thus, pose a threat to the efficacy of SP-Intermittent Preventive Therapy (SP-IPT) in malaria chemoprevention in children, including those with sickle cell anaemia (SCA). This study determined the distinct pattern and prevalence of Pfdhps mutations in children with SCA and in those with homozygous haemoglobin A (HbAA) in Benin City, Nigeria; showing the impact of haemoglobin phenotype. this was a cross-sectional study involving children with SCA and HbAA. Those with successfully amplified Pfdhps genes were included in the study. Point mutations and mutant haplotypes of the Pfdhps gene were identified. Parasite density (PD) was determined by estimating the parasite numbers/μl of blood from the thick film. Descriptive, univariable and multivariable analysis were used appropriately. a total of 146 children: 71 with SCA and 75 with HbAA were recruited, with a mean age of 46.6 ± 13.0 and 36.4 ± 17.6 respectively; proportion of males were 45(63.4%) and 43(57.3%) respectively. I431V, S436A, A437G, A581G, and A613G mutations were present; but the K540E mutation was absent. ISGKAA 41(28.1%) and VAGKGS 61(41.8%) were the most prevalent mutant haplotypes in this study. The prevalence of VAGKGS haplotype 43(57.3%) was significantly higher in HbAA group compared to that 18(25.4%) in the SCA group (p < 0.001). The prevalence of ISGKAA in SCA group 25(35.2%) was significantly higher than that 16(21.3%) in the HbAA group (p=0.032). HbAA phenotype was the only significant predictor for the presence of the VAGKGS mutant haplotype (aOR: 3.0, 95%CI: 1.375 to 6.499; p=0.006). the HbAA phenotype was a significant predictor for the occurrence of the quintuple mutant haplotype (VAGKGS). The K540E mutation was absent; thus, SP-IPT can be explored in children younger than five years with SCA.

ADAMTS13 levels in sickle cell anaemia

Background Sickle cell anaemia (SCA) is a hypercoaguable state characterized by a significant alternation in haemostatic parameters which may predispose to increased risk of vas-occlusive crisis (VOC). The role of ADAMTS13 in the pathogenesis of VOC has not been fully investigated in our environment. Objective The objective of this study is to evaluate the level of ADAMTS13 in SCD subject in Calabar and to determine their role in the pathogenesis of vaso-occlusive crisis. Materials and methods This is a comparative study carried out at the University of Calabar Teaching Hospital (UCTH), Calabar. In all, 60 SCA patients were evaluated in VOC and steady states well are 50 apparently healthy controls. ADAMTS13 Ag was evaluated using Assaypro enzyme-linked immunosorbent assay kits with LOT Nos 04222167 R. Data were analyzed with IBM SPSS Chicago software version 21. The study was approved by the UCTH Institution Ethical Review Board. Results The mean ages of the SCA subjects and controls were 23.5 + 7.2 yrs and 26.5 + 5.6 years respectively (P=0.706). There were 23 (38.3%) males in the SCA group and 21 (42.0%) females in the controls. There was no significant difference in their sex distribution (P=0.063). The mean (SD) of ADAMTS13 in VOC, steady-state, and controls were 0.61 + 0.10 ug/l, 0.44 + 0.06 ug/l, and 0.62 + 0.10 ug/l respectively. ADAMTS13 levels did not differ significantly across the groups (P=0.270). Conclusion ADAMTS13 level is not elevated both in steady-state and vaso-occlusive crisis

A PRELIMINARY ANTHROPOMETRIC STUDY OF THE RELATIONSHIP BETWEEN DERMATOGLYPHICS AND SICKLE CELL ANAEMIA

Dermatoglyphics have been utilized as models for the diagnosis of vast genetic conditions due to their relationship with the genetic make-up of an individual. However, little studies have been conducted worldwide trying to ascertain the relationship between dermatoglyphics and sickle cell anaemia. Therefore, the present study aimed at generating baseline data to elucidate the possible diagnostic value of dermatoglyphics for earlier detection and screening of Sickle Cell Anaemia (SCA) in Ghana. A total of 400 participants including 200 SCA patients from Komfo Anokye Teaching Hospital and 200 control group (CG) from KNUST were recruited for this study. The palmprints and fingerprints of the participants were taken and the sickling status of the control group was determined. Distribution of the three major fingerprint patterns, PIC patterns, ATD angle and total finger ridge count (TFRC) were determined. Loop dominated in both the SCA and control groups followed by whorl and arch. PIC 300 dominated in the SCA group while PIC 310 dominated in the control group, this was statistically significant. Also, the study recorded 5 unreported PIC’s (PIC 400, PIC 410, PIC 430, PIC 500 and PIC 510) in the Ghanaian population. The SCA group recorded a mean ATD angle of 43.62o while the control group recorded 41.61o, this was statistically significant. The SCA group recorded a mean TFRC of 67.17 while the control group recorded 78.49. The results of the present study have shown that, there is a relationship between dermatoglyphics and SCA and this will serve as a reliable indicator for earlier detection and screening of sickle cell anaemia especially in neonates. KEY WORDS: Dermatoglyphics, sickle cell anaemia, Ghana.

Iron overload in steady state, non-chronically transfused children with sickle cell anaemia in Ile-Ife, Nigeria

IntroductionThe estimation of serum ferritin level is a method of determining iron status. We studied the iron status of a group of non-chronically transfused children with sickle cell anaemia (SCA), using their serum ferritin levels. ObjectiveTo determine the iron status among children with SCA in steady state in Ile-Ife, Nigeria. Material and methodsThe study was carried out at the Consultant Paediatric Haematology Outpatient Clinic of the Obafemi Awolowo University Teaching Hospitals' Complex, Ile-Ife between June and September 2013. History and clinical examination were evaluated in 48 HbSS subjects and 48 HbAA controls to ascertain that they did not have features of acute illness. Serum ferritin levels were assayed in both subjects and controls. Results/DiscussionThe age range of both subjects and controls was 1–15 years. The mean age was 8.0 (4.0) for both groups. The serum ferritin levels ranged from 34.2 to 3282.9 μg/l with mean of 381.2 (1.0), median 180 μg/l; this was significantly higher than that for the controls, with range of 3.7–26.6 μg/l, mean 46.1 (0.9) and median of 33.9 μg/l (p = 0.000). Among the SCA group 31 (64.6%) had had blood transfusion while 17 (35.4%) had not. The mean of the serum ferritin levels was not significantly higher in the group with blood transfusion compared with the group without blood transfusion (p = 0.923). Serum ferritin level was greater than 300 μg/l in 16 (33.3%) subjects with SCA, 6 (37.5%) of whom had no history of blood transfusion. There was no significant correlation between serum ferritin levels and age, haemoglobin concentration and number of blood transfusions. ConclusionDespite not being on chronic blood transfusion programme, higher serum ferritin levels indicative of iron overload were found in the SCA group compared with the controls. This suggests a tendency to excessive accumulation of iron within the reticuloendothelial tissues of the SCA group.

Growth and puberty in a prospective cohort of patients with sickle-cell anaemia: an assessment over ten years abstract

Introduction: Hereditary haemoglobinopathies are the most common group of monogenic hereditary diseases in the world. Erythrocytes in sickle form, cellular expression of polymerization of deoxygenated HbS, cause intermittent vascular obstruction, leading to tissue ischaemia and consequent chronic damage in organs and endocrine glands. Objective: The evaluation of the growth pattern and pubertal development of a group of patients with sickle-cell anaemia (SCA) from childhood to adulthood. Method: Thirty patients with SCA between the ages of 10 and 23 years were evaluated in a prospective longitudinal study at three points in time (Te1: 2005; Te2: 2010 and Te3: 2015) and compared with controls. Anthropometric, pubertal and hormonal evaluations were carried out. Age- and gender-specific Z-scores for weight, height and BMI (body mass index) were calculated according to the reference growth standards. Results: Thirty patients with SCA (mean age = 13.93 years) were evaluated at Te1 and 26 patients (mean age = 25.08 years) at Te3. The SCA group lower showed Z-scores for weight (p = 0.0002), height (p = 0.0184) and BMI (p = 0.0011) than the control group at Te1. At Te3, there was no difference in height, but weight (p = &lt;0.0001) and BMI (p = &lt;0.0001) were lower in the SCA group. Men showed greater weight commitment than women at the three study times (Te1: p = 0.0340, Te2: p = 0.0426 and Te3: p = 0.0387) and lower BMI in Te3 (p = 0.0155) in the SCA group. There was a significant increase in weight when comparing Te1 with Te3 (p = 0.0009) and in height when comparing Te1 with Te2 (p = 0.0292) and with Te3 (p = 0.0003) in the SCA group. There was a significant increase in weight when comparing Te1 and Te3 (p = 0.0009) and in height when comparing Te1 and Te2 (p = 0.0292) and Te3 (p = 0.0003) in the SCA group. At Te1, 14 cases and 2 controls were prepubertal. Bone age was delayed in 12 patients. Age at menarche was delayed and lower in the SCA group (mean = 15 years). Five patients had gestated, but no patient had experienced fatherhood. At Te1, TSH levels were higher (p = 0.0080) and T3 levels were lower (p = 0.0020) in the SCA group. At Te3, LH and FSH levels were higher in men with SCA (p = 0.0014; p; 0.0002). IGF-I levels were lower in cases both at Te1 (p = 0.0002) and at Te3 (p = 0.0032). Conclusions: Patients with SCA showed growth impairment and pubertal delay compared with healthy controls. However, albeit belatedly, they reached normal sexual maturation and height in adulthood. Women with SCA showed no fertility problems. The findings highlight the need to investigate the intention of paternity and fertility among men with SCA.

Serum cytokine profile among Brazilian children of African descent with periodontal inflammation and sickle cell anaemia

ObjectiveThe aim of this study was to evaluate possible immunologic relationships between sickle cell anaemia (SCA) and periodontal inflammation and its impact on serum cytokines. DesignTwenty-five Brazilian children of African descent were involved in this study and divided in two groups: SCA (n=10): confirmed diagnosis of homozygous anaemia; and CTR-control (n=15): no sickle anaemia. Clinical examination included comprehensive medical (routine physical evaluation) and periodontal exams: plaque index (PI), bleeding on probing (BoP), and haematological analysis. Serum samples were collected for cytokine evaluation by microarray. Clinical and laboratorial parameters were compared statistically (alpha=5%). ResultsThe higher values of PI and BoP were similar for both groups (p>0.05) confirming a diagnosis of generalized gingivitis for all individuals. Intergroup analysis showed higher levels of interferon gamma (IFNγ), tumour necrosis alpha (TNFα), interleukin (IL)-4, -5, -8, -10 and 13 only in the SCA group (p<0.05). In addition, PI was negatively correlated with IL-2, IL-4, IL-5, IL-6, IL-8 and IL-13, while BoP was positively correlated with IL-10. ConclusionWithin the limits of the present study, it was concluded that SCA increase the levels of serum cytokines regardless of the presence of periodontal inflammation. Therefore, a direct immunological relationship between SCA and periodontal inflammation was not established.

Does pain affect executive function in sickle cell disease?: Abstract G151 Table 1

Aims Research in populations with sickle cell anaemia (SCA) has consistently found differences from healthy controls on tests of executive function (EF), attributed to the elevated stroke risk. In older adults without SCA there is an association between pain and mental flexibility. The aims of this study were to investigate the executive function performance of a group of SCA children across a single test battery and the possibility that chronic pain impacts upon these skills. Methods Patients with SCA were recruited via their consultants at outpatients clinics. Sibling controls and other ethnically matched children were recruited via the community. The Weschler Abbreviated Scales of Intelligence (WASI): two-sub-test version was administered together with five of the nine available sub-tests of the Delis Kaplan Executive Function System (D-KEFS), representing a range of different skills: the trail-making, the design fluency, colour-word interference, sorting and tower tests. Composite scores were created for each of the DKEFS sub-tests. Current pain was measured using the Faces Pain Scale-Revised (FPS-R). Frequency of pain was measured via self-report of the number of pain episodes over the past year. A number of t-tests, followed by correlations, were then employed to compare performance across the two groups. Results Eight controls (3 male), mean age 11 y 3 m (9 y 8 m-14 y 8 m) and 14 (11 male) children with SCA, mean age 13 y 4 m (10 y 7 m-17 y 6 m) were recruited. There was a significant difference between the frequency of pain episodes in the SCA and control groups, t (20)=−2.56, p=0.019. T-tests showed no significant differences at the 95% confidence interval in FPS-R scores, any of the DKEFS sub-tests, or FSIQ. No significant correlations were found between either frequency of pain episodes or current pain, and any of the composite EF sub-test scores. Discussion We found no evidence of impaired performance by the SCA group on any of the EF sub-tests. The SCA group reported significantly more frequent pain episodes, and a wider range of pain levels on the day of testing but there was no association between current pain, or pain frequency, and EF test performance, perhaps explained by the subjective nature of pain, and differences in pain perception.

Mild haemorheological changes induced by a moderate endurance exercise in patients with sickle cell anaemia

The levels and duration of physical activity that can be considered as completely safe in patients with sickle cell anaemia (SCA) is unknown. The present study compared the haemorheological and haematological profile, cell density distribution and basic biochemistry between a group of 17 patients with SCA and 21 healthy subjects before and after a 20 min duration submaximal cycling exercise at the same absolute workload. Blood was sampled at rest and 3 min after the end of exercise for measurement of biological parameters. Exercise did not affect the haematocrit and blood viscosity in the two groups. Plasma viscosity was not different between the two groups at rest and similarly increased with exercise. The proportion of intermediary dense cells (with density between 1·11 and 1·12 g/ml) decreased with exercise in the SCA group resulting in an increase in the proportion of red blood cells with a density >1·12 g/ml. No change was observed in the control group. The present study suggests that mild-moderate exercise is not very harmful for SCA patients. The haemorheological and haematological changes were very mild, except for the formation of dense cells but no clinically significant signs of medical complication were present in any of the patients.

Relationship between Painful Crisis and Serum Zinc Level in Children with Sickle Cell Anaemia

Sickle cell anaemia (SCA) is associated with zinc deficiency; zinc supplementation may ameliorate some of its clinical manifestations including the relief of painful crisis. Subjects and Methods. Serum zinc levels were determined in 71 children with SCA and painful crisis and in equal numbers in steady state. Seventy-one children with AA genotype acted as controls. Qualitative assessment of zinc content of 24-hour dietary recall and the last meal consumed before blood was drawn was taken. Serum zinc was determined using atomic absorption spectrophotometer. Haemoglobin concentration and packed cell volume (PCV) were determined using standard methods. Results. The mean serum zinc concentration in the study was less than international reference range. The controls had significantly higher serum zinc concentrations than the SCA group (42.7 ± 13.6 versus 32.3 ± 14.0 μg/dL, P < .000); this difference was due to the significantly lower values of serum zinc in SCA with painful crisis compared with the remaining two groups F = 30.9, P<.000. There was a positive correlation between serum zinc and haemoglobin concentration only in the control group (r = 0.4; P = .001). Conclusion. The serum zinc levels in this study were low. Painful crisis in SCA may exert greater demand for zinc utilization in children with SCA thereby resulting in lower serum levels.

Pulpal necrosis with sickle cell anaemia.

To investigate radiographic manifestations of sickle cell anaemia (SCA) and whether or not a pulpal necrosis may develop without a pathological history. Thirty-six patients with homozygous SCA were evaluated, and a further 36 individuals without SCA were included in the study as a control group. All 72 patients participating in the study ranged between the ages of 16 and 40 years. General and dental histories of the individuals were recorded. Electrical pulp test, percussion and thermal tests were applied to all the teeth having no restorations. Orthopantomograms of all the subjects were taken. Data obtained from questionnaires, sensitivity tests and radiographic examinations were evaluated by chi-square and Fischer's exact test. Fifty-one (6%) of the teeth having no restorations or history of trauma were determined as being nonvital in the SCA group. In 30 (83%) of these patients orofacial and dental pain with no obvious cause was detected and in 24 (67%) of the patients the quality of the bone tissue as examined radiologically had deteriorated. In eight (22%) of the patients cortical thinning and irregularity in the mandible was noted. A statistically significant difference between the SCA and control groups (P < 0.05) was found in terms of pulpal sensitivity and radiological findings. SCA is a genetic and systemic disease which may cause pulp necrosis without necessarily having an identifiable aetiology. SCA causes radiographically observable differences in jaw structure especially in the mandible.

Urinary tract infection in febrile children with sickle cell anaemia in Ibadan, Nigeria.

A prospective study to determine the prevalence of bacteriuria and bacterial isolates in the urine of febrile children with sickle cell anaemia (SCA) was carried out at University College Hospital, Ibadan. Altogether, 171 febrile children (aged 1-15 years) with SCA and 171 age- and sex-matched controls were studied. After obtaining a history of the illness from the parents or guardians, each child was physically examined and a mid-stream urine specimen collected and subjected to microscopy and culture. The prevalence of bacteriuria in children with SCA was 21.6% compared with 15.8% in the controls. Escherichia coli and Klebsiella species were the predominant isolates from the urine, accounting for 64.9% and 18.9%, respectively, of the isolates from the SCA group and 63% and 22.2%, respectively, in the controls. In the SCA group, significant bacteriuria also occurred with other conditions such as pneumonia and osteomyelitis. Urinary tract infection (UTI) is common in children with SCA. Routine screening for it is therefore recommended during febrile illnesses. Children with fever from other overt causes, however, should not be exempted from the urine screening procedure in case there might be concomitant UTI.