Prevalence of ocular abnormalities in relation to sickle cell disease severity among children in South-western, Nigeria

Introduction: Sickle cell disease (SCD) ranks high among genetic disorders worldwide. It is characterised by repeated vaso-occlusion with resultant end-organ damage. This process can occur in all vascular beds in the body, including ocular blood vessels and may cause irreversible blindness in advanced stages. Little is known of the relationship between the prevalence of ocular abnormalities among children with SCD and their disease severity. Methods: A descriptive cross-sectional study was carried out at the Paediatric Haematology Clinics and the Eye Centre of the Obafemi Awolowo University Teaching Hospitals Complex (OAUTHC), Ile-Ife. Children with SCD in steady state were recruited from the Haematology Clinics and examined for ocular abnormalities at the Eye Centre of the hospital. The subjects SCD severity grade was determined using a previously validated scoring system. Results: One hundred and twenty (120) children aged 5 to 15 years were examined. Of these, 72 had one or more ocular abnormalities giving the prevalence of ocular abnormalities among them to be 60.0%. Though a higher proportion of children with moderate disease, 23 (65.7%) of 35, compared to those with mild disease, 49 (57.6%) of 85 had ocular abnormalities, this difference was not statistically significant, p = 0.412. Conclusion: Ocular abnormalities among Nigerian children with SCD are common even in steady-state, but not significantly associated with disease severity. Periodic screening for ocular abnormalities should thus be done on them irrespective of disease severity.

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Normal Non-HDL Cholesterol, Low Total Cholesterol, and HDL Cholesterol Levels in Sickle Cell Disease Patients in the Steady State: A Case-Control Study of Tema Metropolis

Journal of Lipids ◽

10.1155/2016/7650530 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5 ◽

Cited By ~ 5

Author(s):

Richard K. D. Ephraim ◽

Patrick Adu ◽

Edem Ake ◽

Hope Agbodzakey ◽

Prince Adoba ◽

...

Keyword(s):

Blood Pressure ◽

Steady State ◽

Sickle Cell Disease ◽

Total Cholesterol ◽

Sickle Cell ◽

Density Lipoprotein ◽

Hdl Cholesterol ◽

Lipoprotein Cholesterol ◽

Cell Disease ◽

Cross Sectional

Background.Abnormal lipid homeostasis in sickle cell disease (SCD) is characterized by defects in plasma and erythrocyte lipids and may increase the risk of cardiovascular disease. This study assessed the lipid profile and non-HDL cholesterol level of SCD patients.Methods.A hospital-based cross-sectional study was conducted in 50 SCD patients, in the steady state, aged 8–28 years, attending the SCD clinic, and 50 healthy volunteers between the ages of 8–38 years. Serum lipids were determined by enzymatic methods and non-HDL cholesterol calculated by this formula: non-HDL-C = TC-HDL-C.Results.Total cholesterol (TC) (p=0.001) and high-density lipoprotein cholesterol (HDL-C) (p<0.0001) were significantly decreased in cases compared to controls. The levels of non-HDL-C, low-density lipoprotein cholesterol (LDL-C), and triglyceride (TG) were similar among the participants. The levels of decrease in TC and HDL were associated with whether a patient was SCD-SS or SCD-SC. Systolic blood pressure and diastolic blood pressure were each significantly associated with increased VLDL [SBP,p=0.01, OR: 0.74 (CI: 0.6–0.93); DBP,p=0.023, OR: 1.45 (CI: 1.05–2.0)].Conclusion.Dyslipidemia is common among participants in this study. It was more pronounced in the SCD-SS than in SCD-SC. This dyslipidemia was associated with high VLDL as well as increased SBP and DBP.

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Association between Endothelial Dysfunction, Biomarkers of Renal Function, and Disease Severity in Sickle Cell Disease

Kidney360 ◽

10.34067/kid.0000142019 ◽

2020 ◽

Vol 1 (2) ◽

pp. 79-85

Author(s):

Oluwagbemiga Oluwole Ayoola ◽

Rahman Ayodele Bolarinwa ◽

Chidiogo Chukwunweike Onwuka ◽

Bukunmi Michael Idowu ◽

Adeniyi Sunday Aderibigbe

Keyword(s):

Renal Function ◽

Steady State ◽

Endothelial Dysfunction ◽

Sickle Cell Disease ◽

Disease Severity ◽

Sickle Cell ◽

Negative Correlation ◽

Brachial Artery ◽

Control Group ◽

Cell Disease

BackgroundEndothelial dysfunction (ED), as ascertained by brachial artery flow-mediated dilation (FMD), is a known feature of sickle cell disease (SCD), which is present both in crisis and in steady state. The assessment of FMD was introduced to examine the vasodilator function. Our objective was to establish the relationship between ED determined by FMD, biomarkers of renal dysfunction, and biomarkers of disease severity in SCD subjects asymptomatic of renal disease.MethodsWe enrolled 44 patients with homozygous SCD in steady state and 33 age- and sex-matched controls between 2013 and 2014 in a tropical tertiary hospital. Ultrasonographic FMD of the right brachial artery, renal arterial Doppler, complete blood count, creatinine, fetal hemoglobin, soluble P-selectin, and cystatin C (Cys-C) levels were determined. Using the median FMD value of the control group, the SCD subjects were further classified into two groups for comparison.ResultsThe median FMD in SCD subjects of 3.44 (IQR, 0.00–7.08) was significantly lower than that of controls, which was 5.35 (IQR, 3.60–6.78; P=0.04). There was negative correlation between FMD and Cys-C levels (r=−0.372; P=0.01) along with renal artery resistivity index (RARI; r=−0.307; P=0.04) in SCD subjects. Additionally, Cys-C level was significantly higher in SCD subjects with FMD<5.35.ConclusionsBrachial artery FMD was significantly lower in SCD subjects compared with a control group. Cys-C and RARI show a negative correlation with FMD, indicating that renal function is related to ED in SCD.

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Psychological Study of the Persons Suffering From Sickle Cell Disease in Raigarh District of Chhattisgarh State

International Journal of Indian Psychology ◽

10.25215/0402.104 ◽

2017 ◽

Vol 4 (2) ◽

Author(s):

Dr. Rajesh Kumar Ajagallay ◽

Dr. Gaukaran Janghel ◽

Dr. Vimal Chandra Bhagat ◽

Viyata Chanda ◽

Dr. Rakesh Kumar Agrawal ◽

...

Keyword(s):

Mental Health ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Age Groups ◽

Emotional Stability ◽

Two Dimensions ◽

The Body ◽

Cell Disease ◽

Cross Sectional ◽

Blood Disorder

Background: Sickle cell disease is an inherited blood disorder in which the body produces abnormal shaped red blood cells (RBC). The disease affects both biological and psychosocial aspects of patients. Aim: Present study aimed at investigating the different mental health dimensions used by heterozygous and homozygous sickle cell anemic patients. Method: The cross-sectional study design with the total 100 sickle cell anemic adolescents of both the sexes were selected in 10 to 20 year age groups, from various hospitals and health clinics of Chhattisgarh, India. The correlation analysis was used for analyzing the data. Results: Total 100 patients were selected which consisted of 30 homozygous and 70 heterozygous adolescent patients with sickle cell gene. The Emotional Stability was which higher significantly correlated with the intelligence quotients, IQ (r = .387, p< .001) than the other dimensions. Only two dimensions of mental health viz. emotional stability (t- 2.38; p<.018) and self-concept (t- 2.32; p<.001) of sickle cell patients which differed among heterozygous and homozygous patients.

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Cell-Free Mitochondrial DNA Is Elevated in Sickle Cell Disease Patients, and Serve As a Potential Proinflammatory DAMP

Blood ◽

10.1182/blood-2018-99-118440 ◽

2018 ◽

Vol 132 (Supplement 1) ◽

pp. 1068-1068

Author(s):

Laxminath Tumburu ◽

Shohini Ghosh-Choudhary ◽

Emilia Alina Barbu ◽

Simon Yang ◽

Lauren D Harrison Ware ◽

...

Keyword(s):

Mitochondrial Dna ◽

Steady State ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Acute Pain ◽

Organ Damage ◽

Healthy Controls ◽

Cell Disease ◽

Cross Sectional ◽

Healthy Donors

Abstract Sickle cell disease (SCD) is an inherited hemoglobinopathy characterized by hemolysis and intermittent acute pain with multi-organ damage. Previously, we showed that acute pain in SCD was associated with >10-fold increases in cell-free DNA (cfDNA) when compared to steady state, that were significantly reduced during hydroxyurea therapy. Apoptosis, necrotic cell death and lysis of intact cells in the blood stream have been proposed as sources of plasma cfDNA. Here, we explored if the cfDNA increases could have a role in inflammation, a constant pathological feature of SCD. cfDNA was extracted using QIAamp MinElute ccfDNA Kit (Qiagen), from the platelet-poor plasma processed within 30 minutes from the blood drawn in EDTA tubes, and analyzed using whole genome sequencing (WGS) and targeted quantitative PCR (qPCR). SCD patients are defined as in acute pain if there is no evident cause other than SCD, for which the patient needs hospitalization, either as in- or outpatient, and is treated with parenteral narcotics. Steady state was defined as the period from at any time 8 weeks prior to or after a crisis. A cross-sectional study of 8 healthy controls and 34 SCD patients (18 steady-state; 16 crisis) mapped WGS reads showed significantly higher proportion of cell-free mitochondrial DNA (cf-mtDNA) compared to nuclear cfDNA (cf-nDNA) in SCD patients compared with healthy controls (Fig 1A: steady-state: 14 fold; crisis: 11 fold; p = 0.0001). We used targeted qPCR to quantify both cf-nDNA and cf-mtDNA in another cross-sectional cohort of 13 healthy controls and 92 patients (72 steady-state, 20 crisis) as well as 18 paired HbSS patients (steady-state and crisis) samples with 10 healthy controls. The nuclear reference genes used were GAPDH and TERT and mitochondrial genes were MT-ND1 and MT-ND6. While cf-nDNA (TERT) was significantly increased (> 3.5 fold, p = 0.0251; Fig 1B) in SCD patients compared with healthy controls only during crises, significantly higher levels of cf-mtDNA over cf-nDNA were observed in SCD patients compared with healthy volunteers in both steady-state and crises (Fig 1C: MT-ND1/GAPDH: steady-state >19 fold, crisis > 8 fold; MT-ND1/TERT: steady-state > 8 fold, crisis > 7 fold; MT-ND6/GAPDH: steady-state > 7 fold, crisis > 3 fold; MT-ND6/TERT: steady-state > 4 fold; crisis > 4 fold; p < 0.05). In the paired samples, cf-nDNA (GAPDH andTERT) was significantly increased (> 3 fold; Fig 1D-E) in crisis compared to steady-state (p < 0.05). The differential increase in cf-mtDNA (cf-mtDNA:cf-nDNA ratio) levels in these patients during crises, were significantly higher compared with healthy controls (Fig 1F: MT-ND1/GAPDH: steady-state >9 fold, crisis > 8 fold; MT-ND1/TERT: steady-state > 8 fold, crisis > 9 fold; MT-ND6/GAPDH: steady-state > 8 fold, crisis > 8 fold; MT-ND6/TERT: steady-state > 8 fold; crisis > 7 fold; p < 0.005). Using confocal microscopy and mitochondrial-specific dyes (MitoTracker Green and TMRM), we show that substantial numbers of red blood cells from SCD patients retain their mitochondria in the circulation. We next explored if the elevated cf-mtDNA in SCD could contribute to its pathophysiology, via activating neutrophils to form neutrophil extracellular traps (NETs), a recognized immunological response in inflammation. Initially, we confirmed that mtDNA can induce NETosis by treating neutrophils from healthy donors with mtDNA isolated from human platelets. mtDNA consistently induced a robust NETs response (N=8) while genomic nuclear DNA did not cause any NETosis. SCD plasma containing high levels of cf-mtDNA also caused a strong NETosis response while plasma from healthy donors did not (N=11). Cytosolic adaptor STING has a central role in sensing of cytosolic double stranded DNA. We sought to determine if the downstream STING-TBK1-IRF3 pathway is associated with the mtDNA-mediated formation of NETs. We inhibited the catalytic activity of the STING downstream effector TBK1 with BX795 prior to treating neutrophils with cf-mtDNA-containing plasma (N=5). The TBK1 inhibition consistently reduced the NETs response by at least 70% confirming that cytosolic DNA sensors are involved in promoting mtDNA-mediated formation of NETs. Our findings suggest that cf-mtDNA induces NETosis contributing to the pathological sterile inflammation in SCD patients. Continual release of these mitochondrial DAMPs in hemolysis may serve as key link between inflammation and organ damage in SCD. Disclosures No relevant conflicts of interest to declare.

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GDF -15 and Severity Scores in Sickle Cell Disease Patients Attending Nnamdi Azikiwe University Teaching Hospital, Nnewi, Anambra State, Nigeria

Journal of Advances in Medicine and Medical Research ◽

10.9734/jammr/2020/v32i130347 ◽

2020 ◽

pp. 31-37

Author(s):

Chide Okocha ◽

Patrick Manafa ◽

Joy Anowi ◽

Vera Manafa ◽

Chilota Efobi

Keyword(s):

Sickle Cell Disease ◽

Disease Severity ◽

Sickle Cell ◽

Teaching Hospital ◽

Therapeutic Target ◽

University Teaching ◽

Cell Disease ◽

Potential Therapeutic Target ◽

Anambra State ◽

Ischaemia Reperfusion

Aim: Granulocyte differentiation factor 15 (GDF15) is a growth factor and biomarker for many disorders where Ischaemia Reperfusion Injury (IRI) is pathophysiologically relevant. Hence the need to evaluate GDF-15 as a biomarker in Sickle Cell Disease (SCD). Study Design: This is a cross sectional study. Place and Duration of Study: Department of Haematology, Nnamdi University Teaching Hospital, Nnewi, Anambra state, Nigeria, between January and December 2018. Methods: Ninety subjects were randomly recruited with haemoglobin (Hb) phenotypes SS (test), AS and AA (controls); numbering 30, 28 and 32 respectively. Disease severity was determined by calculating an objective score. 5 mls of blood was collected and used to determine Full Blood Count (FBC), haemoglobin Phenotype and GDF-15 levels (by Enzyme Linked Immunosorbent assay). Data collected was analysed using Statistical Package for Social Sciences software version 20 (SPSS Inc., IL, Chicago, USA). P< 0.05 was considered as significant. Results: GDF-15 level was found to be significantly different in the different HB phenotypes p= 0.005 and correlated negatively with sickle cell disease severity (r= -0.307, p= 0.098). The difference between median GDF-15 levels of HBSS subjects with mild and moderate disease was statistically significant at p= 0.01. Conclusion: We hypothesize that GDF-15 may be a potential therapeutic target for intervention against ischaemia/reperfusion induced micro- vascular injury. Natural GDF-15 mimetics may be useful in taking advantage of this potential therapeutic target.

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Electrocardiographic Study in Adult Homozygous Sickle Cell Disease Patients in Lagos, Nigeria

Journal of Tropical Medicine ◽

10.1155/2016/4214387 ◽

2016 ◽

Vol 2016 ◽

pp. 1-5

Author(s):

Adedoyin Dosunmu ◽

Akinsegun Akinbami ◽

Ebele Uche ◽

Adewumi Adediran ◽

Sarah John-Olabode

Keyword(s):

Steady State ◽

Sickle Cell ◽

Ventricular Hypertrophy ◽

Sickle Cell Anaemia ◽

Left Ventricular ◽

The Body ◽

University Teaching ◽

Cross Sectional ◽

Noninvasive Test ◽

Significant Difference

Background. This study sought to identify the pattern of electrocardiographic changes in steady state adult sickle cell anaemia.Methods.A case-control, cross-sectional study was conducted amongst sickle cell patients attending the sickle cell clinic of Lagos State University Teaching Hospital, Ikeja, and HbAA controls. All consenting participants had haemoglobin electrophoresis done and were subjected to electrocardiography (ECG). The descriptive data were given as means ± standard deviation (SD). The differences were considered to be statistically significant when thepvalue obtained was <0.05.Results.A total of ninety-three sickle cell anaemia (SCA) patients and ninety haemoglobin AA (controls) were enrolled. There was no significant difference in the age of the participants with SCA and that of the controls but the body mass index was significantly higher in controls (p=0.0001). Overall, 73.1% (68 of 93) had abnormal ECG while only 2 of 90 (2.2%) of controls had abnormal ECG. The common abnormalities observed were left ventricular hypertrophy, biventricular hypertrophy, and right ventricular hypertrophy.Conclusion.Patients with SCA in steady state tend to have normal heart rate but about 50% of them would have had ECG changes before the age of 20 years. ECG being a noninvasive test may be used to identify patients at risk for early intervention.

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Comparative study of hypercoagulability change in steady state and during vaso-occlusive crisis among Sudanese patients living with sickle cell disease

African Health Sciences ◽

10.4314/ahs.v20i1.45 ◽

2020 ◽

Vol 20 (1) ◽

pp. 392-396

Author(s):

Elmigdad Abdelgadir Mohamed ◽

Mamoud Mohamed Elgari ◽

Asaad Mohammed Babker ◽

Hisham Ali Waggiallah

Keyword(s):

Steady State ◽

Sickle Cell Disease ◽

Sickle Cell ◽

International Normalized Ratio ◽

P Value ◽

Thrombin Time ◽

Cell Disease ◽

Cross Sectional ◽

Blood Disorder ◽

D Dimer

Background: Sickle cell disease (SCD) is an inherited blood disorder that affects red blood cells. (SCD) is characterized by re- current vaso-occlusive crisis (VOC). Material and methods: This was a descriptive cross sectional study conducted through the period from July 2015 to July 2017 in which a total of seventy two blood specimens were collected in 'EDTA' and citrated vacutainers from Sudanese patients with SCD attending "Fath Elrhman Albasheer" Centre. Both sexes' with different ages were included. Among these samples 49 (68.1%) were in steady state while the remained 23 (31.9%) were in VOC. All samples were tested for coagulation profile Result: There was increase in fibrinogen and D-dimer levels in most patients 67% and 71%, respectively. Significant increase in D-dimers was observed in patients with (VOC) compared with steady state (P. value = 0.006). Protein S was significantly in- creased in males in comparison with females P. value = 0.017. The results of prothrombin time (PT), international normalized ratio (INR) and thrombin time (TT) were within normal range. Conclusion: Significant increase in levels of D-dimer during VOC indicating abnormal coagulation and fibrinolysis activation. Reduced levels of natural anticoagulants proteins C and S can be consider as predictive markers indicate hepatic dysfunction in patients with SCD. Keyword: SCD; VOC; hypercoagulability; Sudanese patients.

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Prevalence of Priapism and Its Awareness amongst Male Homozygous Sickle Cell Patients in Lagos, Nigeria

Advances in Urology ◽

10.1155/2013/890328 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 6

Author(s):

Adewumi Adediran ◽

Kikelomo Wright ◽

Akinsegun Akinbami ◽

Adedoyin Dosunmu ◽

Olajumoke Oshinaike ◽

...

Keyword(s):

Sickle Cell Disease ◽

Sickle Cell ◽

Pathological Condition ◽

Cross Sectional Study ◽

University Teaching ◽

State University ◽

Cell Disease ◽

Cross Sectional ◽

Sexual Stimulation ◽

Male Patients

Background. Priapism is a pathological condition of penile erection that persists beyond, or is unrelated to, sexual stimulation. Impotence and infertility are major problems in male sickle cell disease patients, and priapism has been implicated as a cause of impotence and infertility. The aim of this study is to determine priapism prevalence and assess the knowledge of male homozygous male patients about it in Lagos, Nigeria.Methods. A cross-sectional study was conducted amongst male homozygous sickle cell disease patients of Lagos State University Teaching Hospital. Pretested questionnaires were distributed to determine the prevalence and assess their knowledge on priapism. Data were analyzed using SPSS version 16.0.Results. A total of 114 consenting subjects filled the questionnaires, 85 of 114 (74.6%) had not heard about priapism before this study. A total of 77 of 114 (67.5%) did not know that they are at risk of priapism. Whilst 84 of 114 (73.7%) were not aware that priapism is a complication of SCD. The majority, 94 of 114 (82.5%), were not aware that priapism could cause impotence.Conclusion. There is a need to create more awareness about this complication amongst sickle cell disease patients in order to stem the incidence of impotence and infertility amongst them.

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Increased Inflammatory State and Metabolic Activation in Neutrophils from Patients with Sickle Cell Disease: Comparison of Neutrophil Gene Expression Profiles with Controls.

Blood ◽

10.1182/blood.v104.11.3571.3571 ◽

2004 ◽

Vol 104 (11) ◽

pp. 3571-3571

Author(s):

Sule Bakanay ◽

Ferdane Kutlar ◽

Joshi Ratanmani ◽

Betsy Clair ◽

Leigh Wells ◽

...

Keyword(s):

Gene Expression ◽

Steady State ◽

Sickle Cell Disease ◽

Disease Severity ◽

Sickle Cell ◽

Expression Profiles ◽

Gene Expression Profiles ◽

Cell Disease ◽

Expression Of Genes ◽

Inflammatory State

Abstract Chronic inflammation is a well-established feature of sickle cell disease (SCD) even at steady state, and the degree of inflammation tends to correlate with disease severity. Elevated neutrophil count, as a reflection of the overall inflammatory state, has emerged as an indicator of poor prognosis and has been associated with adverse outcomes including stroke and early mortality. To further delineate the role of neutrophils in the pathogenesis of various complications and in overall disease severity in SCD, we analyzed the gene expression profiles of neutrophils from 5 patients with "severe" disease (>3 vaso-occlusive episodes [VOE] per year), 5 patients with "mild" disease (<3 VOE/year) and compared these to each other and to the gene expression profiles of neutrophils from 5 age and sex matched, healthy, non-sickle cell, African-American individuals. Granulocytes were separated from freshly collected venous blood using Histopaque (Sigma diagnostic) density gradient separation. Total RNA was extracted immediately after cell separation by using Rneasy Mini Kit (Qiagen). 2 micrograms of total RNA was converted to double stranded cDNA (ds-cDNA) by using SuperScript Choice System (Invitrogen). In vitro transcription was performed on the ds-cDNA using Enzo RNA transcript labelling kit. After the fragmentation, labeled RNA was hybridized to a set of oligonucleotide arrays (HG U133A, Affymetrix, Santa Clara, CA) and the data was analysed with the Microarray suite 5.0 software (Affymetrix). Out of the differentially expressed genes (314 genes for severe vs. control, 718 genes for mild vs control), those with greater than two fold expression were analysed with the geneMAPP software for localization into biological pathways. In general, a larger number of genes were differentially expressed between "mild" patients vs. control, compared to that between "severe" vs "mild" patients. Genes related to cellular proliferation, growth and maintenance, DNA repair, DNA replication, and cell cycle progression were expressed at significantly higher levels in SCD patients compared to controls. The most impressive finding was the significantly higher expression of genes leading to NFkB activation and inhibition of apoptosis: IAP-1 (increased 6.7 fold and 4.7 fold in mild and severe patients respectively), IkB (decreased 0.14 fold and 0.3 fold), Apaf-1 (decreased 0.4 fold in mild), and c-jun (decreased 0.4 fold in severe); Traf-2 (TNF receptor associated factor-2; increased 3.5 fold and 2 fold); genes in the MAPK signalling pathway: ERK-2 (increased 3.5 fold and 2-fold), MAP2K3 (increased 3.5 fold and 2 fold). These data show that neutrophils in SCD patients are activated with higher expression of genes in the TNF, MAPK, and NFkB pathways consistent with an inflammatory state. Delayed or inhibited apoptosis of neutrophils further maintains this inflammatory state even during the so-called "steady state" of the disease. We conclude that the analyses of gene expression in neutrophils can be a useful tool in identifying pathways and genes that distinguish SCD patients from controls and in differentiating mild and severe phenotypes.

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Neutrophil gelatinase–associated lipocalin as a biomarker of nephropathy in sickle cell disease

Annals of Hematology ◽

10.1007/s00277-021-04500-4 ◽

2021 ◽

Author(s):

Rajaa Marouf ◽

Adekunle D. Adekile ◽

Hadeel El-Muzaini ◽

Rasha Abdulla ◽

Olusegun A. Mojiminiyi

Keyword(s):

Steady State ◽

Sickle Cell Disease ◽

Sickle Cell ◽

Roc Curve ◽

Screening Tests ◽

Cell Disease ◽

Urine Ngal ◽

Neutrophil Gelatinase Associated Lipocalin ◽

Plasma Ngal ◽

Neutrophil Gelatinase

AbstractSickle cell nephropathy (SCN) develops via altered hemodynamics and acute kidney injury, but conventional screening tests remain normal until advanced stages. Early diagnostic biomarkers are needed so that preventive measures can be taken. This study evaluates the role of neutrophil gelatinase–associated lipocalin (NGAL) as a biomarker of SCN in steady state and vaso-occlusive crisis (VOC). In this case-control study, 74 sickle cell disease (SCD) patients (37 in steady state and 37 in VOC) and 53 control subjects had hematological and biochemical measurements including plasma and urine NGAL. Univariate and logistic regression analyses were used to find the associations between variables. The receiver operating characteristic (ROC) curve was used to determine the diagnostic performance characteristics of plasma and urine NGAL for detection of VOC. Plasma and urine NGAL, urine microalbumin:creatinine ratio, and urine protein:creatinine ratio were significantly higher in VOC. Microalbuminuria was present in 17.1% steady state and 32.0% VOC patients. Microalbuminuria showed significant correlations with age, plasma NGAL, WBC, and hemolytic parameters. Area under the ROC curve for plasma NGAL was 0.69 (95%CI = 0.567–0.813; p = 0.006) and 0.86 (95%CI = 0.756–0.954; p < 0.001) for urine NGAL. Urine NGAL cut-off value of 12.0 ng/mL had 95% sensitivity and 65% specificity. These results confirm the presence of nephropathy during VOC and suggest that plasma and urine NGAL would be useful in the identification of SCN. Urine NGAL should be used as the screening biomarker, and patients with VOC and urine NGAL > 12.0 ng/mL should be selected for aggressive management to prevent progression of renal damage.

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