bims-sicedi 2025-11-09 papers

Ann Hematol. 2025 Nov 07.

Circulating miR-145-5p and miR-133a-3p in pediatric sickle cell disease: biomarker potential for vaso-occlusive crises and disease activity.

Reham Fares, Salwa Bakr, Rehab Galal Abd El-Hamid, Omayma O Abdelaleem, Mai Mahmoud Sharabi, Mostafa Yehia Abdelwahed, Hadeel Sherbieny, Marwa Kamal, Eman Yousief Ali.

MicroRNAs (miRNAs) involving miR-145-5p and miR-133a-3p are increasingly recognized for their roles in modulating inflammation, oxidative stress, and vascular integrity, key processes implicated in sickle cell disease (SCD) pathophysiology. To investigate the expression levels of miR-145-5p and miR-133a-3p in pediatric SCD patients during vaso-occlusive crisis (VOC) as well as steady-state conditions, and to evaluate their potential as clinical biomarkers. A case-control study was conducted including 45 pediatric SCD patients (24 during VOC and 21 in steady-state) and 45 age- and sex-matched healthy controls. Plasma miR-145-5p and miR-133a-3p levels were quantified using real-time quantitative PCR and correlated with clinical characteristics, laboratory parameters, genotype, and hydroxyurea (HU) therapy. Circulating miR-133a-3p and miR-145-5p were significantly higher in SCD patients than in controls (p < 0.001), with a further elevation during VOC compared to steady-state (p < 0.050). While no significant association was found with genotype or HU therapy (p > 0.05), miR-133a-3p levels correlated significantly with fetal hemoglobin (HbF) (p = 0.006), reticulocyte count (p = 0.049), and urine albumin/creatinine ratio (p = 0.043). A strong positive correlation was observed between both miRNAs (r = 0.683, p < 0.001). Receiver Operating Characteristic (ROC) analysis demonstrated reasonable diagnostic accuracy of both miRNAs in distinguishing VOC from steady-state (AUC = 0.69). This study highlights the potential of miR-133a-3p and miR-145-5p as biomarkers for SCD activity. The association of miR-133a-3p with HbF and urine A/C ratio further suggests clinical relevance in vaso-occlusive complications. Larger longitudinal and functional studies are needed to validate these findings.

Keywords: Biomarker.; MiR-133a; MiR-145; MicroRNAs; Sickle cell disease; Vaso-occlusive crisis

DOI: https://doi.org/10.1007/s00277-025-06696-1

Indian J Pediatr. 2025 Nov 04.

Growth Impairment in Pediatric Sickle Cell Disease: Clinical Predictors and Impact of Hydroxyurea Compliance.

Pratigya Meena, Shweta Pathak, Monica Lazarus, Vidya Kumari.

DOI: https://doi.org/10.1007/s12098-025-05833-y

Br J Haematol. 2025 Nov 03.

Inflammatory cytokine profile in patients with sickle cell anaemia and leg ulcers.

M V Diniz, A P Silva, G S Arcanjo, T H C Batista, J V S Rodrigues, M J B M Rêgo, H I Leão, R C Silva, F O Souto, C M L Melo, B R S Barros, A C M Anjos, A S Araújo, I F Domingos, S T O Saad, F F Costa, A R Lucena-Araujo, M A C Bezerra.

Keywords: NLRP3; hydroxycarbamide; interleukins; sickle cell disease; skin wounds

DOI: https://doi.org/10.1111/bjh.70236

PLoS One. 2025 ;20(11): e0335617

Changes in haematological indices among children with sickle cell disease on hydroxyurea treatment for at least 1 year: A cohort study.

Stephen Emuli, Crispus Tegu, Faith Oguttu, Ritah Nantale, Paul Ochieng, George Passi, Julian Abeso, Joan Wamulugwa, Milton W Musaba, Ruth Namazi, Sarah Kiguli, David Mukunya.

BACKGROUND: Sickle cell disease is the 12th cause of under-five mortality in Africa, with over 81,000 deaths attributed to sickle cell disease annually. Hydroxyurea is one of the few disease-modifying therapies available for the management of sickle cell disease. This study aimed to assess changes in haematological indices among children who had been initiated on hydroxyurea for at least one year in a non-trial setting at a regional referral hospital in Eastern Uganda.
METHODS: We conducted a cohort study, which included children who attended the sickle cell clinic from 21/Aug/2024 to 30/Oct/2024. Data were analyzed using Stata version 18.0. We conducted a paired sample t-test comparing the haematological indices of children with sickle cell disease at baseline and at least one year later.
RESULTS: We included 324 children. Nearly half 155/324 (47.8%) of the participants had good monthly adherence to hydroxyurea. The mean haemoglobin level at follow-up increased by 0.77g/dl (p <0.001) from 7.07g/dl (SD 0.10) at baseline to 7.84g/dl (SD 0.09). There was an increase in the mean corpuscular volume [0.97fl (p=0.645)] and mean corpuscular haemoglobin [0.58pg (p=0.120)]. The white blood cell count decreased by 6.17x103/µl (p<0.001) from 20.77x103/µl (SD ± 0.806) at baseline to 14.60x103/µl (SD ± 0.613) at follow-up. The differential white cell counts of neutrophils, lymphocytes, monocytes, and basophils also decreased.
CONCLUSION: Hydroxyurea resulted in an increase in mean haemoglobin level and a decrease in absolute and differential white blood cell count. The benefit was more pronounced among children with good adherence to hydroxyurea. We add our voice to calls for continued advocacy for the availability of hydroxyurea for use by children with sickle cell disease in low-resource settings. We also recommend a routine complete blood count to monitor response to treatment and also guide patient management among children with sickle cell disease initiated on hydroxyurea.

DOI: https://doi.org/10.1371/journal.pone.0335617

Contraception. 2025 Oct 31. pii: S0010-7824(25)00466-4. [Epub ahead of print] 111275

Safety of hormonal contraception among women with sickle cell disease: an updated systematic review.

Antoinette T Nguyen, Preetha Nandi, Andrea H Roe, Lydia H Pecker, Maura K Whiteman, Naomi K Tepper, Kathryn M Curtis.

OBJECTIVE: To update a previous systematic review on sickle cell disease (SCD) and adverse health outcomes associated with hormonal contraception (HC) use.
STUDY DESIGN: We searched multiple databases for articles from inception through December 12, 2022. We included articles that assessed risk of adverse health outcomes (e.g., thrombosis, painful episodes, osteoporosis) among women with SCD using any HC method. We assessed risk of bias for each study and certainty of evidence for all outcomes.
RESULTS: Nine articles met inclusion criteria; four were new. Eight studies had high risk of bias and one had moderate risk. Three studies examined thrombosis among HC users with SCD; one found that 2.9% of 67 women using combined oral contraception (COC) and none of 84 women using other HC methods experienced venous thromboembolism (VTE), another found no VTE among 20 HC users, and another found elevated risk of ischemic stroke among oral contraception users (presumed mostly COC) versus non-users (adjusted hazards ratio 3.6, 95% confidence interval 0.8-16.5). Six studies with disparate study methods generally found that HC users did not report increases in painful episodes, and in some studies but not all, use of progestin-only methods appeared to decrease painful episodes. One study found no differences in bone mineral density between HC users and non-users with SCD, and another found no diagnoses of osteopenia among 20 HC users with SCD.
CONCLUSIONS: Evidence remains limited for adverse health outcomes, especially thrombosis, with HC use among women with SCD. The certainty of evidence was low for all outcomes.
IMPLICATIONS: Sickle cell disease (SCD) increases risk for stroke, venous thromboembolism, and pregnancy complications. Access to safe contraception is essential for women with SCD who are at risk for and do not desire pregnancy or who wish to optimize pre-pregnancy health. This updated review can help providers counsel patients with SCD about safe use of contraception.

Keywords: Contraception; Sickle cell disease; Systematic review; Thromboembolism

DOI: https://doi.org/10.1016/j.contraception.2025.111275