Background Sickle cell disease (SCD) is an autosomal recessive hemoglobinopathy that is very common and causes a great burden in Saudi Arabia and worldwide. This study aims to determine the frequency and benefits of using hydroxyurea in treating children with SCD in King Abdulaziz University Hospital (KAUH) in Saudi Arabia. Method This retrospective observational study included all SCD patients, males and females, who were following up in KAUH and were less than 18 years old. Patients on chronic transfusion therapy or who had previous stem cell transplantation were excluded from the study. The study was approved by the unit of biomedical ethics in KAUH, Jeddah. Result The study included 102 SCD patients; the median age of the sample was 7.5 +/- 4.2, with the majority being female (58 patients; 56.9%). Hydroxyurea users among participants in the study were 62 patients (60.8%). Thirty-seven (37) of the patients using hydroxyurea had an increase in the mean corpuscular volume (MCV). There was a decrease in the level of white blood cells (WBCs) and neutrophil count from 15.81 +/- 10.24 and 48.31 +/- 23.23% to 12.48 +/- 5.48 and 40.81 +/- 15.78%, respectively. Platelet count showed an increase from 345.4 +/- 2.1096 to 359.162 +/- 199.87 after starting hydroxyurea. The incidence of vaso-occlusive crisis (VOC) increased after hydroxyurea initiation from 4.555 +/- 4.08 to 6.288 +/- 9.80. Moreover, the frequency of blood transfusion in patients using hydroxyurea was statistically significant as p-value = 0.048. Conclusion In conclusion, our results showed significant clinical and laboratory benefits of hydroxyurea in children with SCD. Hydroxyurea has been shown to reduce the frequency of VOC and blood transfusion in patients with SCD.

Background/Objectives: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most frequently encountered periodic fever syndrome in pediatric populations. It is characterized by recurrent, stereotyped episodes of fever accompanied by pharyngitis, cervical lymphadenopathy, and aphthous stomatitis, sometimes with other additional nonspecific symptoms. Episodes usually last 3–7 days and recur at regular intervals of approximately 2–8 weeks. The etiopathogenesis of PFAPA remains incompletely understood. Accumulating evidence supports a central role of immune dysregulation with multiple genetic variants that contribute to disease susceptibility. Diagnosis is primarily based on clinical evaluation, although targeted genetic testing may be considered in selected cases particularly when overlapping with other monogenic autoinflammatory disorders. Management of PFAPA aims to decrease the frequency and intensity of febrile episodes. Acute flares are managed with antipyretics, corticosteroids, or, in selected cases, anti-interleukin-1 (IL-1) therapy, while preventive strategies include colchicine, cimetidine, or tonsillectomy. Adjunctive approaches such as vitamin D supplementation, probiotics, and other immunomodulatory interventions have also been investigated. This updated review summarizes current insights into the pathogenesis and therapeutic approaches of PFAPA. Methods: This narrative review was conducted using PubMed, and Scopus to identify English-language publications from 2010 to 2026 addressing PFAPA. Conclusions: Pediatricians should consider PFAPA in children with recurrent fevers and oropharyngeal symptoms, especially when standard treatments fail, to ensure proper management and avoid unnecessary antibiotic use.