Literature DB >> 33552759

Risk Factors and Complications Among Pediatric Patients With Sickle Cell Anemia: A Single Tertiary Center Retrospective Study.

Fatma Alzahrani1, Anas M Fallatah2, Fatimah M Al-Haddad3, Shahad T Khayyat2, Wasayf M AlMehmadi2, Bashaier G AlQahtani4, Rawabi S Alamri4.   

Abstract

Background and objective Sickle cell anemia (SCA) is one of the common genetic diseases in the Kingdom of Saudi Arabia (KSA). This disease results from a genetic mutation that causes malformation of the red blood cells (RBCs), leading to various systemic complications, including vaso-occlusive crisis (VOC), acute chest syndrome (ACS), osteomyelitis, avascular necrosis (AVN), and stroke, to name a few. The leading cause of mortality in SCA is these systemic complications rather than the disease itself. Understanding the risk factors of these complications can help reduce mortality in these patients and improve their quality of life. In this study, we aimed to determine the risk factors of SCA complications among pediatric patients with SCA at King Abdulaziz University Hospital (KAUH) in Jeddah, KSA. Methods This retrospective study was carried out from January 2012 till June end 2019. It was conducted among pediatric patients with SCA. Patients were screened for eligibility, and we excluded those with thalassemia and those who had a medical history of chronic diseases. Data were collected from patients' electronic medical records. Results The study included 102 pediatric patients with SCA; their mean age was 7.88 ±4.22 years; almost half of them were females (56%) and 44% were males. The dominant body mass index (BMI) classification among them was normal (49%). Urinary tract infection (UTI) was the most common complication with 38 cases followed by VOC with 32 cases. Other complications observed were ACS (25.5%) followed by stroke (15.7%). HbSS was the most prominent genotype among these patients, and it was associated with a higher rate of complications. However, there was no significant relationship between genotype and patients developing complications. Finally, patients with high white blood cell (WBC) counts, elevated systolic blood pressure (SBP), and hypoxia developed more complications, and there was a significant relationship between these conditions and the development of complications (p<0.05). Conclusion Based on our findings, patients with high WBC count, elevated SBP, and hypoxia are at greater risk of developing complications. Accordingly, healthcare providers should consider putting in place all measures required to provide a good quality of life for these patients, including raising awareness about the risk factors that lead to these complications, appropriate immunizations, and precautionary measures to promote these patients' welfare.
Copyright © 2021, Alzahrani et al.

Entities:  

Keywords:  jeddah; risk factors; saudi arabia.; sickle cell anemia; sickle cell complications; sickle cell disease

Year:  2021        PMID: 33552759      PMCID: PMC7851883          DOI: 10.7759/cureus.12440

Source DB:  PubMed          Journal:  Cureus        ISSN: 2168-8184


  27 in total

1.  Distribution of sickle-cell hemoglobin in Saudi Arabia.

Authors:  H LEHMANN; G MARANJIAN; A E MOURANT
Journal:  Nature       Date:  1963-05-04       Impact factor: 49.962

Review 2.  A global perspective on sickle cell disease.

Authors:  Banu Aygun; Isaac Odame
Journal:  Pediatr Blood Cancer       Date:  2012-04-25       Impact factor: 3.167

3.  The prevalence of sickle cell disease in Saudi children and adolescents. A community-based survey.

Authors:  Mansour M Al-Qurashi; Mohammad I El-Mouzan; Abdullah S Al-Herbish; Abdullah A Al-Salloum; Ahmad A Al-Omar
Journal:  Saudi Med J       Date:  2008-10       Impact factor: 1.484

4.  Increased prevalence of osteonecrosis of the femoral head in children and adolescents with sickle-cell disease.

Authors:  Kris M Mahadeo; Suzette Oyeku; Benjamin Taragin; Swapnil N Rajpathak; Karen Moody; Ruth Santizo; M Catherine Driscoll
Journal:  Am J Hematol       Date:  2011-09       Impact factor: 10.047

Review 5.  Sickle Cell Disease.

Authors:  Frédéric B Piel; Martin H Steinberg; David C Rees
Journal:  N Engl J Med       Date:  2017-04-20       Impact factor: 91.245

6.  Height and weight reference curves for homozygous sickle cell disease.

Authors:  P W Thomas; A Singhal; M Hemmings-Kelly; G R Serjeant
Journal:  Arch Dis Child       Date:  2000-03       Impact factor: 3.791

Review 7.  Prevention and management of infection in children with sickle cell anaemia.

Authors:  W Y Wong
Journal:  Paediatr Drugs       Date:  2001       Impact factor: 3.022

8.  The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up.

Authors:  Martin H Steinberg; William F McCarthy; Oswaldo Castro; Samir K Ballas; F Danny Armstrong; Wally Smith; Kenneth Ataga; Paul Swerdlow; Abdullah Kutlar; Laura DeCastro; Myron A Waclawiw
Journal:  Am J Hematol       Date:  2010-06       Impact factor: 10.047

9.  Incidence of invasive pneumococcal disease among individuals with sickle cell disease before and after the introduction of the pneumococcal conjugate vaccine.

Authors:  Natasha B Halasa; Sadhna M Shankar; Thomas R Talbot; Patrick G Arbogast; Ed F Mitchel; Winfred C Wang; William Schaffner; Allen S Craig; Marie R Griffin
Journal:  Clin Infect Dis       Date:  2007-04-18       Impact factor: 9.079

10.  Clinical and Geographic Characterization of 30-Day Readmissions in Pediatric Sickle Cell Crisis Patients.

Authors:  Jefferson E McMillan; Emily R Meier; Jeffrey C Winer; Megan Coco; Mary Daymont; Sierra Long; Brian R Jacobs
Journal:  Hosp Pediatr       Date:  2015-08
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  2 in total

1.  Pediatric Sickle Cell Disease in Sudan: Complications and Management.

Authors:  Meysaa Talha; Bashier Osman; Safa Abdalla; Hind Mirghani; Iman Abdoon
Journal:  Anemia       Date:  2022-02-14

2.  Hematopoietic Stem Cell Transplantation Stabilizes Cerebral Vasculopathy in High-Risk Pediatric Sickle Cell Disease Patients: Evidence From a Referral Transplant Center.

Authors:  Abdullah Al-Jefri; Khawar Siddiqui; Amira Al-Oraibi; Amal Al-Seraihy; Ali Al Ahmari; Ibrahim Ghemlas; Awatif Al Anazi; Hawazen Al Saedi; Mouhab Ayas
Journal:  J Hematol       Date:  2022-02-26
  2 in total

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