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Updated: Jul 15, 2025
A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Gastrointestinal symptoms, diagnostic evaluations, and abdominal pathology in children with sickle cell disease
Chinenye R Dike1, Sarah Fittro2, Robert A Oster3
1Department of Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Alabama at Birmingham, Birmingham, Alabama, USA.
Insights
Children with sickle cell disease (SCD) often have abdominal pain, but gastrointestinal (GI) evaluations are infrequent. More GI-specific assessments could improve diagnosis and understanding of abdominal pain in SCD.
Area of Science:
- Pediatric Gastroenterology
- Pediatric Hematology
- Sickle Cell Disease Research
Background:
- Children with sickle cell disease (SCD) frequently experience acute pain, with the abdomen being a common site.
- Gastrointestinal (GI) pathologies can manifest as abdominal pain in children with SCD.
- Limited data exist on the prevalence and diagnostic workup of abdominal pain during acute pain events in pediatric SCD patients.
Purpose of the Study:
- To determine the prevalence of GI symptoms in children with SCD presenting to the emergency department (ED) or hospitalized with abdominal pain.
- To assess the frequency of GI-specific evaluations in these patients.
- To identify risk factors associated with hospitalization for abdominal pain in pediatric SCD.
Main Methods:
- Retrospective study of children under 21 years old presenting to the ED or hospitalized with pain over a two-year period.
- Descriptive statistics were used to analyze clinical characteristics, GI symptoms, and workup by age and genotype (sickle cell anemia vs. non-SCA).
- Logistic regression models identified risks associated with hospitalization.
Main Results:
- Abdominal pain was associated with 23% of encounters analyzed (291 out of 1279).
- Hospitalizations for abdominal pain were more common in older children, those with sickle cell anemia, lower hemoglobin, and higher white blood cell counts.
- Less than 50% of ED patients with abdominal pain received a GI-specific evaluation.
Conclusions:
- Children with SCD frequently present with abdominal pain and GI symptoms, yet GI evaluations are often limited.
- Implementing GI-specific evaluations may enhance the diagnosis and exclusion of GI pathologies.
- Improved GI workup can contribute to understanding the abdomen as a primary pain site in SCD.
Background:
Children with sickle cell disease (SCD) frequently present with acute pain. The abdomen, a common site of acute SCD-related pain, may be present in a variety of gastrointestinal (GI) pathologies. Limited data exist on prevalence and workup of abdominal pain in patients with SCD during acute pain events.
Objectives:
Determine prevalence of GI symptoms, GI-specific evaluation and risks of hospitalization in children with SCD presenting to the emergency department (ED) or hospitalized with abdominal pain.
Methods:
Retrospective study of children less than 21 years presenting to the ED or hospitalized with pain in our center over 2 years. Descriptive statistics were used to report clinical characteristics, frequency of GI symptoms, workup by age (<5 vs. ≥5 years), and genotype (sickle cell anemia [SCA] vs. non-SCA). Logistic regression models were used to identify risks associated with hospitalization.
Results:
A total of 1279 encounters in 378 patients were analyzed; 23% (n = 291) encounters were associated with abdominal pain. More abdominal pain-associated hospitalizations occurred in older children, SCA, children with lower mean hemoglobin (8.7 ± 1.9 vs. 9.6 ± 1.6 g/dL, p < .001) and higher mean white blood cell (WBC) count (14.9 ± 6.6 vs. 13.2 ± 5.3 × 103 /μL, p = .02). We identified that less than 50% of patients presenting to the ED with abdominal pain received a GI-specific evaluation.
Conclusion:
Children with SCD frequently present with abdominal pain and other GI symptoms, with limited GI evaluations performed. GI-specific evaluation may increase diagnosis of GI pathologies, rule out GI pathologies, and contribute to the limited knowledge of the abdomen as a primary site of SCD pain.
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