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Updated: Aug 17, 2025

A Precision Medicine Tool for Measurement and Monitoring of Hemoglobin S in Sickle Cell Disease Patients Receiving Transfusion Therapy
Acute Complications of Sickle Cell Disease in Children Under 5 Years at a Level II Hospital
Joana Lage1, Bernardo Monteiro1, Adriana Costa1
1Hospital Professor Doutor Fernando Fonseca, Amadora, Lisboa, Portugal.
Insights
Children with Sickle Cell Disease (SCD) face frequent hospitalizations for acute complications like vaso-occlusive crisis and infections. These events significantly impact young children, requiring substantial medical care and transfusions.
Area of Science:
- Pediatrics
- Hematology
- Genetics
Background:
- Sickle Cell Disease (SCD) is a genetic blood disorder with significant health implications in early childhood.
- Acute complications requiring hospitalization are a major concern for children diagnosed with SCD.
- Understanding these complications is crucial for effective management and improved patient outcomes.
Purpose of the Study:
- To characterize the acute complications leading to hospitalization in children under five years old with Sickle Cell Disease.
- To identify the most frequent diagnoses and associated factors during hospital admissions for pediatric SCD patients.
- To quantify the burden of hospitalizations, including frequency, duration, and need for interventions like blood transfusions.
Main Methods:
- Retrospective analysis of hospitalization data for 70 pediatric patients (under 5 years) with Sickle Cell Disease.
- Inclusion criteria focused on patients with HbS homozygosity and African ancestry.
- Data analyzed included diagnoses, admission details, hemoglobin levels, and transfusion requirements.
Main Results:
- Vaso-occlusive crisis was the most common diagnosis (36.8%), followed by fever, upper airway infections, bacterial pneumonia, and splenic sequestration.
- The first hospitalization occurred before 12 months in 38.6% of patients, with an average of 3.92 admissions per child.
- 44.2% of patients required at least one blood transfusion, and splenic sequestration was linked to transfusion needs.
Conclusions:
- Acute complications, particularly vaso-occlusive crisis, represent a significant burden for young children with Sickle Cell Disease.
- Splenic sequestration is a notable early complication, often necessitating blood transfusions.
- Pediatric SCD patients experience substantial healthcare utilization, with an average of 3 weeks hospitalized per child annually.
Abstract:
The aim of this study was to characterize the acute complications that required hospitalization in children under 5 years with Sickle Cell Disease. The population included 70 patients, 64% of African ascendency and 95.7% homozygotic for the HbS. We analyzed data from 337 hospitalizations. The first hospitalization occurred before 12 months in 38.6% and the average hospital admission per child was 3.92. The mean duration per hospitalization was 5.36 days. The most common diagnosis was vaso-occlusive crisis (36.8%) followed by fever, upper airway infections, bacterial pneumonia and splenic sequestration. Hemoglobin values at admission were between 6 and 7 g/dL in 22.3%, with 44.2% requiring at least 1 blood transfusion. There are several acute complications of SCD, being vaso-occlusive crisis the most common. Splenic sequestration generally occurs during the first 2 years and is associated with the need of transfusion. They represent a significant burden, with each child spending approximately 3 weeks hospitalized.
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