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[Sickle cell disease in the People's Republic of Angola. 2. Behavior of laboratory parameters]
W Eggert1, L Bernardino, M de Cruz
1Klinik und Poliklinik für Kinderheilkunde, Humboldt-Universität zu Berlin.
Insights
This study in 819 children found a significant decline in hemoglobin (Hb) concentration over six years, particularly during severe disease crises. Anemia negatively impacts the cardiovascular system in pediatric patients.
Area of Science:
- Pediatric Hematology
- Clinical Biochemistry
Context:
- Investigated hemoglobin (Hb) levels and anemia in 819 children over six years.
- Evaluated the relationship between disease course, clinical crises, and Hb concentration.
- Assessed the impact of anemia on the cardio-circulating system.
Purpose:
- To analyze Hb concentration trends and their correlation with disease severity and crises in children.
- To understand the types of anemia (normochromic vs. hypochromic) and their prevalence.
- To determine the effects of anemia on cardiovascular parameters, specifically the thorax-heart quotient.
Summary:
- A significant decline in mean Hb concentration (4.65 +/- 0.5 Hb mmol/l) was observed over six years.
- Severe clinical crises (third degree) correlated with significant Hb decreases.
- Normochromic anemia was observed in nearly half the cases, with hypochromic anemia in 45.7%.
- Mean serum bilirubin increased with disease duration, and anemia showed a positive correlation with the thorax-heart quotient, indicating cardiovascular strain.
Impact:
- Highlights the progressive nature of anemia in this pediatric cohort.
- Identifies severe clinical crises as critical points for Hb level monitoring.
- Underscores the detrimental effects of anemia on pediatric cardiovascular health, necessitating timely intervention.
Abstract:
In 819 children the Hb concentration, its dependence on the course of disease, the impact of various crises on Hb values and the behaviour of MCHC were evaluated. In the mean there was an anemia of 4.65 +/- 0.5 Hb mmol/l. In the course of 6 years a significant decline anemia of 4.65 +/- 0.5 Hb mmol/l. In the course of 6 years a significant decline of the Hb concentration could be observed. Only those crises connected with severe clinical symptoms (third degree) coincide with a significant Hb decrease. Every second child with a mean Hb value of 2.3 mml/l was transfused. In nearly half the cases there is a normochromic anemia, in 45.7% a hypochromic one. The mean serum bilirubin concentration lay at 31.6 mumol/l and increased with growing age of disease. The anemia has negative effects on the cardio-circulating system. The thorax-heart quotient shows a positive correlation to the degree of anemia.