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Factors associated with phosphate homeostasis in children with beta-thalassemia major: An analytical cross sectional
Lena Jafri1, Arsala Jameel Farooqui1, Bushra Moiz2
1Section of Chemical Pathology, Department of Pathology & Laboratory Medicine Aga Khan University (AKU), Karachi, Pakistan.
Insights
Children with beta-thalassemia major experience phosphate imbalances. Fibroblast growth factor 23 (FGF-23) and iron overload are key factors influencing phosphorus levels, necessitating multidisciplinary care for better management.
Area of Science:
- Pediatric Endocrinology
- Hematology
- Metabolic Bone Disease
Background:
- Children with beta-thalassemia major (β-TM) frequently develop metabolic bone diseases.
- Fibroblast growth factor 23 (FGF-23) plays a crucial role in phosphate regulation, and its levels in β-TM patients warrant investigation.
- Understanding phosphate homeostasis in β-TM is vital for managing associated complications.
Purpose of the Study:
- To assess phosphate homeostasis in children with β-TM.
- To investigate the role of FGF-23 and iron overload in phosphate dysregulation.
- To explore factors influencing serum phosphorus variability for improved clinical management.
Main Methods:
- Recruited 143 pediatric patients with β-TM.
- Conducted clinical and biochemical assessments, including serum ferritin, calcium, phosphate, vitamin D, and FGF-23 levels.
- Applied descriptive and inferential statistics, including multivariable regression analysis.
Main Results:
- Elevated FGF-23 (c-FGF23) was observed in 60.8% of patients.
- Vitamin D deficiency/insufficiency was prevalent in 92.3% of participants.
- Multivariable analysis identified c-FGF23, TMP:GFR, corrected calcium, iPTH, and their interaction as significant predictors of serum phosphorus variability.
Conclusions:
- Identified key contributors to serum phosphorus level variations in pediatric β-TM patients.
- Highlights the complex interplay of FGF-23, calcium, and PTH in phosphorus regulation.
- Recommends multidisciplinary care and further research for targeted interventions.
Introduction:
Children with beta-thalassemia major (β-TM) commonly experience metabolic bone diseases. Understanding fibroblast growth factor 23 (FGF-23) levels in these children can shed light on phosphate dysregulation. This study aimed to assess changes in phosphate homeostasis and associated factors, including FGF-23 and explore relationships between iron overload, FGF23 levels, and phosphorus regulation for clinical management of phosphate disorders, in children with β-TM.
Methods:
143 β-TM patients (57.3% male, median age 12 years) were recruited from Fatimid Foundation Karachi, a blood transfusion facility from January to October 2022. Clinical and biochemical evaluations were conducted at Aga Khan University Hospital, including serum ferritin, calcium (Ca), phosphate (P), vitamin D levels, and FGF-23. Descriptive and inferential statistics including multivariable analysis were applied.
Results:
This study enrolled 143 patients, with 57.3% males. The median age was 12 years, with 53% underweight. Blood transfusion rates varied, with 66.4% receiving 2/month. Bone/joint pain was reported by 76.2%, with 60.8% requiring analgesics. Median serum ferritin was 2768.3 ng/mL. Hypophosphatemia and hyperphosphatemia were observed in 5.6% and 3.5% of participants, respectively. Vitamin D deficiency/insufficiency affected 92.3%. Plasma c-FGF23 was elevated in 60.8%, while i-FGF23 was high in 14%. A low TMP-GFR (glomerular filtration rate) was associated with high c-FGF23 and low i-FGF23. Multivariable regression revealed c-FGF23, TMP:GFR, Corrected Ca, iPTH, and an interaction term between corrected Ca and iPTH as predictors of serum P variability (~75%).
Conclusion:
The study identified contributors to the variations observed in serum P levels in individuals with β-TM and recommends multidisciplinary care and prospective future studies to form targeted interventions for this population.
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