Urinary early kidney injury molecules in children with beta-thalassemia major.

BACKGROUND: The aim of this study was to investigate novel urinary biomarkers including N-acetyl-ß-D-glucosaminidase (NAG), neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), and liver-type fatty acid binding protein (L-FABP) in children with ß-thalassemia major (ß-TM). MATERIALS AND METHODS: Totally, 52 patients (29 boys, 23 girls) with ß-TM and 29 healthy controls (3-17 years) were included. Various demographic characteristics and blood transfusions/year, disease duration, and chelation therapy were recorded. Serum urea, creatinine, electrolytes, and ferritin and urinary creatinine, protein, calcium, phosphorus, sodium, potassium, and uric acid in first morning urine samples were measured and estimated glomerular filtration rate (eGFR) was calculated. Routine serum and urinary biochemical variables, urinary NAG to Creatinine (U(NAG/Cr)), U(NGAL/Cr), U(KIM-1/Cr), and U(L-FABP/Cr) ratios were determined. RESULTS: Patients had similar mean serum urea, creatinine and eGFR levels compared with controls (p > 0.05 for all). The mean urinary protein to creatinine (U(Protein/Cr)) ratio was significantly higher in patients compared to the healthy subjects (0.13 ± 0.09 mg/mg and 0.07 ± 0.04 mg/mg, respectively; p < 0.001). Significantly increased U(NAG/Cr) (0.48 ± 0.58 vs. 0.23 ± 0.16, p = 0.026) and U(NGAL/Cr) (22.1 ± 18.5 vs. 11.5 ± 6.17, p = 0.01) ratios were found in ß-TM patients compared with healthy controls. However, no differences were found in serum and urinary electrolytes or U(KIM-1/Cr) and U(L-FABP/Cr) ratios between patients and controls (p > 0.05). Significant correlations were found between urinary biomarkers and urinary electrolytes (p < 0.05). CONCLUSIONS: Our results suggest that urinary NAG and NGAL may be considered to be reliable markers to monitor renal injury in ß-TM patients.

Bone mineral density in children with beta-thalassemia major in Diyarbakir.

Bone mineral status has extensively been investigated in adult thalassemics but less in thalassemic children. This study involves measurements of the bone mineral density (BMD), various demographic and biochemical parameters in 47 thalassemic children and 50 healthy controls with comparable age, sex, socioeconomic and regional distribution. Patients have significantly higher aspartate aminotransferase, alanine aminotransferase, phosphorous, osteocalcin, serum carboxy terminal teleopeptide fragment of type I collagen, intact parathyroid hormone (iPTH) and ferritin levels while they have significantly lower 25-hydroxy vitamin D (25OH-D), alkaline phosphatase and z-scores both at lumbar and femur compared to controls. Patients with high iPTH (30%) had significantly lower z-scores and 25OH-D while larger osteocalcin. We conclude that a significantly lower BMD in beta-thalassemic children compared with their healthy counterparts is a complex process and may partially attributed to their slower physical development, caused by iron overload and chelation therapy which may influence the liver as well as the endocrine tissues.

Assessment of thyroid function in children aged 1-13 years with Beta-thalassemia major.

OBJECTIVE: Hypothyroidism usually appears in the second decade of life and is thought to be associated with iron overload in patients with thalassemia major. This study aimed to evaluate thyroid dysfunctions in patients with beta-thalassemia major and to see if they appear in the earlier period of life. METHODS: Thyroid function and iron load status were evaluated in 90 children with a mean age of 7.17±3.78 years with beta-thalassemia major by measuring serum free thyroxin (FT4), serum free triiodothyronine (FT3), total thyroxin (T3), serum total triiodothyronine (T4), thyroid-stimulating hormone (TSH) and ferritin levels from serum of patients admitted to the Pediatric Department, Faculty of Medicine University of Dicle between March 2005 and July 2009. A control group formed from an age-sex matched healthy children with a mean age of 6.98±3.66 years was also included. A standard thyrotropin releasing hormone test was applied to 3 patients who had high TSH levels and were classified as subclinical primer hypothyroidism. The study was designed according to the Declaration of Helsinki and informed consent was obtained from the parents of all participants. FINDINGS: All thyroid parameters in patients were in the normal ranges compared with the controls except three of them which had high TSH levels. Serum ferritin level (2703±1649 ng/mL) in patients was significantly higher than in controls (81.5±15.5 ng/mL). CONCLUSION: The work implies that hypothyroidism could be even seen in the first decade of life in patients with beta-thalassemia major in spite of improved hematological cares.