Point Shear Wave Elastography Detected Liver Stiffness Increased in Pediatric Patient With Thalassemia Major.

ABSTRACT: Transient elastography (TE) and point shear wave elastography (pSWE) are 2 elastographic ultrasound examinations used in liver stiffness (LS) measurement. It was shown that the LS value detected by TE in pediatric ß-thalassemia major patients has increased, and there was no LS evaluation obtained with pSWE in literature. Thus, in this study, it was aimed to evaluate LS with pSWE examination in children with thalassemia major and to determine LS-related parameters in these patients. Sixty-three schoolchildren with a diagnosis of ß-thalassemia major and 21 healthy controls between the ages of 7 and 18 years were included. In addition to routine anamnesis, physical examination, and laboratory examinations, renal and liver ultrasounds were performed. Liver stiffness values were measured by pSWE examination. Serum levels of urea, aspartate-aminotransferase, alanine-aminotransferase, iron, and ferritin were significantly higher in patients, and serum creatinine, iron binding capacity, and hemoglobin levels were found to be significantly lower (P < 0.05 for each). Liver stiffness values were significantly higher in patients compared with healthy controls. In linear regression analysis, serum iron and iron binding capacity values were found to be closely related with LS (P < 0.001 vs. ß = 0.482 and P = 0.047 vs. ß = 0.237, respectively). Liver stiffness values obtained by pSWE examination increase significantly in patients. According to the results of our study, in addition to the previously known TE method, we think that the LS evaluation obtained by pSWE, a new method that can make more accurate measurements, can be used in the possible early detection of target organ damage in children with thalassemia major.

Ocular biometry, anterior chamber morphometry, and their relationship with serum ferritin levels in children with beta thalassemia major.

Background: Ocular biometry and anterior segment evaluations are important to determine ocular development and pathological changes, especially in thalassemia patients in Mediterranean countries such as Turkey. Objectives: The objectives of this study were to compare ocular biometry and anterior segment parameters in children with thalassemia major and healthy controls and to examine the relationship between ferritin levels, anthropometric measurements, and ocular parameters. Design: This is a prospective case-control study. Methods: The height, weight, body mass index, and occipitofrontal circumference values of the participants were recorded. Anterior and vitreous chamber depth, lens thickness, axial length, central corneal thickness, anterior chamber volume, iridocorneal angle, pupil diameter, and mean keratometry were measured. Measurements were compared between patients and healthy children, and between patients with ferritin levels above and below 1000 ng/mL. Results: This study included 40 patients and 45 controls. Height, weight, and body mass index were significantly lower while ferritin level and occipitofrontal circumference were significantly higher in patients compared with the controls (p < 0.001 for all). There were no statistically significant differences in the other ocular parameters (p > 0.05). In comparisons between patients with ferritin levels below (n = 15) and above 1000 ng/mL (n = 25), there were no significant differences in age, height, weight, body mass index, occipitofrontal circumference, or ocular parameters (p > 0.05). Occipitofrontal circumference and mean keratometry value were positively correlated in patients with ferritin levels below 1000 ng/mL (r = 0.573, p = 0.025), while body mass index was negatively correlated with pupil diameter in patients with ferritin levels above 1000 ng/mL (r = -0.469, p = 0.018). Conclusion: Children with thalassemia showed significant growth retardation and large occipitofrontal circumference but did not differ from controls in terms of biometrics and anterior segment morphology. Our results demonstrated a positive correlation between the occipitofrontal circumference and mean keratometry value in children with ferritin levels below 1000 ng/mL and a negative correlation between body mass index and pupil diameter in children with ferritin levels above 1000 ng/mL.

Treatment of Wilms tumor using carboplatin compared to therapy without carboplatin.

BACKGROUND: Wilms tumor (WT) is the most common pediatric malignant primary renal tumor. One of the main drugs used in treatment is actinomycin-D. This was not readily available in Turkey at one time. Carboplatin was used in the primary treatment of WT in order to prevent delays in treatment. The aim of this study is to present the results of patients with WT receiving carboplatin or actinomycin-D therapy. PROCEDURE: Forty-eight consecutive patients with WT treated between July 2005 and December 2011 were included in this retrospective study. The patients were treated according to Turkish Pediatric Oncology Group guidelines. Nineteen patients were treated with actinomycin-D and 29 with carboplatin (500 mg/m(2) /dose). The two groups were then compared in terms of 2- and 4-year overall survival (OS), event-free survival (EFS) and disease-free survival (DFS). RESULTS: Two- and four-year OS rates in the carboplatin group were 90.0% and 90.0%, compared to 100.0% and 88.0%, respectively, in the non-carboplatin group. Two- and four-year EFS levels in the carboplatin group were 92.0% and 88.0%, respectively, compared to 82.0% and 76.0% in the non-carboplatin group. Two-and four-year DFS levels in the carboplatin group were 92.0% and 86.0%, respectively, compared to 77.0% and 77.0% in the non-carboplatin group. CONCLUSIONS: The findings show that the carboplatin can be used as an alternative drug in the primary treatment of WT in the event that actinomycin-D is unavailable or not tolerated.