Long-term Results of Splenectomy in Transfusion-dependent Thalassemia.

Splenectomy is indicated in transfusion-dependent thalassemia (TDT) only in certain situations. This study aimed to present the effectiveness, complications, and long-term follow-up results of splenectomy in children with TDT. We performed a 30-year single-institution analysis of cases of splenectomy for TDT between 1987 and 2017 and their follow-up until 2021. A total of 39 children (female/male: 24/15) were included. The mean age at splenectomy was 11.2±3.2 years, and their mean follow-up duration after splenectomy was 21.5±6.4 years. Response was defined according to the patient's annual transfusion requirement in the first year postsplenectomy and on the last follow-up year. Complete response was not seen in any of the cases; partial response was observed in 32.3% and no response in 67.6%. Thrombocytosis was seen in 87% of the patients. The platelet counts of 7 (17.9%) patients were >1000 (10 9 /L), and aspirin prophylaxis was given to 22 (56.4%) patients. Complications were thrombosis in 2 (5.1%) patients, infections in 11 (28.2%) patients, and pulmonary hypertension in 4 (10.2%) patients. Our study showed that after splenectomy, the need for transfusion only partially decreased in a small number of TDT patients. We think splenectomy can be delayed with appropriate chelation therapy up to higher annual transfusion requirement values.

Hypothalamic volume and asymmetry in the pediatric population: a retrospective MRI study.

This study investigated age- and sex-related changes in the volumetric development and asymmetry of the normal hypothalamus from birth to 18. Individuals aged 0-18 with MRI from 2012 to 2020 were selected for this retrospective study. Seven hundred individuals (369 [52.7%] Males) who had 3D-T1 sequences and were radiologically normal were included in the study. Hypothalamus volume was calculated using MRICloud automated segmentation pipelines. Hypothalamus asymmetry was calculated as the difference between right and left volumes divided by the mean (in percent). The measurement results of 23 age groups were analyzed with SPSS (ver.23). The mean hypothalamic volume in the first year of life reached 69% of the mean hypothalamic volume between 0 and 18 years (1119.01 ± 196.09 mm3), 88% in the second year. The mean volume of the hypothalamus without mammillary body increased in the five-age segment, while it increased in the six-age segment with mammillary body. Although the hypothalamus volumes of males were larger than females in all age groups, a significant difference was found between the age groups of 3-8 and 12-18 years (p < 0.05). In the pediatric brain, the hypothalamus was right-lateralized between 2.39% and 14.02%. The first 2 years of life were critical in the volumetric development of the hypothalamus. A segmental and logarithmic increase in the hypothalamus volume was demonstrated. In the pediatric brain, asymmetry and sexual dimorphism were detected in the hypothalamus. Information on normal hypothalamus structure and development facilitates the recognition of abnormal developmental trajectories.