Investigation of the color discrimination ability using the Farnsworth-Munsell 100-hue test and structural changes by SS-OCT in patients with transfusion-dependent beta-thalassemia.

AIM: This study aimed to examine the color discrimination ability of patients with transfusion-dependent beta-thalassemia (TDß-T) in detail using the Farnsworth Munsell (FM) 100-hue test and to evaluate structural changes by swept source-optical coherence tomography (SS-OCT). MATERIAL AND METHODS: This prospective, sectional study included 40 patients (79 eyes) with TDß-T and 21 controls (42 eyes). The volunteers underwent a detailed ophthalmological examination and SS-OCT (DRI-OCT, Triton) imaging. Excluded were those with congenital color vision defects detected with the Ishihara pseudoisochromatic test. The patients' color vision was examined using the FM 100-hue test. The total error score (TES), the blue-yellow local error score (b-y LES), and the red-green local error score (r-g LES) were calculated. p <0.05 was considered significant. RESULTS: The mean age was 30.34±6.94 years in the patient group and 32.26±6.43 years in the control group (p = 0.078). The patient group had a significantly lower hemoglobin level (9.25±0.87 g/dL vs. 14±1.79 g/dL, p <0.001) and a significantly higher ferritin level (2665.56±2658.05 µg/L vs. 52.87±69.59 µg/L, p<0.001) compared to the control group. The mean TES, b-y LES, and r-g LES were higher in the patients than in the controls (64.84±30.18 vs. 28.45±16.55, p<0.001, 34.21±17.54 vs. 15.67±10.07, p <0.001, and 29.32±15.72 vs. 12.12±7.94, p<0.001, respectively). The patients had a higher b-y LES than r-g LES (34.21±17.54 vs. 29.32±15.72, p = 0.015). Choroidal thickness was lower in the patients than in the controls (284.34±63.55 µm vs. 324.98±88.05 µm, p = 0.043). CONCLUSION: We found that the color discrimination ability of the patients with TDß-T was reduced in both the r-g and b-y color axes compared to the controls, and their color discrimination ability in the b-y color axis was more affected than in the r-g axis.

Optical coherence tomography angiography findings in transfusion-dependent beta-thalassemia patients with and without splenectomy.

BACKGROUND: Ocular involvement is common in transfusion-dependent beta-thalassemia (TDß-T) patients. We aimed to investigate the effect of splenectomy on optical coherence tomography angiography (OCTA) findings in TDß-T patients. METHODS: The study is a prospective cross-sectional study. A total of 45 eyes of 23 patients with splenectomy (34.04±8.83 years), 18 eyes of 9 patients without splenectomy (27.44±5.43 years), and 54 eyes of 27 controls (33.22±6.44 years) were included. Vessel density in superficial capillary plexus, deep capillary plexus and radial peripapillary capillary, foveal avascular zone, choriocapillaris flow area, choroidal and retinal thickness detected by OCTA were evaluated. p < 0.05 was considered significant. RESULTS: Vessel density of superficial capillary plexus and deep capillary plexus were similar in patients with and without splenectomy, and controls. Choriocapillaris flow area was significantly decreased in patients with splenectomy than that in those without splenectomy and controls (2.02±0.12 vs. 2.17±0.1 and 2.14±0.12; p < 0.001). Choroidal thickness was significantly lower in patients without splenectomy than in patients with splenectomy and controls (260.05±61.02 vs. 305.11±42.13 and 298.89±29.14, p = 0.008). Parafoveal and perifoveal thickness of the full retina and outer retina were significantly lower in patients without splenectomy than in patients with splenectomy and controls (301.06±10.0, 279.78±10.28 vs. 311.04±14.89, 290.87±13.67 and 316.63±13.57, 289.56±9.31, p < 0.001 and p = 0.002; 174.72±7.81, 167.17±6.21 vs. 182.87±8.81, 173.60±7.09 and 185.11±9.26, 173.96±6.79, p = 0.001 and p < 0.001, respectively). CONCLUSIONS: OCTA findings can provide information about the microvascular effects of splenectomy on the retina of patients with TDß-T.

General health status and intelligence scores of children of mothers with acromegaly do not differ from those of healthy mothers.

PURPOSE: To determine the physical status and intelligence scores of children of acromegalic mothers and to compare them with those of children from mothers without acromegaly. METHODS: Six women with acromegaly who became pregnant under follow-up between 2010 and 2014 and their 16 children (group A) were assessed and compared with 16 children of healthy women (group B) and 15 children of women with prolactinoma (group C). The physical examinations of children were performed by the department of pediatric endocrinology and intelligence quotient (IQ) testing was undertaken by adult and pediatric psychiatry departments, using appropriate scales for their ages. RESULTS: Six of the 16 children (girls/boys: 7/9) were born after the diagnosis of acromegaly. Five of the 6 pregnancies occured when the patients were taking somatostatin analogs, none continued taking the drugs during pregnancy. The mean IQ of groups A, B, and C were 106.4 ± 12.5, 105.3 ± 12.5, and 103.2 ± 16.1 respectively (p > 0.05). The mean ages, birth percentiles, recent weight and height standard deviation scores were similar between groups (p > 0.05). Two siblings from group A and 1 child from group B were large for gestational age at birth. At recent follow-up, two children from group A were found tall for their age and one from group C was short for his age and was placed under the care of pediatric endocrinology clinic. CONCLUSIONS: Pregnancies in acromegaly seems to be uneventful and the general health status and IQ scores of children from women with and without acromegaly were found similar.