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

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          Abstract

          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.

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          Most cited references17

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          β-Thalassemia

          β-Thalassemia is caused by reduced (β+) or absent (β0) synthesis of the β-globin chains of hemoglobin. Three clinical and hematological conditions of increasing severity are recognized: the β-thalassemia carrier state, thalassemia intermedia, and thalassemia major, a severe transfusion-dependent anemia. The severity of disease expression is related mainly to the degree of α-globin chain excess, which precipitates in the red blood cell precursors, causing both mechanic and oxidative damage (ineffective erythropoiesis). Any mechanism that reduces the number of unbound α-globin chains in the red cells may ameliorate the detrimental effects of excess α-globin chains. Factors include the inheritance of mild/silent β-thalassemia mutations, the coinheritance of α-thalassemia alleles, and increased γ-globin chain production. The clinical severity of β-thalassemia syndromes is also influenced by genetic factors unlinked to globin genes as well as environmental conditions and management. Transfusions and oral iron chelation therapy have dramatically improved the quality of life for patients with thalassemia major. Previously a rapidly fatal disease in early childhood, β-thalassemia is now a chronic disease with a greater life expectancy. At present, the only definitive cure is bone marrow transplantation. Therapies undergoing investigation are modulators of erythropoiesis and stem cell gene therapy.Genet Med advance online publication 03 November 2016.
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            Phenotype-genotype relationships in monogenic disease: lessons from the thalassaemias.

            The remarkable phenotypic diversity of the beta-thalassaemias reflects the heterogeneity of mutations at the beta-globin locus, the action of many secondary and tertiary modifiers, and a wide range of environmental factors. It is likely that phenotype-genotype relationships will be equally complex in the case of many monogenic diseases. These findings highlight the problems that might be encountered in defining the relationship between the genome and the environment in multifactorial disorders, in which the degree of heritability might be relatively low and several environmental agents are involved. They also emphasize the value of an understanding of phenotype-genotype relationships in designing approaches to gene therapy.
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              Psychosocial implications of Thalassemia Major.

              Many causes including the chronicity of disease, burden of treatment modalities, morbidities, and the expectation of early death resulting from the disease complications, may lead to psychosocial burden in Thalassemia Major (TM) patients. A total of 38 patients with TM and their mothers were recruited to evaluate the psychosocial burden as well as to disclose whether the psychological status of the patients contribute to the compliance with the therapy or to the contrary. Demographic and disease variables were obtained. Child Behavior Check-list (CBCL) was completed by the mothers of the patients. A detailed psychiatric interview based on the 4th edition of the Diagnostic and Statistical Manual diagnostic criteria was performed for each patient. Symptom Distress Checklist 90 (SCL-90) scale was given to all mothers for evaluating their psychopathology. Although CBCL scores remained between the normal ranges, desferrioxamine mesylate (DFO)-compliant patients and the patients with lower ferritin values had significantly higher scores. A total of 24% of the patients had a psychiatric diagnosis including major depression, anxiety disorder, tic disorder, and enuresis nocturnal. The psychiatric diagnosis was significantly higher in the patients who were compliant with desferrioxamine compared with the non-compliant group (P = 0.007). The SCL-90 scores indicated that the mothers who had a child with good adherence to DFO had higher scale scores than the mothers with a poor adherent child. The increase risk of psychosocial and behavioral problems in thalassemics and their parents indicated the importance of a lifelong psychosocial support for the prevention of mental health issues. The patients and their parents, who were more conscious of the illness, were more worried but more compliant with the therapy and need stronger psychiatric support.
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                Author and article information

                Contributors
                Role: ConceptualizationRole: Data curationRole: InvestigationRole: MethodologyRole: Project administrationRole: Writing - original draftRole: Writing - review & editing
                Role: ConceptualizationRole: Data curationRole: Formal analysisRole: InvestigationRole: MethodologyRole: Project administration
                Role: ConceptualizationRole: Data curationRole: InvestigationRole: MethodologyRole: Project administration
                Journal
                Ther Adv Ophthalmol
                Ther Adv Ophthalmol
                OED
                spoed
                Therapeutic Advances in Ophthalmology
                SAGE Publications (Sage UK: London, England )
                2515-8414
                20 April 2023
                Jan-Dec 2023
                : 15
                : 25158414231165824
                Affiliations
                [1-25158414231165824]Department of Ophthalmology, Adana City Training and Research Hospital, Adana, Turkey
                [2-25158414231165824]Department of Ophthalmology, Adana City Training and Research Hospital, Adana, Turkey
                [3-25158414231165824]Department of Pediatric Hematology, Adana City Training and Research Hospital, Adana, Turkey
                Author notes
                Author information
                https://orcid.org/0000-0002-0189-7863
                Article
                10.1177_25158414231165824
                10.1177/25158414231165824
                10126650
                490e9084-22b8-4d9e-a3ea-86e684881c4d
                © The Author(s), 2023

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 28 October 2022
                : 3 March 2023
                Categories
                Original Research
                Custom metadata
                January-December 2023
                ts1

                anterior chamber,anthropometric measurements,biometry,ferritin,thalassemia

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