Is Iron Overload Related to Craniofacial Defects of Transfusion Dependent Thalassemia Patients? Study of Digital Morphometry from Eastern India

铁过载与输血依赖型地中海贫血患者的颅面缺陷有关吗?来自印度东部的数字形态测量学研究

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Abstract

Transfusion dependent thalassemia (TDT) is widely prevalent in India. Significant proportion of TDT patients show craniofacial deformity and also suffer from complications related to iron overload. There is dearth of data on quantitative dimensions of the craniofacial anatomy, more so from the eastern part of the country. We have attempted to obtain quantitative measurements of the craniofacial anatomy by digital method in TDT patients and compare with control. In addition, to also determine relationship of craniofacial defects with increasing serum ferritin (SF) in this group of patients. Sixty two TDT patients (28 males and 34 females), between age 15 and 35 years were studied over a period of four months along with 32 age and sex matched controls. Using a standard protocol, digital photographs were taken and measurements done using photoshop software. Analysis of 15 craniofacial parameters of upper, middle and lower parts of face was done and compared with control. Relationship of the SF level with each of these parameters was also studied. Compared to controls, both male and female TDT patients showed statistically significant difference ( p < 0.05) in bitragion width (BW), nose width (NW), sellion-subnasale length (SSL), lip length (LL), crest angle of nose (CNA)and angle of philtrum protrusion (APP), thus displaying defects in all of upper, middle and lower parts of face. In 44 patients, SF level > 2000 µg/Lwas found to be statistically related to BW and nasal root width (NRW). No such relationship was noted with the other 18 patients with lower SF levels. Measurement of craniofacial parameters by digital method is a convenient method to acquire a comprehensive idea about the anatomical defects of TDT patients. We have also shown that some of these defects may be related to iron overload.

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