Tehran University Medical Journal

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Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 !mso]> ject classid="clsid:38481807-CA0E-42D2-BF39-B33AF135CC4D" id=ieooui> Background: A high percentage of the patients at ENT or plastic surgery clinics have ENT abnormalities, either congenital or those developing later in life. Some are life threatening, while others cause disability and esthetic problems, with profound consequences for the affected child and the family. In addition, ENT abnormalities usually place stress on interpersonal relationships, causing social isolation, unhappiness and depression. The majority of these abnormalities is genetic and follows autosomal recessive pattern of inheritance. Geneticists believe that there is a higher frequency of autosomal recessive disorders among the offspring of consanguineous parents. The aim of this study was to find the frequency of ENT abnormalities among children born of consanguineous parents.
Methods: We studied 3503 files (pedigrees) of patients referred for genetic counseling at the Department of Genetics at Imam Khomeini Hospital, Tehran, Iran, from 1999 to 2000. We studied these cases according to degree of parental consanguinity, patterns of inheritance and gender using SPSS v.15.
Results: Of all 3503 files, 206 had ENT abnormalities, of which 157 had consangui-neous marriages. Among these 157 pedigrees, 496 cases had consanguineous parents. Out of the 496 cases, the four most frequent ENT abnormalities were: 115 cases (23.2%) of deafness, 53 cases (10.7%) of hearing loss, 12 cases (2.4%) of cleft lip and palate, and 5 cases (1%) of cleft palate.
Conclusion: This high frequency of ENT abnormalities indicates that consanguineous marriage should be discouraged by Iranian policy makers in order to reduce the burden of these disorders on society. Further research into the cultural problems that encourage consanguineous marriage and ways of resolving these problems is recommended. 

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Background: Long QT syndromes (LQT) are genetic abnormalities of ventricular repo-larization, with an estimated incidence of about one per 10000 births. It is characterized by prolongation of the QT interval in electrocardiogram (EKG) and associated with a high risk for syncope and sudden death in patients. Type of this syndrome is association with congenital deafness. Our objective was to evaluate QT interval in children with congenital deafness.
Methods: For 219 patients referred to Imam Khomeini Hospital audiometric clinic in 2011, questionnaire were completed. A total of 23 congenitally deaf children were incl-uded. All patients’ examinations were done by a pediatric cardiologist. Electrocardio-gram is conducted in all children (23 patients) with sever and deep congenital deafness. Then the QT interval was measured based on Bazett’s formula. Echocardiography was also performed in these children to assess left ventricular function and the presence of mitral valve prolapse.
Results: The overall patients were two hundred and nineteen children. A total of twenty three congenitally deaf children were included and electrocardiogram was obtained. Three children had obviously prolonged QTc (0.48±0.02) second. The median age of them was 6.1±5 year, the median weight was 18±11.3 kilogram and the median of QT interval was 0.48±0.02 second.
Conclusion: The QT interval obtained 0.48±0.02 second. In the present study we found prolonged QT in congenital deafness, thus we recommend to evaluate the electrocardio-gram of children with congenital deafness.