ازدواج های فامیلی

These days with the improvements in science and technology the number of individuals who suffer from genetic disorders should be drastically less. A crucial method for preventing these disorders, in the first place, is by genetic counseling. Educating the public in regards to how they can avoid producing affected children. In this retrospective cross- sectional study, all the files of the couples attending Genetics Clinic of the Imam Khomeini Hospital Complex, during 1995 to 2004 were studied. The data were collected using a reliable questionnaire and SPSS software was applied for data analysis. The most important variable in this study was the relationship between the couples. The most frequent among the consanguineous marriages was the cross first cousins (45.3%), and the least frequency belonged to non-related marriages (6.4%). The most frequent genetic diseases belonged to children who their parents were parallel first cousins (84.8%). Due to the importance of genetic counseling in relation to reducing the frequency of the genetic disorders in society, educating the general public about compulsory genetic counseling before marriage, is the cheapest and the most effective approach in limiting the number of patients with genetic abnormalities.

To assess the relationship between parental consanguinity and permanent congenital hypothyroidism (PCH). From February 1998 to August 2002, cord dried blood spot samples, viz. only live births, in eight hospitals and a rural birth center in Tehran and Damavand were collected on Whatman BFC 180 filter papers. Samples with cord TSH  20 mU/L (two-site IRMA) were recalled. Between 7-14 days of life or thereafter, congenital hypothyroidism (CH) was confirmed by serum TSH > 10 mU/L and T4 < 6.5 g/dL and L-T4 was immediately started. CH-affected newborns were followed-up until May 2003. Thyroid dysgenesis was determined using 99m technetium pertechnetate thyroid scanning and/or ultrasonography. In thyroid eutopic newborns, dyshormonogenesis was diagnosed by a 4-week discontinuation of L-T4 between 2-3 years of age followed by abnormal serum TSH and T4 values. Of 35067 specimens, 373 (1.06%) were recalled, 25 had PCH (1:1403 births), 18 had thyroid dysgenesis (1:1948 births), and 7 had thyroid dyshormonogenesis (1:5010 births). Twenty-one PCH cases were detected from July 2000 onwards and 6648 (28.6%) of 23227 neonates had parental consanguinity. The odds ratio of parental consanguinity in PCH was 2.75 (95%CI: 1.17-6.47 P=0.02) and in dysgenesis was 3.74 (95%CI: 1.33-10.52 P=0.01) and the odds ratio of first-cousin parental consanguinity in PCH was 2.96 (95%CI: 1.23-7.15 P=0.02) and in dysgenesis was 3.21 (95%CI: 1.14-9.02 P=0.03) compared to non-PCH and non-dysgenetic cases, respectively. Parental consanguinity can be considered as the possible causative factor for the high prevalence of PCH and dysgenesis in Tehran.