ABSTRACT
Objective To understand the status of endemic cretinism in Southern Xinjiang,and to put forward effective measures for diagnosis,prevention and control of the disease.Methods Follow-up visits were carried out to patients of endemic cretinism that at risk from 2006 to 2009,eliminating death and emigration cases of previous findings,increasing new cases,and all of the patients were conducted epidemiological survey and physical examination.Research contents included the history of patient's general situation,cousin marriage,family history,physical examination including mental retardation,hearing speech disorders in patients with thyroid function,nervous system disorder,thyroid palpation,low performance and cretinism faces,at the same time,a 3 ml venous blood sample was used to determine the thyroid function index.Results Totally 216 cases of endemic cretinism patients from 16 counties of Southern Xinjiang were confirmed,including 122 males and 94 females,aged between 11-20 years old.Neural endemic cretinism patients (167) were in the majority;its main performances were mental retardation,hearing speech disorders and nervous system disorders.Gummy swollen endemic cretinism patients(22) had thyroid hypofunction,but no nervous system disorders.Hybrid endemic cretinism patients (27) had manifestations of the neural and gummy swollen.Thyroid function index inspection results showed that the neural and hybrid endemic cretinism patients triiodothyronine [(2.41 ± 0.48),(2.38 ± 0.38) nmol/L],thyroxine [(118.72 ± 20.92),(120.77 ±21.55) nmol/L],free triiodothyronine [(5.25 ± 0.98),(5.14 ± 0.83) pmol/L],and free thyroxine [(15.81 ± 2.60),(15.58 ±2.99) pmol/L] were higher than those of gummy swollen patients [(1.51 ± 0.73),(39.25 ± 16.95) nmol/L,(3.02 ± 1.05),(6.42 ± 1.99) pmol/L,all P ＜ 0.05],but thyroid stimulating hormone [(2.26 ± 1.04),(2.83 ± 0.91) mU/L] was lower than that of gummy swollen patients [(58.56 ± 24.83) mU/L,P ＜ 0.05].Gummy swollen patients bad typical hypothyroidism,which was conformed to clinical manifestations,and all three types of patients had low thyroid antibody positive rate.Conclusions Southern Xinjiang is the prevalence area of endemic cretinism,the work of prevention and control has a long way to go,in addition to the need to improve the environmental iodine deficiency,it is urgent to improve the level of people's economy.
ABSTRACT
Objective To build a case database of endemic cretinism,investigate the distribution of existing cretinism,and provide suggestions for prevention of iodine deficiency disorders.Methods In 2011,an existing endemic cretinism case database was established in Gansu Province by verifying and registering the historical cases before 1985,and searching and diagnosing the cases between 1985-2011 in history cretinism areas and IDD high-risk areas according to endemic cretinism and endemic sub-clinical cretinism diagnosis (WS 104-104) and the IDD monitoring program of the central transfer fund at the same time,and analysis the distribution of existing cretinism.Results In 1985 and 2011,cretinism areas were 53 and 41 in Gansu Province,and the endemic cretinism cases werel9 696 and 4 715,respectively;18 cases of endemic cretinism were born between 1985 to1997,7 new cases were born after 1997;nerve cretinism,myxedematous and mixed types among existing cases were 58.8％ (2 766/4 708),9.8％ (461/4 708) and 31.4％ (1 481/4 708),males and females were 69.3％ (3 261/4 708)and 30.7％ (1 481/4 708),respectively.Cases older than 45 accounted for 73.2％ (3 444/4 708);the 7 new cases were all nerve cretinism and 5 cases distributed in ethnic minority areas.Conclusions There are potential dangers of endemic cretinism in the high-risk areas of iodine deficiency disorders due to multiple factors in Gansu Province;the nervous cretinism is the majority which is caused by insufficient iodine intake in early maternal pregnancy.To prevent new endemic cretinism,it is necessary to strengthen measures of emergency iodine supplementation for pregnant women and nursing mothers in the high-risk areas.
ABSTRACT
We observed the changes of cerebral nuclear T3 and its receptors and T4 5'-deiodinase activity in the fourth or fifth filial generations of the rats with long term iodine deficiency. The cerebrum was dissected on the 20th day postpartum. The results showed that the maximal binding capacity of nuclear T3 receptor was significantly lowered, but the affinity constant was essentially unchanged. Also, the concentration of nuclear T3 and the T4 5'-deiodinase activity were decreased markedly. It was concluded that 1) A decrease of cerebral nuclear T3 receptor was induced by long term iodine deficiency in rats; 2) T4 5'-deiodinase activity rather than serum T3 level could reflect more precisely the cerebral nuclear T3 concentration; and 3) The decrease of T3 receptor may presumably be the initial step of endemic cretinism caused by severe iodine deficiency.