Thyroid ectopia is a Dysgenesis of Thyroid gland and Lingual position represents the most frequent ectopic location accounting up to 90% of ectopic cases. HypoThyroidism is commonly present because of absence of a normal Thyroid gland in most instances. Primary hypoThyroidism in juvenile population generally leads to retardation of linear growth and delay or even arrested puberty. We present a 20 years old female with typical and profound presentation of hypoThyroidism due to lingual Thyroid.

An ectopic Thyroid is a form of Thyroid Dysgenesis in which an entire or parts of the Thyroid gland may be located in another part of the body than what is the usual place. The most frequent location is the base of tongue. Although most cases are asymptomatic, symptoms related to tumor size and its relationship with surrounding tissues, hormonal dysfunction and seldom malignancy may also occur. Any disease affecting the Thyroid gland may involve the ectopic Thyroid, including malignancy.

Objectives: Thyroid hemiagenesis (THA) is a congenital anomaly of unknown origin. The aim of this study was to assess for the firsttime whether there is any seasonality in the date of birth of patients with THA in comparison to general population.Methods: The studied group consisted of 105 patients with THA. The control data regarding total live births in Poland were derived from the Polish Statistical Annals (n=2 421 384). Both groups were subdivided according to the month of birth, season of birth and quarter of the year.Results: Analysis of the months of birth revealed that patients with THA were born significantly less often in October than control subjects (P=0.0217). Although a trend is observed, that more patients with THA were born in the 1st quarter of the year and in winter, while THA patients were less often born in autumn or in 4th quarter of the year, the difference did not reach statistical significance.Conclusions: Month of birth may exert some effect on the risk of developing THA. Studies on larger cohorts are needed to confirm the observation. The results suggest a potential modifying effect of environmental factors in the pathogenesis of THA.

Objective: To determine the prevalence of congenital hypoThyroidism (CH), permanent and transient CH.Methods: From November 2006 to September 2007, 63031 newborns were screened by measuring serum TSH obtained by heel prick. The neonates who had a TSH³5mU/L were recalled for measurement of serum T4, Thyroid stimulating hormone (TSH) and TSH receptor blocking antibodies (TRBAb) in venous samples. In 43 primarily diagnosed as cases of CH, treatment was discontinued at age 2-3 years for 4 weeks and T4 and TSH were measured again. Permanent or transient CH was determined from the results of these tests and radiologic evaluation.Findings: The incidence of congenital hypoThyroidism was found to be 1: 1465 with a female to male ratio of 1.19: 1. The most common clinical findings were prolonged jaundice (73%), large anterior fontanel (56%) and wide posterior fontanel (55%). In 43 patients with CH, prevalence of permanent and transient form of the disorder was 53.6% and 46.4% respectively. Permanent CH was associated with higher initial TSH level than transient hypoThyroidism (P<0.001). The most common etiology of permanent CH was dyshormonogenesis (57%). TRBAb was found in 6.8% of the total 43 cases.Conclusion: Congenital hypoThyroidism in Iran may have different etiologies. Due to higher rate of transient CH than other similar researches, it is reasonable to follow these patients for a longer period to rule out the possibility of permanent hypoThyroidism.

Objective: In Iran Thyroid-stimulating hormone (TSH) based neonatal screening program is included in health care services from 2005 for detection of patients with primary congenital hypoThyroidism (CH). This study was performed for a critical evaluation of the screening program primary congenital hypoThyroidism in Fars province, Iran.Methods: From November 2006 to September 2007, TSH serum concentrations of 63031 newborns, 3 to 5 days old born in Fars province, were measured by heel prick. The newborns with TSH ³ 5mIU/L were recalled for measurement of serumT 4 and TSH in venous blood samples Findings: Of 127 recalled subjects, 43 were confirmed to be hypoThyroid, showing a prevalence of 1: 1465 with F: M ratio of 1.05: 1. The most common clinical and radiological findings were prolonged jaundice (73%), large anterior fontanel (56%), wide posterior fontanel (55%), absence of distal femoral epiphysis (20%), and umbilical hernia (11%). Scintigraphy of the Thyroid with 99m TC revealed eutopia (67.4%), hypoplasia (23.3%), agenesis (4.7%) and ectopia (2.3%).Conclusion: It is concluded that a cut off value of TSH³5mIU/L overestimates recalling the number of patients with CH. The most common cause of congenital hypoThyroidism is not Dysgenesis of the gland and perhaps dyshormonogenesis in Iran is more common than what is reported in other countries.

“pure gonadal Dysgenesis” is applied to one particular variety of defective gonadogenesis. XX gonadal Dysgenesis is a type of female hypogonadism in which no functional ovaries are present to induce puberty in an otherwise normal girl whose karyotype is found to be 46, XX. With nonfunctional streak ovaries she is low in estrogen levels (hypoestrogenic) and has high levels of FSH and LH. The usual phenotype of 46, XX gonadal Dysgenesis includes normal stature, sexual infantilism, bilateral streak gonad, normal female internal and external genitalia, and primary amenorrhea. The streak gonad occasionally produces estrogens or androgens, but malignant transformation is rare. Incomplete forms of this condition may result in hypoplastic ovaries that produce enough estrogen to cause some breast development and a few menstrual periods followed by secondary amenorrhea. This heterogeneous syndrome occurs sporadically or with autosomal recessive inheritance and in some instances is associated with other congenital malformations; some familial cases have been associated with sensorineural deafness (Perrault’s syndrome). we report 3 siblings with pure gonadal dysgenesia with or without normal secondary sexual characteristics in one family. In conclusion, one should think of pure gonadal dysgenesia with different presentation like primary amenorrhea with or without normal secondary sexual characteristics in one family.

Background: Using ionizing radiation in diagnosis and treatment is of great importance. As early diagnosis in some diseases only can be done by using radiation, in treatment phase, radiotherapy is also the main center for healing patients with cancer. Today, one of occupational hazards is ionizing radiation which can cause serious and irreparable damages in radiation workers. This study aimed to count blood cells and evaluate liver enzymes and Thyroid-stimulating hormone (TSH) in radiation workers in hospitals in Kurdistan Province, Iran.Methods: In this case-control study, blood cells, liver enzymes, and TSH levels were compared in 142 radiation staff (cases) and also 142 workers in other sections of hospitals. Matching was done for confounding factors. The statistical analysis was performed using SPSS software at the significance level of P<0.05.Findings: Mean number of white blood cells and the level of serum alanine aminotransferase (ALT) enzyme in radiation staff were significantly different from that of the control group. But no significant difference was observed between other parameters.Conclusion: It seems that working in radiation wards can change some blood factors but cannot predict the recieved dose. In order to increase the safety of radiation workers in radiation wards, monitoring of these individuals should be done annually using cytogenetic methods.

Background: Gonadal Dysgenesis, the most common cause of primary amenorrhea, is characterized by absent or underdeveloped ovaries. Although the coexistence of gonadal Dysgenesis and Mayer-Rokitansky-Kü ster-Hauser (MRKH) has been reported, it is still quite infrequent. To the extent that authors searched, just one study reported the association between Rokitansky sequence and Dandy-Walker malformation. Clinical Presentation and Intervention: We aimed to report a case with gonadal Dysgenesis, MRKH, and the Dandy-Walker variant. In this care report, the authors reported a 15-year-old girl with primary amenorrhea and underdeveloped secondary sexual properties. Her karyotype was 46, XX. The abdominopelvic MRI without contrast demonstrated bilateral ovarian agenesis and no uterus or cervix. Vagina was normal in length. Brain MRI was consistent with the Dandy-Walker variant. Conclusion: Although some affected chromosomal regions have been identified, further genetic analyses should be performed to elucidate the probable association between these anomalies.