effect of quality of care on cardiovascular risk factors in newly diagnosed diabetic patients

In this study, we evaluated the quality of care and control of cardiovascular risk factors in newly diagnosed diabetic patients, identified during diabetes screening program, 1 year after diagnosis. In this prospective study, 83 newly diagnosed diabetic patients identified at screening in Isfahan, were studied. Height, weight, blood pressure, plasma glucose, lipids, and hemoglobin A1c [HbA1c] of these patients were measured 2 times, first at the time of diagnosis and then 1 year later, and the results were compared between two groups, with and without regular course of treatment. Nearly 46.99% and 53.1% of the studied patients have regular and irregular course of treatment. After 1 year, significant improvement in the mean of plasma glucose, cholesterol, triglyceride, low density lipoprotein [LDL], high density lipoprotein and HbA1c was seen in patients with regular course of treatment except for blood pressure [P < 0.05]. Frequency of controlled cardiovascular risk factors including fasting plasma glucose, HbA1c, cholesterol and LDL was significantly improved in patients with regular course of treatment [P < 0.05]. Mentioned changes were not seen in patients with irregular course of treatment. The findings of the current study demonstrated that though diabetes screening program result in earlier diagnosis of patients with type 2 diabetes, but it seems that regular follow-up and proper management of newly diagnosed patients is crucial for appropriate glycemic and metabolic control and preventing its related micro and macrovascular complication

Prevalence of permanent congenital hypothyroidism in Isfahan-Iran

Considering the importance to determine the reasons for the higher occurrence of congenital hypothyroidism [CH] in Iran, in this study we report the prevalence of permanent CH [PCH] in Isfahan province 7 years after initiation of CH screening program in Isfahan. In this cross sectional study, children with a primary diagnosis of CH studied. They clinically examined and their medical files were reviewed by a pediatric endocrinologist. Considering screening and follow-up lab data, radiologic findings and the decision of pediatric endocrinologists the final diagnosis of PCH was determined. A total of 464,648 neonates screened in Isfahan province. The coverage percent of the CH screening and recall rate was 98.9% and 2.1%, respectively. A total of 1990 neonates were diagnosed with primary CH. PCH was diagnosed in 410 neonates. The prevalence of PCH and transient CH [TCH] was 1 in 1133 and 1 in 294 live births. The most common etiology of CH was thyroid dyshormonogenesis. Though the prevalence of PCH is high, but the higher prevalence of CH in Isfahan is commonly due to cases with TCH. Hence, the necessity of determining new strategies for earlier diagnosis of patients with TCH is recommended

G395R mutation of the sodium/iodide symporter [NIS] gene in patients with dyshormonogenetic congenital hypothyroidism

Considering the high prevalence of congenital hypothyroidism [CH] in Isfahan and its different etiologies in comparison with other countries, the high rate of parental consanguinity, and the role of NIS gene in permanent CH due to dyshormonogenesis, the aim of this study was to investigate the G395R mutation of the NIS gene in patients with permanent CH due to dyshormonogenesis. In this case-control study, patients diagnosed with permanent CH due to dyshormonogenesis during CH screening program were selected. Venous blood sample was obtained to determine the G395R mutations of NIS gene using polymerase chain reaction [PCR] sequencing method. In this study, 35 CH patients with permanent CH due to dyshormonogenesis and 35 neonates with normal screening results as a control group were studied. We did not find any changes of the mentioned mutation of NIS gene in the patients' group. Considering the findings of the current study, it seems that further studies with larger sample size and with consideration of other gene mutations such as pendrin and thyroglobulin are needed for more accurate conclusion

role of maternal thyroid stimulating hormone receptor blocking antibodies in the etiology of congenital hypothyroidism in Isfahan, Iran

Considering the role of maternal thyroid stimulating hormone [TSH] receptor blocking antibody [TRAb] in the etiology of congenital hypothyroidism [CH], this study aimed to determine TRAb among patients with CH in Isfahan, Iran. In this case-control study, patients with CH and their mothers were compared with a group of healthy neonates and their mothers. Venous blood samples were obtained for measurement of TRAb using enzyme-linked immunosorbent assay [ELISA] method among mothers and their neonates. TSH of mothers was also determined. The case group consisted of 65 patients with CH and their mothers; controls were 148 healthy neonates and their mothers. The prevalence of positive TRAb in patients with CH and their mothers was higher than in the control group [81.5% vs. 1.3% in mothers and 80% vs. 0% in neonates, respectively, P<0.05]. The relationship between the TRAb and occurrence of CH was significant [P<0.05], whereas the corresponding figure was not significant for TRAb and the level of maternal and neonatal TSH in case and control groups [P>0.05]. It seems that autoimmunity has an important role in the etiology of CH. Further studies are necessary to determine other autoantibodies in CH patients

Hearing impairment in congenitally hypothyroid patients

Thyroid hormone is necessary for normal development of the auditory system. The aim of this study was to investigate the rate of hearing impairment in congenitally hypothyroid [CH] patients, and its relation with factors such as CH severity and age at starting treatment, during CH screening program in Isfahan. Hearing acuity was assessed in two groups of children with [94 patients aged 4 months - 3 years] and without CH [450], between 2000-2006. Otoacostic emission [OAE] was performed by a two step method. After two tests without OAE signals bilaterally, they were referred for auditory brainstem response [ABR] test. Subjects with both OAE and ABR abnormal test results were considered to have hearing problem. Obtained data was compared in case and control group and also CH patients with and without hearing impairment. Three [3.2%] of patients and 1 of control group [0.2%] were diagnosed with sensorineural hearing loss. The rate of hearing loss was not different significantly in two studied groups [P>0.05]. There was no difference between age of starting treatment and first T4 and TSH level in CH patients with and without hearing loss [P>0.05]. CH neonates with hearing impairment had thyroid dyshormonogenesis according to the follow up results. The rate of hearing loss was low among our studied CH patients. It may be due to proper management of CH patients. In view of the fact that all CH neonates were dyshormonogentic and considering the relation between certain gene mutations and hearing impairment in CH patients, further studies with larger sample size, with regard to different etiologies of CH should be investigated to indicate the possible gene mutations related to hearing loss in CH

case of congenital lipoid adrenal hyperplasia

Lipoid congenital adrenal hyperplasia [lipoid CAH], a rare disorder of steroid biosynthesis, is the most severe form of CAH. In this disorder the synthesis of glucocorticoids, mineralocorticoids and sex steroids is impaired which result in adrenal failure, severe salt wasting crisis and hyperpigmentation in phenotypical female infants irrespective of genetic sex. In this report, we presented a 28-day-old phenotypic female infant, which referred with lethargy, failure to thrive and electrolyte abnormalities. Considering the clinical and biochemical findings, lipoid CAH was diagnosed and replacement therapy with standard doses of glucocorticoid and mineralocorticoid and sodium chloride was initiated. During follow-up, she had good clinical condition, but at 6 years of age, she refers with hypertension and adrenal insufficiency because of arbitrary drug discontinuation by mother. In ultrasonography an abdominal mass [the testicles] was reported. Chromosome study showed 46XY pattern. Orchiectomy was performed. We recommended that in cases with clinical presentation of adrenal insufficiency if there is not the facility to determine the karyotype, repeated ultrasonography perform during follow-up. In addition, investigating the genetic bases of the disorder would help us to determine the pathogenesis of lipoid CAH in our community. It would be helpful in prenatal diagnosis and treatment of the disorder to prevent its related comorbidities

case of atypical mccune-albright syndrome with vaginal bleeding

McCune-Albright syndrome [MAS] is a rare non-inherited disorder characterized by the clinical triad of precocious puberty, cafe-au-lait skin lesions, and fibrous dysplasia of bone. We report a girl with MAS, presenting initially with vaginal bleeding at the age of 17 months. Ultrasonography revealed unilateral ovarian cysts and ureteral and ovarian enlargement. Considering the clinical and paraclinical findings, the patient diagnosed as a case of gonadotropin-independent precocious puberty was treated with medroxy-progestrone acetate [MPA] for three months. During the follow up, recurrent episodes of bleeding, ovarian activation and cyst formation, as well as breast size development were reported. At the age of 5.5 years, fibrous dysplasia was detected, which in coexistence with precocious puberty confirmed the diagnosis of MAS. The patient had no cafe-au-lait skin macles during follow up. Considering that clinical manifestations of MAS appear later in the course of recurrent periods of ovarian activation and cyst formation, a careful clinical observation and follow up of patients is necessary and the diagnosis of MAS must be kept in mind in cases with gonadotropin-independent precocious puberty

High frequency of IgM antiphospholid antibodies in young Iranian patients with stroke

To investigate the presence of IgG and IgM types of anticardiolipin [aCL] and antiphospholipid [aPL] antibodies in younger Iranian patients with ischemic stroke. Both IgG and IgM types of aPL [cardiolipin, anti phosphatidyl inositol, anti phosphatidyl serine, anti phosphatidic acid and beta 2-glycoprotein I [B2-GPI]] and aCL alone [cardiolipin and B2-GPI] were measured in 117 patients with ischemic stroke [aged <45 years] during an 18-month period from September 2002 to March 2004 in Al-Zahra Hospital, Isfahan, Iran. The demographic, clinical, and laboratory characteristics of patients with a positive titer were recorded. Seven men and 16 women [23 patients, 19.6%] had increased IgG types of aPL antibodies. Increased titers of IgM and IgG were found in 19 [82.6%] and 6 [26%] patients for aPL antibodies and in 15 [83.3%] and 8 [44.4%] cases for aCL alone. Despite European studies, high titers of IgM aPL antibodies found in a large number of patients can be caused by the presence of unknown triggering factors [infections or poisons], that are more prevalent in developing countries compared to developed countries. This hypothesis remains to be investigated further

[Relationship between pancreas echogenisity and insulin sensitivity during the course of OGTT in children with beta-thalassemia major aged 10-20 years]

Background: increased echogenicity of pancreas, due to hemosiderosis, is a frequent finding in beta thalassemic paitents. Hemosiderosis also leads to beta - cell dysfunction. So diabetes and glucose intolerance are common consequences of hemosiderosis. The aim of this study was to investigate the association of increased pancreas. echogenicity [IPE] with insulin sensitivity in beta - thalassemic children aged 10-20 years

Methods: after exclusion of thalassemic paitents with diabetes or familial history of diabetes, pancreas ultrasonography was performed in 42 beta-thalassemic children and they were divided into 2 groups with normal [21] and increased [21] pancreas echogenicity. Serum ferritin was measured, as well as serum insulin and glucose values, during an OGTT, at 0, 30, 60 and 120 minutes. A control group was selected randomly [n= 23]. Insulin Sensitivity Index and Fasting Glucose/Insulin Ratio were calculated and the data were analysed using t-test and ANOVA statistical methods

Results: serum feritin differed significantly between 2 groups of thalassemic paitents [P<0.005], but the insulin and glucose values were not significantly different among studied population [P>0.05]. Serum feritin was inversely correllated with ISI in patients IPE and 28.6% of them had IFG, as compared to patients with normal echogenicity [P<0.05]

Conclusion: regarding the detection of all IFG cases among thalassemic paitents with IPE and the relation of feritin with IS1 in this group, pancreas ultrasonography may be used to investigate the early stages of diabetes in these patients. however after conducting further studies with larger sample size and on older paitents are recommended