[Comparison of anthropometric measurements as substitutes for McNamara's cephalometric maxillomandibular unit measurements]

Cephalometric measurements cannot be performed for screening purposes, therefore orthodontists usually have to use either photographic or anthropometric measurements in such occasions. Finding a valid and reliable alternative could be of great value. The purpose of this study was to evaluate the correlation between McNamara's cephalometric unit difference and a proposed soft tissue equivalent. An anthropometric ruler was redesigned and used in this study. Soft tissue measurements were performed on 36 randomly selected specimens by two investigators. The soft tissue measurements included external auditory meatus to subnasale [Ext-Sn], external auditory meatus to soft tissue pogonion [Ext-Pog], and the difference between them [UDMA_]. These measurements were considered as equivalents to cephalometric indices in McNamara's anteroposterior measurements, including maxillary [Co-A] and mandibular [Co-Gn] unit length and their difference [UDMA], respectively. All soft tissue variables had an intra-class correlation coefficient [ICC] above 0.90. There was a high and significant correlation between cephalometric and anthropometric measurements [P<0.01]. The ICC between [Ext-Sn] and [Co-A] was 0.890, 0.869 between [Ext-Pog] and [Co-Gn], and 0.819 between UDMA and UDMA. The proposed anthropometric method showed a good correlation with cephalometric equivalents and the results show that this method could be used for screening purposes, especially when a low-cost, non-invasive method is required. However it cannot be considered as a substitute for cephalometry in diagnostic and treatment purposes

[ effect of hudroxyurea on extramedullary hematopoietic masses in patients with beta-thalassemia intermediate]

Since the need for transfusions in thalassemia intermediate [TI] patients is limited into some rare occasions they are almost always referred complaining of problems due to chronic anemia, bone marrow hyper-functioning [such as gall stones, osteoporosis] and also extramedullary hematopoiesis masses [EHM]. Different treatment modalities have been reported for managing the latter complication. We carried out this study to evaluating the effects of Hydroxyurea in reducing the size of such masses. The study was clinical trial comparing before and after characteristics of the patients referred to the Talassemia Research center from 2003 to 2006. Presentation of all was pain, weakness, paresthesia of legs and paraparesia/paraplegia. Diagnosis of EHM was confirmed by Spinal MRI [a 1.5 Tesla, Toshiba machine]. Hydroxiurea [Syrea, Medac, Germany] was administered 10-20 mg/kg/day. CBC, Urea, Creatinin were checked monthly. A Neurologist was observing the progression of patients. The second MRI was performed 3-6 months later. Another radiologist unaware of the sequence of imaging reported the changes of EHM during the period. Paired-t test and McNemar test were applied to compare the results. P< 0.05 was considered as statistically significant. Eight patients [7 males and 1 female] were enrolled in our study. Their age ranged from 26-44 years old. None had undergone transfusion, nor had they used pain killers. Neurologic symptoms were initiated from one week to a couple of months prior to their admission. They had administered Hydroxyurea in recommended dosage. The follow up takes 0.7 - 4.7 years. All subjects improved within 2 weeks, whereas none of them needed surgical procedures and radiotherapy. Both the MCV and NRBC changes were significant [P<0.02]. No considerable side-effects were noticed within the follow up period. HU is proved to be both effective and safe in treatment of EHM amongst patients with thalassemia intermediate who develop neurological symptoms. We suggest hydroxyurea as a "prophylactic agent" for several complications of TI

study of relationships between beta-globin gene mutations in beta-thalassaemia patients, in response to hydroxyurea treatment

Beta-thalassaemia is the most frequent inherited disorder in the world, especially in Iran and Mazandaran Province. It is caused by mulation in beta-globin gene on chromosome 11 with more than 150 different mulations causing beta-thalassaemia, has been identified in the beta-globin gene to date. Hydroxyurea, is one of the drugs used in Thalassemia patient's treatment, however, it is not effective in all patients. The mechanisms of the hydroxyuea effect in not clear yet. This study compared different beta-globin gene mutations in beta-thalassaemia patients who were referred to the Thalassemia Research Center in Sari in two groups, good responder and non-responder, to the hydroxyurea. This was a case-control study, comparing two groups of 30 thalassaemic patients who received hydroxyurea. Two groups were included, 30 good responders to hydroxyurea treatment [control] and 30 who did not respond to the treatment [case]. First, DNA was extracted from peripheral blood. Then, two different methods for mutation detection were used. In the Thalassaemia Research Center in Sari, mutations in 60 patients were identified using ARMS-PCR. Also the results were confirmed in Genetic laboratory of Amirkola, using two mutation detection methods, reverse-dot blot hybridization and ARMS-PCR. In the group of good responder [control], the average patient's age were 28/1 +/- 7/78 years, and the average age at the onset of blood transfusion was reported to be 8/5 +/- 8/56 year. In this group, the mean comparison of the hemoglobin level and red blood size [MCV] prior and after drug consumption were statistically significant. In the group of non-responder [case], the mean age was 21.3 +/- 6.43, the mean age starting blood transfusions was 3.3 +/- 3.75, and the mean of drug consumption was 2.3 +/- 0.8 months. From the mutations identified, IVSII-1G>A was the most common type in both case and control group, while of 30 of control group, 22 individuals were homozygous, and 7 individuals were heterozygous for this mutation [frequency% 42.5]. For the 30 case patients, 11 individuals were homozygous, while 11 were heterozygous [frequency% 27.5]. Comparison between two groups, case and control group, were statistically significance [P < 0.008]. The correlations of IVSII-1G>A mutation in good responder patients to hydroxyurea as compared to the non responder group, is significant and similar to the previous findings

[Therapeutic effects of carvedilol on systolic function in children with dilated cardiomyopathy and heart failure]

Heart Failure [HF] and Dilated Cardiomyopathy [DCMP] are two common heart diseases among children. Carvedilol is the third generation of beta-blocker and although it has been approved in adults, very little is known about its safety, efficacy and dosing profile in children. This study is to evaluate the efficacy and safety of Carvedilol in children with HF or DCMP. This is a randomized double-blind study. We studied 30 patients with HF or DCMP for 6 months. 16 patients [2 DCMP and 14 HF] underwent standard medical therapy in addition to Carvedilol and the remained ones [2 DCMP and 12 HF] received the same regimen, except the placebo instead of Carvedilol. We visited all patients on a monthly basis program and echocardiographically, evaluated them for their systolic function indices including LVEF [Left Ventricular Ejection Fraction], EPSS [E Point Septal Separation], LVESV [Left Ventricular End Systolic Volume] and LVEDV [Left Ventricular End Diastolic Volume]. Data were analyzed by t-test and Mann-Whitney test. After 6 months of therapy, most of systolic function indices showed a more significant improvement in the Carvedilol group, including increase of LVEF [P=0.033] and decrease of EPSS [P=0.008] and LVESV [P=0.019]. No significant side-effects were observed in our patients. Pearson correlation coefficient between some variables showed further improvement of LVEF and EPSS in males. Also, response of cardiomyopathic patients to Carvedilol was more than patients with heart failure. The Carvedilol is an effective and safe remedy for treatment of children with HF and DCMP

[Niemann pick type A, a case report]

Niemann Pick type A is a very rare hereditary disease with an incidence 1 in 20000-40000 live birth, which is calassified as a shingolipidoses. The disease is marked by the abnormal accumulation of sphingomyelin in most tissues, secondary to sphingomylinase deficiency. The most clinical manifestations are: Splenohepatomegaly-cherry red maculae-neuropathologic findings. This is a case report of an infant with clinical manifestation of Niemann Pick disease type A

Problems in standardization of orthodontic shear bond strength tests: a brief review

Bonding brackets to the enamel surface has gained much popularity today. New adhesive systems have been introduced and marketed and a considerable increase in research regarding bond strength has been published. A considerable amount of these studies deal with shear bond strength of adhesives designed for orthodontic purpose. Previous studies have used variety of test designs. This diversity in test design is due to the fact that there is no standard method for evaluating shear bond strength in orthodontics. Therefore comparison of data obtained from different study is almost impossible. This article tries to briefly discuss the developments occurred in the process of shear bond strength measurement of orthodontic adhesives with an emphasis on the type of test set up and load application. Although the test designs for measuring shear bond strength in orthodontics are still far from ideal, attempts must be made to standardize these tests especially in order to make comparison of different data easier. It is recommended that test designs be set up in such a manner that better matches with the purpose of the study