سال:1391 | دوره: | شماره: |تعداد مقالات:8

Background: The present randomized clinical trial evaluated the efficacy of a topical lidocaine-prilocaine cream (EMLA) to relief pain during spinal puncture in pediatric oncology patients.Materials and Methods: Sixty patients with malignancy candidate for spinal puncture were selected for this study. The patients were randomly allocated to two groups. Group A had topical EMLA cream and group B had placebo, which they received 60 to 90 minutes before spinal puncture. Pain score was rated using the Wong-Baker Faces rating score (0: no pain to 10: worst pain).Results: Pain score does not reduce significantly in the topical EMLA cream in compared with placebo (p-value=0.126). No signs of infection were noted at the spinal puncture sites 24 or 48 hours after the procedure.Conclusion: Results obtained from this study represents that the EMLA cream has not more analgesic effect than placebo in reducing the pain for spinal puncture.

Background: Thalassemia is common in the Iranian population, and it must be considered in the differential diagnosis of the microcytic hypochromic anemia. The molecular analysis of b-thalassemia is necessary for prenatal molecular diagnosis. Α-thalassemia caused by loss of function of either one of the two duplicated a-globin genes or in less frequent non deletion mutations mostly located in the a2-globin gene.Materials and Methods: DNA were extracted from 100 whole blood using salting out method. The PCR was performed in two segments for entire b-globin gene and the a1 and a2-globin genes separately. Direct sequencing was carried out. The Gap-PCR was performed using published primers.Results: Clinical application of DNA analysis on thalassemic patients showed 42 persons have various b-thalassemia mutations, 48 persons with aa/-a3.7 deletion and 8 persons with non deletion mutations of a1 and a2-globin genes. These mutations determined by direct sequencing of entire b-globin, a1 and a2-globin genes and Gap-PCR for detection of deletions. Thirteen different b-thalassemia alleles were identified, the most common being IVS I-5 (G>C) and CAP+1 (A>C). The most a –globin mutation being aa/-a3.7 deletion.Conclusions: The frequency of mutations in North-east of Iran shows that these mutations are not the same as frequent mutation in other province of Iran. Feature study could determine molecular analysis of frequent mutations, which is useful for differentiating mild from severe alleles. In addition, mutation definition in carriers should be necessary for prenatal testing and genetic counseling.

Background Autologous platelet gel is easy to prepare and is relatively low cost. The aim of this study was to prepare and evaluate in vitro efficacy of autologous platelet gel.Materials and Methods In this experimental study, platelet concentrate and platelet poor plasma were prepared with aphaeresis method. Thrombin was prepared by mixing the plasma with calcium. Thrombin activity level was determined by spectrophotometric method. The platelet gel was obtained by adding thrombin and calcium to the platelet rich plasma. The concentration of growth factors was measured using the enzyme-linked immunosorbent assay technique.Results Platelet rich plasma contained a total average of 1×1011 platelet in the 100 ml product. The highest activity of prepared thrombin was achieved at a ratio of 5 vol (5 ml) platelet poor plasma to 1 volium (1 ml) calcium gluconate (p=0.02). Produced thrombin was stable for two hours at 24oC, six hours at 4oC, and more than three months at -20oC (p=0.04). Concentration of platelet derived growth factor and transforming growth factors-b in platelet gel supernatants were generally high (23.2±6 ng/ml, 18.2 ± 5ng/ml respectively), whereas epithelial growth factor and fibroblast growth factor were present at low concentration (870±110 pg/ml, 24.7±3 pg/ml respectively).Conclusion With proper production, large numbers of platelets in the platelet rich plasma will be activated, hence high concentration of growth factors will be produced.

Background: The aim of the present study was to determine the endocrine dysfunction in patients with major thalassemia, who receive hyper transfusion.Materials and Methods: This cross sectional study was performed during one year, which included 65 major thalassemia patients (31 females and 34 males), aged between 14 month to 27 years old (median 10, 3).Growth assessment was measured by height and weight according to age and BMI. For all patients serum ferrites concentration, serum calcium and phosphorus, alkaline phosphates, fasting blood sugar, thyroid stimulating hormone, free thyroxin, FSH, LH, dehydroepiandrosterone sulfate, testosterone, estradiol were measured.Results: In this study, 44.9% of patients were found to be shorter than the height of 5th percentile for their age.Diabetes mellitus and impaired fasting glucose were seen in 16% and 28.6% of patients respectively. There was significant correlation between serum ferritin level and impaired glucose tolerance test (p-value=0.043) in them. Frequency of hypocalcaemia and hyperphosphatemia were 36.9% and 10.7% respectively. Frequency of hypogonadism was 54.8%. Fourthy percent of the patients had no endocrine abnormalities.Conclusion: Endocrine dysfunctions are extremely frequent in patients with thalassemia, but chelating therapy can prevent or limit these complications. Impaired glucose tolerance was one of the most frequent endocrine complications, which were related to ferritin level.

Background: Prolonged P-wave duration and P-wave dispersion indicate interatrial conduction disturbance and early predict arrhythmia especially atrial fibrillation. Iron deposition in the atrial tissue might prolonged the atrial conduction time, and this study evaluated the P-wave prolongation and increased P-Wave dispersion in these patients.Materials and Methods: Fifty patients with major thalassemia and fifty healthy controls were studied, which age and sex matched between cases and controls. All patients and the controls were examined and had an ECG. The P-wave dispersion defined as the difference between the maximum and minimum P-wave duration. P-wave dispersion was compared between two groups. T-test and s correlation were used as needed.Results: There was a significant difference between the P-wave dispersion of the 2 groups (P=0.019). Maximum P-wave duration and P-wave dispersion in patients had strong correlation with age (disease duration). P-wave duration and P-wave dispersion had no correlation with regularity or irregularity of desferal use.Conclusion: Maximum P-wave duration and P-wave dispersion were seen in older cases, which significantly get prolonged. This could show that the myocardial involvement and dysfunction was probably more in the older patients.

Background: Phenyl ketonuria (PKU) is a congenital metabolic disease. Irreversible brain damage is the result of phenyl alanin accumulation, so its amount should be restricted in patients diet. In their diet, trace elements such as hem Iron, folic acid, vitamin B12 and, etc are also limited, and could represent anemia. In this study, the frequency of anemia in PKU patients in Yazd was investigated and compare with non-affected individuals.Materials and Methods: In this case-control study, all the PKU patients of Yazd who were under diet at least for 6 months were selected. The controls were selected from non PKU population and matched with cases according to age, gender and socioeconomic level. Hematologic factors were measured in both groups and analyzed using SPSS software using T-Test.Results: This study consisted of 18 patients aged between 1 to 18 years old. Full blood count and blood indices did not show significant difference (p>0.05), except MCHC (p<0.05). The blood level of vitamin B12 and Folic acid were significantly higher in the cases than controls (p<0.05), but ferritin was not significantly different between them (P>0.05).Conclusion: Our results did not show significant difference in presentation of anemia in patients with PKU and normal controls. Screening for anemia among PKU patients and taking supplements is recommended.

Introduction Cancers are one of the most common chronic diseases in children that cause fear and anxiety in them and their families. Praying is one of the methods can be used for reducing anxiety. In this study, we examined praying effect on anxiety in mothers with children suffer from cancer.Materials and Methods This randomized clinical trial was done on 60 mothers divided in two groups (n=30). In first group prayer therapy was done for three weeks by mothers. In control group prayer therapy was not done. Data was collected by state anxiety spilberger test and mean anxiety was compared between two groups by analytical statistical test.Results The results showed that difference between mean anxiety in two groups was significant (p=0.001), and mean anxiety reduced after praying from 56.2±13.9 to 40.9±12.4 in case group (p=0.001).Conclusion The results of this study showed that praying can reduce anxiety in mothers with children suffer from cancer and could be useful for them.

Histologically confirmed meningiomas in first year of life are quite rare. However, review of the sporadic cases in the literature reported meningioma in infants. Those have unique features in common.Case presentation An 11 month-old Iranian male infant presented with a several weeks history of decrease in movement of his right sided upper and lower extremities. Brain MRI revealed a large well-defined extra axial, Dural-based mass lesion, which is contained solid and cystic components. It is originated from falx, and showed intense contrast enhancement. The patient underwent a central craniotomy. The mass was removed sub totally. Microscopic examination of the specimen revealed a meningioma. Radiotherapy was deferred because of the young age. Patient follow up at 4 months later showed that the child was well.Conclusion Pediatric meningiomas show a higher frequency of cyst formation in compared with adult meningiomas. In addition, they show more frequent sarcomatous changes, which is male predominance. Absence of dural based attachment and higher incidence of intraventricular location were seen.