Exercise testing in children with Wolff-Parkinson-White syndrome: what is its value?

This study was conducted to evaluate the accuracy of exercise testing for predicting accessory pathway characteristics in children with Wolff-Parkinson-White (WPW) syndrome. The study enrolled 37 children with WPW syndrome and candidates for invasive electrophysiologic study (EPS). Exercise testing was performed for all the study participants before the invasive study. Data from the invasive EPS were compared with findings from the exercise testing. The sudden disappearance of the delta (Δ) wave was seen in 10 cases (27 %). No significant correlation was found between the Δ wave disappearance and the antegrade effective refractory period of the accessory pathway (AERP-AP) or the shortest pre-excited RR interval (SPERRI). The sensitivity, specificity, and positive and negative predictive values of Δ wave disappearance, based on AERP-AP as gold standard, were respectively 29.4, 80, 71.4, and 40 %. The corresponding values with SPERRI as the gold standard were respectively 23.8, 71.4, 71.4 and 23.8 %. Exercise testing has a medium to low rate of accuracy in detecting low-risk WPW syndrome patients in the pediatric age group.

Major beta-thalassemia, use of desferiexamine and renal proximal tubular damage.

BACKGROUND AND PURPOSE: Thalassemia is a quantitative hereditary hemoglobinopathy, which is one of common hereditary diseases in the Mediterranean region including Iran. Homozygotic beta-thalassemia patients have manifestations and complications such as severe anemia and multiorgan dysfunction. Recently there have been some concerns about renal complications in â-thalassemia but some controversies have been demonstrated. The aim of this study was to investigate the renal function of major â-thalassemia patients in comparison with control group. METHODS AND MATERIALS: This was a historical cohort. Study population consisted of major beta-thalassemia patients who were under a management plan of thalassemia ward of Bo Ali sina hospital sari, IRAN (case group ) and uninvolved brothers or sisters of the patients (the control group). Two groups (case and control) were matched. Renal function of patients was investigated by measurement of renal and serum markers. Demographic and therapeutic data of patients were extracted from medical records. The data were analyzed using SPSS 11. FINDINGS: Eighty-four samples were studied in total, while 42 of them were from the case group and the rest from the control group. The case and control groups were matched according to gender and age. Mean age of patients was 21.3 +/- 5.2 years. Desferiexamin was dosed in amount of 70 +/- 19 mg/kg. There was no significant difference in the measures of 24-hour urine protein (beta-2 microglobin and fraction excretion of Na and K between case and control groups. Significant differences were however in the measures of Bun serum and creatinin as well as K (from both urine and serum). Gender, Hb, and level of ferritin related significantly with differences. CONCLUSION: This study demonstrates no evidence of proximal tubular damage in major â-thalassemia patients. Nevertheless, the patients with severe anemia, high dose of desferal and high level of ferritin yielded an increase in the levels of Bun, serum K, and in some cases in uric acid (Tab. 4, Ref. 33).

Assessment of coagulation state and its related factors in thalassemia intermedia patients referred to thalassemia research center at Booali Sina Hospital Sari/IR Iran in 2007.

A high incidence of thromboembolic events have been reported among thalassemia intermedia patients especially in splenectomized patients. This study has been conducted to evaluate the coagulation state of patients referred to thalassemia research center at Booali Sina Teaching Hospital, Sari, Iran. This descriptive-cross sectional study was performed in 2007. Sixty thalassemia intermedia patients aged older than 10 years were enrolled. After recording demographic and therapeutic data of the participants, the plasma levels of Coagulant factors were measured. Obtained Data were analyzed using SPSS 13 software and t-test and chi-square tests. Of 60 studied patients, 62% were female. The mean age of the patients was 26.6 +/- 9.3 years. ASA tablet were used in 53.3%. None of the patients have experienced thromboembolic events. Decrease in protein C has been observed in 60%. Antithrombin III was decreased in 42% and protein S declined in 10% of the patients. Mutation in factor V leiden was seen in 3%. Splenectomy performed in 53.3% and there was no significant relation between low level of protein C and protein S, antithrombin III and presence of factor V Leiden with splenectomy (p > 0.05). Regarding to the high incidence of low levels of protein C, protein S and antithrombin III among thalassemia intermedia patients, there is an increased risk of thromboembolic events in the patients.

Prevalence of hemoglobin alpha-chain gene deletion in neonates in North of Iran.

OBJECTIVES: Alpha-thalassemia (alpha-thal) is one of the most common genetic disorders and in some populations has prevalence as high as 30%. Disorders in hemoglobin (Hb) synthesis lead to mild to severe reduction in alpha-chain synthesis. Diagnosis of alpha-thal by examining fresh blood taken from umbilical cord is a simple and appropriate approach, while in later stages its diagnosis will be difficult and costly. MATERIAL AND METHODS: This study examined the prevalence of alpha-thal gene deletion in neonates in Sari, Iran. Screening study was carried out by examining fresh blood samples obtained from excised umbilical cords of neonates born in Sari hospitals from June 2007 to March 2008. Complete blood count (CBC) was done and Hb electrophoresis and High Performance Liquid Chromatography (HPLC) were performed for detection of Hb Bart's band. For each case two slides were stained by vital stain, 20 and 120 minutes post blood collection. Prevalence of alpha-thal was calculated and statistically analyzed (p < 5%). RESULTS: 69 cases out of 680 (10.1%) were positive for Hb Bart's. In 16 out of 69 positive cases (22.3%) the results of two methods, electrophoresis and slide staining were in conformity. In 53 positive cases (77%) there was no visible band in Hb electrophoresis; however Hb Bart's was detected via vital staining method. If the ratio of mean corpuscular volume (MCV) to red blood cell (RBC) count is smaller than 23, risk of alpha-thal is 2.8 fold greater than cases with an MCV/RBC ratio below 23 (p < 0.05). None of the cases were reported to be positive for Hb H disease and hydrops fetalis. CONCLUSIONS: Considering high prevalence of alpha-thal gene deletions in neonates in Sari hospitals, it is recommended to screen newborns for alpha-thal in this city and similar areas with such a high prevalence. The sensitivity of cellulose acetate electrophoresis and HPLC methods is not adequately high to detect Hb Bart's in all positive cases and staining and examination of peripheral blood slides stained with vital staining is necessary.