[Serum folate levels in major beta Thalassemia patients]

Beta major thalassemia is a variant of beta thalassemia syndrome which could be treated with bone marrow transplantation or if not available, regular blood transfusion. In the latter case, supportive therapy is the mainstay of treatment because of low folate intake or absorption. But the main cause of insufficient supportive therapy is the increasing need of bone marrow for ineffective erythropoiesis in the absence of regular blood transfusion. The purpose of regular blood transfusion in beta major thalassemia patients is to maintain the range of hemoglobin level between 9 and 11 gr/dl to stop insufficient erythropoiesis completely. Therefore, by regular blood transfusion, supportive therapy with folic acid would not be needed. The aim of this study is to determine serum folate level in regular transfused [3 major thalassemia patients in Mofid Children's Hospital during 2006. This is a cross sectional descriptive-analytic study performed on 100 beta major thalassemia patients receiving regular blood transfusion and desferal. Post-storage leukodepleted blood is used for transfusion. Patients' data is achieved from information data sheets. Serum folate level is determined with Electrochemiluminescence method in one of the most reliable laboratory centers. Normal serum folate level was 3-17.5 ng/ml in this laboratory with the sensitivity of 0.6 ng. Data analysis is performed with SPSS analysis software, and with chi squared, T-test and Spearman test. 56 [56%] girls and 44 [44%] boys entered this study with a median age of 156 [ +/- 71.2] months and an age range of 14-288 months. Patients' median hemoglobin level was 9.5 [ +/- 0.87] g/dl, with minimum of 7.5 and maximum of 11.9 g/dl. Mean MCV was 84.2 [ +/- 4.20] fl, with the range of 73.4 -95.3 fl. Seaim folate level was in the range of 1-19 ng/ml and median of 9 [ +/- 4.9] ng/ml. Serum folate was less than 3 ng/ml in 3% of evaluated patients. Hemoglobin level was equal or more than 9 g/dl in 73% of patients. It seems that if major [3 thalassemia patients receive regular blood transfusion, their serum folate level would be in normal range and supplementation therapy with folate will not be necessary

Frequency of red cell alloimmunization in patients with beta-major thalassemia in an Iranian referral hospital

Frequency of red cell alloimmunization in patients with ?-major-thalassemia in Mofid children's hospital. Tehran. IranThis is a cross-sectional descriptive study conducted in Mofid children's hospital, March 2007. A total of 121 major thalassemia patients on regular blood transfusion were included in this study. Clinical and laboratory data were collected and analyzed to find out the frequency, pattern and factors influencing red cell immunization secondary to multiple blood transfusions in these patients. Mean age of patients was 13 [6.19] from 2-26 years. They had received regular blood transfusions during periods ranging from 1 to 25.5 years. Red cell alloimmunization was found in 9 patients [7.4%]. In female group, 5 out of 66 [7.6%] patients and in male group 4 out of 55 [7.3%] patients had evidence of alloimmunization.The mean age of patients with alloimmunization was 9.6 [6.5] years [range 3.7-20]. Four patients [44.4%] with alloimmunization were more than 3 years old at the time of first blood transfusion. The mean age at first blood transfusion in patients with alloimmunization and without alloimmunization was 2.8 [2.4] and 1.7 [2.0] years [P=0.1]. The differential rate of splenectomy as a risk factor between patients with and without alloimmunization [11.1% and 8% respectively] was not statistically significant [P=0.5]. Direct or indirect antiglobulin tests were positive in 5 [62.5%] patients. The blood alloantibodies by a panel of antibodies using standardized blood bank methods were detected in 4 patients, which were of anti-K and anti-D types. he rate of red blood cell alloimmunization is relatively low in our patients. The age at first blood transfusion and splenectomy were not statistically significant as risk factors for alloimmunization in this study

[Hearing evaluation in children with meningitis by auditory brainstem response and otoacoustic emissions]

Meningitis is one of the most important causes of sensorineural hearing loss in childhood. Because of the critical situation of patients, proper attention is not usually paid to hearing assessment in meningitis. By early detection and medical intervention at proper time, the retadation of lingual development can be avoided. The hearing function of 40 children with meningitis was evaluated at 24-72 hours after diagnosis [acute period] and 24 hours before discharging from hospital [recovery period] with auditory brainstem responses [ABR]. All patients 7-14 days after discharge underwent thorough investigation by detecting otoacoustic emissions test [OAE]. Using the ABR test, during the acute period, 35 [87.5%] patients had normal hearing and in 5 [12.5%] patients severe to profound sensorineural hearing loss was detected. The same results were obtained during recovery period. OAE test showed normal hearing in 37 [92.5%] patients and impaired cochlear function in 3 [7.5%] patients. Early detection of hearing loss in the acute and recovery period of meningitis can be made by ABR and OAE tests

[Audiological assessment in neonates and children suffering from meningitis]

To assess neonates and children suffering from meningitis , during the critical and recovery periods , using ABR , EOAE and Behavioral Audiometry. 40 neonates to 12-year-old children were assessed. Using ABR test, the hearing of these cases was evaluated during the critical period-that is , 24-72 hours following the diagnosis. This test was repeated when patients recovered from meningitis-that is 24 hours before discharge [recovery period] Hearing function , again , was assessed 7-14 days following discharge through Immittance Audiometry, Behavioral Audiometry [PTA , SF and BOA]. And EOAE [TEOAE and DPOAE] tests procedures. During the critical period , ABR test revealed normal hearing in 35 patients [87.5%] and severe to profound sensorineural hearing loss [unilateral and bilateral] in 5 patients [12.5%-girl=4, boy=1]. The same result were obtained in reevaluation in the recovery period. On the other hand , 7-14 days after of discharge , Immittance Audiometry and Behavioral Audiometry [including PTA , S.F and BOA tests], confirmed the mentioned results [cross - gcheck]. EOAE test [DPOAE and TEOAE] in 35 normal hearing patients [according to ABR test] , showed normal cochlear function. 2 out of 5 cases suffering from severe to profound sensorineural hearing loss revealed normal cochlear function. According to EOAE results , abnormal cochlear function was evident in 3 patients. ABR , EOAE tests , and Behavioral Audiometry in children meningitis during the critical period and recovery period can be used to diagnose any degree of hearing loss with a high level accuracy. The results of persent study is confirmed by previous investigations