Background: b Thalassemia major is because complications in many organs of patients that it is due to anemia and iron overload in the body due to repeated injections of blood. Our country is on the thalassemia belt. About of kidney dysfunction in thalassemic patients in Iran has been only one study and in worldwide, researches are very limited. Hence, the purpose of this study is about impaired renal tubular function in pediatric patient with b thalassemia major. Methods: In this study, using the regular search published articles about of Impaired renal tubular function in pediatric patient with b thalassemia major has been studied and collected during 1997-2004. These studies were obtained by using the databases as ISI, SID. Results: Researches done in this field reviews were NAG levels and 2 b micro globulin for impaired renal tubular function. Results show that the mean NAG in patients with b thalassemia is more than healthy people, also between NAG and the duration of the disease was observed a significant relationship. Conclusion: The results of this study showed that is disturbed the pipes of renal function in patients with b thalassemia, which is characterized by increased concentrations of urinary NAG. Thus, increased urinary this enzyme should be considered as a primary marker of the pipes of renal damage in patients with βthalassemia major.

Introduction: To study the prevalence of most common  b-thalassemia mutations in Lorestan province and use the results for epidemiologic study and prenatal diagnosis of b_thalassemia major.Material and methods: 130 chromosomes from 65 unrelated homozygous b_thalassemia patients from Lorestan province of Iran (west-central) were investigated for b globin gene mutations by ARMS PCR.Results: Most common mutations of the Mediterranean region were examined in this study. We found a different mutation spectrum in Iran compared to the data obtained by other authors. Our results showed that codons 36/37 (-T) with a frequency of 33.84% represented the most common mutation followed by the following four mutations in the Mediterranean region, IVS-II-1 (G-->A), IVS-I-110 (G-->A), frameshift codons (FSC) 8/9 (+G) and IVS-I-5 (G-->C) with frequencies 27.69%, 11.53%, 10.76% and 4.47% respectively. The less frequent alleles, IVS-II-745 (C-->G), Codon 5(-CT), IVS-I (25 bp deletion) and Frameshift CD44 (-C) with the following frequencies 1.59%, 0.76%, 0.76% and 0.76%, respectively, accounted for only 3.87% of the mutations. No mutations in Codon 30 (G-->C), Codon 39 (C-->T), IVS-I-6(T-->C) and IVS-I-1(G-->A), were detected. The unknown alleles were 7.63%.Conclusion: These data suggest that the pattern of mutations in Lorestan province differs from those reported for the Mediterranean and other thalassemic regions in Iran.

objectives: Splenectomy sometimes needed in the path of longterm treatment of Thalassemia disease.Post SpleneetoiPortal vein thrombosis is a complication of splenectomy, one of the interesting cases with the aim faster diagnosis and management have been discussed here.Case Report: The patient is a 23 years old female with B-thalassemia major, who was admitted in Thalassemia ward, with complaints of fever, limb and, abdominal pain, dysuria and diarrhea. She had history of splenectomy (2 years ago), cholecystectomy (1.5 years ago) and diabetes mellitus.In the first and second gray scale ultrasonography which performed in the first and second weeks of admission except hepatomegaly due to iron deposition otherwise was unremarkable Portal and splenic venous thrombosis with cavernous fonnation with extension to superiormesentric vein was noted, in third grayscale ultra -sonography which performed 3 days after second ultrasonography. These findings were confinned with colour Doppler ultrasonography.In MRA , vascular network around stomach (endoscopy showed gastric fundal varices) in addition to extensive cavernous formation around portal vein was seen.For primary prevention of bleeding due to gastric varices propranolol equivalent to Img/kg/day was started.Discussion and conclusion: In major B-thalasemia patients, portal vein thrombosis have been seen after splenectomy, as two distinct acute and chronic forms.Due to hypercoagulability state(thrombocytosis,leukocytosis and increased rigidity of RBC) and splenic vein ectasia (due to splenomegaly) possibility of portal vein thrombosis in major B- thalassemic patients is high.Abdominal CT Scan with contrast is the best diagnostic method. In acute phase anticoagulative drugs (heparin, warfarin), thrombolytic drugs (t-P A) are useful. But in chronic phase(which is characterize with cavernous formation) a primary or secondary prevention of bleeding due to varices is regarded.

Dear Editor, The case report by Mirhosseini et al. explores the noteworthy association between Turner syndrome and βthalassemia major, emphasizing the clinical manifestations and the potential genetic factors that may clarify the relationship between these two rare conditions. While the findings warrant further consideration, a more detailed evaluation of the evidence and its implications is essential...