Gino Schilirò, Antonio Russo, Cattedra di Ematologia Pediatrica, Istituto di Clinica Pediatrica I, Università di Catania, Viale A. Doria 6, I-95125 Catania (Italy) It is now widely accepted that the best treatment for thalassemia major is a precocious and regular transfusional regimen with the aim of maintaining pretransfusional Hb levels of 10.511 g/dl [1]. Such an approach, by minimizing the ...

Microcytosis is decrease of red blood cells (RBCs) size. The RBCs size is measured by the mean corpuscular volume (MCV). In the children, MCV varies by age and sex and so it must be always compared with sex and age-based norms. A MCV less than the 5 percentile defines the children microcytosis . The most frequent causes of microcytosis are iron deficiency anaemia (IDA) and haemoglobinopathies [...

The thalassemias are a heterogeneous group of disorders characterized by accumulation either of unmatched alpha or beta globin chains. These in turn cause the intramedullary and peripheral hemolysis that leads to varying anemia. A partial explanation for the hemolysis came our of our studies on material properties that showed that beta-thalassemia (beta-thal) intermedia ghosts were very rigid b...

BACKGROUND AND OBJECTIVE After the first National Census of transfusion-dependent thalassemics (1984), in 1992 the Italian Association of Pediatric Hematology and Oncology and the National Health Institute organized its 3rd edition. Here, results concerning Latium are presented and discussed. DESIGN AND METHODS Data for Latium, as in the rest of Italy, were collected by a single reference cen...

One hundred seven adult patients with thalassemia aged from 17 through 35 years and transplanted from HLA-identical siblings between November 1988 and September 1996 were evaluated on December 31, 1997. The outcome experience of 20 consecutive patients transplanted between November 13, 1988 and January 10, 1991 and reported in September 1992 is updated after 5 additional years. The experience o...

Dengue hemorrhagic fever (DHF) causing by dengue viral infection is endemic in Thailand and Southeast Asian countries where thalassemias are prevalent. Thalassemic patients are also at risk to acquire dengue viral infections and to develop DHF. However, they can have different clinical manifestations and complications as well as more severity than general population requiring special awareness ...

Tooth transposition, which is a rare condition, is ascribed to the disturbance of tooth eruption and the subsequent abnormal occlusal relationships. Transpositions mostly involve the upper jaw and more frequently occur between the maxillary canine and first premolar. Herein, we present a case of a maxillary canine‒second molar transposition in a thalassemic patient.

OBJECTIVE Thalassemia is one of the most common genetic disorders worldwide. Cap +1 mutation which causes 'silent beta thalassemia' is present around all ethnic groups of Pakistan. This study was designed to detect the frequency of Cap+1 mutation in Pakistani Population. METHODS Molecular genetic for Cap+1 beta thalassemic mutation was done by extracting DNA from whole blood by using Genomic ...

INDIAN PEDIATRICS 1072 VOLUME 41OCTOBER 17, 2004 In developing countries including Pakistan, due to lack of resources for universal and effective screening of blood donors for antibodies to hepatitis C virus (anti-HCV), blood transfusion is still a major source of HCV transmission. Therefore, in addition to other potential recipients of blood transfusion, the thalassemic patients on a long-term...

Preclinical and clinical studies demonstrate the feasibility of treating β-thalassemia and Sickle Cell Disease (SCD) by lentiviral-mediated transfer of the human β-globin gene. However, previous studies have not addressed whether the ability of lentiviral vectors to increase hemoglobin synthesis might vary in different patients.We generated lentiviral vectors carrying the human β-globin gene wi...