الفهرس 
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Abstract
The thalassemia syndromes are heterogeneous groups of inherited anemias characterized by defects in the synthesis of one or more of the globin chain subunits of the haemoglobin tetramer.
The clinical syndromes associated with thalassemia arise from the combined consequences of inadequate haemoglobin production and imbalanced accumulation of globin subunits. The former causes hypochromia and microcytosis and the latter leads to ineffective erythropoiesis and haemolytic anaemia.
Types of ß-thalassemia syndromes include thalassemia major, thalassemia intermediate, thalassemia trait (minor), and thalassemia in association with Hb variants.
Clinical manifestations are diverse, ranging from asymptomatic hypochromia and microcytosis to profound anaemia, which can be fatal in utero or in early childhood if untreated. This heterogeneity arises from the variable severities of the primary biosynthetic defects and coinherited modifying factors. Palliative treatment of the severe forms by blood transfusion is eventually compromised by the concomitant problems of iron overload, alloimmunization and blood-borne infections.
Severe cases of beta thalassemia necessitate regular blood transfusion to diminish the chronic anaemia. Multiple blood transfusions, increased haemolysis and augmented gastrointestinal iron absorption led to iron overload, The excess iron saturates the capacity of the transferrin iron transport system that labile plasma iron circulate in blood with subsequent deposition in susceptible cells and generate reactive oxygen species (ROS).
Reactive oxygen species (ROS) increase lipid peroxidation inducing cellular dysfunction, apoptosis, and necrosis. Increase in lipid peroxidation products, apart from myocardial parenchymal damage from iron loading, is correlated with the occurrence of vascular endothelial complications in thalassaemic patients.
Endothelial damage and vascular dysfunction are associated with thalassemia, as shown by increased numbers of circulating endothelial cells (ECs), increased arterial stiffness, plays an essential role in cardiovascular diseases that starts at early age in thalassemic patients and leads to serious cardiovascular complications in further years that is the most common cause of death in patients with transfusion-dependent thalassemia.
Pulmonary hypertension is rather common in thalassaemic syndromes. Splenectomy, blood transfusion intensity (frequency and pre-transfusion haemoglobin), and degree of iron overload are the strongest predictors of pulmonary hypertension.
Recent studies have revealed that one of the novel markers of the vascular endothelial damage is pentraxin-3 (PTX3). Pentraxins-3 (PTX3), also known as tumoral necrosis factor (TNF)-Inducible gene 14 protein, is a 45 kDa protein that assembles to form high molecular weight multimers linked by interchain disulphide bounds. PTX3 is abundantly secreted by vascular endothelial and smooth muscle cells in response to inflammation and oxidized low-density lipoprotein (LDL). Vascular endothelial cells (EC) are a major source of PTX3 in response to inflammatory signals.
Current knowledge suggests that PTX3 plays important and not fully understood roles in vascular inflammation. Numerous studies conducted to this purpose have not completely elucidated the mechanisms involving this molecule. PTX3 interacts with a series of cellular receptors and substances, thus generating molecular effects especially on the vascular endothelium.
The aim of this study was to estimate the level of serum pentraxin-3 in patients with β thalassemia major in relation to pulmonary arterial hypertension.
We collected forty (40) adult patients with β thalassemia major treated in the Hematology Department of the Medical Research Institute. They were divided into 2 groups; group A which included 20 patients with pulmonary hypertension and group B which included 20 patients without pulmonary hypertension. Forty (40) healthy individuals with matched age and sex were recruited as a control group (group C).
Patients were subjected to the following:
• Full history taking
• Complete physical examination.
• Laboratory investigations:
- Complete blood picture. (Bain et al.,2011)
- Serum ferritin level. (Burtis et al., 2015)
- High sensitive CRP. (Burtis et al., 2015)
- Lipid profile. (Burtis et al., 2015)
• Estimation of serum pentraxin-3 by ELISA.
• ECHO cardiography.
There was a statistically significant difference between group A and group B as regards Desferal (P=0.028) and Jadnue (P< 0.001).
There was a statistically significant difference between group A and group B as regards the HCV (P=0.027), dyspnoea (P<0.001), palpitation (P< 0.001).
There was a statistically significant difference as regard to Hb level (p= 0.018), MCV (p=0.033), and MCH (p= 0.023) between group A and group B.
There was a statistically significant difference regarding Ferritin in group A when compared to group B (P<0.001). where it was higher in group A patients compared to group B.
There was a statistically significant difference regarding some lipid profile parameters in group A when compared to group B where HDL which was lower in group A than group B (P=0.014) and TG (P=0.004) and VLDL (P=0.003) which were higher in group A than group B.
There was a statistically significant difference regarding Hs CRP t in group A when compared to group B (P<0.001). where group A patients were higher than group B.
There was a statistical significance among the three studied groups with regards to Pentraxin-3(P<0.001), where group A patients recorded the highest level, then group B higher than group C.
There was a statistically significant difference regarding Tricuspid velocity (TRV) between the two studied groups (p<0.001) with group A higher than group B.
There was a statistically significant difference regarding Pulmonary artery systolic pressure (PASP), between the two studied groups (p<0.001) with group A higher than group B.
There was a statistically significant correlation between pentraxin 3 and hemoglobin in group A (p<0.001)
There was a statistically significant correlation between pentraxin 3 and Ferritin in group A (p<0.001)
There was a statistically significant negative correlation between pentraxin 3 and Cholesterol (p<0.001), LDL (p<0.001), HDL (p<0.001) and a statistically significant positive correlation between pentraxin 3 and TG (p<0.001) and VLDL (p<0.001) in group A.
There was a statistically insignificant correlation between pentraxin 3 and Hs CRP in group A patients (p=0.789).
There was a statistically significant correlation between pentraxin 3 and Haemoglobin (p<0.001) in group A.
There was a statistically insignificant correlation between pentraxin 3 and LVEF (p=0.687), TRV( p=0.546), PASP (p=0.546) and TAPSE(p=0.924) in group A patients.
There was a statistically significant correlation between Ferritin and Cholesterol (p<0.001), LDL (p<0.001), HDL (p<0.001), TG (p<0.001) and VLDL (p<0.001) in group A.
There was a statistically significant correlation between Hemoglobin and Cholesterol (p=0.001), LDL (p=0.004), HDL (p<0.001), TG (p=0.005) and VLDL (p=0.005) in group A.