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Cardiac disease is the main cause of death in both forms of thalassemia; thalassemia major (TM) and thalassemia intermedia (TI). Pulmonary hypertension (PH) is one of the cardiopulmonary morbidities with high mortality that, if not treated, may trigger right-sided heart failure and premature death. PH is defined as a mean pulmonary artery pressure of =25 mmHg at rest or =30 mmHg during exercise. The prevalence of PH is known to be higher in TI than in TM. Moreover, the pathophysiology of PH in thalassemia appears to be sophisticated and complex. Risk factors for occurrence of PH consists of non-transfusion dependent thalassemia (NTDT), sub-optimally transfused transfusion dependent thalassemia (TDT), splenectomy, thrombocytosis, anemia, NRBC = 300 × 106, iron accumulation, history of thrombosis and older age. Other parameters which aggravate the risk of PH include hemolysis, oxidative stress, hypoxemia, alteration of erythrocyte membrane, decline of nitric oxide biological availability, arginine abnormal regulation and arginase excess. The screening method for PH is Doppler echocardiography but the gold standard for detection of PH is right heart catheterization (RHC). Current medical therapeutic options in PH comprise hydroxyurea, L-Carnitine, sildenafil, calcium channel antagonists, endothelin 1-receptor blockers and prostacyclin agonists. The only curative surgical method for the refractory and severe cases of PH is pulmonary endarterectomy. In this article, the etiology, pathophysiology, diagnostic methods and novel therapies of thalassemia associated PH are discussed. [ABSTRACT FROM AUTHOR]

Improved survival among transfusion dependent thalassemia patients in recent years has led to the manifestation of morbidities such as renal dysfunction. Renal injury is still an underestimated complication in ß thalassemia major patients. Chronic anemia, iron overload due to repeated transfusion, and specific iron chelators are the main factors in pathogenesis of renal dysfunction in ß thalassemia. Early identification of this morbidity allows us to delay the progression of kidney damage and therefore reduce renal impairment. In recent decades, novel biomarkers for early recognition of renal dysfunction have been studied in thalassemic patients, such as cystatin C, beta 2 microglobulin, alpha 1 microglobulin, N-acetyl beta-D-glucosaminidase (NAG), neutrophil gelatinase associated lipocaline (NGAL), kidney injury molecule 1 (KIM-1), liver type fatty acid binding protein (LFABP), and retinol binding protein (RBP). In this review, renal aspects of thalassemia with focus on novel biomarkers were discussed. [ABSTRACT FROM AUTHOR]

Background: Hepatic tumors are rare in children comprising only 1% - 4 % of all childhood solid tumors. Primary sarcomas of the liver are extremely rare and represent approximately 0.2 % of all liver tumors. Undifferentiated embryonal sarcoma of liver is an aggressive tumor with an unfavorable prognosis. Here we reported a case of undifferentiated embryonal sarcoma of liver in a 9-year-old boy who was misdiagnosed as hydatid cyst of liver. Case Report: An 8-year-old male patient was referred to our hospital following unsuccessful surgery for presumed hydatid disease of the right lobe of liver. Computed tomography (CT) and ultrasonography showed a large hepatic mass. Following the surgery, our patient received adjuvant chemotherapy. At the time of writing, it has been one year after the termination of adjuvant chemotherapy. Follow up imaging studies, including ultrasonography, CT scan, and MRI show no evidence of recurrence. Conclusion: Here we reported a case of undifferentiated embryonal sarcoma of liver in a 9-year-old boy mistaken for hydatid cyst of liver. Based on our observation, undifferentiated embryonal sarcoma of liver should be considered as a differential diagnosis among patients suspected of hydatid cyst of liver. [ABSTRACT FROM AUTHOR]