The Relation between Serum Hepcidin, Ferritin, Hepcidin: Ferritin Ratio, Hydroxyurea and Splenectomy in Children with ß-Thalassemia.

BACKGROUND: Hepcidin, a small peptide hormone, is established as the main regulator of iron homeostasis. AIM: To estimate serum hepcidin, ferritin, and hepcidin: ferritin ratio in ß-thalassemia patients and to determine the effect of splenectomy and hydroxyurea on serum hepcidin. METHODS: A study was conducted on 30 thalassemia major (ßTM), 29 thalassemia intermedia (ßTI) and 29 healthy children's controls. Data were collected by patient interviewing where detailed history-taking and thorough clinical examinations were carried out. Serum ferritin and hepcidin were measured by ELISA assay (Bioneovan Co. Ltd Beijing, China). RESULTS: Βeta-thalassemia patients had higher serum ferritin, serum hepcidin and lower Hb and hepcidin: ferritin ratio compared to the controls (p < 0.001, 0.010, 0.001, 0.001) respectively. Β-TM patients had higher mean serum hepcidin and serum ferritin compared to ß-TI, with statistically significant difference (P = 0.042, P < 0.001, respectively). Twenty-one patients out of 29 ßTI was on hydroxyurea therapy; these patients had significantly lower levels of serum ferritin (P < 0.004) and significantly higher levels of Hb (P < 0.004). Serum ferritin was statistically significantly higher in splenectomized patients P < 0.009. Serum hepcidin level was insignificantly higher in splenectomized patients than non-splenectomized patients (21.6 ± 14.75, 17.76 ± 10.01 ng/mL). Hepcidin showed a significantly positive correlation with hepcidin: ferritin ratio in all studied groups. CONCLUSION: Serum hepcidin was elevated in ß-thalassemia children with more evident elevation in ßTM patients. Splenectomy played no major role in hepcidin regulation. Knowing that hepcidin in serum has a dynamic and multi-factorial regulation, individual evaluation of serum hepcidin and follow up, e.g. every 6 months could be valuable, and future therapeutic hepcidin agonists could be helpful in management of iron burden in such patient.

Prevalence and Anti-Microbial Susceptibility of Hospital Acquired Infections in Two Pediatric Intensive Care Units in Egypt.

BACKGROUND: Hospital-acquired (nosocomial) infection is a common serious health problem worldwide, especially in pediatric intensive care units and is associated with high mortality and morbidity, prolonged hospital stays and high cost. AIM: To determine the types of organisms involved in hospital-acquired an infection in two pediatric intensive care units during the one-year study and its anti-microbial susceptibility. MATERIAL AND METHODS: This study was carried out in the pediatric intensive care units (PICU) of Ain Shams & Cairo Universities, where 86 pediatric patients were recruited. Their age ranged from 1 month to 156 months with mean 20.7 ± 25.8 months. Male to female ratio was 37:29. Four samples were collected from each child for culture and sensitivity: blood, endotracheal aspirate, urine and skin swab. RESULTS: The most common microorganism was staphylococcus while Gram-negative bacteria were the commonest group. Amikacin and imipenem are the most sensitive antibiotics. Risk estimate for different risk factors among studied patients revealed no significance. CONCLUSION: Staphylococcus was the commonest micro-organism while Gram-negative infections were the commonest group among PICU with a predominance of Acinetobacter and Klebsiella. Respiratory infections were the most common, followed by blood-borne infection. Risk factors for mortality were not significant.

Red cell alloimmunization and autoantibodies in Egyptian transfusion-dependent thalassaemia patients.

INTRODUCTION: The objective of this study was to explore the frequency of red cell alloantibodies and autoantibodies among ß-thalassaemia patients who received regular transfusions. MATERIAL AND METHODS: This study included 501 patients with ß-thalassaemia. This work planned to study the presence of alloantibodies and autoantibodies to different red cell antigens in multitransfused thalassaemia patients using the ID. Card micro typing system. RESULTS: Of a total of 501 ß-thalassaemia patients included in the study, 11.3% of patients developed alloantibodies; 9.7% of these alloantibodies were clinically significant. The most common alloantibodies were anti-K, anti-E and anti-C. The rate of incidence of these alloantibodies was 3.9%, 3.3% and 1.7% respectively. Autoantibodies occurred in 28.8% of the patients and 22.1% of these antibodies were typed IgG. There was a significant association between splenectomy with alloimmunization and autoantibody formation (p = 0.03, p = 0.001 respectively). There was no significant association between alloantibody, autoantibody formation and number of transfused packed red cells. CONCLUSIONS: Alloimmunization to minor erythrocyte antigens and erythrocyte autoantibodies of variable clinical significance are frequent findings in transfused ß-thalassaemia patients. There is an association between absence of the spleen and the presence of alloimmunization and autoantibody formation.