Renal function in ß-thalassemia major patients treated with two different iron-chelation regimes.

BACKGROUND: Renal injury in transfusion dependent ß thalassemia patients (TDT) has been attributed to iron overload, chronic anemia and iron-chelation therapy (ICT) toxicity. We studied renal function in TDT patients treated with two different ICT regimes. PATIENTS AND METHODS: We studied 36 TDT patients: 26 received deferasirox (DFX) and 10 were treated with deferoxamine (DFO) +/- deferiprone (DFP). RESULTS: Increased uNAG was found in 30% of the DFX group vs. 10% of the DFO+/-DFP group, the mean uNAG level in the DFX group was significantly higher than in the DFO+/-DFP group, (P < 0.05). A moderate negative correlation was found between uNAG levels and mean serum ferritin for the prior 10 years (P = 0.03), more pronounced for the DFO+/-DFP group. Twenty nine patients had had their renal function evaluated 10 years earlier; eGFR significantly declined in patients switched to DFX (P = 0.0093) but not in patients who continued DFO+/-DFP. CONCLUSIONS: A high prevalence of renal tubular damage was observed in our TDT patients, particularly those treated with DFX; uNAG was negatively associated with mean 10-year serum ferritin, suggesting ICT's involvement in tubular injury. A significant decline in eGFR compared to a decade earlier was observed only in patients currently treated with DFX. Strict follow-up of renal function in TDT patients is warranted.

Dream Doctor Intervention Instead of Sedation: Performing Radionuclide Scanning Without Sedation in Young Children: A Study in 142 Patients.

BACKGROUND: Dream Doctors (DDs; professional medical clowns) are expanding their activities in pediatric wards. DDs were introduced as an alternative to sedation among children undergoing kidney imaging after urinary infection. The imaging requires that the patient lie completely still under the camera during the scan; otherwise the image cannot be interpreted. It is extremely difficult to get these children to cooperate, and pediatricians have to provide sedation to get good results. Giving sedation requires medical observation, and it can take hours until the child can be safely released from the hospital. METHODS: A DD intervention was introduced in an attempt to avoid sedation in these young children. The DD tried to gain the child's cooperation during the procedure. Each study was subsequently scored by a radionuclide physician to assess study quality and interpretation. RESULTS: A total of 142 patients were studied over a 14-month period. The mean age was 2 ± 1.6 years. During the study, in the presence of the DD, only five (3.2%) patients required pharmacologic sedation compared with 100% before the study. CONCLUSIONS: The introduction of DDs proved to be a good alternative to sedation in cases where the procedure does not involve pain and only requires the child's cooperation.

Iron Deficiency and Iron-deficiency Anemia in Toddlers Ages 18 to 36 Months: A Prospective Study.

In young children, iron deficiency (ID)-the most common cause of anemia-may adversely affect long-term neurodevelopment and behavior. We prospectively evaluated the prevalence of ID and iron deficiency anemia (IDA) in 256 healthy 18- to 36-month-old children in Northern Israel. Complete blood count and ferritin evaluation were performed, and risk factors were assessed. Hemoglobin (Hgb) was compared with first-year routine screening. Complete data were obtained from 208 children: 56.2% were boys; the mean age was 26.1±5.27 months. A prevalence of 5.8% IDA, 16.3% ID without anemia, 9.6% anemia with normal ferritin, and 68.3% normal Hgb and ferritin was found. In nonanemic infants at 1 year of age (n=156), ID/IDA was found in 19.9%, and 12.8% became anemic at study evaluation. Despite iron supplementation in the first year, and normal Hgb at first-year screening, ID and IDA were still prevalent, and might develop during the second year of life. Recognition of this child subset and consideration of iron supplementation are mandatory.

Non-transferrin bound iron in Thalassemia: differential detection of redox active forms in children and older patients.

Non-transferrin bound iron (NTBI) is commonly detected in patients with systemic iron overload whose serum iron-binding capacity has been surpassed. It has been perceived as an indicator of iron overload, impending organ damage and a chelation target in poly-transfused thalassemia patients. However, NTBI is a heterogeneous entity comprising various iron complexes, including a significant redox-active and readily chelatable fraction, which we have designated as "labile plasma iron" (LPI). We found that LPI levels can be affected by plasma components such as citrate, uric acid, and albumin. However, the inclusion of a mild metal mobilizing agent in the LPI assay (designated here as "eLPI"), at concentrations that do not affect transferrin-bound iron, largely overcomes such effects and provides a measure of the full NTBI content. We analyzed three distinct groups of poly-transfused, iron overloaded thalassemia patients: non-chelated children (3-13 yrs, Gaza, Palestine), chelated adolescents-young adults (13-28 yrs, Israel), and chelated adults (27-61 yrs, Israel) for LPI and eLPI. The eLPI levels in all three groups were roughly commensurate (r(2) = 0.61-0.75) with deferrioxamine-detectable NTBI, i.e., DCI. In older chelated patients, eLPI levels approximated those of LPI, but in poly-transfused unchelated children eLPI was notably higher than LPI, a difference attributed to plasma properties affected by labile iron due to lack of chelation, possibly reflecting age-dependent attrition of plasma components. We propose that the two formats of NTBI measurement presented here are complementary and used together could provide more comprehensive information on the forms of NTBI in patients and their response to chelation.

Non-transferrin-bound labile plasma iron and iron overload in sickle-cell disease: a comparative study between sickle-cell disease and beta-thalassemic patients.

BACKGROUND: Blood transfusions are the standard of care in b thalassemia and transfusions are also indicated in sickle cell disease (SCD) patients with hypersplenism, recurrent vaso-occlusive crises and for stroke prevention. Iron overload caused by blood transfusions in thalassemia and in SCD may affect morbidity and mortality. Recent studies of iron overload in SCD suggest that the biologic features of SCD and the chronic inflammatory state may protect SCD patients from iron damage. DESIGNS AND METHODS: In view of the controversy regarding the effect of iron overload in patients with SCD we studied the iron status, including non-transferrin bound iron (NTBI) and labile plasma iron (LPI) levels in a cohort of 36 SCD patients and compare the results with 43 thalassemia patients. RESULTS: Our results indicate that none of the SCD patients had clinical symptoms of iron overload. Only two SCD patients had NTBI values in the gray zone (0.4 units) and none had positive values. By contrast, 14 patients with thalassemia major and three with thalassemia intermedia had NTBI values above 0.6, level that are in the positive pathological range. Similarly, four thalassemia patients, but only one SCD patient had positive LPI levels. CONCLUSIONS: The parameters of iron status in SCD patients, even after frequent transfusions are different when compared to patients with thalassemia. The low NTBI and LPI levels found in patients with SCD are in keeping with the absence of clinical signs of iron overload in this disease.

The incidence of anemia in an Israeli population: a population analysis for anemia in 34,512 Israeli infants aged 9 to 18 months.

OBJECTIVES: The purpose of this work was to use the comprehensive computerized database of Clalit Health Services to analyze the prevalence and contributing factors of anemia among the population of Clalit Health Services-insured Israeli infants aged 9 to 18 months, characterized by ethnic sector. METHODS: This was a cross-sectional retrospective study for the year 2003 using the computerized database of Clalit Health Services for 34,512 infants aged 9 to 18 months insured by the Clalit Health Services sick fund. Children with abnormal white blood counts at the time of the hemoglobin test and with chronic diseases were excluded. The data were analyzed for age, infant hemoglobin level, ethnic origin, district distribution, type of clinic where the infant received treatment, the number of iron prescriptions dispensed to each child, and the mother's last hemoglobin level before giving birth. Anemia was defined as a hemoglobin level <105 g/L. RESULTS: The prevalence of anemia among Israeli infants is 15.5%. The prevalence is significantly higher in the non-Jewish population (22.5%) as compared with the Jewish population (10.5%). The lowest prevalence of anemia was found in pediatric health centers (10.7%). A significant correlation was found between the presence of anemia in infants and the presence of anemia found in their mothers. Infants with anemia used significantly less iron preparations. CONCLUSIONS: This study is one of the first studies to use a comprehensive computerized database to perform a population-based analysis of anemic infants. We found a considerable percentage of infants to be anemic and identified a specific population to be at high risk for anemia. We describe 2 factors that have the potential to be altered through intervention: improving compliance of iron intake and maternal anemia. Major national efforts should be made to minimize the prevalence of anemia, especially in the non-Jewish population, and to learn more about the causes of iron-deficiency anemia in this group. This study provides a base for an intervention study.