Re-examining ferritin-bound iron: current and developing clinical tools

Iron is a highly important metal ion cofactor within the human body, necessary for haemoglobin synthesis, and required by a wide range of enzymes for essential metabolic processes. Iron deficiency and overload both pose significant health concerns and are relatively common world-wide health hazards. Effective measurement of total iron stores is a primary tool for both identifying abnormal iron levels and tracking changes in clinical settings. Population based data is also essential for tracking nutritional trends. This review article provides an overview of the strengths and limitations associated with current techniques for diagnosing iron status, which sets a basis to discuss the potential of a new serum marker – ferritin-bound iron – and the improvement it could offer to iron assessment.

Growth Failure in Children with Sickle Cell Anaemia

Abstract:-  Background Sickle cell disease is a group of inherited sickling disorders consequent upon abnormalities of haemoglobin synthesis. It is one of the common causes of retardation in both physical growth and sexual maturation in children, as a result of chronic anaemia and consequent hypoxia that occur in several organs.  Objectives To determine the anthropometric indices (weight, height and Body Mass Index) of children with sickle cell anaemia (SCA) and compare them with that of children with normal haemoglobin; haemoglobin AA (HbAA).

Kelainan pada Sintesis Hemoglobin: Thalassemia dan Epidemiologi Thalassemia

Hemoglobinopathy includes structural abnormalities and haemoglobin synthesis disorders (thalassemia), is a single gene disorder that was originally found in malaria endemic areas but nowadays can be found all over the world. The birth rate of homozygous or compound heterozygous hemoglobinopathies, including alpha and beta thalassemia is less than 2.4 per 1000 births. Sickle cell anemia is the most prevalent compared to beta major and HbE-beta thalassemia. In Southeast Asia with more than 600 million people, abnormalities in hemoglobin including thalassaemia, HbE and HbCS are the most common and highly prevalent genetic disorders. Indonesia, has several areas that are endemic to malaria, there are many cases of abnormalities in Hb including thalassemia. If the percentage of carriers is associated with the birth rate and the number of Indonesian population and based on the study, it is estimated that the number of thalassemia patients born each year around 2500 children. As the case of thalassemia is increasing from year to year, it is necessary that prevention starts with screening in individuals who have relatives known as a carrier or thalassemia patient.

Efficacy and safety of high dose hydroxyurea in transfusion dependent thalassemic children: a quasi experimental study

Background: This study was conducted to find out whether high dose hydroxyurea is an effective and safe modality, in inducing haemoglobin synthesis to decrease blood transfusion requirement in transfusion dependent thalassemics.Methods: This quasi experimental un-control before and after comparison study was conducted in Thalassemia Day Care Centre, Department of Pediatrics over a period of six months after obtaining an approval from the Institute’s ethical committee. Fifty transfusions dependent thalassemic children belonging from 2 to 18 yrs were given hydroxyurea in dose of 20mg/kg after getting consent. Pre and post intervention haemoglobin and HbF levels were obtained using Hb electrophoresis by HPLC. Paired t test was applied to find out statistical significance and p value <0.05 was taken as significant.Results: Significant rise in haemoglobin pre and post intervention (p<0.001) but the rise in HbF was not significant (p=0.110). One patient had bone marrow depression which was reversible with drug withdrawal and one patient had rise in s. creatinine.Conclusions: High dose hydroxyurea is an effective and safe drug in inducing Hemoglobin synthesis in transfusion dependent thalassemics.

Haemoglobinopathies and their occurrence in South East Asia

A great number of hereditary abnormalities in the rate of haemoglobin synthesis and in structure of haemoglobins are known at the moment. Most of these abnormalities occur only in a few families and are not very important for the health of a population. Some forms occur, however, rather frequently. In Eastern Asia both alfa and beta thalassaemia cases are rather common. The alfa thalassaemia gene seems to occur in a rather high frequency in people from Chinese extraction. The beta thalassaemia gene, too, is not rare in the population of many etnic groups in South- East Asia.The symptoms of homozygocity, heterozygocity and double-heterozygocity for the different thalassaemia genes are described. In most Asia countries genetic abnormalities, causing abnormal haemoglobins, are not as common as they are for instance in many African groups. Forms of Lepore haemoglobin, Hb E, Hb Thai, Hb O, Hb S, have been found in East Asian populations, Hb Thai being rather frequent in Thailand, Hb E in Ceylon.The different clinical pictures of heterozygocity, homozygocity and double-heterozygocity for these genetic abnormalities are described.

The Effect of Adding Hesperidin, Diosmin, Quercetin and Resveratrol Extracts to Feed for Turkey Hens on Selected Immunological and Biochemical Blood Indices

It was postulated that naturally occurring phenolic compounds obtained from various plant species may have potential use as feed additives for poultry. Therefore the aim of the study was to compare extracts of hesperidin, diosmin, quercetin and resveratrol in terms of their health-promoting (particularly immunostimulatory) effect on turkeys at different ages. The experiment was conducted on 720 Big 6 turkey hens assigned to 6 experimental groups of 120 individuals (6 repetitions with 20 birds each). The turkey hens in group G-C were the control, receiving a basal compound feed with no experimental additives. The turkey hens in the remaining groups, from the first to the 16th week of life, received a basal diet containing hesperidin (group G-H), diosmin (group G-D), quercetin (group G-Q) or resveratrol (group G-R) in the amount of 200 g per tonne of feed. Ht, Hb, RBC, WBC, lysozyme activity, %PC, IgA, IL-6, GLU, TP and minerals were determined in blood samples. The addition of quercetin or resveratrol in the amount of 200 g per tonne of feed was found to have a beneficial effect on haemoglobin synthesis and phosphorus availability, and may also modulate immunity in turkey hens.