Associated Complications In Beta Thalassemia Patients

Grow, Kirti

doi:10.9790/3013-31302225

Cited by 5 publications

(8 citation statements)

References 12 publications

(10 reference statements)

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“…in thalassemic patients with a median age of 10 years 14 . On the contrary, Grow et al ., reported lower prevalence of hepatic complications (6.8%) and this can be explained based on that 59.3% of their patients were below 10 years 15 .…”

Section: Discussionmentioning

confidence: 91%

Association between genotype and disease complications in Egyptian patients with beta thalassemia: A Cross-sectional study

Hassan

Zakaria

Fathy

et al. 2018

Sci Rep

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In beta thalassemia, the degree of globin chain imbalance is determined by the nature of the mutation of the β-gene. β° refers to the complete absence of production of β-globin on the affected allele. β+ refers to alleles with some residual production of β-globin. The homozygous state results in severe anemia that necessitates regular blood transfusion. On the other hand, frequent blood transfusion can lead to iron overload resulting in progressive dysfunction of the heart, Liver as well as multiple endocrinopathies. We studied the impact of genotype on the development of disease complications in patients with β thalassemia. A Cross sectional study was carried on 73 patients with beta thalassemia. Genotyping was determined by DNA sequencing technique. Routine investigations as well as MRI liver and heart were performed to assess iron overload. We found that β+β+ was the most common genotype in our patients followed by β°β° and β°β+. Mean Liver iron content (LIC) was significantly higher in β°β° compared to β°β+ and β+β+ genotypes and mean cardiac T2* was significantly lower in β°β° compared to β°β+ and β+β+ genotypes. Hepatic complications, hepatitis C, cardiac complications and some endocrinopathies were significantly higher in patients with β°β° genotype compared to other genotypes which explain the role of the underlying genetic defect in thalassemia patients in development of disease complications.

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Section: Discussionmentioning

confidence: 91%

Association between genotype and disease complications in Egyptian patients with beta thalassemia: A Cross-sectional study

Hassan

Zakaria

Fathy

et al. 2018

Sci Rep

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“…In the present study, 41 cases of hemolytic anaemia between ages 6 months-12 years have been studied. The commonest congenital hemolytic anemia was beta thalassemia major (19), sickle beta thalassemia(4), thalassemia intermedia(4), thalassemia minor(1).…”

Section: Discussionmentioning

confidence: 99%

“…Similar growth retardation was seen with Anitha Saxena [18], growth faltering was seen in 75% of thalassemic patients obvious after the age of 8 years. Study by Kirti Grow [19] showed growth faltering as the most common complication of thalassemia major. The reasons for growth retardation could be due to hypoxia, iron overload, compounded by undernutrition, and low SES.…”

Section: Discussionmentioning

confidence: 99%

Clinicohematological profile of hemolytic anaemia in tertiary care hospital in rural Andhra Pradesh

2016

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Introduction: Hemolytic anaemias are a group of disorders that cause significant morbidity in children. Method: A cross sectional study was conducted at NRI Medical College for a period of 1 year. All cases of newly diagnosed and old cases of hemolytic anaemia on follow up were included. Results: The study showed beta thalassemia as the most common hemolytic anaemia; followed by malaria, sickle beta thalassemia, thalassemia intermedia, beta thalassemia minor, Sickle cell disease, sickle cell trait, auto immune hemolytic anaemia, and hereditary spherocytosis. The mean hemoglobin at presentation was 5.39 gm/dl. Anthropometric measurements in 32 cases of congenital hemolytic anaemias revealed height < 3 rd centile in 9 cases. Weight less than 3 rd centile was seen in 11 cases. 13 children had hemolytic facies. Massive splenomegaly causing discomfort, gall stones, heart failure were seen in 2 cases each. In thalassemia major, 10 cases required frequent transfusions, [10-12 per year]. 9 came for less frequent transfusions [6 per year]. Sickle thalassemia, thalassemia intermedia, required one transfusion every 1-2 years. Occasional transfusions were given in sickle cell anaemia, hereditary spherocytosis. Serum ferritin levels varied between 220-1427. Conclusion: Hemoglobin electrophoresis remains the main investigation of choice in diagnosis of hemolytic anaemia. Thalassemia major is the most severe among other hemolytic anaemias encountered in this series. The study emphasizes the need to improve awareness regarding hemoglobinopathies among population, prenatal screening, blood transfusion policies, chelation policies to prevent complications in transfusion dependent patients.

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“…In this re- search, respondents in the MBT group had got regular PRC transfusion every 1-2 months for many years. The older MBT patiens are more susceptible to complications, such as heart, liver and endocrines disturbance [10]. Eight out of 11 children in the MBT group were male, the male to female ratio being 8:3.…”

Section: Statistical Analysesmentioning

confidence: 96%

“…A majority of children (9 / 11) in the MBT group had short stature, whereas, all children in the control group had normal stature. In MBT patients, the short stature was caused by growth hormon disturbance, chronic hypoxia due to chronic anemia and cardiovascular disturbance [10]. A majority of the children (9 / 11) in the MBT group suffered from Cooley's face caused by erythropoitic expansion in the bone marrow leading to the skull and face bone deformities [13].…”

Section: Statistical Analysesmentioning

confidence: 99%

Correlation between Serum Ferritin and Cardiac Troponin I in Major Beta Thalassemia Children

Shodikin

Suwarniaty

Nugroho

2016

JTLS

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Major beta thalassemia (MBT) is a hereditary disease which synthesies defects in beta chains of haemoglobin, it is causes red blood cell destruction and the symptoms of anemia. Red blood cell destruction, frequent blood transfusion and low adherence to routine use of iron chelator lead to iron accumulation in the heart, liver and endocrine organs. Accumulation of iron in the myocard can lead acute myocardial infarction. One of cardiac markers that had been used for the diagnosis of myocardial infarction was cardiac troponin I (cTnI). The aim of this research is find the correlation between serum ferritin levels and cTnI in MBT children. A descriptive analytic research was conducted using a cross sectional design. The subjects were divided into 2 groups, the MBT group and the control group. In both groups, the serum ferritin and cTnI levels ere evaluated. Data were analyzed using t-test and Pearson correlation test. Eleven children in the MBT group and 11 children in the control group were involved in this study. In the MBT group, the mean of serum ferritin and cTnI levels were 4292.5 µg/L and 0.20 ng/mL respectively. The mean of serum ferritin levels in the MBT group were higher than in the control and statistically significant (p= 0.0004). The mean of serum ferritin levels in the MBT group were higher than in the control and statistically significant (p= 0.0004). The mean of serum cTnI in the MBT group were higher than in the control, but statistically not significant (p= 0.82). In the MBT group, there was a weak corellation between serum ferritin and cTnI levels (r= 0.34).

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Associated Complications In Beta Thalassemia Patients

Cited by 5 publications

References 12 publications

Association between genotype and disease complications in Egyptian patients with beta thalassemia: A Cross-sectional study

Association between genotype and disease complications in Egyptian patients with beta thalassemia: A Cross-sectional study

Clinicohematological profile of hemolytic anaemia in tertiary care hospital in rural Andhra Pradesh

Correlation between Serum Ferritin and Cardiac Troponin I in Major Beta Thalassemia Children

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