European Journal of Molecular & Clinical Medicine

Abstract

Congenital hemolytic anaemia is mostly caused by hemoglobinopathy and
thalassemia. They contribute significantly to morbidity, moderate to severe haemolytic
anaemia in young children, and a number of fatalities in India. In India, according to
UNICEF estimates from 1996, there are 29.7 million beta thalassemia trait carriers and
10,000 newborns are born each year with homozygous beta thalassemia.
Materials and Methods: All instances of hereditary hemolytic anaemia seen as
outpatients and inpatients between the ages of 1 and 20 years were included in this
group for the purposes of the study. The patient was disqualified if they had other
systemic illnesses such heart failure, liver disease, major infections, etc. With the aid of
a pre-designed and pre-tested standard proforma, all the data regarding their sociodemographic
profile, pertinent clinical history, and clinical examination were gathered.
Data was compiled in an excel spreadsheet and properly statistically assessed.
Results: 50 cases of hereditary hemolytic anaemia were identified. Hereditary hemolytic
anaemias are passed to the progeny through a specific pattern of inheritance.A total of
27 patients were born out of consanguineous marriage, in which 1st degree was 2 and
2nd/3rd degree were19 and 6 respectively.
Conclusion: The majority of hereditary hemolytic anaemias are hemoglobinopathies,
which place a heavy strain on families and society. Although important, haemoglobin
electrophoresis establishes the disease in hemoglobinopathies and the osmotic fragility
test in hereditary spherocytosis when differentiating between hereditary hemolytic
anaemias. Despite some recent optimistic initiatives in developing nations, hereditary
hemolytic anaemia control rarely receives the attention it requires.