Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disease, characterized by partial oculocutaneous albinism, frequent pyogenic infections, and the presence of abnormal large granules in leukocytes and other granulecontaining cells. The abnormal granules are readily seen in blood and marrow granulocytes. Other clinical features include silvery hair, photophobia, nystagmus and hepatosplenomegaly. However, the presence of abnormal giant intracytoplasmic granules in neutrophils and their precursors are diagnostic of CHS. Here, we present a series of five cases, out of which four presented in the accelerated phase. In all the five cases, the giant granules were noted predominantly in the cytoplasm of lymphocytes, which is a rare occurrence compared to those present in the granulocytes.

Introduction: Dengue is a mosquito borne infection caused by flavivirus and prevalent for more than a century in tropical countries. The aim of the study is to correlate hematological, biochemical and radiological parameters in serologically proven children of dengue at a tertiary care pediatric institute. Material and Methods: A study was done on 100 children with dengue up to 18 years of age admitted to our hospital. The serological tests used for diagnosis were NS1 antigen (rapid test) and IgM antibody detection by ELISA. Children were categorized into non-severe and severe dengue based on clinical features. Laboratory parameters were analyzed in these children. Result: Out of 100 children, 78 were categorized as non-severe dengue which included dengue fever (both with and without warning signs) and 22 children of severe dengue which included children of DHF and DSS as per WHO 2009 guidelines. Among hematological parameters, hemoconcentration, leukopenia, thrombocytopenia, presence of reactive lymphocytes in the peripheral smear, prolonged prothrombin time and activated partial thromboplastic time were useful indicators. Among biochemical parameters, raised SGOT and SGPT were seen both in severe and non-severe children. SGOT was found to be more elevated than SGPT. Gall bladder thickening was the commonest radiological finding followed by pleural effusion and ascites. Case fatality rate was 6%. Conclusion: Dengue infection is responsible for frequent epidemics with fatal outcome especially in the pediatric age group. The highly complex and non-specific presentation of dengue infection highlights the importance of laboratory testing which will help in improving the outcome.

Introduction: Thalassaemia and other structural haemoglobinopathies are the major genetic disorders that cause significant morbidity in children. Haemoglobinopathies need to be diagnosed at the earliest in order to offer suitable treatment, carrier identification and counseling which will help to decrease the incidence of haemoglobinopathies. Aim: To study the clinicohaematological spectrum of children with thalassaemia and other haemoglobinopathies, followed by family screening and counseling. Materials and Methods: This retrospective cross-sectional study was taken up at Department of Pathology, Indira Gandhi Institute of Child Health, Bangalore, Karnataka, India from January 2018 to December 2020, during which records of 235 cases were studied, out of which 100 were the children visiting institute and 135 were the parents and siblings of these children. Diagnosis of these cases was followed by counseling for these families. Results were calculated by data analysis. Percentages were calculated from the frequency of the variables. Results: Amongst the records of 235 cases studied, β thalassaemia major was the most common haemoglobinopathy found in 75 patients, followed by sickle cell anaemia in 10 patients. β thalassaemia trait was the most common haemoglobinopathy among the parents of these children, which was found in 125 individuals. Conclusion: β thalassaemia major is the most common disorder amongst the children with haemoglobinopathy, followed by sickle cell anaemia. β thalassaemia trait is a common entity amongst the carriers of haemoglobinopathy.

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