Context: Silvery gray hair syndromes consist of three conditions, Chediak–Higashi syndrome (CHS), Griscelli syndrome (GS), and Elejalde syndrome (ES), which are rare autosomal recessive diseases presenting in childhood. They have similar skin and hair changes but differ with respect to the other clinical findings. Aim: This retrospective study was taken up to identify the spectrum of silvery gray hair syndromes diagnosed over a period of 10 years (2006-2015) in a super specialty pediatric institute and also to identify characteristic clinical and diagnostic findings. Methods: A total of 17 children diagnosed to have silvery gray hair syndrome over a period of 10 years (2006-2015) at Indira Gandhi Institute of Child Health Hospital, Bengaluru and were studied retrospectively. Complete blood counts, peripheral smear study, and microscopic examination findings of the hair were analyzed. Radiological findings were also considered. Results: The most common syndrome in the study was GS Type II (10 cases) followed by CHS (5 cases), with one case each of GS Type III and ES. Conclusion: This study emphasizes the importance of silvery gray hair as a clue to diagnose these rare genetic disorders with proper clinical examination and simple diagnostic procedures.
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.
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