RESUMEN
Objectives: To correlate the Full outline of unresponsiveness (FOUR) score and Glasgow coma scale (GCS) in the assessment of children with acute encephalitis syndrome (AES). Method: This observational study was conducted in the department of pediatrics of a public sector tertiary care center from January, 2019 to March, 2020. All consecutive patients of AES admitted during the study period (n=150) were recruited. Subjects were analyzed using the FOUR score and GCS on admission, and then 12-hourly till discharge/ death. Treatment-related and demographic variables were collected and analyzed. Correlation between FOUR score and GCS scores was calculated using spearman correlation coefficient. Results: Positive correlation was observed between the GCS score and the FOUR score (n=0.82; P<0.001). Conclusion: FOUR score and GCS were comparable to assess the level of consciousness in patients with AES. The possibility of using FOUR score as an alternative to GCS in children with AES needs to be considered.
RESUMEN
Harlequin ichthyosis (HI) is the rarest and the most severe form of congenital ichthyosis. It is inherited in an autosomal recessive manner and associated with mutations in gene ABCA12. There have been reports of several families with siblings affected with harlequin icthyosis. Affected infants have severe ectropion, eclabium, diffuse decreased range of motion, thick, dry hyperkeratotic plates, over entire body and scalp and associated deep erythematous fissures. Infants with HI have historically succumbed in the perinatal period to sepsis, respiratory failure and infections, poor nutrition and electrolyte imbalances. It is a rare entity. We report here a case of harlequin Icthyosis infant born to consanguineous parents whose 3 siblings were also affected with the same disorder. Keywo