Background: Congenital adrenal hyperplasia (CAH) is an inherited disorder of adrenal glands, the most common type being due to a deficiency of the enzyme 21-?-hydroxylase. Girls affected with it are virilized to a variable extent. The taboo associated with ambiguous genitalia in female babies poses an immense societal pressure on parents for keeping the disease a secret. Objective: The aim of the study was to generate the evidence on the knowledge, stress, and coping strategies used by the parents of CAH children. Methodology: In a cross-sectional descriptive survey using mixed methods approach, 30 parents of children with CAH attending the outdoor department of pediatric medicine and surgery in a tertiary care hospital were enrolled. The CAH knowledge questionnaire, parental stress scale (PSS), and COPE inventory were the standardized tools used along with demographic profile sheet to assess the knowledge of parents related to CAH, stress, and coping strategies used by them. Results: Majority of the parents (67%) had poor knowledge (<7) and moderate stress (42-65) scores. Knowledge and stress were negatively correlated (r=?0.39, p=0.035). Coping strategies used by the parents in dealing with stress in moderate amount were mainly problem-focused (active coping [60%] and planning [60%], instrumental social support [53%], suppression of competing activities [50%]), and emotion-focused (positive reinterpretation [60%], emotional social support [67%], acceptance [53%], and religious coping [50%]). Less useful coping strategies such as focus on and venting of emotions, behavioral disengagement, mental disengagement, humor, and substance abuse were not used by the majority parents. Conclusion: Parents of children with CAH had poor knowledge and moderate stress related to the disease condition of their children and were using problem focused and emotion focused coping strategies to reduce their stress.
Recurrent aphthous stomatitis (RAS) is a common mucosal condition producing painful ulcerations in the oral cavity and considerable clinical morbidity. The etiology remains obscure, though local trauma, psychologic stress, hematinic deficiencies and immune dysregulation have been implicated. Though the primary goals of therapy are symptomatic relief of pain, the clinicians are aiming toward reducing the frequency, duration, number of ulcerations and increasing ulcer free periods with systemic drug therapy if topical medications appear ineffective. Levamisole, an antihelminthic drug has been tried with promising results in patients with severe RAS providing long-term benefits.
Aim: To report our experience with four neonates with Shah-Waardenburg syndrome (SWS) and highlight the spectrum of intestinal aganglionosis in this disorder. Method: Four consecutive cases of SWS that were managed over last five years at two pediatric surgical centres are described. The syndrome was identified by virtue of its various components.
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