Triple “A” Syndrome Presenting As Recurrent Chronic Sinusitis With Pneumonia, Septic Shock And Meningoencephalitis In A 13 Years Old Boy
Journal Title: Journal Of Pediatric Critical Care - Year 2018, Vol 5, Issue 1
Abstract
Background : Triple A syndrome (All grove syndrome) is a rare autosomal recessive disorder characterized by the clinical triads of adrenal insufficiency, achalasia cardia and alacrima.It was first described by Allgrove in 1978 but in 1995, it was diagnosed as a syndrome with variable association of autonomic and neurological manifestations. This disorder manifests in the early childhood with life threatening complications like severe hypoglycemia, shock and recurrent infection.The syndrome usually presents during the first decade of life with dysphagia, while other signs may be delayed until adulthood. The onset of adrenocortical Impairment is usually before puberty. We present 13 years old boy with chronic recurrent sinusitis with failure to thrive without lacrimation since birth who has presented pneumonia, septic shock, and acute meningoencephalitis with adrenal insufficiency. He had all three cardinal features and underwent cardiomyotomy and was started on oral hydrocortisone replacement therapy. He recovered well and gaining weight now. Catastrophic complications can be prevented with adequate cortisol and specific measures such as cardiac pneumatic dilatation or myotomy along with other supportive management. The prognosis for health and quality of life can be significantly improved by early diagnosis and treatment.
Authors and Affiliations
Ramaning Loni
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