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
Keywords
Related Articles
- EP ID EP540434
- DOI -
- Views 45
- Downloads 0
Drug Induced Hypersensitivity Syndrome (DIHS) with Multi-organ Failure with Type I Diabetes in Infant : A Case Report
.
Thoracic and abdominal trauma in children
Thoracic and abdominal trauma is relatively uncommon in children experiencing polytrauma. Survival depends on early recognition and timely management. Current review is to review literature about the thoracic and abdomin...
Successful endovascular occlusion of ruptured intracranial mycotic aneurysm in a child with Ventricular Septal Defect and infective endocarditis – A case report
Infective endocarditis is associated with significant morbidity and mortality. Neurologic complications associated with infective endocarditis are cerebral infarction, bacterial meningitis, intracerebral haemorrhage, and...
Salvaging Young ‘Brains’: The need of the hour !
.
Approach to Acute Valproic Acid Intoxication: A Case Presentation
Valproic acid (VA) is a broad spectrum antiepileptic that is commonly used in epilepsy, mood disorders and migraine prophylaxis. Although it is not encountered frequently, VA intoxication can be seen during childhood. In...