The Wenckebach Phenomenon and Auricular-ventricular Dissociation in Children

Shnier, M. H.

doi:10.1136/adc.33.169.246

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“…Renal PHA type I is generally milder and tends to remit with age, whereas systemic PHA type I persists and requires lifelong treatment [2–4]. Cheek and Perry first described PHA in 1958 [5]. Since then, several patients have been reported and our understanding of the disease has expanded dramatically.…”

Section: Introductionmentioning

confidence: 99%

Systemic Pseudohypoaldosteronism Type I: A Case Report and Review of the Literature

Nur

Lang

Hodax

et al. 2017

Case Reports in Pediatrics

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Systemic pseudohypoaldosteronism (PHA) type I is a rare genetic disorder resulting from mutations in the subunits of the epithelial sodium channel that manifests as severe salt wasting, hyperkalemia, and metabolic acidosis in infancy. In this article we report a patient with systemic PHA type I presenting with severe dehydration due to salt wasting at 6 days of life. She was found to have a known mutation in the SCNN1A gene and subsequently required treatment with sodium supplementation. We also review the clinical presentation, differential diagnosis, and treatment of systemic PHA type I and summarize data from 27 cases with follow-up data.

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Section: Introductionmentioning

confidence: 99%