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Pseudohypoaldosteronism in Children

Meta Description: Pseudohypoaldosteronism has been classified into three distinct types, primary hereditary, namely Type 1 and Type 2, and secondary. It is a rare condition which is characterized by hyponatremia, hyperkalemia, metabolic acidosis and is associated with high levels of plasma aldosterone.

Pseudohypoaldosteronism is a rare condition which is characterized by hyponatremia, hyperkalemia, metabolic acidosis and is associated with high levels of plasma aldosterone. Because of hyponatremia and hyperkalemia, the differential diagnosis should include congenital adrenal hyperplasia, isolated aldosterone synthase deficiency and adrenal insufficiency. Pseudohypoaldosteronism can be primary or secondary. Pseudohypoaldosteronism has been classified into three distinct types, primary hereditary, namely Type 1 and Type 2, and secondary.

Type 1 pseudohypoaldosteronism is a rare syndrome mainly starts during the neonatal period, either as renal form of autosomal dominant inheritance due to a mutation of the mineralocorticoid receptor gene or a severe systemic form of autosomal recessive inheritance due to a mutation of the epithelial sodium channel (ENaC) gene. Type 2 pseudohypoaldosteronism is a rare familial renal tubular defect characterized by hypertension and hyperkalemic metabolic acidosis in the presence of low renin and aldosterone levels. Secondary causes of pseudohypoaldosteronism include, urinary tract infection, nephropathy, medications and gastrointestional losses.

For more details:

https://www.omicsonline.org/open-access/pseudohypoaldosteronism-secondary-to-high-output-ileostomy-a-unique-report-in-an-infant-2161-0665.1000224.php?aid=33660

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