Lessons from Wolfram Syndrome: Initiation of DDAVP Therapy Causes Renal Salt Wasting Due to Elevated ANP/BNP Levels, Rescued by Fludrocortisone Treatment

Επιστημονική δημοσίευση - Άρθρο Περιοδικού uoadl:3119687 27 Αναγνώσεις

Μονάδα:
Ερευνητικό υλικό ΕΚΠΑ
Τίτλος:
Lessons from Wolfram Syndrome: Initiation of DDAVP Therapy Causes Renal Salt Wasting Due to Elevated ANP/BNP Levels, Rescued by Fludrocortisone Treatment
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Initiation of desmopressin acetate (DDAVP) for untreated diabetes insipidus (DI) in Wolfram syndrome (WS) causes abrupt volume expansion resulting in particularly high secretion of Atrial Natriuretic Peptide (ANP) and/or Brain Natriuretic Peptide (BNP), which in turn blocks all stimulators of zona glomerulosa steroidogenesis, resulting in secondary mineralocorticoid deficiency and acute hyponatremia, causing renal salt wasting (RSW). Two sisters, a 19-y-old girl (A) and a 7-y-old girl (B) with WS, presented with severe polyuria-polydipsia due to never treated DI. Both had neurogenic bladder and “B” had severe hydronephrosis secondary to untreated grade III bilateral vesicoureteral reflux. They initiated therapy with oral melt DDAVP which resulted in RSW. ANP was found ×50 and BNP ×2–4 fold elevated. Fludrocortisone 100–200 × 2 μg/d controlled natriuresis and restored electrolytes to normal within 48 h. Fludrocortisone treatment rescues otherwise potentially life-threatening hyponatremia due to RSW and the secondary mineralocorticoid deficiency driven by elevated ANP and/or BNP, caused by sudden volume expansion following DDAVP initiation. © 2020, Dr. K C Chaudhuri Foundation.
Έτος δημοσίευσης:
2021
Συγγραφείς:
Kleanthous, K.
Maratou, E.
Spyropoulou, D.
Dermitzaki, E.
Papadimitriou, A.
Zoupanos, G.
Moutsatsou, P.
Mastorakos, G.
Urano, F.
Papadimitriou, D.T.
Περιοδικό:
Indian Journal of Pediatrics
Εκδότης:
Springer-Verlag
Τόμος:
88
Αριθμός / τεύχος:
6
Σελίδες:
582-585
Λέξεις-κλειδιά:
atrial natriuretic factor; brain natriuretic peptide; desmopressin; electrolyte; fludrocortisone; argipressin[1 deamino]; atrial natriuretic factor; fludrocortisone, adult; Article; case report; child; clinical article; controlled study; disease severity; female; human; hydronephrosis; hyponatremia; natriuresis; neurogenic bladder; polydipsia; polyuria; salt wasting; school child; steroidogenesis; vesicoureteral reflux; Wolfram syndrome; young adult; zona glomerulosa; hyponatremia; Wolfram syndrome, Atrial Natriuretic Factor; Deamino Arginine Vasopressin; Female; Fludrocortisone; Humans; Hyponatremia; Wolfram Syndrome
Επίσημο URL (Εκδότης):
DOI:
10.1007/s12098-020-03538-y
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