Severe syncope and sudden death in children with inborn salt-losing hypokalaemic tubulopathies.
BACKGROUND
Potassium deficiency may cause cardiac arrhythmias culminating in syncope or sudden death.


METHODS
An inquiry performed among physicians caring for a total of 249 patients with inborn salt-losing tubulopathies revealed that acute cardiac complications occurred in seven children.


RESULTS
Four patients died suddenly and three had severe syncope. These episodes occurred in the context of severe chronic hypokalaemia (< or =2.5 mmol/l) or were precipitated by acute diseases, which exacerbated hypokalaemia (< or =2.0 mmol/l).


CONCLUSIONS
In conclusion, severe chronic or acute hypokalaemia is hazardous in inborn salt-losing tubulopathies.
Pediatric Renal Unit, University of Milan Medical School, De Marchi Hospital, Milan, Italy.
Cinzia
C
Cortesi
Alberto
A
Bettinelli
Francesco
F
Emma
Michel
M
Fischbach
Paolo
P
Bertolani
Mario G
MG
Bianchetti
D002648:Child; D003645:Death, Sudden; D005260:Female; D006801:Humans; D007223:Infant; D007683:Kidney Tubular Necrosis, Acute / Q000401:mortality*; D008297:Male; D008661:Metabolism, Inborn Errors / Q000150:complications* / Q000401:mortality; D011191:Potassium Deficiency / Q000150:complications*; D016019:Survival Analysis; D013575:Syncope / Q000453:epidemiology*