05. tbl. 96.árg. 2010

A case report – Severe electrolyte disturbances in an eight week old boy

Sjúkratilfelli - lífshættulegar truflanir á blóðsöltum hjá átta vikna dreng

Hyponatremia is the most common electrolyte abnormality in children and underlying causes are many. It is most often caused by excessive salt loss from the gut but is also associated with severe systemic disorders in which there is actual or apparent aldosterone deficiency, such as congenital adrenal hyperplasia (CAH), which is the most common inherited disorder of aldosterone synthesis, and pseudohypoaldosteronism (PHA). Abscent aldosterone activity also leads to hyperkalemia which is characteristic for PHA and can result in life threatening arrythmias. This is a case report about a boy presenting with life threatening electrolyte disturbances in conjunction with PHA resulting from pyelonephritis and vesicoureteral reflux.

Table I.

  At birth 8 weeks Normal weight gain after 8 weeks (i.e. +30g per day):
Weight 4545 g 5145 g 1680 g
First lab results:   # 1

# 2

(after 60 min)

Normal range
  Na+ (mmol/L) 116 115 137-145
  K+ (mmol/L) 7,5 7,3 3,5-5,0
  Cl- (mmol/L) 86 84 98-110
  Kolsýra (mmol/L) 8 12 22-31
  CRP (mg/L) 8 8 <10
  Kreatínín (mmol/L) - 17 12-35
Second lab results: Hormones # 1 Normal range
  Cortisol (nmol/L) 535 200-700
  17a-OHP (nmol/L) 30,6 1,8-10,4
  Aldosterone (pmol/L) > 3892 111-860
  Renin (pmól/L) 92  

Table I: Tests taken and their results. Normal range also shown.


Figure 1.
Micturition cystourethrogram (MUCG) shows fourth degree vesicoureteral reflux into the right ureter.


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