Normal prostaglandinuria E2 in Gitelman's syndrome, the hypocalciuric variant of Bartter's syndrome.
In familial Bartter's syndrome, hyperprostaglandinuria is considered a constant feature and prostanoid synthetase inhibition often positively influences the disease course. The urinary calcium excretion distinguishes two clinically and biochemically different variants, namely, classic Bartter's syndrome (normocalciuric or hypercalciuric variant; urinary calcium to creatinine > or = 35.3 mg/mg 10(-3)) and Gitelman's syndrome (hypocalciuric variant; urinary calcium to creatinine < 35.3 mg/mg 10(-3)). In the hypocalciuric variant of Bartter's syndrome prostanoid synthetase inhibition is of little benefit. Since the production of prostanoids has not been extensively studied in Gitelman's syndrome, the urinary excretion of prostaglandin E2 was assessed by radioimmunoassay in 11 untreated patients with Gitelman's syndrome (aged 10 to 21 years; five females and six males) and in 11 healthy controls (aged 11 to 20 years; five females and six males). The urinary excretion of prostaglandin E2 was similar in both study groups. The study provides the rationale for the poor effect of prostanoid synthetase inhibition in the hypocalciuric variant of Bartter's syndrome. The assessment of urinary excretion of prostaglandin E2 does not represent a diagnostic sine qua non in the context of familial Bartter's syndrome.
Lüthy
C
C
Department of Pediatrics, Abteilung für Kindernephrologie, Bern, Switzerland.
Bettinelli
A
A
Iselin
S
S
Metta
M G
MG
Basilico
E
E
Oetliker
O H
OH
Bianchetti
M G
MG
D000293:Adolescent; D000328:Adult; D001477:Bartter Syndrome / Q000175:diagnosis / Q000235:genetics / Q000652:urine*; D002118:Calcium / Q000652:urine*; D016022:Case-Control Studies; D002648:Child; D003937:Diagnosis, Differential; D015232:Dinoprostone / Q000652:urine*; D005260:Female; D006801:Humans; D008297:Male; D011863:Radioimmunoassay; D013577:Syndrome
D015232:Dinoprostone; D002118:Calcium