5-oxoprolinuria

5-oxoprolinuria

 [ok″so-pro″lin-u´re-ah]
an inborn error of metabolism marked by abnormally increased levels of 5-oxoproline in the urine, metabolic acidosis, and an increase in the rate of hemolysis. Called also pyroglutamicaciduria.

5-ox·o·pro·lin·ur·i·a

(oks'ō-prō'lēn-yu'rē-ă),
Elevated levels of 5-oxoproline in the urine.
Mentioned in ?
References in periodicals archive ?
5-oxoprolinuria is caused by GSSD or 5-oxoprolinase deficiency.
Five Chinese patients with 5-oxoprolinuria due to glutathione synthetase and 5-oxoprolinase deficiencies.
Transient 5-oxoprolinuria (pyroglutamic aciduria) with systemic acidosis in an adult receiving antibiotic therapy.
Por ejemplo, el sindrome de hiperamonemia-hiperornitinemia-homocitrulinemia se detecta en once estados, la hiperglicinemia no cetosica en seis, y la 5-oxoprolinuria en cinco.
High anion gap metabolic acidosis secondary to pyroglutamic acidosis (5-oxoprolinuria): association with prescription drugs and malnutrition.
Transient 5-oxoprolinuria and high anion gap metabolic acidosis: clinical and biochemical findings in eleven subjects.
Pyroglutamic acidemia, or 5-oxoprolinuria, is caused by a glutathione synthetase deficiency, an inherited metabolic condition that may show in early infancy as persistent or acute metabolic acidosis associated with chronic hemolytic anemia (1).
The two adult subjects with 5-oxoprolinuria presented acutely with symptoms not associated with either of these two genetic diseases.
The subjects with 5-oxoprolinuria described in this report clearly represent a heterogeneous and distinct group compared with subjects with 5-oxoprolinuria produced by genetic enzyme deficiencies in the [gamma]-glutamyl cycle, although the concentrations of 5-oxoproline were similar to those seen in the latter group (19).
None of the subjects were taking vigabatrin or on artificial feeds known to induce 5-oxoprolinuria. Although it was not possible to completely exclude functional glycine deficiency in all subjects, it was considered unlikely to be the primary cause of the 5-oxoprolinuria.
5-Oxoprolinuria (pyroglutamic aciduria) is a condition characterized by increased production and urinary excretion of 5-oxoproline.
An enzymatic block here, therefore, leads to accumulation of 5-oxoproline, which is reflected as an increase in plasma concentration and urinary excretion (5-oxoprolinuria).