A new method for screening for hyperammonemia

Tada, Keiya; Okuda, Kiyoshi; Watanabe, Kyoko; Iimura, Yasuo; Yamada, Shigeki

doi:10.1007/bf00442347

Cited by 28 publications

(6 citation statements)

References 2 publications

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“…The search for a grossly elevated blood ammonia concentration has been facilitated by the use of a method presented by Tada et al [5]. In our experience, mild elevations (up to twice the upper reference limit) might, however, be overlooked; in addition, one should be aware that the ammonia concen trations (above 120 pmol/1) measured by this method in whole capillary blood tend to be lower than when assayed by the enzymatic method in venous plasma.…”

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confidence: 76%

Treatment of Congenital Hyperammonemias

Bachmann¹

1984

Enzyme

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A rapid recognition of congenital hyperammonemia, a clear diagnostic workup and institution of a combined treatment without delay, by restriction of nitrogen supply, adequate caloric supply, substitution of missing metabolites, and use of alternate routes of nitrogen excretion will help to control hyperammonemic crises and improve the prognosis. For long-term treatment the use of essential amino acid mixtures and perhaps of antiserotoninergic agents is needed.

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confidence: 76%

Treatment of Congenital Hyperammonemias

Bachmann¹

1984

Enzyme

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“…Plasma ammonia levels were measured by an enzymatic methods (Mondzac et al 1965) or using a commercial kit "Amitest" (Tada et al 1979). …”

Section: Methodsmentioning

confidence: 99%

Enzyme replacement therapy in a patient with hyperargininemia.

Mizutani

Hayakawa

Maehara

et al. 1987

Tohoku J. Exp. Med.

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In a patient with hyperargininemia, enzyme replacement therapy such as whole blood exchange transfusion or erythrocyte transfusion was performed, and its effect was confirmed in vitro as well as in vivo. The patient has been treated with the restriction of protein intake, oral administration of an essential amino acid mixture, and sodium benzoate or phenylacetic acid. With these treatments, his plasma ammonia levels were controlled.On the other hand, plasma and CSF concentrations of arginine were not so well controlled. With whole blood exchange transfusion and erythrocyte transfusion, plasma arginine concentrations and plasma ammonia levels were controlled.These effects have continued for about 3 months. Furthermore, the effect of exchanged erythrocytes on the blood arginine levels was also confirmed by in vitro experimentation that mixes arginine with erythrocytes in the medium RPMI 1640. Arginine concentration of the medium did not decrease when arginine was mixed with erythrocytes of the patient, but when arginine was mixed with erythrocytes of mother or normal controls, it decreased rapidly.In calculation, 10' of erythrocytes of the mother or normal controls was presumed to metabolize 4-5 nmole of arginine per day. From these results, the erythrocyte exchange transfusion is considered to be effective for the control of clinical and biochemical abnormalities in this disorder.blood exchange transfusion ; enzyme replacement therapy ; erythrocyte transfusion ; hyperargininemia

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“…Ammonia was determined by a modification of the method used by Okuda and Fujii (1966). During the time that a laboratory was not available, Amitest (Tada et al 1979) was used to detect and monitor the level of blood ammonium. Orotic acid in urine was determined by the Roger and Porter method (1968).…”

Section: Methodsmentioning

confidence: 99%