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Literature DB >> 436855

Hyperdibasicaminoaciduria in a Turkish infant without evident protein intolerance.

Abstract

The first patient of Turkish descent with hyperdibasicaminoaciduria is described. Recurrent diarrhea was observed only during the first three months of life. The infant exhibited low plasma levels of ornithine and arginine. Intestinal absorption of lysine was decreased. Hyperammonemia was noticed only after an i.v. alanine load. It was prevented by addition of arginine.

Entities: Chemical Disease Species

Mesh：

Substances：
Arginine
Ornithine
Lysine

Year: 1979 PMID： 436855 DOI： 10.1007/bf00442783

Source DB: PubMed Journal: Eur J Pediatr ISSN： 0340-6199 Impact factor: 3.183

26 in total

1. Prolonged coma and isoelectric electroencephalogram in a child with lysinuric protein intolerance.

Authors: H Chan; G J Billmeier; S V Molinary; H N Tucker; B C Shin; A Schaffer; K Cavallo
Journal: J Pediatr Date: 1977-07 Impact factor: 4.406

2. Lysinuric protein intolerance.

Authors: N A Carson; O A Redmond
Journal: Ann Clin Biochem Date: 1977-05 Impact factor: 2.057

3. Leucocyte glutaminase in familial protein intolerance.

Authors: J Malmquist; B Hetter
Journal: Lancet Date: 1970-07-18 Impact factor: 79.321

4. Hyperlysinuria with hyperammonemia. A new metabolic disorder.

Authors: J H Brown; L F Fabre; G L Farrell; E D Adams
Journal: Am J Dis Child Date: 1972-07

5. Familial protein intolerance with deficient transport of basic amino acids. Report on an adult patient with chronic hyperammonemia.

Authors: M Kekomäki; E Toivakka; V Häkkinen; M Salaspuro
Journal: Acta Med Scand Date: 1968-04

Hyperdibasicaminoaciduria in a Turkish infant without evident protein intolerance.

1. Prolonged coma and isoelectric electroencephalogram in a child with lysinuric protein intolerance.

2. Lysinuric protein intolerance.

3. Leucocyte glutaminase in familial protein intolerance.

4. Hyperlysinuria with hyperammonemia. A new metabolic disorder.

5. Familial protein intolerance with deficient transport of basic amino acids. Report on an adult patient with chronic hyperammonemia.

6. Hyperdibasicaminoaciduria: an inherited disorder of amino acid transport.

7. Protein intolerance with deficient transport of basic aminoacids. Another inborn error of metabolism.

8. Enzymes of urea synthesis in familial protein intolerance with deficient transport of basic amino acids.

9. Hyperdibasicaminoaciduria, hyperammonemia, and growth retardation: Treatment with arginine, lysine, and citrulline.

10. Renal handling of diamino acids in lysinuric protein intolerance.

1. Growth hormone studies in lysinuric protein intolerance.