pylori) infection], menstruation, gastroesophageal reflux disease, Trimethylaminuria, hypermethioninemia
Infantile hypermethioninemia and hyperhomocystinemia due to high methionine intake: A diagnostic trap.
Hypermethioninemia: A metabolic disorder associated with cirrhosis, islet cell hyperplasia and renal tubular degeneration, Pediatrics 1965; 36 (2): 236-250.
Reduction of gangliosides, phospholipids and cholesterol content in cerebral cortex of rats caused by chronic hypermethioninemia
Also, a Scandinavian group (23) and a Canadian group (24) found that hypermethioninemia occasionally occurred in patients with HRT, especially in the acute stage.
It is recognised that the odor in patients with HRT is associated with hypermethioninemia, and this, together with aforementioned symptoms and signs, are seen frequently in acute type HRT.
Table IV Causes of blood-borne halitosis Odorant Systemic diseases * Hepatic failure/liver cirrhosis * Dimethyl sulfide * Uremia/kidney failure * Dimethylamine, trimethy lamine * Diabetic ketoacidosis/diabetes mellitus * Acetone Metabolic disorders * Isolated persistent * Dimethyl sulfide hypermethioninemia
* Trimethylamine * Fish odor syndrome, trimethy laminuria Medication * Disulfiram * Carbon disulfide * Dimethyl sulphoxide * Dimethyl sulfide * Cysteamine * Dimethyl sulfide Food * Garlic * Ally1 methyl sulfide * Onion * Methyl propyl sulfide Tangerman A.
Many cases of homocystinuria have secondary hypermethioninemia
. Neonatal screening for homocystinuria by measurement of increased methionine concentrations in dried blood spots (DBS) has been performed in some centers but has poor sensitivity.
In newborns and infants, food formulas and amino acid solutions may cause hypermethioninemia
and hyperhomocysteinemia (3,62), and severe hypermethioninemia
is often found in babies who are premature, have low birth weights, or who are in neonatal intensive care units (40, 62).
When increased, methionine is the metabolite used in MS/MS analysis to identify cases of isolated hypermethioninemia
or homocystinuria, which often result from deficiencies of methionine adenosyltransferase and cystathionine [beta]-synthase, respectively (63).
Quantitative plasma amino acid analysis 1 week later disclosed isolated hypermethioninemia
. Subsequent plasma and urine amino acid analysis in specimens promptly delivered to our laboratory, however, revealed the presence of homocystine and the mixed disulfide in addition to the increase in methionine.
Similar methods have been adopted on a limited scale for diseases such as maple syrup urine disease, isolated hypermethioninemia
, homocystinuria, and galactosemia [10, 13].