Literature DB >> 1088261

The use of 5-hydroxytryptophan in a child with Lesch-Nyhan syndrome.

R D Ciaranello, T F Anders, J D Barchas, P A Berger, H M Cann.   

Abstract

The effects of 5-hydroxytryptophan (5-HTP), a serotonin precursor, were studied in a boy with the Lesch-Nyhan syndrome. During the course of drug treatment, self-mutilation, crying, sleep state architecture, serum dopamine B-hydroxylase (DBH), and cerebrospinal fluid levels of 5-hydroxyindolylacetic acid and homovanillic acid were studied. Treatment with 5-HTP failed to affect this child's biting behavior. However, the drug significantly reduced irritability as measured by crying time. Moreover, 5-HTP may have affected sleep state architecture, making it more normal in character. Serum DBH levels were normal throughout the study. Neither the patient's mother nor his maternal grandmother showed a hypertensive response on the cold pressor test.

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Year:  1976        PMID: 1088261     DOI: 10.1007/bf01464036

Source DB:  PubMed          Journal:  Child Psychiatry Hum Dev        ISSN: 0009-398X


  11 in total

1.  A FAMILIAL DISORDER OF URIC ACID METABOLISM AND CENTRAL NERVOUS SYSTEM FUNCTION.

Authors:  M LESCH; W L NYHAN
Journal:  Am J Med       Date:  1964-04       Impact factor: 4.965

2.  Proportionally between dopamine-beta-hydroxylase activity and immunoreactive protein concentration in human serum.

Authors:  G F Wooten; R D Ciaranello
Journal:  Pharmacology       Date:  1974       Impact factor: 2.547

3.  Letter: Self mutilation in Lesch-Nyhan syndrome.

Authors:  T I Mizuno; Y Yugari
Journal:  Lancet       Date:  1974-04-20       Impact factor: 79.321

4.  Reduced plasma dopamine-beta-hydroxylase activity in familial dysautonomia.

Authors:  R M Weinshilboum; J Axelrod
Journal:  N Engl J Med       Date:  1971-10-21       Impact factor: 91.245

5.  Urine uric acid to creatinine rtio--a screening test for inherited disorders of purine metabolism. Phosphoribosyltransferase (PRT) deficiency in X-linked cerebral palsy and in a variant of gout.

Authors:  J M Kaufman; M L Greene; J E Seegmiller
Journal:  J Pediatr       Date:  1968-10       Impact factor: 4.406

6.  Evidence for the release of vesicle-proteins together with noradrenaline upon stimulation of the splenic nerve.

Authors:  W P de Potter; A F de Schaepdryver; E J Moerman; A D Smith
Journal:  J Physiol       Date:  1969-10       Impact factor: 5.182

7.  X-linked hypoxanthine-guanine phosphoribosyl transferase deficiency: heterozygote has two clonal populations.

Authors:  B R Migeon; V M Der Kaloustian; W L Nyhan; W J Yough; B Childs
Journal:  Science       Date:  1968-04-26       Impact factor: 47.728

8.  Enzyme defect associated with a sex-linked human neurological disorder and excessive purine synthesis.

Authors:  J E Seegmiller; F M Rosenbloom; W N Kelley
Journal:  Science       Date:  1967-03-31       Impact factor: 47.728

9.  5-hydroxytryptophan (5-HTP) in Down's syndrome.

Authors:  M W Partington; M R MacDonald
Journal:  Dev Med Child Neurol       Date:  1971-06       Impact factor: 5.449

10.  Lesch-Nyhan syndrome: evidence for abnormal adrenergic function.

Authors:  S Rockson; R Stone; M Van der Weyden; W N Kelley
Journal:  Science       Date:  1974-12-06       Impact factor: 47.728

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  5 in total

Review 1.  Heberden oration 1979: human aberrations of purine metabolism and their significance for rheumatology.

Authors:  J E Seegmiller
Journal:  Ann Rheum Dis       Date:  1980-04       Impact factor: 19.103

Review 2.  Lesch-Nyhan Syndrome: Models, Theories, and Therapies.

Authors:  Scott Bell; Ilaria Kolobova; Liam Crapper; Carl Ernst
Journal:  Mol Syndromol       Date:  2016-09-24

3.  Contributions of Lesch-Nyhan syndrome to the understanding of purine metabolism.

Authors:  J E Seegmiller
Journal:  J Inherit Metab Dis       Date:  1989       Impact factor: 4.982

Review 4.  Pharmacological treatment of self-injurious behavior in mentally retarded persons.

Authors:  N N Singh; C J Millichamp
Journal:  J Autism Dev Disord       Date:  1985-09

5.  Genital self mutilation in alcohol withdrawal state complicated with delirium.

Authors:  Sri Hari Charan; C M Pavan Kumar Reddy
Journal:  Indian J Psychol Med       Date:  2011-07
  5 in total

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