Literature DB >> 12233049

[Clinical manifestations for early diagnosis of the patient with classical Menkes disease].

Hiroshi Ozawa1, Natsue Nakamoto, Hiroko Kodama.   

Abstract

Early treatment reportedly prevents neurological disturbance in patients with Menkes disease. To promote early diagnosis, a questionnaire concerning symptoms during the neonatal period and at the age of 1 month was sent to the mothers of 14 patients with Menkes disease. The height, body weight and head circumference were almost normal in most of the Menkes patients. At the age of one month, all patients had abnormal hair. The mothers noticed it in 8 cases, and in the remaining 6 cases, the abnormalities were confirmed by the photos at the age of one month. At this age, the mothers noticed weak activity in 5, jaundice in 5, dyspnea in 4 cases, feeding disturbance in 3, hypothermia in 2 and eczema in 2. These results suggest that recognizing hair abnormalities at a one-month check-up may provide a clue to the early diagnosis of Menkes disease.

Entities:  

Mesh:

Year:  2002        PMID: 12233049

Source DB:  PubMed          Journal:  No To Hattatsu        ISSN: 0029-0831


  2 in total

Review 1.  Peptidylgycine α-amidating monooxygenase and copper: a gene-nutrient interaction critical to nervous system function.

Authors:  Danielle Bousquet-Moore; Richard E Mains; Betty A Eipper
Journal:  J Neurosci Res       Date:  2010-09       Impact factor: 4.164

2.  Interactions of peptide amidation and copper: novel biomarkers and mechanisms of neural dysfunction.

Authors:  Danielle Bousquet-Moore; Joseph R Prohaska; Eduardo A Nillni; Traci Czyzyk; William C Wetsel; Richard E Mains; Betty A Eipper
Journal:  Neurobiol Dis       Date:  2009-10-06       Impact factor: 5.996

  2 in total

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