title: Changes in body weight and height in survivors of Menkes disease.
abstract: OBJECTIVE
  To explore the changes in the body weight and height of Menkes disease (MNK) patients treated with long-term copper-histidine.
  
  
  METHODS
  A survey involving a retrospective review of medical records or summaries of MNK patients was conducted. Patients were 44 males born after 1994, and their feeding method and genetic analysis of the ATP7A gene were reviewed. We compared the data of body weight and height from birth until 6 years between classical MNK patients and the general population obtained from national data and between patients who received early treatment and patients who received late treatment.
  
  
  RESULTS
  Although five patients who received early treatment reached some developmental milestones, the body weight and height did not differ from patients who received late treatment in the mode of oral nutrition, and were lower in comparison to the national data (<3 percentile).
  
  
  CONCLUSIONS
  We reported changes in the body weight and height of MNK patients who received early and late treatment. Although early treatment with copper-histidine had favorable effects on neurological development, it did not result in improvements in body weight and height. We suggest that the establishment of sufficient nutritional support is necessary along with early parenteral copper treatment to improve whole body condition in MNK patients.
authors.affiliation: Graduate School of Public Health, Teikyo University, Tokyo, Japan; Department of Hygiene and Public Health, Teikyo University, Tokyo, Japan. Electronic address: gyh@med.teikyo-u.ac.jp.
authors.forename: Yan-Hong
authors.initials: YH
authors.lastname: Gu
authors.affiliation: Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Hiroko
authors.initials: H
authors.lastname: Kodama
authors.affiliation: Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Eishin
authors.initials: E
authors.lastname: Ogawa
authors.affiliation: Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Sato
authors.affiliation: Department of Pediatrics, Teikyo University School of Medicine, Tokyo, Japan.
authors.forename: Kahoko
authors.initials: K
authors.lastname: Motoyama
authors.affiliation: Department of Pediatrics, Nikoniko House Medical and Welfare Center, Kobe, Japan.
authors.forename: Mariko
authors.initials: M
authors.lastname: Yagi
authors.affiliation: Department of Pediatrics, Nara Prefecture General Medical Center, Nara, Japan.
authors.forename: Sayaka
authors.initials: S
authors.lastname: Yoshida
authors.affiliation: Department of Hygiene and Public Health, Teikyo University, Tokyo, Japan.
authors.forename: Takayoshi
authors.initials: T
authors.lastname: Ohkubo
mesh_terms: D000293:Adolescent; D001827:Body Height / Q000502:physiology*; D001835:Body Weight / Q000502:physiology*; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000503:physiopathology*; D012189:Retrospective Studies; D055815:Young Adult
keywords: Growth; Intractable diseases; Long-term treatment; Nutrition; Percentile