Page last updated: 2024-11-02

pamidronate and Menkes Kinky Hair Syndrome

pamidronate has been researched along with Menkes Kinky Hair Syndrome in 1 studies

Menkes Kinky Hair Syndrome: An inherited disorder of copper metabolism transmitted as an X-linked trait and characterized by the infantile onset of HYPOTHERMIA, feeding difficulties, hypotonia, SEIZURES, bony deformities, pili torti (twisted hair), and severely impaired intellectual development. Defective copper transport across plasma and endoplasmic reticulum membranes results in copper being unavailable for the synthesis of several copper containing enzymes, including PROTEIN-LYSINE 6-OXIDASE; CERULOPLASMIN; and SUPEROXIDE DISMUTASE. Pathologic changes include defects in arterial elastin, neuronal loss, and gliosis. (From Menkes, Textbook of Child Neurology, 5th ed, p125)

Research Excerpts

ExcerptRelevanceReference
"Pamidronate treatment was associated with an increase in bone mineral density and may be an effective treatment modality for the management of osteoporosis in children with Menkes disease."2.70Pamidronate treatment improves bone mineral density in children with Menkes disease. ( Boneh, A; Kanumakala, S; Zacharin, M, 2002)

Research

Studies (1)

TimeframeStudies, this research(%)All Research%
pre-19900 (0.00)18.7374
1990's0 (0.00)18.2507
2000's1 (100.00)29.6817
2010's0 (0.00)24.3611
2020's0 (0.00)2.80

Authors

AuthorsStudies
Kanumakala, S1
Boneh, A1
Zacharin, M1

Trials

1 trial available for pamidronate and Menkes Kinky Hair Syndrome

ArticleYear
Pamidronate treatment improves bone mineral density in children with Menkes disease.
    Journal of inherited metabolic disease, 2002, Volume: 25, Issue:5

    Topics: Adolescent; Bone Density; Calcium; Child, Preschool; Diphosphonates; Humans; Infusions, Intravenous;

2002