title: Camurati-Engelmann disease: failure of response to bisphosphonates: report of two cases.
abstract: Camurati-Engelmann disease is a rare bone disorder characterized by cortical thickening of the diaphysis of tubular bones, with sparing of the epiphysis. It has variable degrees of penetrance and expression, but may be very disabling for the affected individuals who manifest the painful symptoms. The authors report on two women with typical presentation of severe Camurati-Engelmann disease whose treatment with bisphosphonates failed to add any improvement beyond that elicited by corticosteroids alone.
authors.affiliation: Departamento de Clínica Médica, Faculdade de Ciências Médicas/ UNICAMP, Campinas, SP, Brazil. appenzel@unicamp.br
authors.forename: Glaucio R W
authors.initials: GR
authors.lastname: Castro
authors.forename: Simone
authors.initials: S
authors.lastname: Appenzeller
authors.forename: João Francisco
authors.initials: JF
authors.lastname: Marques-Neto
authors.forename: Manoel B
authors.initials: MB
authors.lastname: Bértolo
authors.forename: Adil M
authors.initials: AM
authors.lastname: Samara
authors.forename: Ibsen
authors.initials: I
authors.lastname: Coimbra
mesh_terms: D000328:Adult; D015519:Bone Density / Q000502:physiology; D003966:Camurati-Engelmann Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D003720:Densitometry; D004164:Diphosphonates / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D035583:Rare Diseases; D012720:Severity of Illness Index; D017211:Treatment Failure
chemical_list: D004164:Diphosphonates