title: Conventional Therapy in Adults With X-Linked Hypophosphatemia: Effects on Enthesopathy and Dental Disease.
abstract: BACKGROUND
  Treatment of X-linked hypophosphatemia (XLH) with active vitamin D metabolites and phosphate can partially correct skeletal deformities. It is unclear whether therapy influences the occurrence of two major long-term morbidities in XLH: enthesopathy and dental disease.
  
  
  OBJECTIVE
  The objective of the study was to investigate the relationship between treatment and enthesopathy and dental disease in adult XLH patients.
  
  
  METHODS
  The study was designed as observational and cross-sectional.
  
  
  METHODS
  The study was conducted at an academic medical center's hospital research unit.
  
  
  METHODS
  Fifty-two XLH patients aged 18 years or older at the time of the study participated in the study.
  
  
  METHODS
  There were no interventions.
  
  
  METHODS
  The number of enthesopathy sites identified by radiographic skeletal survey and dental disease severity (more than five or five or fewer dental abscesses), identified historically, were measured.
  
  
  METHODS
  Associations between proportion of adult life and total life with treatment and number of enthesopathy sites were assessed using multiple linear regression, whereas associations between these exposure variables and dental disease severity were assessed using multiple logistic regression. All models were adjusted for confounding factors.
  
  
  RESULTS
  Neither proportion of adult nor total life with treatment was a significant predictor of extent of enthesopathy. In contrast, both of these treatment variables were significant predictors of dental disease severity (multivariate-adjusted global P = .0080 and P = .0010, respectively). Participants treated 0% of adulthood were more likely to have severe dental disease than those treated 100% of adulthood (adjusted odds ratio 25 [95% confidence interval 1.2-520]). As the proportion of adult life with treatment increased, the odds of having severe dental disease decreased (multivariate-adjusted P for trend = .015).
  
  
  CONCLUSIONS
  Treatment in adulthood may not promote or prevent enthesopathy; however, it may be associated with a lower risk of experiencing severe dental disease.
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Jessica
authors.initials: J
authors.lastname: Connor
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Elizabeth A
authors.initials: EA
authors.lastname: Olear
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Karl L
authors.initials: KL
authors.lastname: Insogna
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Lee
authors.initials: L
authors.lastname: Katz
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Suher
authors.initials: S
authors.lastname: Baker
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Raghbir
authors.initials: R
authors.lastname: Kaur
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Christine A
authors.initials: CA
authors.lastname: Simpson
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: John
authors.initials: J
authors.lastname: Sterpka
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Robert
authors.initials: R
authors.lastname: Dubrow
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Jane H
authors.initials: JH
authors.lastname: Zhang
authors.affiliation: Departments of Epidemiology and Public Health (J.C., R.D.), Medicine (K.L.I., C.A.S., J.S.), Pediatrics (E.A.O., T.O.C.), and Diagnostic Imaging (L.K.), Yale University School of Medicine, Yale-New Haven Hospital Dentistry Program (S.B., R.K.), New Haven, Connecticut 06520; and the Veterans Administration Cooperative Studies Program Coordinating Center, Veterans Affairs Connecticut Healthcare System (J.H.Z.), West Haven, Connecticut 06516.
authors.forename: Thomas O
authors.initials: TO
authors.lastname: Carpenter
mesh_terms: D000328:Adult; D002117:Calcitriol / Q000627:therapeutic use*; D003430:Cross-Sectional Studies; D053098:Familial Hypophosphatemic Rickets / Q000150:complications / Q000000981:diagnostic imaging / Q000188:drug therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010710:Phosphates / Q000627:therapeutic use*; D011859:Radiography; D012216:Rheumatic Diseases / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology; D012720:Severity of Illness Index; D009057:Stomatognathic Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000209:etiology; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D010710:Phosphates; D002117:Calcitriol