title: The prevalence of isolated growth hormone deficiency among children of short stature in Jordan and its relationship with consanguinity.
abstract: OBJECTIVE
  The prevalence of isolated growth hormone deficiency (IGHD) among short-statured children in Jordan, where consanguineous marriage (CM) is common, is unknown. No studies have investigated the relationship between degrees of consanguinity and IGHD. This study aimed to determine the prevalence of IGHD among short-statured children referred to a university hospital in Jordan and its relationship with different degrees of consanguinity.
  
  
  METHODS
  We conducted a 24-month cross-sectional observational trial at an outpatient tertiary care center in Amman, Jordan.
  
  
  METHODS
  We obtained detailed family histories, medical evaluations and laboratory tests for 94 short-statured children (50 boys and 44 girls aged 6-16 years).
  
  
  METHODS
  Complete and partial GHD were defined as peak GH responses of 5 and 7 μg/l (15 and 21 mIU/l) [IRMA/DiaSorin®], respectively, in both exercise and insulin tolerance tests.
  
  
  RESULTS
  GHD was diagnosed in 69·1% of the short children, including 86% (43/50) of the children of consanguineous parents (83·3%, 93·8% and 81·8% of children of first cousins, first cousins once removed and second cousins, respectively) and 50% (20/44) of the children of nonconsanguineous parents (P = 0·039, 0·002 and 0·013, respectively). However, there was no statistically significant difference in the prevalence of small pituitary MRI between GH-deficient children of consanguineous parents and those of nonconsanguineous parents (28·6% vs 13·6%, P = 0·3).
  
  
  CONCLUSIONS
  The prevalence of IGHD among referred short children in Jordan was exceptionally high and significantly higher in the children of CM. In countries where CM is common, preconception counselling and rigorous surveillance for GHD in short children may be indicated.
authors.affiliation: Division of Endocrinology and Metabolism, Department of Internal Medicine, School of Medicine, The University of Jordan, Amman, Jordan.
authors.forename: Ayman A
authors.initials: AA
authors.lastname: Zayed
authors.forename: Moaath K
authors.initials: MK
authors.lastname: Mustafa Ali
authors.forename: Mohammad A
authors.initials: MA
authors.lastname: Al-Ani
authors.forename: Munther S
authors.initials: MS
authors.lastname: Momani
authors.forename: Al-Motassem F
authors.initials: AM
authors.lastname: Yousef
mesh_terms: D000293:Adolescent; D002648:Child; D003241:Consanguinity; D003430:Cross-Sectional Studies; D004393:Dwarfism, Pituitary / Q000453:epidemiology* / Q000235:genetics; D005260:Female; D006130:Growth Disorders / Q000453:epidemiology / Q000235:genetics; D006801:Humans; D007597:Jordan; D008297:Male; D015995:Prevalence; D062606:Tertiary Care Centers