title: Psychiatric illness and intellectual disability in the Prader-Willi syndrome with different molecular defects--a meta analysis.
abstract: OBJECTIVE
  Several studies have suggested a difference in clinical features of intellectual ability and psychiatric illness in the Prader-Willi syndrome (PWS) with the 15q11-q13 paternal deletion and maternal uniparental disomy (mUPD). Our objective was to appraise evidence on this association through a meta-analysis.
  
  
  METHODS
  The electronic records PubMed and EMBASE from 1956 to 2012 were extracted for meta-analysis. Meta-analyses were performed by using fixed effect model. Mean difference, odds ratio, and 95% confidence interval were calculated.
  
  
  RESULTS
  We retrieved a total of 744 PWS cases from 13 studies. These include 423 cases with paternal 15q11-q13 deletions and 318 cases of mUPD. Compare to the PWS cases with mUPD, PWS patients with the paternal 15q11-q13 deletion associated with significantly lower full scale IQ (FSIQ) [mean difference (MD), -2.69; 95%CI, -4.86 to -0.52; p=0.02] and verbal IQ (VIQ) (MD, -7.5; 95%CI, -9.75 to -5.26; p<0.00001) but higher performance IQ (PIQ) (MD, 4.02; 95%CI, 1.13 to 6.91; p=0.006). In contrast, PWS patients with mUPD are associated with significantly higher risk of psychiatric illness [odds rate (OR), 0.14; 95%CI, 0.08 to 0.23; p<0.00001] and higher risk of bipolar disorder (OR, 0.04; 95%CI, 0.01 to 0.23; p=0.0002).
  
  
  CONCLUSIONS
  Significant different clinical features of cognitive development and psychiatric illness are associated with PWS with different molecular defects. These findings provide support for evidence based practice to evaluate and manage the PWS syndrome with different molecular defects.
authors: Children's Hospital, Fudan University, Shanghai, China.
authors: Lin
authors: L
authors: Yang
authors: Guo-dong
authors: GD
authors: Zhan
authors: Jun-jie
authors: JJ
authors: Ding
authors: Hui-jun
authors: HJ
authors: Wang
authors: Duan
authors: D
authors: Ma
authors: Guo-ying
authors: GY
authors: Huang
authors: Wen-hao
authors: WH
authors: Zhou
mesh_terms: D001714:Bipolar Disorder / Q000150:complications; D002872:Chromosome Deletion; D006801:Humans; D008607:Intellectual Disability / Q000150:complications* / Q000235:genetics; D001523:Mental Disorders / Q000150:complications* / Q000235:genetics; D011218:Prader-Willi Syndrome / Q000150:complications* / Q000235:genetics*; D024182:Uniparental Disomy
references: Pediatrics. 2001 Nov;108(5):E92
references: 11694676
references: J Intellect Disabil Res. 2004 Feb;48(Pt 2):172-87
references: 14723659
references: J Intellect Disabil Res. 2007 Jan;51(Pt 1):32-42
references: 17181601
references: Nat Genet. 1997 Jan;15(1):74-7
references: 8988172
references: Res Dev Disabil. 2011 Sep-Oct;32(5):1729-35
references: 21454045
references: Nat Genet. 1997 Jan;15(1):70-3
references: 8988171
references: Eur Child Adolesc Psychiatry. 2004 Feb;13(1):42-50
references: 14991431
references: Am J Med Genet A. 2005 Jul 15;136(2):140-5
references: 15940679
references: Neurogenetics. 2007 Aug;8(3):169-78
references: 17415598
references: Am J Med Genet A. 2006 Jun 1;140(11):1136-42
references: 16646032
references: Am J Hum Genet. 1997 Apr;60(4):928-34
references: 9106540
references: Pediatrics. 1993 Feb;91(2):398-402
references: 8424017
references: J Intellect Disabil Res. 2001 Aug;45(Pt 4):317-25
references: 11489053
references: J Med Genet. 2009 Feb;46(2):86-93
references: 18835857
references: J Intellect Disabil Res. 2005 Aug;49(Pt 8):613-25
references: 16011554
references: Am J Med Genet. 1990 Sep;37(1):97-9
references: 2240051
references: Am J Med Genet. 1996 Oct 16;65(2):133-6
references: 8911605
references: Psychol Med. 2003 Jan;33(1):141-53
references: 12537045
references: Sci Transl Med. 2011 Oct 5;3(103):103ra97
references: 21974935
references: Cell. 1993 Nov 5;75(3):495-505
references: 8221889
references: Proc Natl Acad Sci U S A. 2001 Jul 17;98(15):8844-9
references: 11438699
references: N Engl J Med. 1981 Feb 5;304(6):325-9
references: 7442771
references: Psychiatr Genet. 2005 Dec;15(4):243-54
references: 16314754
references: Nat Genet. 2008 Jun;40(6):719-21
references: 18500341
references: Nature. 1989 Nov 16;342(6247):281-5
references: 2812027
references: J Endocrinol Invest. 2011 Jul-Aug;34(7):493-7
references: 20651469
references: Psychol Med. 2008 Oct;38(10):1505-14
references: 18177526
references: J Med Genet. 2001 Nov;38(11):792-8
references: 11732491
references: Am J Med Genet. 1990 Mar;35(3):319-32
references: 2309779
references: J Clin Endocrinol Metab. 2008 Nov;93(11):4183-97
references: 18697869
references: J Child Psychol Psychiatry. 2008 Sep;49(9):1001-8
references: 18665884
references: J Intellect Disabil Res. 2007 Jun;51(Pt. 6):478-87
references: 17493030
references: J Intellect Disabil Res. 2000 Feb;44 ( Pt 1):25-30
references: 10711647
references: J Child Psychol Psychiatry. 2005 Oct;46(10):1089-96
references: 16178933
references: Hum Mol Genet. 2009 Sep 1;18(17):3257-65
references: 19498035
references: Eur J Hum Genet. 2010 Nov;18(11):1196-201
references: 20588305
references: J Intellect Disabil Res. 2010 Oct;54(10):906-17
references: 20636465
references: Pediatrics. 2004 Mar;113(3 Pt 1):565-73
references: 14993551
references: Lancet. 2002 Jan 12;359(9301):135-6
references: 11809260
references: Am J Med Genet A. 2007 Mar 1;143A(5):460-8
references: 17163531
references: Am J Ment Retard. 1999 Jan;104(1):67-77
references: 9972835
references: Nat Genet. 1995 Apr;9(4):395-400
references: 7795645
references: Am J Med Genet A. 2008 Aug 15;146A(16):2041-52
references: 18627066
references: Nat Rev Genet. 2011 Jul 18;12(8):565-75
references: 21765458
references: Hum Mol Genet. 2008 Jan 1;17(1):111-8
references: 17940072
references: Am J Hum Genet. 1995 Jul;57(1):40-8
references: 7611294