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Pituitary stalk interruption syndrome

MedGen UID:
797683
Concept ID:
CN206776
Disease or Syndrome
Synonyms: Ectopic neurohypophysis; PSIS
 

Definition

Pituitary stalk interruption syndrome (PSIS) is a congenital abnormality of the pituitary that is responsible for pituitary deficiency and is usually characterized by the triad of a very thin or interrupted pituitary stalk, an ectopic (or absent) posterior pituitary (EPP) and hypoplasia or aplasia of the anterior pituitary visible on MRI. In some patients the abnormality may be limited to EPP (also called ectopic neurohypophysis) or to an interrupted pituitary stalk. [from ORDO]

Recent clinical studies

Etiology

Tatsi C, Sertedaki A, Voutetakis A, Valavani E, Magiakou MA, Kanaka-Gantenbein C, Chrousos GP, Dacou-Voutetakis C
J Clin Endocrinol Metab 2013 Apr;98(4):E779-84. Epub 2013 Mar 8 doi: 10.1210/jc.2012-3982. [Epub ahead of print] PMID: 23476075
Guo Q, Yang Y, Mu Y, Lu J, Pan C, Dou J, Lv Z, Ba J, Wang B, Zou X, Yang L, Ouyang J, Yang G, Wang X, Du J, Gu W, Jin N, Chen K, Zang L, Erickson BJ
PLoS One 2013;8(1):e53579. Epub 2013 Jan 14 doi: 10.1371/journal.pone.0053579. PMID: 23341953Free PMC Article
Pham LL, Lemaire P, Harroche A, Souberbielle JC, Brauner R
PLoS One 2013;8(1):e53189. Epub 2013 Jan 7 doi: 10.1371/journal.pone.0053189. PMID: 23308160Free PMC Article
Yang Y, Guo QH, Wang BA, Dou JT, Lv ZH, Ba JM, Lu JM, Pan CY, Mu YM
Clin Endocrinol (Oxf) 2013 Jul;79(1):86-92. Epub 2013 Apr 27 doi: 10.1111/cen.12116. [Epub ahead of print] PMID: 23199197
Reynaud R, Albarel F, Saveanu A, Kaffel N, Castinetti F, Lecomte P, Brauner R, Simonin G, Gaudart J, Carmona E, Enjalbert A, Barlier A, Brue T
Eur J Endocrinol 2011 Apr;164(4):457-65. Epub 2011 Jan 26 doi: 10.1530/EJE-10-0892. [Epub ahead of print] PMID: 21270112

Diagnosis

Dekeyzer S, Herregods N, Meersschaut V, De Schepper J
JBR-BTR 2013 Nov-Dec;96(6):393. PMID: 24617191
Guo Q, Yang Y, Mu Y, Lu J, Pan C, Dou J, Lv Z, Ba J, Wang B, Zou X, Yang L, Ouyang J, Yang G, Wang X, Du J, Gu W, Jin N, Chen K, Zang L, Erickson BJ
PLoS One 2013;8(1):e53579. Epub 2013 Jan 14 doi: 10.1371/journal.pone.0053579. PMID: 23341953Free PMC Article
Pham LL, Lemaire P, Harroche A, Souberbielle JC, Brauner R
PLoS One 2013;8(1):e53189. Epub 2013 Jan 7 doi: 10.1371/journal.pone.0053189. PMID: 23308160Free PMC Article
Qi Wei P, Hui Juan Z, Feng Ying G, Nai Shi L, Tao Z, Gang B, Hui P, Xian-wei Z
Zhongguo Yi Xue Ke Xue Yuan Xue Bao 2011 Feb;33(1):9-13. doi: 10.3881/j.issn.1000-503X.2011.01.003. PMID: 21375930
Gascoin-Lachambre G, Brauner R, Duche L, Chalumeau M
PLoS One 2011 Jan 27;6(1):e16367. doi: 10.1371/journal.pone.0016367. PMID: 21298012Free PMC Article

Therapy

Tsai SL, Laffan E, Lawrence S
Pediatr Radiol 2012 Jul;42(7):799-804. doi: 10.1007/s00247-012-2421-3. PMID: 22763804
El Chehadeh S, Bensignor C, de Monléon JV, Méjean N, Huet F
Ann Endocrinol (Paris) 2010 Mar;71(2):102-10. Epub 2009 Dec 30 doi: 10.1016/j.ando.2009.11.007. [Epub ahead of print] PMID: 20044069
Louvel M, Marcu M, Trivin C, Souberbielle JC, Brauner R
BMC Pediatr 2009 May 6;9:29. doi: 10.1186/1471-2431-9-29. [Epub ahead of print] PMID: 19419564Free PMC Article
Reynaud R, Gueydan M, Saveanu A, Vallette-Kasic S, Enjalbert A, Brue T, Barlier A
J Clin Endocrinol Metab 2006 Sep;91(9):3329-36. Epub 2006 May 30 doi: 10.1210/jc.2005-2173. [Epub ahead of print] PMID: 16735499
Tauber M, Chevrel J, Diene G, Moulin P, Jouret B, Oliver I, Pienkowski C, Sevely A
Horm Res 2005;64(6):266-73. Epub 2005 Oct 15 doi: 10.1159/000089425. [Epub ahead of print] PMID: 16260897

Prognosis

Reynaud R, Albarel F, Saveanu A, Kaffel N, Castinetti F, Lecomte P, Brauner R, Simonin G, Gaudart J, Carmona E, Enjalbert A, Barlier A, Brue T
Eur J Endocrinol 2011 Apr;164(4):457-65. Epub 2011 Jan 26 doi: 10.1530/EJE-10-0892. [Epub ahead of print] PMID: 21270112
Louvel M, Marcu M, Trivin C, Souberbielle JC, Brauner R
BMC Pediatr 2009 May 6;9:29. doi: 10.1186/1471-2431-9-29. [Epub ahead of print] PMID: 19419564Free PMC Article
Lemaire P, Brauner N, Hammer P, Trivin C, Souberbielle JC, Brauner R
Med Sci Monit 2009 Jan;15(1):MT5-10. PMID: 19114977
Duché L, Trivin C, Chemaitilly W, Souberbielle JC, Bréart G, Brauner R, Chalumeau M
BMC Pediatr 2008 Jul 16;8:29. doi: 10.1186/1471-2431-8-29. [Epub ahead of print] PMID: 18631396Free PMC Article
Arrigo T, De Luca F, Maghnie M, Blandino A, Lombardo F, Messina MF, Wasniewska M, Ghizzoni L, Bozzola M
Eur J Endocrinol 1998 Jul;139(1):84-8. PMID: 9703383

Clinical prediction guides

Tatsi C, Sertedaki A, Voutetakis A, Valavani E, Magiakou MA, Kanaka-Gantenbein C, Chrousos GP, Dacou-Voutetakis C
J Clin Endocrinol Metab 2013 Apr;98(4):E779-84. Epub 2013 Mar 8 doi: 10.1210/jc.2012-3982. [Epub ahead of print] PMID: 23476075
El Chehadeh-Djebbar S, Callier P, Masurel-Paulet A, Bensignor C, Méjean N, Payet M, Ragon C, Durand C, Marle N, Mosca-Boidron AL, Huet F, Mugneret F, Faivre L, Thauvin-Robinet C
Eur J Med Genet 2011 May-Jun;54(3):369-73. Epub 2011 Mar 30 doi: 10.1016/j.ejmg.2011.03.001. [Epub ahead of print] PMID: 21397059
Gascoin-Lachambre G, Brauner R, Duche L, Chalumeau M
PLoS One 2011 Jan 27;6(1):e16367. doi: 10.1371/journal.pone.0016367. PMID: 21298012Free PMC Article
Reynaud R, Albarel F, Saveanu A, Kaffel N, Castinetti F, Lecomte P, Brauner R, Simonin G, Gaudart J, Carmona E, Enjalbert A, Barlier A, Brue T
Eur J Endocrinol 2011 Apr;164(4):457-65. Epub 2011 Jan 26 doi: 10.1530/EJE-10-0892. [Epub ahead of print] PMID: 21270112
El Chehadeh S, Bensignor C, de Monléon JV, Méjean N, Huet F
Ann Endocrinol (Paris) 2010 Mar;71(2):102-10. Epub 2009 Dec 30 doi: 10.1016/j.ando.2009.11.007. [Epub ahead of print] PMID: 20044069

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