Van Wyk-Grumbach综合征临床特点及发病机制分析

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Abstract Objective To summarize the c1inica1 feature of Van Wyk-Grumbach syndrome and ana1yze the possib1e pathogenesis.Methods C1inica1 data of 7 Van Wyk-Grumbach syndrome of our hospita1 from August 2013 to September 2015 was retrospective ana1yzed.Re1ated Documents review was conducted.Results A11 of patients had hypothyroidism with precocious puberty,and with different c1inica1 examination resu1ts.The 1eve1 of LH,FSH,PRL cou1d not be increased.The GNRH stimu1ation test was negative or positive.Most of them have retardation of bone age,pituitary hyperp1asia,po1ytrichia,anemia or gonad en1argement,but these indexes and symptoms cou1d be recovered by treatment with thyroid hormone.Conclusion The cognition to Van Wyk-Grumbach syndrome shou1d be improved in the process of c1inica1 diagnosis and treatment.For the chi1dren with precocious puberty（gir1s breast deve1opment,vagina1 b1eeding, boys testic1e en1argement）with retardation of bone age,growth dece1erated,ovarian cyst,en1arged testic1e or pituitary hyperp1asia, etc.,thyroid function shou1d be paid attention to screening.Precocious puberty of Van Wyk-Grumbach syndrome is not a rea1 gonadotropin- independent in most of patients.Thyroid hormone treatment can ame1iorate the c1inica1 symptom of chi1dren,avoid unnecessary drugs and surgica1 treatment.

Key words： Primary hypothyroidism Precocious puberty Ovarian cyst Testic1e en1argement Pituitary hyperp1asia

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[2]	中华医学会儿科学会内分泌遗传代谢学组.中枢性性早熟诊断与治疗共识（2015）[J].中华儿科杂志，2015，53（6）：412-418.
[1]	Christens A，Sevenants L，Toe1en J，et al.Van Wyk and Grumbach syndrome：an unusua1 form of precocious puberty[J].Gyneco1 Endocrino1，2014，30（4）：272-276.
[3]	Cabrera SM，DiMeg1io LA，Eugster EA.Incidence and characteristics of pseudoprecocious puberty because of primary hypothyroidism[J].J Pediatr，2013，162（3）：637-639.
[4]	Van Wyck JJ，Grumbach MM.Syndrome of precocious menstruation and ga1actorrhoea in juveni1e hypothyroidism：an examp1e of hormona1 over1ap in pituitary feedback[J].J Pediatr，1960，57（3）：416-435.
[5]	Baranowski E，Hoeg1erW.Anunusua1presentationofacquired hypothyroidism：the Van-Wyk Grumbach syndrome[J].Eur J Endocrino1，2012，166（3）：537-542.
[6]	Patni N，Cervantes LF，Diaz A.E1evated α-fetoprotein 1eve1s in Van Wyk-Grumbach syndrome：a case report and review of 1iterature[J].J Pediatr Endocrino1 Metab，2012，25（7-8）：761-767.
[7]	Chattopadhyay A，Kumar V，Maru1aiah M.Po1ycystic ovaries，precocious puberty and acquired hypothyroidism：the Van Wyk and Grumbach syndrome[J].J Pediatr Surg，2003，38 （9）：1390-1392.
[8]	Weber G，Vigone MC，Stroppa L，et al.Thyroid function and puberty[J].J Pediatr Endocrino1 Metab Mar，2003，16（2）：253-257.
[9]	Ryan GL，Feng X，d′A1va CB，et al.Eva1uating the ro1es of fo11ic1e-stimu1ating hormone receptor po1ymorphisms in gonada1 hyperstimu1ation associated with severe juveni1e primary hypothyroidism[J].J C1in Endocrino1 Metab，2007，92（6）：2312-2317.
[10]	Jannini EA，U1isse S，D′Armiento M.Thyroid hormone and ma1e gonada1 function[J].Endocr Rev，1995，16（4）：443-459.
[11]	Omran A，Peng J，Shrestha B，et al.Ma1e chi1d with Van Wyk Grumbach′s Syndrome and other comp1ications of 1ong standing primary hypoyhyroidism：a case report[J].Case Rep Pediatr，2012，2012：352751.
[12]	Franceschi R，Rozzaningo U，Fai1o R，et al.Pituitary hyperp1asia secondary to acquired hypothyroidism：case report[J].Ita1 J Pediatr，2011，37（4）：15-18.
[13]	Eom KS，See-Sung C，Kim JD，et al.Primary hypothyroidism mimicking a pituitary marcroadenoma：regression after thyiod hormone rep1acement therapy[J].Pediatr Radio1，2009，39（2）：164-167.
[14]	Khawaja NM，Taher BM，Barham ME，et al.Pituitary en1argement in patients with primary hypothyroidism[J].Endocr Pract，2006，12（1）：29-34.
[15]	Chiamo1era MI，Wondisford FE.Minireview：thyrotropinre1easing hormone and the thyroid hormone feedback mechanism[J].Endocrino1ogy，2009，150（30）：1091-1096.