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0, Calvani L, Montuori M, Cipolletta E, Di Mario U, Bonamico M. The circulating insulin-like ... Feliciani M, Cicognani A, Partesotti S, Azzaroni D, Picazzoli P, Pisi.

ShortStature in Children withCoeliac Disease MeazzaCristina,PhD,PaganiSara,phD,LaarejKamitia,BS CantoniFrancesca, MD,Civalteropaota,MD, BoncimÍno Agata,MD,Bozzota Mauro,MD PaediatricDepartment,Universityof pavìa, FondazioneRCCSSanÀ atteo, rrary Corresponding author:MauroBozzola,l D, Pa€diatric Department, Universjty of Pavìa,Fondazton€ TRCCS SanMatteo,plazzate C. Golgi2, 27100Pavìa,taty,Tel:+390382502891, Fax:+39038252797ó,E maìl:mauro.bozzotaountovit j ndi vi duats. susceptj bte l t h a mul ti factori aL dl sorderthat depends0n b0th genetj cand envi ronmenral factofsfor oelia. dlseose(CD)k a Seneticolly detemined expressi on. The maj or predi sposl ng genesare l ocatedon gluten 5ensitiyeentetopothyresultihgin the H LAregìonof chfomosome ó. n fact, 95%of patj ent s nutrientmaLabsarption, with an increasing showsp€ciricHLA-DQ atteles,nameLyDQ2190%) and/or DQ8 íncldencewotlll.wide. Clinlcal presentattonin (51) (1).Thecoeljac€nt€ropathy is characterjzed by a variabte eatly chlldhoodtnoyincLude classicnaLobsorptton d€greeol villousatrophy,crypt hyperptasla, and af ìncreasea synptons,whercas olderCDchildrcnólten pretent ìntraepl th€tj al l ymphocyt€ counr. Intestj nat damagets the extta-intestlnol synptont inclùdlnsthort stoture jmmune end.stage lesion of an ìiapproprjate T,cett.mediated and pubertal deloy. A gluten.free diet (CFD) response gl !ten peptj d€s agai nst genetolLy rhat are modj fìed i n the leodtta a rapidcdtch.up in grcwthondto lamjnaproprjaby trafsglutaminase notndlizotion enzymes ol thepltultotyfunclian. l2). Thepothogenesis The ìnci dence oÍ CD-ossocldted of thìs dj sease short stotùre is hasgreatl yj ncreased w ìt h still uncleor.Begides the invol'r'enent af the grcwth a concomrtant decreasei n the severi tyof the symptof ir s growthldctorl oxis,o rcle hornone(GH)linsulin.like at djagnosjs(l). Prevat€nce of clinjcatlyovert CD has b€en lot ghrellnvns rccentlyprcposed. repo ed to be 1/99i n Fìnl and (4)andbetw een 1/285and1/77 Furthernore, some CD potlents do nor snow in Sweden(5,ó).lr ltalianschootchlldren the frequencyvarjes catch.!p 9towtn du,i.9 GFD.despite,eue,sian from 1/210(7) ro 1/r0ó (8). In the l j nj tedS tares C Daffect s to setonegotl\tltylot CD natkett lncluding 1/104chjtdrenby 5 yea6 of age(9). Despjtethe reportedhìgh ontiendonysialond ontl-tisguetronsglutaninase preval-Ànce of CD, only a few casesare cuf€ntly recognjz€d ontlbodies. fhesesubjects showGHdefi.lencyond grounds on cliricat alonè,because of the scarcityanddiversity couldpatentiallybeneiitfrom tecÒnbinant hunan (10). of symptoms Both the djversjry of symptoms and djsease GHtherapy. severityhas led to the concepiof a ,toetiac ic€b€rg"(1j) fhis reviewdeolswith the nonogenentoÍ short (Fi gure1). stotureand the evoluotíanoÍ grovtthaxisfunctionin CDchildren. fhe clinlcalsp€ctrumof CDinctudesrypjcal,atyptcat,site|r Ref:Ped,Èndoctlnol. Reu2009;ó(4):457 463 and potenti alformsof the di sèase.P atìentsw ìth typj cat Key words:Caelloc Disease; Ghrclin,Gluten;Short sl mpl or