Žemas ūgis: ankstyva diagnozė ir optimalus gydymas – geresnė prognozė

Žemas ūgis: ankstyva diagnozė ir optimalus gydymas – geresnė prognozė

Įvadas

 

Vaikų ūgio ir svorio augimas yra jų visokeriopos sveikatos atspindys. Vaiko augimas labiausiai priklauso nuo augimo hormono (AH), lytinių ir skydliaukės hormonų. Žmogaus augimą veikia ir kiti veiksniai – genetiniai ir aplinkos, visavertė mityba, fizinė ir psichinė sveikata, miegas, imuninė sistema. Sunki ar ilgai užsitęsusi lėtinė liga, kūdikystėje arba vaikystėje patirta galvos trauma gali silpninti augimą. Aišku, lemiamą vaidmenį atlieka genetika, ir tai ne tik genų nulemtos įvairios ligos ir būklės, pasireiškiančios žemu ūgiu, bet ir visiems puikiai suprantama tiesa, kad aukštesni tėvai paprastai augina aukštesnio ūgio vaikus, o žemesni – žemesnius.

Kas yra tas žemas ūgis? Remiantis statistika, medicinoje priimta, kad 95 proc. populiacijos yra normalaus ūgio, o likę 5 proc. yra arba žemo, arba aukšto ūgio. Todėl visi, kūdikiai, vaikai, paaugliai ir suaugusieji, kurių ūgis apytiksliai yra žemesnis už trečiąją procentilinę kreivę, yra žemo ūgio. Tačiau ūgio augimo sutrikimas gali būti nustatytas gerokai anksčiau, jei pastebėsime, kad vaiko augimas (ūgio augimo braižoma kreivė) neatitinka tėvų ūgio, taip vaidinamojo taikininio ūgio (TŪ) arba vidutinio ūgio pagal tėvus. Jį galima apskaičiuoti pagal formulę:

 

berniukų TŪ (cm) = [tėvo ūgis (cm) + motinos ūgis (cm) + 13] / 2;

mergaičių TŪ (cm) = [tėvo ūgis (cm) + motinos ūgis (cm) – 13] / 2.

 

Normalu, jei vaikai auga ±5 cm intervale apie TŪ procentilinę kreivę, tačiau atsiradus nukrypimui daugiau nei 5 cm ir šiam nukrypimui ryškėjant reikia sunerimti.

Svarbiausios endokrininės priežastys, kada žemas ūgis gydomas AH, yra izoliuotas AH trūkumas, kombinuotas hipofizės hormonų trūkumas, vaikai, gimę maži pagal nėštumo laiką, idiopatinis žemas ūgis, Turnerio, Noonano ir Praderio-Willio sindromai.

 

Klinikinis vaikų, kuriems sutrikęs augimas, ištyrimas

 

Turintiems nusiskundimų dėl žemo ūgio pacientams ir blogai augantiems vaikams ir paaugliams pirmiausia rekomenduojama nubraižyti individualias ūgio ir svorio augimo kreives (1 ir 2 pav.). Apskaičiuojamas vaiko augimo greitis per pastaruosius 4–15 mėnesių. Reikia sužinoti abiejų tėvų ūgį, jų lytinio brendimo ypatumus, paveldimąsias ligas. Apskaičiuojamas TŪ, įvertinama vaiko ūgio procentilės padėtis TŪ procentilės atžvilgiu. Svarbūs perinatalinio laikotarpio duomenys, ypač gimimo svoris ir ūgis, gestacijos amžius, nėštumo ir gimdymo charakteristikos.

Valgymo ypatumai bei išmatų pobūdis gali padėti išaiškinti kai kuriuos virškinimo sutrikimus. Reikia surinkti anamnezę ir apie persirgtas ligas, ypač apie onkologines, skirtą gydymą, vartotus vaistus…

Apskaičiuojamas ir įvertinamas kūno masės indeksas (KMI, kg/m2). Per mažas KMI gali būti nulemtas mitybos sutrikimų ar virškinimo sistemos ligų. Nustatoma lytinio brendimo stadija pagal Tannerį. Apžiūrima, ar nėra galimų dismorfinių bruožų ir lėtinių ligų simptomų. Atliekama plaštakos rentgenograma kaulų amžiui nustatyti.

 

Laboratoriniai tyrimai

 

Kadangi žemo ūgio priežastys gali būti labai įvairios, atliekami laboratoriniai tyrimai: bendrojo kraujo ir eritrocitų nusėdimo greičio (žarnyno uždegiminėms ligoms nustatyti), elektrolitų ir šlapimo tyrimas (inkstų ligoms), antikūnų prieš transgliutaminazę (dėl celiakijos). Endokrininės kilmės žemas ūgis pradedamas tirti nuo skydliaukės funkcijos (tirotropinio hormono ir tiroksino) nustatymo. Jei įtariamas AH trūkumas (mažas augimo greitis, kaulų amžiaus atsilikimas, žema pirmojo insuliniškojo augimo faktoriaus (IGF-1) koncentracija), AH sekrecijai įvertinti atliekami du stimuliaciniai mėginiai su insulinu, klonidinu ar gliukagonu. Žemo ūgio mergaitėms būtina genetiko konsultacija ir kariotipo tyrimas dėl įtariamo Turnerio sindromo.

Nustačius AH ir / ar kitų hipofizės hormonų trūkumą, atliekamas galvos smegenų branduolių magnetinio rezonanso tyrimas dėl įtariamos centrinės nervų sistemos organinės patologijos.

 

Gydymas

 

Siekiant kuo geresnių žemo ūgio gydymo rezultatų, labai svarbu pastebėti ūgio augimo sutrikimus, nustatyti priežastį ir atitinkamą gydymą pradėti kuo anksčiau. Visiškai nepateisinama laukimo ir pasyvaus stebėjimo taktika (prasidės brendimas – užaugs) arba bandymas aiškinti žemą paciento ūgį neaukštu tėvų ūgiu.

Kalbant apie žemo ūgio gydymą AH, ypač aktualus yra pagrindinis medicinos principas – primum non nocere (liet. pirmiausia nepakenkti). Svarbu atkreipti dėmesį į tai, kad žemo ūgio gydymas AH skiriamas ir kompensuojamas tik tada, kai somatropino sekrecijos sumažėjimas iki 20 mTV/l yra nustatytas dviem stimuliaciniais mėginiais. Tik kai yra organinė centrinės nervų sistemos patologija, užtenka vieno mėginio. Antra tarp svarbiausių po hipopituitarizmo AH skyrimo indikacija yra Turnerio sindromas. Kitos indikacijos: intrauterinis augimo sulėtėjimas, idiopatinis žemas ūgis, Praderio-Willio sindromas, lėtinė inkstų liga. Jokiu būdu negalima skirti AH, jei tik norima aukštesnio ūgio, pavyzdžiui, žaidžiant krepšinį.

Heterogeniškos grupės pacientams, turintiems izoliuotą AH trūkumą, dauginį hipofizės hormonų trūkumą, intrauterinį augimo sulėtėjimą, idiopatinį žemą ūgį, AH efektyvumo tyrimų metu nustatyta, kad geriausių rezultatų pasiekiama, kai gydymas pradedamas anksčiau ir kai per pirmus 4 mėnesius pacientai paauga daugiau (Lee ir kt., ANSWER Program®, 2011). Tai patvirtino ir vėlesni tyrimai. Geresni rezultatai pastebėti ir pacientams, kurių gydymo pradžioje KMI buvo didesnis, o ūgis ir IGF-1 koncentracija žemesni (Ross ir kt., 2015).

Backeljauw ir bendraautorių tyrime, pristatytame 2016 metais, buvo vertinami mergaičių, sergančių Turnerio sindromu, gydymo rezultatai. Teigiama, kad pacienčių, sergančių Turnerio sindromu, atsakas į gydymą AH ilgiau nei 4 metus priklauso nuo amžiaus gydymo pradžioje: ankstesnė iki 9 metų pacienčių gydymo pradžia nulėmė geresnius gydymo rezultatus, o vyresnėms nei 11 metų pacientėms ūgio augimo pagreitėjimas buvo susijęs su estrogenų šioje amžiaus grupėje vartojimu.

Ketverių metų trukmės izoliuoto AH trūkumo gydymas somatropinu, pradėtas iki 5 metų, lėmė ne tik geresnius rezultatus, bet ir buvo ekonomiškai naudingesnis, nes reikėjo mažesnių brangaus vaisto dozių (Miller ir kt., 2016).

Remiantis dar vienu tyrimu (Polak ir kt., 2016), esant izoliuotam AH trūkumui anksti (mergaitėms – iki 8 metų, berniukams – iki 9 metų) pradėtas gydymas AH taip pat davė geresnius rezultatus (suaugusio ūgis didesnis 4–5 cm), palyginti su gydymo pradžia vyresnio amžiaus pacientais. 3 pav. palyginimui pateikiame dviejų klinikinių izoliuoto AH trūkumo gydymo rezultatus, kada vienu atveju gydymas buvo pradėtas gana anksti – nuo 4 metų, o kitu – tik nuo 13 metų.

Kiti veiksniai, lemiantys geresnius gydymo rezultatus, buvo aukštesnis ūgis gydymo pradžioje, aukštesnis pacientų tėvų ūgis, žemesnė stimuliuoto AH koncentracija (Polak ir kt., 2016).

Plačiau skaitykite žurnale „Internistas“ Nr.7, 2017 m.

Gyd. Romualdas Tomas Preikša

Lietuvos sveikatos mokslų universitetas


413 minčių apie “Žemas ūgis: ankstyva diagnozė ir optimalus gydymas – geresnė prognozė

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