Hipopituitarizmas: fiziologija, etiologija, klinika, diagnostika ir gydymas

Hipopituitarizmas: fiziologija, etiologija, klinika, diagnostika ir gydymas

Įvadas

Hipopituitarizmas – tai hipofizės hormono trūkumo sukeltas klinikinis sindromas, kuris gali būti susijęs su hipofizės, hipotalamo arba aplinkinių struktūrų pažeidimu [1]. Panhipopituitarizmas – tai visų hipofizinių hormonų trūkumas (dažniau – vieno ar kelių), kuris sukelia izoliuotą arba dalinį panhipopituitarizmą.

Šioje apžvalgoje aptarsime bendrąsias hipopituitarizmo priežastis, klinikinius simptomus, diagnostiką ir gydymą.

 

Hipofizės fiziologija

Hipofizės liauka yra sudaryta iš priekinės (adenohipofizė) ir užpakalinės (neurohipofizė) dalies. Priekinė hipofizės dalis gauna signalus iš hipotalamo, kurie stimuliuoja arba slopina hipofizės hormonų sekreciją. Hipofizės hormonai cirkuliuoja sisteminėje kraujotakoje ir veikia organus taikinius. Hipofizės ir organų taikinių hormonai gali veikti hipotalamusą atgaliniu ryšiu, taip padidindami ar sumažindami hipofizės, hipotalamo sekretuojančių hormonų kiekį. Dažnai būna sumažėjusi organų taikinių hormonų koncentracija ir normali arba sumažėjusi atitinkančio tropinio hormono koncentracija. Pavyzdžiui, maža laisvojo tiroksino koncentracija ir normali arba sumažėjusi tirotropinio hormono koncentracija rodo, kad yra sutrikusi hipofizė, o ne skydliaukė, dėl to centrinės kilmės hipotirozė liks nediagnozuota atliekant tik tireotropinio hormono tyrimą.

 Hormonai, kuriuos sekretuoja adenohipofizė:

  • tireotropinis hormonas (TTH);
  • gonadotropinai arba folikulus stimuliuojantis hormonas (FSH) ir liuteinizuojantis hormonas (LH);
  • somatotropinis arba augimo hormonas (AH);
  • kortikotropinas arba adenokortikotropinis hormonas (AKTH);

Hipotalamas gamina du hormonus, vazopresiną ir oksitociną, kurie saugomi neurohipofizėje ir, esant reikalui, sekretuojami į sisteminę kraujotaką. Vazopresinas, kitaip vadinamas antidiuretiniu hormonu (ADH), pirmiausia veikia inkstų distalinių kanalėlių V2 receptorius, reabsorbuodamas vandenį, padidina vandens, šlapimo osmoliariškumą ir sumažina šlapimo tūrį. Kitas hormonas – oksitocinas. Jis stimuliuoja nėščiųjų moterų gimdos lygiųjų raumenų susitraukimus ir yra svarbus maitinimo krūtimi procesui [2].


 

Etiologija

Remiantis tarptautiniais tyrimais, sergamumas hipopituitarizmu yra 4,2 atvejo 100 tūkst. žmonių per metus [3].

1 lentelė. Hipopituitarizmo priežastys [4]

Navikai, kurie sukelia hipofizės destrukciją:

   Intraseliariniai navikai (adenomos, kraniofaringiomos)

   Paraseliariniai navikai (meningiomos, regos nervo gliomos)

   Metastaziniai navikai (krūties, plaučių, melanoma,inkstų ląstelių karcinoma)

Hipofizės išeminė nekrozė

Po gimdymo (Sheehanso sindromas)

Cukrinis diabetas

Hipofizės apopleksija

Kavernozinio sinuso trombozė

Intrakranijinių galvos smegenų arterijų aneurizmos

Infekcinės ligos (tuberkuliozinis meningitas, grybelinės ligos, maliarija, ŽIV)

Infiltracinės ligos (hemohromotozė, antrinė amiloidozė)

Uždegiminės ligos (limfocitinis ar granuliomatozinis hipofizitas, sarkoidozė)

Tuščio turkiabalnio sindromas

Jatrogeninis

Nazofaringinis, hipofizės ir smegenų apšvitimas

Chirurginė destrukcija

Genetinės ligos (PIT-1, GH, β-LH, GHRH-R mutacijos, vazopresino ir neurofizino geno, KAL1 geno mutacijos)

Idiopatinis (GH, ACTH, TSH ir kt.): dažnai monohormoniniai

Galvos smegenų trauminis pažeidimas

Nervinė anoreksija

Egzogeninių hormonų sukeltas (gliukokortikoidai, gonadiniai steroidai)

Mityba (badavimas, nutukimas)

Sunki sisteminė liga

 

Klinikiniai simptomai

Klinikiniai simptomai priklauso nuo pagrindinės patologijos lokalizacijos, išsivystymo greičio ir sunkumo proceso. Pavyzdžiui, hipopituitarizmą sukėkės navikui, pacientą vargins galvos skausmas, suprastės rega, taip pat gali išsivystyti asmenybės sutrikimų. Klinikiniai simptomai gali pasireikšti per kelias valandas (necukrinis diabetas) arba dienas (antinksčių nepakankamumas) po hipofizės apopleksijos, hipofizektomijos, tačiau daugeliui pacientų simptomai progresuoja lėtai ir ilgą laiką liga lieka nediagnozuota…

Gyd. Tatjana Bašmakova

Vilniaus universiteto Medicinos fakulteto Vidaus ligų, šeimos medicinos ir onkologijos klinika

Plačiau, skaitykite žurnale „Endokrininių ligų aktualijos“, 2017 m.


137 mintys apie “Hipopituitarizmas: fiziologija, etiologija, klinika, diagnostika ir gydymas

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