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Novini vo pedijatrijata / Godi[na revija 2009<br />

SOVREMENA KLASIFIKACIJA I PRIOD KON<br />

DIJAGNOZATA I TRETMANOT NA HRONI^NA<br />

INTERSTICIELNA BELODROBNA BOLEST VO DETSTVOTO<br />

Emilija Vla[ki<br />

Univerzitetska klinika za detski bolesti<br />

Skopje, R. Makedonija<br />

Apstrakt<br />

Hroni~nata intersticielna bolest na belite drobovi vo detstvoto pretstavuva<br />

heterogena grupa na bolesti so zaedni~ki i sli~ni histolo[ki obele`ja<br />

i so poznata ili nepoznata etiologija. Tie mo`at da bidat familijarni,<br />

primarni ili idiopatski i sekundarni pri sistemska bolest ili belodroben<br />

insult. Retkata pojava i posledi~no ograni~eniot broj na klini~ki<br />

i istra`uva~ki studii predizvikuvaat seu[te dilemi vo dijagnostikata<br />

i terapijata na ovaa grupa na bolesti. Klini~ki ovaa grupa na bolesti<br />

mo`e da se manifestira od doene~ka vozrast do adolescencija so postepena<br />

pojava na tahipneja, gu[ewe, hipoksemija i kislorodna hiposaturacija, i<br />

~esto so difuzno postoewe na krepitacii. Naj~estite dijagnosti~kite ispituvawa<br />

vklu~uvaat pulsna oksimetrija/krvni gasovi, belodrobni funkcionalni<br />

testovi, rentgengrafija na beli drobovi, ECG/Ehokardiogram,<br />

imunolo[ka procenka i anti-HIV antitela, ANA/ANCA, serumski angiotenzin-konvertira~ki<br />

enzim (sACE), precipitira~ki IgG antitela za potencijalni<br />

alergeni, hloridi vo pot, pH-metrija na ezofagus i bronhoskopija so<br />

bronhoalveolarna lava`a. HR-CT skenot i otvorenata belodrobna biopsija<br />

ili video torakoskopskata biopsija se neophodni za utvrduvawe na dijagnozata.<br />

Terapijata obi~no se sostoi od oksigenoterapija, prolongirano koristewe<br />

na kortikosteroidi i/ili hydroxychloroquine, belodrobna transplantacija<br />

i kaj idiopatska belodrobna alveolarna proteinoza granulocyte<br />

macrophage colony-stimulating factor so lava`a na celoto belodrobie.<br />

Abstract<br />

Chronic interstitial lung disease in children represent a heterogeneous group of diseases<br />

that share common histological features and are of both known and unknown<br />

aetiology. They can be familial, primary or idiopathic and secondary as a part of a<br />

systemic illness or pulmonary insult. The rare frequency and consequently limited<br />

number of clinical and research studies still cause dilemmas in diagnostics and<br />

treatment of this group of diseases. Patients can present at any age from infancy to<br />

adolescence with gradual onset of tachypnoea, breathlessness, hypoxia and oxygen<br />

hyposaturation, and commonly with diffuse crackles. The most frequently used<br />

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