md-medicaldata

Authors

Emilija Vujičić^1,2, Miroslav Ilić^1,3, Dragica Kovačević^1,2, Dejan Miljković^1,4, Jovan Javorac^1,3, Nevena Savić¹, Dejan Đekić <sup>1

1</sup>Institut za plućne bolesti Vojvodine, Sremska Kamenica, Srbija
²Univerzitet u Novom Sadu, Medicinski fakultet Novi Sad, Srbija
³Univerzitet u Novom Sadu, Katedra za internu medicinu, Medicinski fakultet Novi Sad, Srbija
⁴Univerzitet u Novom Sadu, Katedra za histologiju i embriologiju, Medicinski fakultet Novi Sad, Srbija

 

UDK: 616.24:616.993.1
(497.113)"2013/2023"

The paper was received / Rad primljen: 14.02.2025.

Accepted / Rad prihvaćen: 31.03.2025.

 

Correspondence to:

Emilija Vujičić
Univerzitet u Novom Sadu, Medicinski fakultet Novi Sad, Srbija
Institut za plućne bolesti Vojvodine, Sremska Kamenica
Put doktora Goldmana 4,
21204 Sremska Kamenica
Hajduk Veljkova 3, 21000 Novi Sad
e-mail: emilija.vujicic@institut.rs

 

 

Sažetak

 

 

Uvod: Plućna alveolarna proteinoza je retka respiratorna bolest uzrokovana malfunkcijom alveolarnih makrofaga, što dovodi do akumulacije surfaktantna u alveolarnom prostoru, te nastanka hipoksemične respiratorne insuficijencije, recidivantnih sekundarnih infekcija i/ili plućne fibroze. Cilj: Analiziranje kliničkih karakteristika pacijenata tokom desetogodišnjeg perioda (2013-2023.), lečenih u Intitutu za plućne bolesti Vojvodine. Materijal i metode: Retrospektivna studija koja je obuhvatila 8 pacijenta. Analizirani su pol, starost, komorbiditeti, pušački status, simptomi, parametri plućne funkcije, način dokazivanja bolesti, radiološka prezentacija bolesti pre lavaže, prisutnost lavaža, biohemijski parametar-laktat dehidrogenaza, remisija bolesti, zanimanje i smrtni ishod. Korišćene su neparametarske metode za testiranje statističkih hipoteza (α=0,05). Rezultati: Prosečna starost bila je 53,25±12,56 godina. Broj muških i ženskih ispitanika je bio jednak. Dominantni su bili aktivni pušači i pacijenti koji nikada nisu pušili. Kod svih pacijenata dijagnoza je bila postavljena uz pomoć patohistološkog nalaza. Najčešći simptomi bili su otežano disanje i kašalj. DSS skor 4 je bio prisutan kod svakog drugog pacijenta. Karakterističnu prezentaciju bolesti na CT pregledu grudnog koša je imalo 75% pacijenata. Lavaža oba plućna krila je urađena kod 50% pacijenata. Spontanu remisiju imalo je 50% pacijenata, remisiju nakon lavaže 37,5%. Smrtni ishod je bio prisutan kod 37,5%. Svi pacijenti su umrli od egzacerbacije osnovne bolesti. Zaključak: Potrebna su dalja istraživanja kako bi se poboljšale dijagnostičko-terapijske strategije, što bi sve trebalo da rezultira poboljšanju kvaliteta života onih koji su pogođeni ovom retkom bolešću.

 

Ključne reči:

plućna alveolarna proteinoza; plućni surfaktant; bronhoalveolarna lavaža; faktor stimulacije kolonija granulocita i makrofaga

 

 

 

Abstract

 

Introduction: Pulmonary alveolar proteinosis is a rare respiratory disease caused by the malfunction of alveolar macrophages, which leads to the accumulation of surfactant in the alveolar space, and the occurrence of hypoxemic respiratory insufficiency, recurrent secondary infections and/or pulmonary fibrosis. Aim: Analyzing the clinical characteristics of patients during ten years (2013-2023.), treated at the Institute for Pulmonary Diseases of Vojvodina. Material and methods: This retrospective study included 8 patients. Analyzed variables included gender, age, comorbidities, smoking status, symptoms, pulmonary function, diagnostic methods, radiological presentation before lavage, presence of lavage, lactate dehydrogenase levels, disease remission, occupation, and death outcome. Non-parametric statistical methods were used for analysis (α = 0,05). Results: The average age was 53.25±12.56 years. The number of male and female patients was equal. Dominant were active smokers and patients who had never smoked. Pathohistological findings in all patients confirmed the diagnosis. The most common symptoms were shortness of breath and cough. A DSS score of 4 was present in 50% of patients. 75% had a characteristic chest CT presentation. Whole lung lavage was performed in 50% of patients. Precisely 50% of patients experienced spontaneous remission, while 37.5% had remission after lavage. Death occurred in 37.5% of patients, all due to disease exacerbation. Conclusion: Further research is needed to improve diagnostic and therapeutic strategies, enhancing the quality of life for patients with this rare disease.

 

Key words:

pulmonary alveolar proteinosis; pulmonary surfactant; bronchoalveolar lavage; granulocyte-macrophage colony-stimulating factor

 

 

 

 

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