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master's thesis
Kongenitalna dijafragmalna hernija
Zagreb: University of Zagreb, School of Medicine, 2015. urn:nbn:hr:105:116393
Matić, Marija
University of Zagreb
School of Medicine
Department of Pediatrics

Institutional repository: Dr Med - University of Zagreb School of Medicine Digital Repository

Cite this document

Matić, M. (2015). Kongenitalna dijafragmalna hernija (Master's thesis). Zagreb: University of Zagreb, School of Medicine. Retrieved from https://urn.nsk.hr/urn:nbn:hr:105:116393

Matić, Marija. "Kongenitalna dijafragmalna hernija." Master's thesis, University of Zagreb, School of Medicine, 2015. https://urn.nsk.hr/urn:nbn:hr:105:116393

Matić, Marija. "Kongenitalna dijafragmalna hernija." Master's thesis, University of Zagreb, School of Medicine, 2015. https://urn.nsk.hr/urn:nbn:hr:105:116393

Matić, M. (2015). 'Kongenitalna dijafragmalna hernija', Master's thesis, University of Zagreb, School of Medicine, accessed 26 November 2024, https://urn.nsk.hr/urn:nbn:hr:105:116393

Matić M. Kongenitalna dijafragmalna hernija [Master's thesis]. Zagreb: University of Zagreb, School of Medicine; 2015 [cited 2024 November 26] Available at: https://urn.nsk.hr/urn:nbn:hr:105:116393

M. Matić, "Kongenitalna dijafragmalna hernija", Master's thesis, University of Zagreb, School of Medicine, Zagreb, 2015. Available at: https://urn.nsk.hr/urn:nbn:hr:105:116393

Metadata
TitleKongenitalna dijafragmalna hernija
Title (english)Congenital diaphragmatic hernia
AuthorMarija Matić
MentorRuža Grizelj (mentor)
Committee memberJurica Vuković (predsjednik povjerenstva)
Committee memberMarija Jelušić (član povjerenstva)
Committee memberRuža Grizelj (član povjerenstva)
GranterUniversity of Zagreb
School of Medicine
(Department of Pediatrics)
Zagreb
Defense date and country2015-07-15, Croatia
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Pediatrics
Abstract
Kongenitalna dijafragmalna hernija (KDH) razvojni je poremećaj dijafragme u sklopu kojeg dolazi do prolapsa trbušnih organa u prsnu šupljinu. Defekt nastaje zbog poremećaja u razdvajanju prsne i trbušne šupljine tijekom 8. do 10. tjedna gestacije, u isto vrijeme kada se dešavaju ključna zbivanja u razvoju pluća i plućnih krvnih žila. Prolaps trbušnih organa sprječava normalan razvoj pluća i plućnih krvnih žila s posljedičnom parenhimnom i vaskularnom plućnom hipoplazijom. Klinička slika odraz je hipoplazije pluća, malpozicije srca, malrotacije crijeva i otvorenog duktusa Botalli. Defekt se češće nalazi na lijevoj strani i u većini je slučajeva ograničen na posterolateralni dio dijafragme. Incidencija iznosi oko 1 na 2500 živorođenih. Etiologija je nepoznata, ali novija istraživanja ukazuju na postojanje poremećaja retinoičkih signalnih puteva tijekom organogeneze. U 50-60% slučajeva KDH se javlja kao izolirana anomalija. Trećina bolesnika ima pridružene kardiovaskularne anomalije, a manji dio anomalije drugih organa i organskih sustava. KDH se može javiti u sklopu monogenskih bolesti ili kromosomskih aberacija kao što su Turnerov sindrom, trisomija 13, trisomija 18 i Fryns sindrom. Anomalija se u većini slučajeva dijagnosticira prenatalno. U slučajevima izražene plućne hipoplazije moguće je prenatalno liječenje reverzibilnom opstrukcijom fetalne traheje. Postnatalna terapija sastoji se od protektivne mehaničke ventilacije, agresivnog liječenja plućne hipertenzije te kirurškog zatvaranja dijafragmalnog defekta nakon kliničke stabilizacije bolesnika. Iako se preživljenje bolesnika kreće i do 80%, prema epidemiološkim studijama ono iznosi svega 30-40%.
Abstract (english)
Congenital diaphragmatic hernia (CDH) is a developmental defect in the formation of the diaphragm, which allows abdominal organs to herniate into the chest cavity. The defect is caused by a disturbed separation of thoracic and abdominal compartments between weeks 8 and 10 of gestation, in the critical period of bronchial and pulmonary artery branching. Herniated abdominal organs cause impaired lung growth and development, resulting in pulmonary parenchymal and vascular hypoplasia. Most common pathological findings are pulmonary hypoplasia, displacement of the heart, intestinal malrotation and patent ductus arteriosus. A defect occurs more often on the left side and in most cases it is limited to the posterolateral area. CDH occurs with an incidence of ∼ 1 case per 2500 live births. The etiology is unknown although new studies point to disturbance in the retinoid-signaling pathway during organogenesis. In 50-60% of cases, CDH occurs as an isolated finding. About one third of cases have cardiovascular malformations and lesser proportions have other defects. CDH may be associated with single gene disorders or chromosomal aberrations, as in Turner syndrome, trisomy 13, trisomy 18 and Fryns syndrome. Most cases are diagnosed antenatally. Reversible fetoscopic tracheal obstruction may be indicated in cases with severe lung hypoplasia and poor prognosis. The postnatal management consists of „gentle“ ventilation, aggressive treatment of pulmonary hypertension and surgical closure of the diaphragmatic defect when medical stability is achieved. The best hospital centres report 80% survival but it remains around 30-40% in population-based studies.
Keywords
Keywords (english)
Languagecroatian
URN:NBNurn:nbn:hr:105:116393
Study programmeTitle: Medicine
Study programme type: university
Study level: integrated undergraduate and graduate
Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
Type of resourceText
File originBorn digital
Access conditionsOpen access
Terms of useLicence In copyright icon
Created on2016-08-16 09:45:43