Title Ishodi liječenja djece s Hirschsprungovom bolesti u Kliničkom bolničkom centru Split
Title (english) Treatment outcomes in children with Hirschsprung's disease in the University Hospital of Split
Author Marijana Božinović-Karauz
Mentor Branka Polić (mentor)
Committee member Ivana Mudnić (predsjednik povjerenstva)
Committee member Davor Todorić (član povjerenstva)
Committee member Doris Rušić (član povjerenstva)
Granter University of Split School of Medicine Split
Defense date and country 2024-07-24, Croatia
Scientific / art field, discipline and subdiscipline BIOMEDICINE AND HEALTHCARE Clinical Medical Sciences Pediatrics
Abstract Cilj istraživanja: Cilj ovog istraživanja je analizirati demografske, epidemiološke, antropometrijske, kliničke i dijagnostičke karakteristike te ishode liječenja djece oboljele od Hirschsprungove bolesti liječene u KBC-u Split u periodu od 2002. do 2022.
Materijali i metode: Istraživanje je provedeno na 28 djece s Hirschsprungovom bolesti koja su liječena u KBC-u Split u razdoblju od 1. siječnja 2002. do 31. prosinca 2022.
Rezultati: 23 (82,1%) ispitanika muškog je spola od ukupno 28 djece s dijagnozom Hirschsprungove bolesti. Medijan gestacijske dobi je bio 40 tjedana, a porođajne mase 3700 grama. Polovina bolesnika, njih 14 (50%) nisu samostalno praznili mekonij u prvih 48 sati života. Najveći broj djece prvi put je hospitalizirano zbog opstipacije, njih 14 (50%). Za 16 (57,1%) bolesnika nalaz prve irigografije se uklapa u dijagnozu Hirschsprungove bolesti. Prva biopsija rektuma je potvrdila dijagnozu za 26 (92,9%) bolesnika. Medijan dobi djece pri potvrdnoj biopsiji bio je 6 mjeseci. Stomu prije konačnog operacijskog zahvata imalo je 19 bolesnika (67,9%). Epizodu eneterokolitisa u anamnezi prije konačnog zahvata imalo je 14 (50%) bolesnika. Toksični kolitis u anamnezi prije konačnog zahvata imalo je 10 (36%) bolesnika. Veći broj bolesnika, njih 21 (75%) imalo je elektivan prvi operacijski zahvat u usporedbi s njih 7 (25%) kojima je učinjen hitan prvi operacijski zahvat. Medijan ukupnog broja operacijskih zahvata zbog osnovne bolesti je 3. Medijan vremena proteklog od rođenja do konačnog operacijskog zahvata je 14 mjeseci. U najvećem broju slučajeva, njih 25 (89,3%), konačni zahvat izveden je operacijskom tehnikom po Soave-u. Medijan ukupnog broja dana intenzivne njege nakon konačnog zahvata bio je 5 dana, dok je u JILD-u bio 2 dana. Medijan uvođenja peroralne prehrane nakon konačnog zahvata iznosi 4 dana. Sve poslijeoperacijske komplikacije su nastale u djece operirane tehnikom po Soave-u transabdominalnim pristupom. Od ukupno 28 djece njih 27 (96,4%) je preživjelo dok je 1 (3,6%) dijete umrlo od sepse i komplikacija nakon višestrukih operacijskih zahvata.
Zaključci: Hirschsprungova bolest predstavlja izazov u dijagnosticiranju i liječenju te može značajno utjecati na kvalitetu života djece koja žive s njom. Timski rad liječnika različitih specijalnosti, pravovremeno postavljanje dijagnoze i adekvatno kirurško liječenje imaju ključnu ulogu u konačnom ishodu.
Abstract (english) Objectives: The aim of this research is to analyze the demographic, epidemiological, anthropometric, clinical and diagnostic characteristics and treatment outcomes of children with Hirschsprung's disease treated in the Univerity Hospital of Split in the period from 2002 to 2022.
Materials and methods: The research was conducted on 28 children with Hirschsprung’s disease, which were treated in the Univerity Hospital of Split during the period from 2002 to 2022.
Results: From a total of 28 children with a diagnosis of Hirschsprung's disease 23 (82,1%) subjects were male. The median gestational age was 40 weeks and median birth weight was 3700 grams. Half of the patients, 14 (50%) of them did not empty meconium independently in the first 48 hours of life. Most of the children were hospitalized for the first time because of constipation, 14 of them (50%). For 16 (57.1%) patients, the findings of the first irrigography fit in the diagnosis of Hirschsprung's disease. The first rectal biopsy confirmed the diagnosis for 26 (92.9%) patients. The median age of the children at the time of the confirmatory biopsy was 6 months. 19 patients (67.9%) had a stoma before the final surgical procedure. 14 (50%) patients had an episode of enterocolitis in their medical history, before the final surgical procedure. There was history of toxic colitis in 10 (36%) patients, before the final procedure. A larger number of patients, 21 of them (75%), had an elective first surgical procedure compared to 7 (25%) who had an urgent first surgical procedure. The median time that passed from birth to the final procedure is 14 months. In the largest number of cases, 25 of them (89.3%), the final surgical procedure was performed using the Soave technique. The median total number of intensive care days after the final procedure was 5 days, while the median total number of days in the PICU after the final procedure was 2 days. Median introduction of oral nutrition after the final procedure is 4 days. All postoperative complications occurred in children that underwent the Soave procedure via transabdominal approach. Out of the total 28 children, 27 of them (96.4%) survived, while 1 (3.6%) child died of sepsis and complications.
Conclusions: Hirschsprung's disease represents a significant challenge in diagnosis and treatment, and can significantly affect the quality of life of the children who are affected by it. Teamwork of doctors with different specialties, timely diagnosis and adequate surgical treatment play a key role in the final outcome.
Keywords
Hirschsprungova bolest
Keywords (english)
Hirschsprung Disease
Language croatian
URN:NBN urn:nbn:hr:171:113737
Study programme Title: Medicine Study programme type: university Study level: integrated undergraduate and graduate Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
Type of resource Text
File origin Born digital
Access conditions Open access Embargo expiration date: 2024-07-24
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Created on 2024-07-24 07:36:58