bluuebunny/crossref_metadata_2025 huggingface dataset:
Our experience in the podological analysis of children with motor disorders (20 cases)
Authors: Mansi Zied , Aymen Ben Mahmoud , Abdelkader Tounsi , Ahmed Mahmoudi , Wajdi Chermiti , Ali Haggui , Bacem Zaidi
Published: November 2024

Introduction and Importance: Pediatric patients with neuromotor disorders frequently experience various podiatric conditions. The effectiveness and implications of treatments like orthotics and insoles in this  demographic are not thoroughly understood, highlighting a significant gap in podiatric research and clinical practice.Case Presentation: This observational study analyzed 20 pediatric patients with neuromotor disorders. The focus was on common foot conditions such as ingrown toenails and hyperhidrosis, and their association with treatments like orthotics, insoles, and surgical interventions like Achilles tendon lengthening.Clinical Discussion: Statistical analysis using chi-square tests revealed significant associations. Notably, orthotic use was linked to a higher incidence of ingrown toenails (χ² = 5.69, p = 0.017). In contrast, insole usage correlated with increased hyperhidrosis (χ² = 4.44, p = 0.035). Additionally, a significant tendency for orthotic use was observed in patients who underwent Achilles tendon lengthening (χ² = 8.15, p = 0.017). The study also highlighted the prevalence of brittle nails and hyperkeratosis among the participants.Conclusion: The findings emphasize the critical role of podiatrists in the management of foot conditions in pediatric neuromotor disorder patients. While interventions like orthotics are generally beneficial, they may also exacerbate or contribute to other conditions, necessitating regular and careful podiatric monitoring. The study advocates for future research with larger sample sizes and controlled study designs to further validate and expand upon these observations.
Utility and benefits of Camp for Children with Epilepsy: a retrospective
Authors: Kukuruzović Monika , Cvitanović-Šojat Ljerka , Divčić Branka , Batista Maja , Kužnik Kristina , Šeparović Iva , Malenica Maša
Published: November 2024

Objective: The aim of this study was to evaluate the benefits of educational and recreational camping programs for children with epilepsy.Method: A retrospective review of data collected from all children attending annual epilepsy camps organized by the Croatian Epilepsy Association and the Zagreb City Office for Health during 8 years was performed. Children included were between 6 and 18 years of age. The staff of the camp includes child neurologists, a psychologist, neurology nurses and trained volunteers. Educationalactivities are designed to improve children’s knowledge of epilepsy, importance of taking medication, academic choices, suggested employment, and regulations regarding driving laws. These issues were discussed in groups or in single sessions, depending on thecase. Other activities included supervised swimming, team sports, fun activities and games, arts and crafts and entertainment such as disco or movie night.Each of the participants filled out three questionnaires 1. General data questionnaire and pre-camp knowledge test; 2. Camp satisfaction questionnaire and post-camp knowledge test; 3. Questionnaire on adaptation to epilepsy in young people.Results: The analyses of surveys and tests showed that only 75% of children in camp knew that they had epilepsy. In 10% of children, friends and distant family members did not know about epilepsy. Comparing the knowledge test before and after the camp, theaverage percentage of correctly solved tasks on the first test is 65%, and on the second 87%. The answers in the questionnaire on adaptation to life with epilepsy are indicators of strong stigma in children with epilepsy.Conclusions: The main accomplishment of the camp was for children to gain confidence that they can reach their full potential in life and to feel equal to their peers in every aspect of life.
Učestalost odštetne odgovornosti kod povrede pravnog standarda dužne pažnje u pedijatriji kao zdravstvenoj djelatnosti
Authors: Vuletić Dominik
Published: November 2024

Objective: The phenomenology of legal practice records emergence of the entire industry of medical liability lawsuits or litigation on global level. Consequently, there has been an increasing trend of legal proceedings against health care providers. Relevant research shows that the risk of exposure to compensation claims for doctors in paediatrics is higher than in other areas of medicine.Methods: In order to determine whether the aforementioned global trend was also reflected in Croatian legal practice, this researchproduces a survey of the frequency of final judicial decisions on compensation claims due to medical errors in the period from 2019 to 2023. The research also took into account the strongest correlation factor: malpractice. Methodologically research encompassedcase law of domestic courts in the mentioned period recorded in the Ius info database and recorded the number of decisions on medical error and malpractice by year, then narrowing the results down to paediatric cases, i.e. cases where a decision was made on the existence of a paediatrician’s medical error.Results: A total of 54 decisions on the existence or non-existence of a medical error were observed in the relevant period, of which the largest number (31.5%) was recorded in 2021. The total number of decisions on malpractice is half that (N=26), with the highest frequency (27%) recorded in the first year of the observed period. In the entire period of interesr, only one decision refers to a paediatric case (N=1).Conclusions: The results of this research show that in Croatia there is no significant increase in the number of compensation claims for damages due to medical errors in paediatrics. In other words, the trend in the Republic of Croatia does not follow the trends of global legal practice in that area.
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Authors: Bukvić Blaženka Kljaić , Ivković-Jureković Irena , Navratil Marta , Rogulj Marijana , Bandić Davor , Kelečić Jadranka , Šegulja Silvije , Richter Darko
Published: November 2024

Teška astma je složena i heterogena bolest obilježena neprimjerenom kontrolom unatoč visokom stupnju liječenja. Procjenjuje sekako je prisutna u 2 - 5% djece s astmom, češće u djece starije od 10 godina i u dječaka. Iako je teška astma rijetka, ova skupina djeceima povećani rizik nuspojava liječenja, kao i teških i po život opasnih egzacerbacija. Povećani su izravni i neizravni troškovi u vidupotrošnje lijekova, češćih redovitih i hitnih posjeta liječniku, hospitalizacija, izostanaka s nastave i roditeljskih odsustava s posla.Defi nicije teške astme nisu ujednačene, ali im je svima zajednička potreba za kombinacijskim liječenjem inhalacijskim kortikosteroidima i bronhodilatatorima dugog djelovanja u visokim dozama neophodnima za kontrolu simptoma, ili, čak nedostatnima zapunu kontrolu bolesti. Nekontrolirana astma zahtijeva iscrpno preispitivanje koje uključuje diferencijalno dijagnostičku reevaluacijui nedvojbenu potvrdu dijagnoze astme, otkrivanje otegotnih čimbenika poput neredovitog uzimanja i/ili loše tehnike primjene inhalacijskih lijekova, utjecaja okoliša (izloženost alergenima i iritansima) te komorbiditeta (bolesti gornjeg dišnog puta, gastroezofagusni refl uks, pretilost i anksioznost). Ukoliko je dijagnoza astme potvrđena, a otegotni čimbenici ispravljeni, te je postignuta kontrolabolesti, radi se o teško lječivoj astmi (engl. diffi cult-to-treat asthma). Ako se ovim mjerama ne postigne kontrola, radi se o teškoj rezistentnoj astmi (engl. severe, therapy-resistant asthma).U pristupu djetetu s teško lječivom astmom slijedimo sistematičnu evaluaciju kojom u približno dvije trećine bolesnika djelovanjemna modifi cirajuće čimbenike ispunjavamo dugoročne ciljeve liječenja kroz postizanje kontrole simptoma te smanjenje rizika za pogoršanja i nepovratna oštećenja bronha/komplikacija liječenja. U preostale djece, u koje primjena ovih mjera ne dovodi do uspostave kontrole, radi se o teškoj, na terapiju rezistentnoj astmi. U njih slijedi, kroz određivanje fenotipskih obilježja, izbor biološkog liječenjatemeljnog na pretpostavljenom endotipu.
Sindrom stražnjeg sraza gležnja u adolescenata
Authors: Bojanić Ivan , Karakaš Tin , Knežević Igor , Dimnjaković Damjan
Published: November 2024

Cilj ovog rada jest ukazati na moguću pojavu stražnjeg sindroma sraza gležnja (SSSG) u populaciji adolescenata te provjeriti tvrdnju dase u toj populaciji njegova dijagnoza postavlja s vremenskom odgodom. Osim toga, željeli smo prikazati i rezultate provedenog kirurškog liječenja SSSG-a artroskopskim zahvatom u adolescenata te usporediti dobivene rezultate s rezultatima objavljenim u literaturi.U istraživanju je sudjelovalo 16-ero ispitanika koji su u trenutku zahvata imali ≤19 godina i kod kojih je zbog SSSG-a načinjen artroskopski zahvat samo u stražnjem dijelu gležnja. Sva dostupna medicinska dokumentacija sustavno je pretražena s ciljem utvrđivanja razdoblja od pojave prvih si mptoma do točne dijagnoze te uzroka nastanka SSSG-a, kao i traženja zabilješki o komplikacijamaliječenja. Na pregledu provedenom u sklopu istraživanja procjena funkcijskog stanja gležnja provedena je upitnikom Američkogortopedskog društva za stopalo i gležanj (AOFAS upitnik), dok je zadovoljstvo ispitanika načinjenim zahvatom ocijenjeno pomoćuAbdelatifova upitnika.Prosječna životna dob ispitanika u trenutku zahvata iznosila je 16,8 godina, a medijan trajanja simptoma do postavljanja točnedijagnoze iznosio je 8,5 mjeseci. Zahvat je u svih protekao bez komplikacija, a one nisu uočene ni u poslijeoperacijskom razdoblju.Utvrđeno je značajno poboljšanje rezultata AOFAS upitnika s prosječno 70,9 prije zahvata na 97,0. Svi su ispitanici izrazili izrazitozadovoljstvo načinjenim zahvatom i istaknuli da im se funkcija gležnja popravila nakon načinjenog zahvata.Ovo je istraživanje potvrdilo konstataciju iz literature da se dijagnoza SSSG-a u populaciji adolescenata postavlja s vremenskomodgodom. Osim toga, ovo je istraživanje potvrdilo iznimnu uspješnost liječenja SSSG-a artroskopskom kirurgijom.
A Case of Pediatric Multisystem Inflammatory Syndrome Temporally Associated with SARS-CoV-2
Authors: Pavlyshyn Halyna , Horishna Ivanna , Slyva Viktoriia , Dyvonyak Olga , Olga Panchenko
Published: November 2024

Introducion. COVID-19 in children is a relatively mild disease. Though a more serious condition characterized by systemic inflamma- tion does occur in children in a Kawasaki disease-like form named multisystem inflammatory syndrome (MIS-C). MIS-C in children is a less common pathology. Case Report. A clinical case of multisystem inflammatory syndrome in children (MIS-C) temporally associated with SARS-CoV-2 in- fection in a previously healthy preschool-age girl is presented. The disease had an incomplete Kawasaki disease-like form corre- sponding to this syndrome in children and adolescents as defined by WHO and CDC criteria. Conclusion. This clinical case draws the attention of general practitioners and pediatricians to the peculiarities of this type of syn- drome diagnosis and management.
Unexpected inconsistency of cranial MRI and cranial US measurements: new normative values in the neonatal period
Authors: Coban Gokcen , Silahlı Musa , Cifci Bilal Egemen
Published: November 2024

Cranial ultrasonography (US) enables diff erentiation of normal from abnormal in a reasonable scanning time and without radiation. However, there is not enough data comparing measurements of cranial US and magnetic resonance imaging (MRI) for some intracranial structures in the neonatal period. We aimed to assess the reliability of cranial US compared with brain MRI. We also sought reference values of the pituitary gland and infundibulum, which is important in searching for growth retardation or abnormal growth in this population. Normative measurements of the width of the 4th, 3rd and lateral ventricles, length and width of the corpus callosum (CC), biventricular width (BVW), and biparietal diameter (BPD) were prospectively obtained from 95 healthy fullterm neonates on both cranial US and brain MRI. Besides the well-known structures, the height of the pituitary gland, thickness of the infundibulum, height and width of the cerebellum were measured on MRI. Comparison of cranial US and brain MRI showed good correlation in the BPD, width of the splenium and genu of CC, anteroposterior length of CC, and width of the 3rd ventricle (κ=0.88- 0.99). Correlation was poor in BVW, width of the corpus of CC, and width of the lateral ventricles (κ=0.28-0.56). New normative values of the height of pituitary gland and thickness of the stalk in the neonatal period are novel fi ndings of this prospective study. In conclusion, cranial US and MRI measurements were not consistent in some brain structures. It is crucial to know the normative values, and to diff erentiate congenital and developmental brain anomalies in the neonatal period. The new reference values of the pituitary gland and infundibulum were also established in this period. Key words: MAGNETIC RESONANCE IMAGING; INFANT, NEWBORN; PEDIATRICS; ULTRASONOGRAPHY
A retrospective study on health-related quality of life in congenital clubfoot (with patient-reported outcomes)
Authors: Bohner-Beke Aliz , Jámbor Szilvia , Vass Lívia , Kránicz János , Végh Veronika , Pusztafalv Henriette
Published: November 2024

When treating clubfoot, doctors prefer objective measurements, while knowing the subjective quality of life of a patient is secondaryrather than observing the two in parallel. The aim was to highlight the significance of patient reporting in the quality of life questionnaires.This retrospective study included 75 patients with clubfoot (44 boys and 28 girls, mean age 12.45 (range 2-30 years; 3 missing).The treatment was typical in 55 patients (posteromedial release), while Ponseti method was used in 20 patients. The followingquestionnaireswere used: EQ-5D-3L and Clubfoot Disease Specific Instrument for Health Science, Rosenberg Scale for PsychologicalSapiens, and our own sociological questionnaire. The mean EQ-5D index was 0.86 and mean EQ-VAS was 79.92, scoring lower thanthe Hungarian population data. According to the results of the special quality of life questionnaire, foot pain and finding shoes that fitcaused the biggest problems for the study patients. The quality of life questionnaire results showed that finding shoes that fit withoutexacerbating pain was the biggest problem for study participants. The mean Rosenberg scale score was 22.2 (maximum 30) points.There were no significant findings in individual cases, however, correlations were found when examining low-self-evaluation participantsin the group. Internal consistency of the instruments employed was appropriate (Cronbach alpha: 0.831-0.939), and combinedquestionnaires used to identify each aspect as indicated by the medium and high Spearman correlation values (rs=0.539-0.916).Key words: clubfoot; quality of life; patient reported outcome measures
Pripadnost mediteranskoj prehrani studenata riječkog sveučilišta
Authors: Žeželj Sandra Pavičić , Zubalj Nataša Dragaš , Fantina Daria , Krešić Greta , Jovanović Gordana Kenđel
Published: November 2024

Cilj: Tradicionalna mediteranska prehrana povezuje se sa smanjenim rizikom od nastanka mnogih kroničnih nezaraznih bolesti. Ciljovog rada bio je utvrditi kakvoću prehrane studenata u Rijeci, odnosno odrediti pripadnost mediteranskom načinu prehrane uzpomoć dvaju indeksa za ocjenu mediteranskog načina prehrane: Mediteranskog indeksa kvalitete prehrane i Ocjene mediteranskeprehrane.Metode: Istraživanje je provedeno od siječnja do svibnja 2018. godine i obuhvatilo je 455-ero studenata s riječkog Sveučilišta, 336žena i 119 muškaraca. Podatci o dobi, tjelesnoj aktivnosti, antropometriji, pušenju i prehrambenim navikama dobiveni su iz anketnih upitnika. Za određivanje pripadnosti mediteranskom načinu prehrane primijenjena su dva indeksa: Mediteranski indeks kvaliteteprehrane i Ocjena mediteranske prehrane.Rezultati: Prosječna ocjena kakvoće prehrane studenata ocijenjena prema Mediteranskom indeksu kvalitete prehrane iznosila je9,61, što označava srednju do nedostatnu kakvoću prehrane. Prema Ocjeni mediteranske prehrane kakvoća prehrane ocijenjena jekao nedostatna i iznosila je 3,70. Žene su imale značajno bolji Mediteranski indeks kvalitete prehrane (9,36) od muškaraca (p<0,001)i studenti koji ne puše također su imali značajno bolji Mediteranski indeks kvalitete prehrane (9,11) od pušača (p<0,001).Zaključci: Prema dobivenom Mediteranskom indeksu kvalitete prehrane i Ocjeni mediteranske prehrane možemo zaključiti da jeprehrana studenata ocijenjena srednjom do nedostatnom te je vidljivo kako postoji potreba za povećanjem pridržavanja mediteranskog načina prehrane te promjenom prehrambenih navika studenata. Prema dobivenim rezultatima postoji jasna potreba zastvaranjem programa promicanja zdravlja, s posebnim naglaskom na edukaciju o prehrani i na prehrambene navike.
Prevalence of malnutrition and energy intake in hospitalized children
Authors: Mamić Marinela , Kučan Petra , Vukman Diana , Niseteo Tena
Published: November 2024

The main aim of the present study was to report the seven-year prevalence of malnutrition in a tertiary children’s hospital and toassess the diff erence between recommendations and actual intake in paediatric inpatients. The study included 606 patients, agerange from 0 months to 18 years, admitted to the Children’s Hospital Zagreb. Energy intake by all routes was assessed and comparedwith daily requirements recommended by the Croatian nutritional standard for hospital diets. Weight and height were measured,and body mass index (BMI) with standard deviation (SD) was calculated. Malnutrition was defi ned using the World Health Organization(WHO) cut-off BMI Z-score SD as the most widely used criteria. Overall, 28.6% of the study population were malnourished,including 19.7% of overweight and obese patients, 8.9% of undernourished patients and 6.9% of stunted patients. Study resultsshowed that 37% of all study patients had an average daily energy intake between 60% and 90%, and 33% above 90% of the recommendedvalues. In conclusion, the prevalence of malnutrition, including undernutrition and overnutrition, in hospitalized children inCroatia is similar to that in Europe. It is of great importance to identify the children at risk at an early stage, so that appropriatenutritional intervention can be introduced. A simple nutritional screening tool should be implemented and used in all childrenadmitted to the hospital. Key elements in improving dietary intake are a combination of screening, appropriate nutritional assessment,further patient monitoring, and improving the hospital menu.
Nekrotizirajući enterokolitis – prikaz slučajeva i pregled literature
Authors: Bašković Marko , Župančić Božidar , Car Andrija , Višnjić Stjepan , Ježek Davor
Published: November 2024

Nekrotizirajući enterokolitis je gastrointestinalna bolest novorođenačke dobi opasna za život. Do dana današnjeg patogeneza bolesti nije jasna, ali je prihvaćeno da je multifaktorska. Rizični čimbenici za nastanak ove bolesti su u prvom redu nedonošenost iniska porođajna masa. Bolest se dijagnosticira na temelju kliničke slike upotpunjene radiološkim i laboratorijskim nalazima. Načinliječenja ovisi o težini kliničke slike i stadiju bolesti. U većini slučajeva dostatno je simptomsko i antimikrobno liječenje uz praćenjelaboratorijskih i radioloških parametara. Novorođenčad s teškim oblikom bolesti i perforacijom crijeva zahtijeva hitno kirurško liječenje. Kako bismo približili ovo stanje, prikazali smo troje pacijenata liječenih u Klinici za dječje bolesti Zagreb u 2016. godini.
Comparison of body mass index percentiles for schoolchildren in Croatia with international reference values
Authors: Jureša Vesna , Musil Vera , Tiljak Mirjana Kujundžić , Majer Marjeta
Published: November 2024

The aim was to determine body mass index (BMI) percentiles in Croatian children aged 6.5-18.5 years and to compare them with theUnited States Centers for Disease Control and Prevention (USCDC 2000) and World Health Organisation (WHO 2007) BMI referencevalues in order to show the pattern of diff erences. A representative cross-sectional national survey with standardized body heightand weight measurements included 12,389 schoolchildren (6372 boys and 6017 girls) aged 6.5-18.5 years (response rate 94.5%). BMIpercentile curves were established using the Lambda-Mu-Sigma method. BMI percentiles of the Croatian schoolchildren diff eredfrom analogical percentiles in two international reference populations. The 5th percentile in boys and girls was similar to both referencepopulations until the age of about 13 years, and in the age onwards it showed an upward shift. The 50th, 85th and 95th percentilesin boys showed an upward shift in boys in all ages observed; in girls, an upward shift was observed until the age of about 14years and downward shift in the age onwards. The diff erences observed between BMI percentiles of Croatian schoolchildren andUSCDC 2000 and WHO 2007 references imply that BMI percentiles developed on a nationally representative sample would be moreappropriate for assessment of nutritional status at the national level, whereas international standards could be used for comparisonof nutritional status among diff erent populations.
Provedba cijepljenja protiv HPV-a u Republici Hrvatskoj
Authors: Posavec Marija
Published: November 2024

Dostupnost cjepiva protiv humanog papiloma virusa (HPV) generacijama mladih omogućila je zaštitu od karcinoma u odraslojdobi. U Hrvatskoj je cjepivo protiv HPV-a registrirano 2007. godine. Hrvatsko društvo za školsku i sveučilišnu medicinu Hrvatskog liječničkog zbora provelo je u 2016. godini istraživanje online upitnikom. Rezultati upućuju na brojna i različita iskustva specijalistaškolske medicine u organizaciji, provedbi i odazivu na cijepljenje.Razlozi odbijanja cijepljenja protiv HPV-a su mnogobrojni, no istraživanja i iskustva upućuju na to da su najčešći sumnja u pouzdanost cjepiva, preispitivanje njegove učinkovitosti, povezivanje cijepljenja s rizičnim spolnim ponašanjem, koncept besplatnog, preporučljivog, dobrovoljnog cjepiva uz obvezan informirani pristanak kao novitet u provedbi cijepljenja u Hrvatskoj. Mogućnost cijepljenja protiv HPV-a u Hrvatskoj postoji više od desetljeća, no još i danas je veliki izazov za profesionalce u pogledu informiranja ieduciranja roditelja, djece i mladih o mogućnosti i važnosti prevencije bolesti uzrokovanih HPV-om jednostavnim, pouzdanim iučinkovitim postupkom – cijepljenjem.
Pristup predškolskom djetetu s ponavljajućom sipnjom – kada postaviti dijagnozu astme i kako liječiti bolesnika?
Authors: Štefanović Iva Mihatov , Vrsalović Renata , Čičak Biserka , Lesar Tatjana , Ribičić Rebeka
Published: November 2024

Sipnja je jedan od najčešćih respiratornih simptoma u predškolskoj dobi. U većine djece sipnja je vezana isključivo za virusne infek cijegornjih dišnih putova i s vremenom je prolazna, no trećina djece s ponavljajućom sipnjom ima postavljenu dijagnozu astme u školskoj dobi. Prema smjernicama stručnih društava ponavljajuća sipnja se u predškolskoj dobi najčešće klasifi cira u fenotipove,ovisno o učestalosti simptoma i njihovoj povezanosti s određenim okidačima. Upalna patogeneza fenotipova sipnje u predškolskojdobi zasad nije poznata, ali vjerojatno je riječ o različitim upalnim mehanizmima. Većina djece s ponavljajućom sipnjom u predškolskoj dobi može se liječiti samo bronhodilatatorima. Prema smjernicama Europskog respiratornog društva protuupalnu terapiju treba započeti u bolesnika koji imaju učestale simptome i tešku kliničku sliku. Mjerenje objektivnih parametara kao što su plućnafunkcija i hiperreaktivnost dišnih putova otežano je zbog nesuradnje, tako da je uvođenje terapije i dobar klinički odgovor vjerojatnonajpouzdaniji put za dijagnozu astme. U ovom trenutku ne postoje strategije liječenja koje bi modifi cirale tijek bolesti i spriječile razvoj perzistentne sipnje odnosno astme.
Premature twins with acute renal injury – it is not always what it seems to be: case report
Authors: Ahel Ivona Butorac , Milardović Ana , Tomulić Kristina Lah , Verbić Arijan , Raspor Sanja Flajšman , Košuljandić Đurđica , Romić Ivana Štrk
Published: November 2024

Acute kidney injury (AKI) is common in critically ill premature infants. They are more susceptible to renal injury than older infants andchildren because of the functional and developmental immaturity of neonatal kidney. There is no unifi ed defi nition for neonatal AKI.AKI in neonates is often multifactorial and may result from prenatal, perinatal, or postnatal insults as well. Serum creatinine (SCr)concentration at birth is similar to the mother’s value. We present a case of prematurely born twins who were admitted to thepaediatric intensive care unit because of AKI. Laboratory examination showed equally elevated levels of blood urea nitrogen (BUN)and SCr and metabolic alkalosis in both twins. High values of BUN and SCr were the result of the mother’s unrecognized renaldisease. On the seventh postnatal day, SCr and BUN in twins were within the normal ranges. In all cases with high SCr levels inneonates in the fi rst 72 hours after birth, it is mandatory to check the mother’s renal function.Key words: acute kidney injury; infant; premature; uraemia
Cardiorespiratory complications in patients with osteogenesis imperfecta
Authors: Turkalj Mirjana , Miranović Vesna , Lulić-Jurjević Rajka , Juraški Romana Gjergja , Primorac Dragan
Published: November 2024

Osteogenesis imperfecta (OI) is a hereditary connective tissue disorder, usually caused by dominant mutations of genes coding forcollagen type I alpha chains, COL1A1/A2. Although skeletal manifestations of OI are most readily observable, cardiopulmonarydisorders in patients with OI are increasingly recognized as life-threatening but treatable disorders. Unfortunately, the majority ofpatients with moderate to severe types of OI die from or with cardiopulmonary complications. The lungs and the heart are oftenunrecognizable and neglected organs in patients with OI. In monitoring of patients with OI, attention is mostly focused on monitoringlong bone and spine deformities, and indirectly deformities of the chest wall, which have consequences on the development oflung and the airway diseases. Lung disorder is frequently ignored until breathing problems become severe. An important componentin patients with OI is obstructive lung disease, sleep disordered breathing, as well as acute and chronic infection often connectedwith resultant bronchiectasis. In addition to respiratory complications, some patients with OI have serious cardiovascularproblems, including severe mitral valve prolapse, aortic valve insuffi ciency and dilation of the aorta, which require cardiac surgery.The diagnosis and management of the lung and cardiovascular complications in some patients with OI are quite diffi cult. In allpatients with OI, it is important to recognize and monitor respiratory and cardiovascular manifestations in order to prevent furtherprogression of any complications.Key words: osteogenesis imperfecta; pulmonary disease; pulmonary function; lung infections; cardiovascular manifestation; prevention
Molecular genetics analysis of osteogenesis imperfecta in clinical practice
Authors: Stubbe Annika , Primorac Dragan , Höppner Wolfgang
Published: November 2024

Osteogenesis imperfecta (OI) is characterized by fractures with minimal or absent trauma, representing a continuum ranging fromperinatal lethality through individuals with severe skeletal deformities to nearly asymptomatic individuals with mild predispositionto fractures. Diagnosis of OI is an interdisciplinary task based on family and/or patient history of fractures combined with characteristicphysical fi ndings. Radiographic examination reveals fractures of varying ages and stages of healing, wormian bones, and osteopenia.As there is no defi nitive test for OI, molecular genetic testing by next generation sequencing (NGS) of COL1A1 and COL1A2 andup to 12 other genes is essential to confi rm the genetic background. Therefore, we designed a NGS gene panel comprising 12 genesinvolved in OI or severe osteoporosis. Here we report results in a cohort of 11 apparently sporadic young patients with OI, all off springof unaff ected parents, who were referred to orthopaedic surgery at Sv. Katarina Special Hospital (Zabok/Zagreb, Croatia). Tenof these 11 patients could be classifi ed genetically. Overall, three genes with diff erent percent relating to the whole cohort wereinvolved: COL1A1 (63.6%), COL1A2 (18.18%) and WNT1 (9.09%).Key words: osteogenesis imperfecta; molecular genetics - analysis
Chronic infl ammatory bowel diseases (IBD) including Crohn’s disease, ulcerative colitis and IBD unclassifi ed have been ever more frequently diagnosed in childhood and adolescence. This tendency is highly important because the clinical picture, spread an
Authors: Kolaček Sanja , Hojsak Iva
Published: November 2024

Kronične upalne bolesti crijeva (IBD) - Crohnova bolest, ulcerozni kolitis i neklasifi cirana upalna bolest crijeva sve se češće dijagnosticiraju u dječjoj i adolescentnoj dobi. Navedeno je posebice važno, jer se klinička slika, proširenost i težina bolesti uvelike razlikuju akoje bolest nastala u pedijatrijskoj populaciji. Štoviše, navedeno je to izraženije što je dob kad se bolest javila mlađa. Baš je porast incidencije ovih bolesti, ali i njihova specifi čnost u dijagnostici i liječenju, dovela do razvoja specifi čnih algoritama za dijagnostiku i liječenje IBD-a u djece. Velik je broj istraživanja koja pokušavaju odgovoriti na pitanje zašto se bolest sve češće javlja, koji su etiopatogenetski mehanizmi njezina nastanka te što je to što čini kliničku sliku i terapijski odgovor različitim u dječjoj dobi. Namjena je stogaovog preglednog rada prikazati i raspraviti specifi čnosti IBD-a u djece, poglavito u odnosu na epidemiologiju, fenotipska obilježja ispecifi čnosti dijagnostičkog i terapijskog pristupa.
Obostrana prirođena dislokacija koljena udružena s obostranim razvojnim poremećajem kuka – prikaz bolesnika
Authors: Miličić Gordana , Šimić Marijana , Bojić Davor , Vlaić Josip , Ribičić Omislav , Vrdoljak Ozren , Antičević Darko , Matić Ida , Miličić Marko
Published: November 2024

Prirođena dislokacija koljena je relativno rijetka deformacija, a pogotovo udružena s deformacijom drugih zglobova. Djevojčica uovom prikazu, rođena je s obostranom dislokacijom koljena i obostranim razvojnim poremećajem kuka u smislu iščašenja. Prvi pregled u ortopedskoj ambulanti obavljen je 21. dan nakon rođenja. Učinjena je radiološka i ultrazvučna dijagnostika i genetska obrada.Nisu nađeni elementi Larsenova sindroma kao ni artrogripoze. Djevojčica je liječena trakcijom na vertikalnom okviru i sadrenim redresmanom oba koljena. Nakon repozicije kukova aplicirao se aparat Hilgenreiner. Tek nakon repozicije oba kuka omogućila se redresmanom repozicija i puna fl eksija koljena. Nakon stabilizacije kukova i koljena aplicirani su remenčići prema Pavliku, koji su nošenidva mj. Liječenje je trajalo do sedam mj. života djevojčice a svi postupci su praćeni ultrazvukom i RTG snimkama. Praćenje još traje.
Mikrodelecija 8q23.3-q24.11 povezana sa sindromima Langer-Giedion i Cornelia de Lange-4
Authors: Ivankov Ana-Maria , Bobinec Adriana , Boban Ljubica , Sansović Ivona , Barišić Ingeborg
Published: November 2024

Mikrodelecija kromosomske regije 8q23.3-q24.11 povezuje se sa sindromom Langer-Giedion ili trihorinofalangealnim sindromomtipa II., rijetkim genetičkim poremećajem uzrokovanim prvenstveno delecijom gena TRPS1 i EXT1. Fenotip je sukladan trihorinofalangealnom sindromu tipa 1, ali uključuje i višestruke osteohondrome tipa 1. Kako je riječ o sindromu delecije susjednih gena, klinička slika varira ovisno o veličini deletirane regije. U navedenoj se regiji nalazi i gen RAD21, čije se heterozigotne delecije i muta cijepovezuju sa sindromom Cornelia de Lange-4. U ovom radu prikazujemo dva bolesnika s delecijom koja obuhvaća kromosomskuregiju 8q23.3-q24.11, kod kojih je molekularnom i citogenetskom analizom utvrđeno da imaju dva različita poremećaja – sindromLanger Giedion i sindrom Cornelia de Lange-4
Klinička slika konverzivnog poremećaja u hitnoj pedijatrijskoj službi: prikaz triju bolesnica
Authors: Santrić Lena , Richter Darko , Begovac Ivan
Published: November 2024

Konverzivni poremećaj u adolescenata može imati dramatičnu kliničku prezentaciju te ga je u prvi mah teško razlikovati od organskih ili od drugih psihijatrijskih bolesti. Prikazujemo tri bolesnice u dobi od 11 do 13 godina koje su dovedene u hitnu pedijatrijskuambulantu pod slikom neepileptičkih psihogenih napadaja. Zbog dramatične kliničke prezentacije, odsutnosti kontakta i sličnosti spravim epileptičkim napadajima, primijenjene su reanimacijske mjere te antikonvulzivna terapija. Tek naknadno, detaljnom dijagnostičkom obradom, isključeno je organsko podrijetlo simptoma. Psihološkim testiranjem te pregledom dječjeg i adolescentnog psihijatra postavljena je dijagnoza konverzivnog poremećaja, a liječenje i praćenje nastavljeno je na dječjoj i adolescentnojpsihijatriji. Kako bi se izbjegle nedoumice te primjena rizičnih i nepotrebnih terapijskih postupaka, u svakodnevnoj hitnoj pedijatrijskoj praksi potrebno je misliti i na moguće neepileptičke psihogene napadaje.
Utjecaj pasivnog pušenja na kontrolu astme u djece
Authors: Hajdić Josip , Pavlov Neven , Slavica Dragišić-Ivulić Slavica x
Published: November 2024

Izloženost duhanskom dimu je rizični čimbenik za nastanak ili pogoršanje mnogih poremećaja, uključujući astmu. Cilj ovog istraživanja je ustvrditi pogoršava li pasivno pušenje u djece vrijednosti spirometrijskih i alergijskih parametara mjerenih iz krvi. Provedenoje retrospektivno istraživanje parova u razdoblju od 2011. do 2013. godine, u Alergološkoj ambulanti Klinike za dječje bolesti KBC-aSplit. U istraživanje je uključeno 60-ero djece u dobi od 5 do 18 godina s dijagnozom astme (postavljenom prema GINA smjernicamaiz 2006. godine), koje smo podijelili u dvije skupine: 36-ero djece čiji su ukućani pušili i 24-ero kojoj su ukućani bili nepušači. Analizi ranisu parametri krivulje protok-volumen: PEF, FVC, FEV1, FEF25, FEF50 i FEF75 prije i poslije obavljenog bronhodilatacijskog testa sa dvaudaha salbutamol (Ventolin) spreja, 100 μg po udahu; alergijski markeri iz krvi: ukupna konc. IgE-a u serumu, apsolutni i relativnibroj eozinofi la u krvi te broj eozinoifi la u brisu nosa. Podatci su prikupljeni od roditelja putem upitnika te uvidom u elektroničku medicinsku dokumentaciju. Nije dokazana statistički značajna razlika između skupine djece izložene pasivnom pušenju i kontrolneskupine u promatranim vrijednostima. Broj pušača u kućanstvu također nije pokazao značajan utjecaj na spomenute vrijednosti.Pronađena je značajna razlika u vrijednosti ECP-a između skupine djece čiji su ukućani pušili u domu i one kojoj su ukućani pušiliizvan njega (P = 0,009). Broj na dan popušenih cigareta u domu statistički značajno korelira s relativnim brojem eozinofi la u krvi(P = 0,048). Pasivno pušenje nije se pokazalo povezanim s brojem alergena na koje je dijete alergično.
Skin of the very premature newborn – physiology and care
Authors: Jurica Sonja Anić , Čolić Ana , Gverić-Ahmetašević Snježana , Lončarević Damir , Filipović-Grčić Boris , Stipanović-Kastelić Jasminka , Rešić Arnes
Published: November 2024

Skin is a multifunctional human organ. It has a protective, regulatory and sensory function. Skin of the very premature newborn is underdeveloped with defi cient functionality and has to be observed and treated as an immature organ in need of special care and interventions. Appropriate prevention and compensation of water and heat losses is obligatory as preserving the integrity of the skin and therefore of the entire body.
Reference values for cardiopulmonary exercise testing in children and adolescents in northwest Croatia
Authors: Dilber Daniel , Malčić Ivan , Ćaleta Tomislav , Zovko Ana
Published: November 2024

The already published reference values for cardiopulmonary exercise test (CPET) might not be representative for our population. Our aim is to create reference values for CPET in children and adolescents in northwest Croatia. Exercise testing according to modifi ed Bruce treadmill protocol was performed in 164 healthy children aged 11-17 years. Results are presented as mean and standard deviation. Mann-Whitney test was used for continuous variables without normal distribution. Boys reached signifi cantly higher peak oxygen consumption (VO2) at anaerobic threshold (AT) with mean 37.13±8.9 mL/kg/min compared with 31.95±6.6 mL/kg/ min in girls. The same fi nding was recorded for VO2 at peak exercise (peak VO2) where boys reached signifi cantly higher peak VO2 of 51.3±8.9 as compared with 41.49±6.51 mL/kg/min in girls. During incremental work, systolic pressure increased from mean 109.86±14.2 mm Hg to 145.43±11.9 mm Hg, with no signifi cant diff erence between girls and boys. During incremental work, together with linear increase in heart rate and systolic pressure, oxygen pulse that represents cardiac output, showed linear increase with signifi cantly higher values in boys. Compared with girls, boys had signifi cantly higher tidal volume at rest and reached signifi - cantly higher peak minute ventilation. This study has comprehensively provided a reference set of data for the most important cardiopulmonary variables in a population of healthy children and adolescents in northwest Croatia. Our fi ndings showed strong correlation with previous reports and gave a basis for further research.
Osobitosti cistične fi broze odrasle dobI
Authors: Tješić-Drinković Dorian , Tješić-Drinković Duška
Published: November 2024

Životni vijek bolesnika sa cističnom fi brozom stalno se produžuje zahvaljujući medicinskim spoznajama i njihovoj implementaciji upraksi, što utječe na porast broja odraslih bolesnika. Dužinom životnog vijeka mijenjaju se epidemiološki pokazatelji bolesti te seuočava sve više njenih komplikacija na drugim organskim sustavima. Stoga strategija za daljnje poboljšanje kakvoće života iživotnog vijeka bolesnika sa cističnom fi brozom mora uključiti i preventivno djelovanje, kako bi se izbjegle, umanjile ili odgodilekomplikacije u kasnijoj dobi.
Pasivno pušenje i poremećaji dišnog sustava u djece
Authors: Pavić Ivan , Vlahović Irma Golmajer , Pavić Ivana , Čurlin Mirela
Published: November 2024

Iako je brojnim istraživanjima dokazan štetan utjecaj pasivnog pušenja na dišni sustav djece, činjenica je da značajan broj roditeljai dalje izlaže svoju djecu duhanskom dimu. Glavni cilj ovog preglednog članka je upozoriti na štetne posljedice pasivne izloženostiduhanskom dimu na dišni sustav u dječjoj dobi te utvrditi koji bi bili mogući mehanizmi njihovog nastanka. Brojnim epidemiološkimistraživanjima dokazano je da su djeca izložena pasivnom pušenju sklonija infekcijama dišnog sustava, imaju povećanu učestalostporemećaja plućne funkcije i astme, kao i statistički značajno nižu motoričku spremnost i fi zičku kondiciju od djece čiji roditelji nisupušači. Dokazano je da čak djeca majki koje su pušile tijekom trudnoće kasnije u djetinjstvu imaju povećani rizik za pojavu bolestidišnog sustava. Podatci o štetnim učincima duhanskog dima trebali bi pomoći u javnozdravstvenim aktivnostima usmjerenim prema smanjenju izloženosti djece pasivnom pušenju i svih negativnih utjecaja izloženosti pasivnog pušenja na zdravlje djece.
Lavage with diluted surfactant as a treatment option for atelectasis in pediatric intensive care patients
Authors: Ilić Maja Karaman , Goran Madžarac Goran x , Babić Irena , Milavić Boris
Published: November 2024

The aim of this study was to assess the effect of fiberoptic aspiration and diluted surfactant administration on affected lungs inchildren with atelectasis. A convenience sample of 18 mechanically ventilated children were analyzed in this single center prospectivestudy. The children had fi rst been treated unsuccessfully by respiratory physical therapy, after which they underwent fi beropticaspiration. After aspiration, nine children were randomly selected to receive therapeutic lavage with diluted porcine surfactant; theremaining nine received the same quantity of saline solution. Several parameters of lung function, including positive end-expiratorypressure (PEEP) and oxygenation index (OI), as well as lung x-ray images were determined before aspiration and lavage, then at 6and 12 h after lavage. In both groups, most of the measured parameters showed improvement from baseline at 6 h after treatment.Improvement was even more signifi cant 12 h after treatment. The surfactant group showed signifi cant improvement in comparisonto the saline group. The impact of surfactant administration was most visible on the following parameters 12 h after treatment(p<0.001 in all cases): PEEP 5.22 (SD 0.44) in the saline group vs. 3.44 (SD 0.73) in the surfactant group; OI 3.84 (SD 1.13) vs. 2.1 (SD0.38); and mean airway pressure (MAP) 8.56 (SD 0.88) vs. 6.33 (SD 0.5). In conclusion, fi beroptic aspiration is an effi cient treatment inpediatric intensive care patients. The observed benefi cial eff ects of therapeutic lavage with diluted surfactant should be confi rmedprospectively in a larger number of patients.
Vizualno funkcioniranje djece sa spastičnom cerebralnom paralizom
Authors: Alimović Sonja , Katušić Ana
Published: November 2024

Cerebralna paraliza je neurološki razvojni poremećaj motoričke kontrole položaja i pokreta tijela, često udružen s drugim teškoćama, od kojih je jedno i oštećenje vida. Cilj našeg istraživanja bio je utvrditi razinu funkcionalnog vida (vizualno funkcioniranje) djecesa spastičnom cerebralnom paralizom. Daljnji cilj bio je utvrditi povezanost između vidnih funkcija (oštrine vida i kontrastne osjetljivosti) i vizualnog funkcioniranja. U uzorak je uključeno 70-ero djece sa spastičnom cerebralnom paralizom, srednje dobi 4,8 godina. Procjena oštrine vida i osjetljivosti na kontraste obavljena je preferencijalnim testovima, a razina funkcionalnog vida procijenjena je kroz četiri područja: zadatci izbliza, komunikacija, orijentacija i kretanje te svakodnevne vještine. Rezultati pokazuju kakoznatan broj djece sa spastičnom cerebralnom paralizom ima teškoća u svim područjima vizualnog funkcioniranja, a osobito upodručju komunikacije. Uz nedostatno razvijenu vidnu oštrinu, prisutan je i smanjen prag kontrastne osjetljivosti. Oštrina vida iosjetljivost na kontraste značajno su povezane samo s vizualnim funkcioniranjem prilikom rješavanja zadataka izbliza (rs=0,267;p=0,049 / rs=0,444; p=0,001). Osjetljivost na kontraste značajno je povezana i s uporabom vida u vještinama svakodnevnog života(rs=0,432; p=0,002). Djeca sa spastičnom cerebralnom paralizom mogu razviti teškoće u vizualnom funkcioniranju, osobito u situacijama zamjećivanja vizualnih naznaka u komunikaciji. Procjena funkcionalnog vida trebala bi biti nužan element procjene i habilitacije djeteta sa spastičnom cerebralnom paralizom.
Are radiologic studies necessary in differentiating rare diseases in children?
Authors: Potočki K. , Prutki M.
Published: November 2024

Skeletal dysplasias is a general term that refers to a large group of hereditary disorders characterized by abnormal bone and cartilage development. There are almost 400 different disorders classified according to clinical and radiographic features and mode of genetic transmission. In this paper, radiologic characteristics, different imaging modalities and differential diagnosis are presented, since distinction among the various skeletal dysplasias is based largely on radiographic findings. Since radiographic examination and clinical manifestations play significant role in establishing the diagnosis, a multidisciplinary approach to the diagnosis of skeletal dysplasia is required.
Prevalence of insufficient physical activity in children and adolescents: review
Authors: Jurakić D. , Pedišić Ž.
Published: November 2024

The aim of this review is to summarize the results of recently published studies on physical activity prevalence among children and adolescents. Literature search was conducted through Academic Search Complete, CINAHL, ERIC, Health Source, Hrčak, PubMed/ MEDLINE, SocINDEX, and Web of Science. A total of 32 articles (published 2001-2011) were reviewed. The prevalence rates of insufficient physical activity among girls ranged from 2.4% to 39.3% in the accelerometer-based studies, and from 19.5% to 95% in the questionnaire-based studies. The prevalence rates among boys in the accelerometer-based studies and questionnaire-based studies ranged from 2.6% to 18.1% and from 18.2% to 89%, respectively. Insufficient physical activity was more prevalent among high school students compared with primary school students. Prevalence rates of insufficient physical activity were by 5%-30% higher among girls than among boys. Lower prevalence rates of insufficient physical activity were determined in the accelerometerbased studies than in the questionnaire-based studies. The high prevalence of insufficient physical activity in children and adolescents addresses the need for systematic physical activity promotion. Taking into consideration that insufficient physical activity is most prevalent among girls and high school students, physical activity interventions should mainly target these groups.
Physical activity and type 1 diabetes in children and adolescents
Authors: Stipančić G.
Published: November 2024

Type 1 diabetes, the third leading chronic disease in pediatric population, significantly affects life expectancy and quality of life. Only good metabolic control of the disease, including maintaining target blood glucose levels, will prevent the development of diabetes complications. The basis of treatment consists of insulin, diet and physical activity combined with good education. Patients must understand how the body responds to physical activity, its positive and possible negative effects. Therefore, the patient has to prepare for physical activity taking into account its type and duration, regime of insulin therapy, current blood glucose level and need of carbohydrate replacement. Only in this manner, the patients’ participation in physical activity will be enjoyable with a favorable health effect.
Congenital heart disease in childhood and physical activity
Authors: Batinica M.
Published: November 2024

Regular physical activity is associated with a range of health benefits and should be a regular part of almost anyone’s life, including most people with heart disease. However, despite the benefits of physical activity, exercise may provoke acute cardiac events in susceptible individuals. In every single patient, one must consider the individual, the lesion and its hemodynamic implications, and the type and level of exercise contemplated. Children with heart disease should be encouraged to participate in physical activity within the limits provided by their cardiovascular impairment. In small children, motor experience not only determines their physical and motor development, but also influences their emotional, psychosocial and cognitive development. Regular exercise at recommended levels can be performed and should be encouraged in all patients with congenital heart disease. Some patients should be excluded from all but mild regular exercise because of the known high risk. Other patients should be encouraged to participate in sports without any limitation. A simple physical activity intervention like regular walking is feasible, safe and significantly increases the exercise capacity of young patients at all stages of congenital heart disease. It is also helpful in upgrading the quality of life by improving physical self-perception, satisfaction with life, physical activity levels and general health. From the preventive point of view, physical inactivity is a major atherosclerosis and obesity risk factor. Additionally, adopting a physically active lifestyle early in life increases the likelihood that children will become active adults.