While there is a variety of surgical and non-surgical treatment options for obstructive sleep apnoea (OSA) in adults, surgery remainsthe option of choice in paediatric patients due to the fact that more than 90% of childhood OSA is associated with adenotonsillarhypertrophy. Age under three years is often described as one of the most common risk factors for developing postoperative complications.The most important additional risk factor is severity of the disease as measured by polysomnography (PSG). The authorsreport a case of a 20-month-old boy with extremely severe OSA and a history of repeated respiratory cessation during sleep lastingfor up to 30 seconds and lowest recorded saturation during PSG of 67% with the apnoea-hypopnoea index 58.43/h. As classical adenotonsillectomyis recommended only after the child has reached three years, due to the small circulating volume of the blood,several solutions were considered but, ultimately, classical ‘cold steel’ adenotonsillectomy was performed when the child reachedtwo years. This case shows the importance of surgical treatment in severe cases of OSA even in very small children, despite the recommendations,due to the increased risks in neurocognitive and behavioural development.

Nenamjerno otrovanje biljkama nerijetko susrećemo u svakodnevnoj praksi, posebice u pedijatrijskoj populaciji. Kozlac (latinskiArum Maculatum) jedan je od rijetko opisanih uzroka otrovanja, a uz mnogobrojne simptome može dovesti i do smrtnog ishoda.Najvažniji toksin kozlaca je cijanoglikozid triglohinin koji dovodi do klasičnih simptoma otrovanja cijanidima, a zahtijeva specifi čniantidot. U ovom radu prikazujemo dvoje maloljetnih pacijenata, 13-godišnjeg dječaka i njegovu 11-godišnju sestru, koji su slučajnopojeli nepoznatu količinu listova kozlaca. Prezentirali su se ranim simptomima otrovanja ovom biljkom koji uključuju žarenje u usnojšupljini i prsištu te smetnje gutanja. Navedeni simptomi posljedica su djelovanja kristala kalcijeva oksalata u listovima kozlaca kojidjeluju lokalno nadražujuće, a oštećujući kožno-sluzničku barijeru omogućuju ulazak ostalih toksina u organizam. Liječeni su, uzsimptomatsku terapiju, aktivnim ugljenom, te su 24 sata opservirani kako bi se navrijeme uočili znakovi otrovanja cijanidima kao štosu dispneja, poremećaji srčanog ritma te različiti neurološki poremećaji. Nasreću, djeca nisu razvila kasne simptome otrovanja, štopripisujemo činjenici da je biljka prethodno bila skuhana, a toksični cijanoglikozid u njenom sastavu je termolabilan. Cilj ovog prikaza je uputiti na mogućnost otrovanja kozlacem, objasniti kompleksnu simptomatologiju do koje takvo otrovanje dovodi te liječenje koje takav pacijent zahtijeva.

Osteohondritis disekans (OCD) lakta je žarišno oštećenje zglobne hrskavice i pripadajuće subhondralne kosti. U laktu je OCD najčešće lokaliziran na kapitulumu humerusa. OCD lakta se uobičajeno javlja kod športaša u adolescenciji, posebice kod onih koji sebave „bacačkim“ športovima, te kod gimnastičara. Nastanak OCD-a lakta posljedica je kombinacije ponavljajućih mikrotrauma,biomehaničke neusklađenosti i oskudne vaskularizacije distalnog dijela nadlaktične kosti. OCD lakta se najčešće otkriva na magnetskoj rezonanciji i računalnoj tomografi ji, iako se oštećenje može uočiti i na standardno načinjenim radiološkim snimkama. Od najveće je važnosti razlikovati stabilni od nestabilnog OCD-a lakta kako bi se odredio najbolji način liječenja. Stabilni OCD laktamože se započeti liječiti neoperacijski, i to poštedom i modifi kacijom aktivnosti. Kirurško je liječenje potrebno kad je riječ o nestabilnom OCD-u lakta, nakon neuspješnog neoperacijskog liječenja te ako je prisutno slobodno zglobno tijelo u zglobu koje čini mehaničke simptome. Osnovne metode kirurškog liječenja OCD-a lakta su stimulacija koštane srži, fi ksacija koštano-hrskavičnog fragmenta te prijenos autolognog koštano-hrskavičnog presatka. Cilj ovog rada je prikazati dosadašnje spoznaje o etiologiji, kliničkoj slici, dijagnostici, mogućnostima liječenja te ishodu liječenja OCD-a lakta.

Pfeiff er syndrome is a rare autosomal dominant congenital disorder with the main features of acrocephalic skull, midfacial hypoplasia,syndactyly of hands and feet, broad thumbs and big toes. Three clinical subtypes (with diff erent diagnostic and prognostic implications)have been identifi ed. We report on two cases of Pfeiff er syndrome type 2 (one of them diagnosed prenatally and theother detected in the newborn), identifi ed through screening for congenital defects as an activity of the Pleven Registry of CongenitalAnomalies (member of EUROCAT). Case 1 was a male foetus from terminated pregnancy following prenatal diagnosis. Foetal morphologyultrasound in the 28th week of gestation revealed craniosynostosis, cloverleaf skull, fl at face, exophthalmos, macroglossia,short ribs, deformed spine, and abnormally shortened limbs (micromelia). Clinical examination after birth confi rmed the diagnosisof Pfeiff er syndrome type 2. Case 2 was a boy (born at term, 40th week of gestation) diagnosed with Pfeiff er syndrome type 2 at birth.Multiple malformations were presented including cloverleaf skull, craniosynostosis of coronal suture, midfacial hypoplasia, depressednasal bridge, high palate, exophthalmos, down-slanted palpebral fi ssures, hypertelorism, low-set ears, short neck, broad andmedially deviated thumbs and hallux, clinodactyly V, skin syndactyly of II-III toe, short thorax with sternal depression, and anal atresia.The patient died from respiratory distress syndrome on the ninth day after birth. Severe malformations, high mortality andproblems in clinical management determine poor prognosis in most cases of Pfeiff er syndrome type 2. Early prenatal diagnosis inpregnancies with severe foetal malformations and poor prognosis gives parents more time and opportunities for reproductivechoices.

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.

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.

Osteogenesis imperfecta (OI) or brittle bone disease is a metabolic bone disease characterized by bone fragility, low bone mass, andincreased rate of bone fractures and deformities. Clinical presentation in OI patients shows wide variability ranging from mild to severeand lethal OI types. Advances in molecular biology and studies on animal OI models found at least 16 new genes involved in OIpathogenesis. The majority of mutations are autosomal dominant aff ecting COL1A1 and COL1A2 genes responsible for collagensynthesis. The remaining 10%-15% of mutations in OI are autosomal recessive and aff ect genes involved in various metabolic boneprocesses. Progress in understanding bone metabolism and genetic engineering off ers new potential therapeutic opportunities thatare under diff erent stages of investigation.Key words: osteogenesis imperfecta, type I collagen, molecular genetics, gene therapy, stem cell