Tumori jetre u dječjoj dobi

Liver tumors count for approximately 2% of all childhood tumors and almost 70% of them are malignant. Most of them present as palpable abdominal mass. Correct diagnosis considering type, size and localization of the tumor is crucial for the right treatment strategy. Although surgical resection still remains the most important factor for survival, when combined with chemotherapy, the survival rates will raise. Liver transplantation is also considered in some cases of liver tumors. From 1991 to 2008 we treated 13 children with liver tumors. Our experience together with the review of recent literature is presented here.Tumori jetre čine ukupno 2% svih tumora dječje dobi, a preko dvije trećine su zloćudni. Većina se prezentira kao palpabilna tvorba u abdomenu. Za odabir ispravnog plana liječenja nužno je postaviti točnu dijagnozu i odrediti stupanj bolesti. Iako je kirurška resekcija i dalje najbitniji faktor prognoze, u kombinaciji s kemoterapijom postotak preživljenja značajno raste. U određenim slučajevima neresektabilnih tumora transplantacija jetre daje dobre rezultate. Od 1991. godine do 2008. liječili smo 13 djece s tumorom jetre i u ovom radu prikazujemo naše rezultate uz pregled recentne literature

Endoscopic treatment of vesicoureteral refluks in children

Vezikoureteralni refluks prisutan je u oko 1 % djece i povezan je s povećanim rizikom nastanka pijelonefritisa i oštećenja bubrega. Glavni cilj liječenja refluksa je spriječiti pojavu uroinfekcije i na taj način sačuvati bubrežnu funkciju. Unatoč njegovoj učestalosti, mnogi aspekti dijagnostike i liječenja još su uvijek kontroverzni. Endoskopsko liječenje je minimalno invazivno liječenje koje je postalo široko prihvaćeno zbog kratkoće vremena potrebnog za izvođenje zahvata, kratkotrajnog boravka u bolnici, visoke učinkovitosti, niske stope komplikacija kao i smanjenih troškova liječenja. Iako postoje brojni prijepori u vezi s njegovom učinkovitosti, endoskopsko liječenje korisna je metoda liječenja i dobra alternativa dugoročnoj uroprofilaksi. U preglednom radu donosimo današnje spoznaje o nastanku vezikoureteralnog refluksa, njegovoj dijagnostici i endoskopskom liječenju.Vesicoureteral reflux is present in approximately 1% of children and is associated with high risk of developing pyelonephritis and renal scarring. The main goal in treating vesicoureteral reflux is to prevent uroinfection and thus preserve renal function. Despite it’s prevalence, many aspects in diagnosis and treatment are still controversial. Endoscopic treatment has become widely accepted due to short operative time, short hospital stay, high success rate, low complication rate and lower overall cost of treatment. Although there are numerous arguments about it’s efficacy, endoscopic treatment is a valuable alternative to long-term antibiotic profilaxis. In this review article we present current knowledge about the genesis of vesicoureteral reflux, diagnosis and endoscopic treatment

Diagnosis and surgical treatment of intestinal malrotation in a patient with Cornelia de Lange syndrome

Prikazujemo žensko dojenče s fenotipskim karakteristikama sindroma Cornelia de Lange kod kojeg je dokazana i uspješno kirurški liječena pridružena malrotacija crijeva. Svrha je rada upozoriti na činjenicu da malrotacija crijeva, iako ne pripada skupini učestalih simptoma sindroma Cornelia de Lange, ne smije biti izostavljena u diferencijalnoj dijagnostici gastrointestinalnih tegoba u navedenih bolesnika.We report on a female infant with phenotypic characteristics of Cornelia de Lange syndrome and associated, successfully surgically treated, intestinal malrotation. The purpose of this report is to point out that intestinal malrotation, as a rare element of Cornelia de Lange syndrome, should not be left out on the diff erential diagnosis of gastrointestinal symptoms in these patients

Abdominal aortic thrombosis in a healthy neonate

Abdominal aortic thrombosis is a rare entity in neonates and has mostly been associated with umbilical artery or cardiac catheterization. We present a complicated case of an otherwise healthy neonate who developed thrombosis of abdominal aorta with renal failure. Therapy with intravenous heparin was unsuccessful, and thrombolysis was contraindicated because of disseminated intravascular coagulation so we decided to perform open thrombectomy using the left retroperitoneal approach. The following day, thrombosis recurred in the same extent and despite high risk of bleeding Alteplase was eventually given, which resulted in recanalization of the aorta 6 hours later. Renal function recovered, dialysis was discontinued, and further course was uneventful. The treatment of abdominal aortic thrombosis in neonates should be considered on a case-by-case basis because the available data on the condition are limited to case report and series. If open thrombectomy is performed, retroperitoneal approach should be preferred because it allows for easy institution of peritoneal dialysis should the need arise

Quality of communication at the Division of Pediatric Surgery, Zagreb University Hospital Center

Istraživanje je provedeno kako bi se ispitalo zadovoljstvo i utvrdilo iskustvo roditelja hospitalizirane djece s različitim aspektima komunikacije s medicinskim osobljem za vrijeme bolničkog liječenja. U istraživanju je sudjelovalo osamdeset pet roditelja djece hospitalizirane na Zavodu za dječju kirurgiju i urologiju Klinike za kirurgiju KBC Zagreb. Za potrebe istraživanja primijenjen je prilagođeni „Upitnik o iskustvima roditelja djece s teškoćama u razvoju u komunikaciji s medicinskim osobljem“. Upitnik su ispunjavali roditelji na dan djetetova otpusta iz bolnice. Roditelji medicinsko osoblje najčešće ocjenjuju kao profesionalno, ljubazno i empatično. Informacije koje su dobili u razgovoru bile su im razumljive, a to su provjerili liječnici. Najviše informacija dobili su o djetetovom trenutnom stanju i o tijeku i postupcima liječenja. Svi roditelji smatraju važnim da na Zavodu postoji stručna osoba koja pruža psihosocijalnu potporu. Najveći broj ispitanih roditelja izjavio je da su im od medicnskog osoblja najveću potporu pružili liječnik i medicinske sestre. Na pitanje o zadovoljstvu s komunikacijom s medicinskim osobljem općenito, većina ispitanika izjasnila se kao vrlo zadovoljni (75% ih je vrlo zadovoljno s komunikacijom s liječnikom, 82% ih je vrlo zadovoljno s komunikacijom s medicinskim sestrama). Odnos medicinskog osoblja i obitelji važan je u procesu liječenja djece i adolescenata. Primjerena komunikacija medicinskog osoblja i roditelja umanjuje stres roditelja zbog bolesti i liječenja i unapređuje tijek i rezultate liječenja.The study was designed to assess the level of parental satisfaction and experience with diff erent aspects of communication with medical personnel during hospitalization of their children. The study included eighty-fi ve parents of children hospitalized at the Division of Pediatric Surgery and Urology, Department of Surgery, Zagreb University Hospital Center in Zagreb. On the day of discharge from the hospital, the parents voluntarily fi lled in the specially designed questionnaire. In most cases, parents rated medical personnel as professional, kind and sympathetic. They understood information provided by medical personnel and physicians made sure that the parents fully understood what had been communicated to them. Most of the given information was about the current medical condition of the child, and the course and procedures of treatment. All participating parents consider professional psychosocial support as an important asset on the ward. Regarding the support given by medical personnel, most parents stated that it was provided by both the physician and the nurse. When asked to rate their satisfaction with communication with medical personnel in general, the majority of parents stated that they were very satisfi ed (75% very satisfi ed with communication with physicians and 82% very satisfi ed with communication with nurses). In conclusion, the relationship between medical personnel and patient family is important in the process of the child and adolescent treatment. Appropriate communication between medical personnel and parents reduces the parents’ stress caused by their children sickness and treatment and improves the course and outcome of treatment

Psoas abscess as the first manifestation of Crohn’s disease – case report

A case of 16-year-old female with secondary psoas abscess as the complication of the Crohn’s disease was analysed. Her pre-hospital, long lasting, non-specific symptoms led to ultrasound (US) and radiologic imaging confirming the diagnosis. The antibiotic treatment was insufficient and worsening of the patient’s condition required surgical treatment including descendent colectomy, abscess drainage and negative pressure wound therapy (NPWT). In spite of numerous complications the condition of the child gradually improved and she was discharged from the hospital on the 71st postoperative day. Two years after the colectomy, the occlusion of her colostomy was performed

SURGICAL TREATMENT OF THYROID GLAND DISEASES IN CHILDHOOD – OUR RESULTS

Bolesti štitne žlijezde jedna su od najčešćih endokrinopatija u djece. Većina njih uspješno se liječi konzervativnom terapijom, no u određenim slučajevima potrebno je kirurško liječenje. Odluka o kirurškom liječenju rezultat je suradnje pedijatra endokrinologa i kirurga, ovisi o prirodi bolesti, a opseg kirurškog zahvata o patološkoanatomskim promjenama u žlijezdi. Prikazani su rezultati kirurškog liječenja 41 djeteta provedenog u Zavodu od 1991. do 2009. godine te današnja stajališta u kirurškom liječenju djece s različitim bolestima štitne žlijezde.Thyroid gland diseases are the most common endocrinopathies in children. Vast majority of these conditions are treated with medical therapy but in individualised cases surgery is indicated. Decision about surgical treatment is made in cooperation of pediatric endocrinologist and surgeon, treatment options depend on the nature of the disease while the extent of surgical procedure is determined by the pathological changes present in the gland. In this paper we represent the results of surgical treatment of 41 children at our department from 1991 to 2009 and current trends in surgical treatement of thyroid gland disease

Rijetka lokalizacija neuroblastoma u zdjelici

Neuroblastom je najčešći ekstrakranijalni maligni solidni tumor kod djece koji se većinom pojavljuje u trbuhu (nadbubrežna žlijezda, simpatički lanac) i medijastinumu, dok su druge lokacije rijetke. Tumor se liječi kirurški, kemoterapijom i radioterapijom. U ovom članku prikazan je rijedak slučaj neuroblastoma sakralnog pleksusa u zdjelici 9-mjesečnog ženskog dojenčeta sa širenjem u desni ishijadični i opturatorni kanal. Učinjena je ekstirpacija tumora s paraaortalnom i ilijakalnom limfadenektomijom, dok je manji dio tumora zaostao u opturatornom otvoru jer bi pokušaj potpunog odstranjenja nosio visok rizik od neurološkog oštećenja. Pokušaj potpunog odstranjenja tumorskog tkiva nije preporučiv jer nosi visok rizik od znatnih neuroloških oštećenja, a ne poboljšava preživljenje

FRONTAL, AXILLARY AND TYMPANIC TEMPERATURE MEASUREMENTS IN CHILDREN

Svrha ovoga prospektivnog istraživanja, provedenog u jednom istraživačkom centru, bila je usporediti vrijednosti tjelesne temperature izmjerene dvjema metodama: standardnim staklenim termometrom u aksilarnoj regiji i infracrvenim temperaturama timpanične i frontalne regije u afebrilne djece. Studija obuhvaća 345-ero afebrilne djece životne dobi od 4 do 16 godina, koja su radi elektivnog zahvata boravila na odjelu dječje kirurgije. Temperature su mjerene u 1000 navrata simultano aksilarno, u slušnom kanalu i frontalno. Koristili smo se dvama različitim infracrvenim termometrima; jednom vrstom za timpaničnu, drugom za frontalnu temperaturu. Aksilarna temperatura definirana je kao standard i mjerena je klasičnim staklenim termometrom. Svaki je pacijent bio izložen konstantnoj temperaturi okoliša minimalno 10 minuta prije simultanog mjerenja temperatura. Prosječna frontalna temperatura bila je 36,9 ± 0,38 °C i jednaka je aksilarnoj tempera­turi, 36,9 ± 0,16 °C. Prosječna timpanična temperatura bila je 36,3 ± 0,98 °C. Srednja je razlika timpanične i aksilarne temperature –0,4 °C. Izmjereni niz timpaničnih temperatura u skupini naših ispitanika ima trostruko veću disperziju nego frontalni niz i pet puta veću nego aksilarne temperature. Aksilarne temperature, mjerene klasičnim staklenim termometrom, imaju najmanju disperziju izmjerenih vrijednosti, slijede frontalne temperature mjerene infracrvenim termometrom, a najmanje su pouzdane izmjerene timpanične temperature.The purpose of this study was to compare the results of body temperature measurements obtained by standard axillary thermometers with the results of infrared tympanic and frontal skin thermometry in afebrile children. This study comprises a single-center, prospective comparison trial. A total of 345 afebrile children aged 4 to 16 years hospitalized in the pediatric surgery department for elective surgery were included. One thousand axillary, tympanic and frontal measurements were obtained and compared. We used two different infrared thermometers in this study; one type measured the tympanic temperature, the other the temperature on the forehead. The axillary temperature measured with the glass thermometer was set as the standard. Each patient was exposed to a constant environmental temperature for a minimum of 10 min before simultaneous temperature measurements. The mean frontal temperature 36.9± 0.38 °C was equal to the axillary temperature 36.9± 0.16 °C. The mean tympanic temperature was 36.3 ± 0.98 °C. The mean difference between the tympanic and axillary temperatures was –0.4 °C. The tympanic temperature had a threefold greater dispersion than frontal and a fivefold greater dispersion than axillary temperature. The results of this study suggest that the axillary temperature measured with glass thermometer has the least dispersion. Somewhat less reliable is the frontal temperature measured with infrared thermometer. The least reliable is tympanic temperature measurement

CONGENITAL HYPERINSULINISM – NOVEL INSIGHTS INTO ETIOLOGY, DIAGNOSIS AND TREATMENT

Kongenitalni hiperinzulinizam (KHI) najčešći je uzrok tvrdokornih hipoglikemija u novorođenačkom i ranome dojenačkom razdoblju. Iako je bolest razmjerno rijetka s pojavnosti od oko 1 : 25 000–50 000 živorođene djece, bolest se ne smije podcijeniti zbog trajnih neuroloških oštećenja do kojih dolazi ako se bolest brzo ne otkrije i ne započne promptno liječiti. Uzrok su mutacije nekog od 7 gena, ključnih u regulaciji inzulinske sekrecije u b-stanicama gušterače. Odmah nakon postavljanja dijagnoze nužno je u terapiju uvesti antihipoglikemike koji djeluju specifično na -stanice gušterače. Kod teških neonatalnih oblika nerijetko postoji rezistencija na antihipoglikemike. Tada preostaje kirurško liječenje prije kojeg je potrebno odrediti histološki oblik bolesti, što se danas uspješno postiže kombinacijom genskog testiranja i scintigrafske pretrage. Kirurškim se zahvatom najčešće postiže izlječenje kod fokalnog KHI, dok difuzni oblik ima lošiju prognozu. Ovaj je tekst pregled novijih spoznaja o KHI, kojim želimo naglasiti važnost što ranijeg postavljanja dijagnoze kao preduvjeta uspješnoga ciljanog liječenja bolesti, koje je posljednjih godina obogaćeno novim mogućnostima.Congenital hyperinsulinism (CHI) is a major cause of persistent hypoglycemia in the neonatal and early infancy periods. Althought the disease is relatively rare with incidence of about 1: 25 000–50 000 live births, the importance of the disease should not be underestimated. Namely, prompt recognition and management of patients with CHI is essential, if permanent neurological impairment is to be avoided. CHI is caused by mutations in one of the 7 genes involved in the regulation of insulin secretion in pancreatic b-cells. It is important to introduce specific medical therapy as soon as diagnosis is established. Severe, neonatal forms of CHI are often resistant to medications, thus they require surgical procedure. The preoperative genetic testing and scintigraphy are indicated to distinguish histological subtypes of the disease (focal vs. diffuse CHI). Patients with focal disease are usually cured after pancreatic resection, while diffuse disease has much worse prognosis. This manuscript offers novel insights into CHI and emphasizes the role of early diagnosis as crucial for succesful treatment that was recently enriched with novel options