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Estimated glomerular filtration rate (eGFR) is one of the best-performing methods in evaluating kidney function. There are limited data regarding the estimated glomerular filtration rate in children and young adults with hemophilia. The aim of this study was to determine the difference between three commonly used estimated glomerular filtration rate equations in the pediatric population in a cohort of patients with hemophilia. Our prospective study included 36 pediatric patients with moderate or severe hemophilia. eGFR was calculated for each patient using the original creatinine-based “bedside Schwartz” equation, the cystatin C-based equation and the creatinine-cystatin C-based equation. The difference between the equations, calculated using the one-way repeated ANOVA test, was statistically significant (p, Procjena glomerularne filtracije jedna je od najboljih metoda ocjene bubrežne funkcije. Postoje oskudni podaci o procjeni glomerularne filtracije u djece i mladih odraslih oboljelih od hemofilije. Cilj našeg istraživanja je utvrditi razliku između tri često korištene metode za procjenu glomerularne filtracije u pedijatrijskoj populaciji u skupini pacijenata oboljelih od hemofilije. U naše prospektivno istraživanje uključili smo 36 djece s hemofilijom umjerenog ili teškog stupnja. Svakom pacijentu procijenjena je glomerularna filtracija koristeći jednostavnu, kreatinin baziranu jednadžbu po Schwartzu, cistatin C baziranu jednadžbu i kreatinin - cistatin C baziranu jednadžbu. Razlika između tri jednadžbe koristeći jednosmjerni ANOVA test bila je statistički značajna (p

Our retrospective analysis included 85 children with newly diagnosed NHL from January 1, 1997 to December 31, 2016. They all have been diagnosed and treated at the Department of Pediatric Hematology and oncology, University Hospital Centre Zagreb.

Fanconi anemia (FA) is a rare inherited disorder associated with congenital abnormalities, progressive bone marrow failure and a predisposition for hematological and nonhematological malignant disease. Bone marrow failure initially presents with thrombocytopenia, erythrocyte macrocytosis, followed by granulocytopenia and anemia. Allogeneic hematopoietic stem cell transplantation (HSCT) is the only curative option for the hemato-logical diseases of FA. We present a boy with pancytopenia as a first manifestation of the FA at the age of 5. The boy is the first FA patient in our country treated with HSCT. The bone marrow from a matched unrelated donor was transplanted. Conditioning regimen we used is based on GEFA03 protocol which includes fludara-bine, cylophosphamide and busulfan together with alemtuzumab and mycophenolate mofetil as GVHD prophy-laxis.

Analiza rezultata ovog istraživanja nije dosegla statističku značajnost oštećenja bubrega u promatranim skupinama. Stoga planiramo u daljnjem istraživanju uključiti i mlađe odrasle osobe s očekivanjem postizanja statistički značajnih rezultata. U tijeku je procjena oštećenja bubrega osjetljivijim dijagnostičkim metodama, te u tom slučaju očekujemo razliku u bubrežnoj funkciji.

Sve mutacije opisane u ovom istraživanju su blagog fenotipa po Mahidol thalassemia severity score indeksu i bez daljnje progresije bolesti. U ovoj skupini pacijenata nismo pronašli mutacije beta globinskog gena klinički izraženijeg fenotipa. Nužno je provesti sveobuhvatno genetičko istraživanje beta- talasemija u Republici Hrvatskoj kako bi se upotpunila populacijska studija beta globinskog gena.

Limfomi su treća najčešća maligna bolest u djece. Standardno liječenje Bstaničnog NHL u odraslih pacijenata je kemoterapija s monoklonskim protutijelom rituksimabom (anti CD 20). Kada je rituksimab pridružen CHOP kemoterapijskom protokolu došlo je do povećanja stope preživljenja u odraslih. Zbog nedostatka većih studija, liječenje B-staničnog NHL uz dodatak monoklonalnih protutijela u djece još nije nedvojbeno prihvaćeno. U ovom radu 36 djece s novo dijagnosticiranim B-NHL praćeno je u razdoblju od 1. siječnja 1998. do 31. prosinca 2012. Liječeni su na Klinici za pedijatriju Kliničkog bolničkog centra Zagreb. Uključeno je 8 djevojčica i 28 dječaka (u dobi 3-17 godina). Sveukupno preživljenje za cijelu skupinu pacijenata je 80,6 %. Preživljenje temeljeno na spolu prednost daje djevojčicama – 87,5 %, naspram 78,6 % u dječaka. Prema kliničkom stadiju djeca su podijeljena u dvije skupine: A – stadij I i II i skupinu B: stadij III i IV: Preživljenje tih dviju skupina pokazuje statistički značajnu razliku. Preživljenje skupine A bilo je 92,0 % prema 54,5 % za skupinu B. Djeca liječena standardnim protokolom NHL-BFM-95 u kombinaciji s rituksimabom imaju bolje preživljenje (85,7%) od onih koji nisu primili liječenje rituksimabom uz kemoterapiju (73,3%). Navedeni rezultati nisu dosegli statističku značajnost. Kako bi donijeli konačan zaključak o korisnosti monoklonalnih protutijela u liječenju B-NHL u djece potrebna nam je veća skupina pacijenata., Lymphomas are the third most common malignant disease in childhood. Standard treatment of B-cell NHL in adult patients is chemotherapy with monoclonal antibody – rituximab (anti CD 20). Rituximab added to CHOP chemotherapy protocol improved survival rates in adults. Treatment of B cell NHL with monoclonal antibodies in children has not yet been determined. In total 36 children with newly diagnosed B-NHL were followed from January 1, 1998 to December 31, 2012. They have been treated at the Department of Paediatrics University Hospital Centre Zagreb. There were 8 girls and 28 boys (3-17 yrs). Overall survival (OS) for the entire group was 80.6%. Gender based survival is in favour of girls - 87,5% versus boys 78,6%. According to clinical stage children were divided in two groups: A: stage I and II, and group B: stage III and IV. Survival time of those two groups showed statistically significant difference. Survival rate of group A was 92,0 % versus 54,5 % for group B. Children treated with standard protocol NHL-BFM-95 combined with rituximab have higher survival rate (85,7%) than those who did not receive rituximab treatment together with chemotherapy (73,3%). But there were no statistical significance. To make definitive conclusion of usefulness of monoclonal antibodies in treatment of paediatric B-NHL we need larger cohort of patients.

Currently, persons with hemophilia are living longer and the life expectancy is reaching almost that of general male population. However, having in mind that their health condition dramatically deteriorate with age, we investigated how these changes affect their everyday-life, particularly the level of personal well-being and happiness. We conducted an empirical survey among adults with hemophilia in Croatia (N=135). The sample was divided into four age groups: ≤ 30 years ; 31-40 years, 41-50 years, ≥ 51 years. Subjective well-being was measured by using the Personal Wellbeing Index - Adult (PWI-A), which measures satisfaction within life domains. The affective component of subjective well-being was examined by using the Happiness Measure from the Fordyce scale. The level of happiness and all domains of subjective well-being in relation to age showed a statistically significant negative correlation. However, the significance was reversed if the influence of disability level and/or movement impairment was excluded. The most significant decrease in well-being and personal happiness level (lowest score in all analyzed domains) was observed among hemophiliacs in the third age group (41-50 years). The average educational and the employment levels in that group were significantly lower than in the previous group (31-40 years), but the degree of disability was significantly higher. These results suggest that aging per se is not a direct predictor of lower levels of personal well-being and happiness, unless it is accompanied by increased movement impairment and higher degree of disability, which in hemophiliacs often correlates with age. Moreover, our results show that the third age group is the most vulnerable among hemophiliacs and requires special attention. In the subsequent analysis we revealed an objective cause for such finding, and our results clearly emphasize the importance of a prompt and an adequate treatment to prevent excessive bleeding that can result in musculoskeletal disorder and increased level of the disability. At the same time, this is a clear indication of a strong need to encourage education among young persons with hemophilia and to orientate them towards professions in which a reduction of the body's motor functions would not significantly limit the performance that can lead to loss of the employment.