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Immunocompromised patients are susceptible to multiple severe viral infections. This paper describes a 4-year-old boy with newly diagnosed B-cell precursor acute lymphoblastic leukaemia. The 4-year-old patient developed haemorrhagic cystitis, obstructive nephropathy and renal failure due to human polyomavirus BK and human adenovirus co-infection. Cidofovir should be used only in life-threatening cases.

Hematopoietic cell transplantation (HCT) is widely used as a treatment for acquired and congenital disorders. In recent years, a significant increase in transplant activity around the world has been observed, especially in Eastern European countries. This article aimed to assess progress and trends in pediatric HCT in Central-Eastern European countries between 2013 and 2018. Transplant activity survey in 2013 and 2018 in nine Central-Eastern European countries (Czech Republic, Croatia, Hungary, Lithuania, Poland, Romania, Slovakia, Slovenia, and Ukraine) was performed. The highest transplant rates in total were found in the Czech Republic and Hungary. When calculated per 10 million of the pediatric population, a 25.9% increase in the number of allo-HCT was observed with the highest in Croatia, Romania, Lithuania, and Poland; and a 12.2% increase in the number of auto-HCT was observed with the highest in Slovenia, Slovakia, Romania, Poland, Ukraine, and Croatia. We have shown, over the years 2013 and 2018, in some countries of Central-Eastern Europe that there was a significant increase in transplant activity, especially in those with the lower rates. This increase was observed mainly in centers already existing in 2013, especially in the allo-HCT setting. The rise of activity was significantly less influenced by the creation of new transplant centers or the increase in the number of pediatric transplant beds. In conclusion, our analysis indicates that in the Czech Republic, Hungary, Lithuania, and Slovenia, the actual infrastructure and the number of HCTs cover the needs, whereas in other countries, especially in Romania and Ukraine, the number of HCT needs to be increased.

Introduction: This study reevaluated von Willebrand disease (vWD) diagnosis in a Croatian paediatric cohort by combining bleeding scores (BS), phenotypic laboratory testing, and next- generation sequencing (NGS). Materials and methods: A total of 25 children (11 males and 14 females, median age 10 years, from 2 to 17) previously diagnosed with vWD were included. BS were calculated using an online bleeding assessment tool. Phenotypic laboratory analyses included platelet count, platelet function analyser closure times, prothrombin time, activated partial thromboplastin time, von Willebrand factor antigen (vWF:Ag), vWF gain-of- function mutant glycoprotein Ib binding activity (vWF:GPIbM), vWF collagen binding activity (vWF:CBA), factor VIII activity (FVIII:C) and multimeric analysis. Next-generation sequencing covered regions of both vWF and FVIII genes and was performed on MiSeq (Illumina, San Diego, USA). Results: Disease-associated variants identified in 15 patients comprised 11 distinct heterozygous vWF gene variants in 13 patients and one novel FVIII gene variant (p.Glu2085Lys) in two male siblings. Four vWF variants were novel (p.Gln499Pro, p.Asp1277Tyr, p.Asp1277His, p.Lys1491Glu). Three patients without distinctive variants had vWF:GPIbM between 30 and 50%. Patients with identified vWF gene variants had statistically significant lower values of vWF:GPIbM (P = 0.002), vWF:Ag (P = 0.007), vWF:CBA (P < 0.001) and FVIII:C (P = 0.002), compared to those without. Correlations between BS and phenotypic laboratory test results were not statistically significant for either of the tests. Conclusion: The applied diagnostic approach confirmed the diagnosis of vWD in 13 patients and mild haemophilia A in two. Limited utility of BS in the paediatric population was evidenced.

A 6-year-old boy was referred to a hematologist due to excessive mucocutaneous bleeding. Diagnostic assessment for von Willebrand disease (VWD) was indicated and included both coagulation and genetic testing. Laboratory testing revealed proportionally decreased von Willebrand factor (VWF) glycoprotein Ib-binding activity (23.6%) compared to VWF antigen (24.7%), similarly decreased VWF collagen-binding activity (24.2%), and normally distributed VWF multimers, with decreased intensity of all fractions. Diagnosis of type 1 VWD was established. Genetic analysis by means of next-generation sequencing (NGS) of VWF and coagulation factor VIII genes did not identify any causative mutations. Additionally, multiplex ligation-dependent probe amplification (MLPA) of VWF gene exons revealed a heterozygous deletion of exons 1 to 6, which is reported in type 1 VWD for the first time. Application of MLPA was crucial for revealing the genetic basis of type 1 VWD in this case, which would have remained undetected if only NGS was used.

Introduction This study analyses real-world data on 144 previously untreated patients (PUPs) with severe Haemophilia A, from seven countries in Central and Eastern Europe (CEE: Bulgaria, Croatia, Czech Republic, Hungary, Latvia, Serbia, and Slovenia), over a period of 11 years. Aim To analyse the risk factors associated with development of inhibitors to factor VIII concentrates. Methods Cox proportional hazard models were used to estimate the hazard risk of factors possibly influencing the development of inhibitors. Patients were followed for up to 100 exposure days (EDs). Results Cumulative inhibitor incidence at the time of 100 EDs was 18.7%, slightly lower than the 25–35% incidence reported in most studies. Of PUPs who developed inhibitors, a majority (56%) developed them within the first 20 EDs and 88% by the 50th ED. FVIII class (recombinant or plasma-derived) did not influence the inhibitors' incidence rate (p = 0.64). We found a significant protective effect of prophylaxis compared to on-demand treatment (p = 0.003). PUPs who had an intensive peak treatment during the first 50 EDs were at significantly higher risk for inhibitor development (HR (95% CI) 5.3 (2.3–12.5), p Conclusion Inhibitors are and will continue to be the most significant complication of haemophilia treatment with factor concentrates. This is particularly true for haemophilia A. In our cohort, we were able to show that the treatment regimen used during first 50EDs influenced significantly the inhibitor risk, but the class of the factor concentrate did not play an important role. Real world data will remain one of the important resources for improving our knowledge of haemophilia.

We present a case of a 16-year-old male with large pericardial effusion due to Hodgkin Lymphoma (HL). Shortly after drainage of pericardial effusion he developed a low cardiac output syndrome which had to be treated with extracorporeal membrane oxygenation (ECMO). This 9-day ECMO support helped the patient to recover his cardiac function, and thereafter a remission of his primary disease was successfully achieved with chemotherapy. It is a matter of discussion whether a large pericardial effusion with moderate symptoms in patients with HL should be evacuated or just observed since the effusion should ameliorate with chemotherapy. But based upon our experience in this case of hemodynamic instability due to a large effusion requiring evacuation, we propose that pericardiocentesis procedure should be performed with caution at a slow drainage rate of 0.5-1 ml/kg/hour with a maximum rate of 50 ml/hour, to help avoid the low cardiac output syndrome in patients with similar disease conditions.

Invasive fungal infections (IFI) are an important problem of modern medicine. The reason is growing population of immunocompromised patients and high morbidity and mortality of these infections. Timely diagnosed IFI is of utmost importance because the delay of antifungal treatment has impact on treatment outcome. Cultivation as a conventional diagnostic method has low sensitivity, long duration and demands obtaining invasive samples. Therefore, in the last two decades fungal biomarkers are investigated for earlier and more sensitive diagnostics. 1,3-β-D-glucan (BDG) is a fungal biomarker in patients’ sera that enables detection of the following fungal pathogens: Candida spp., Aspergillus spp., Acremonium, Coccidioides immitis, Fusarium spp., Histoplasma capsulatum, Trichosporon spp., Sporotrix schenckii, Saccharomyces cerevisiae and Pneumocystis jirovecii. Low level and absence of BDG in the cell wall unables the detection of Cryptococcus spp. and order Mucorales with this test. High negative predictive value of BDG can be used when deciding to stop antifungal treatment and be a part of strategy for antifungal stewardship in intensive care units. In hematological patients BDG can be used as a screening method or as a part of diagnostic work-up when IFI is suspected. Reliability of test result is higher when two or more consecutive samples are positive. Influence of antifungal prophylaxis on BDG test results is still unclear. BDG kinetics and its relation to clinical outcome are still investigated. For pediatric population cut-off values for interpretation are still not defined, although many studies have been published investigating this issue. Although still not recommended by pediatric guidelines, this test can help in certain situations having in mind its limitations. BDG as a fungal marker represents the significant progress in IFI diagnostics. With simultaneous application of other diagnostic methods, exact interpretation and rational use, it can help earlier and more successful diagnostics and treatment of IFI.

Thiamine-responsive megaloblastic anemia (TRMA) is an autosomal recessive disorder characterized by the development of megaloblastic anemia, diabetes mellitus, and sensorineural deafness. We report on the first two Croatian patients with TRMA, compound heterozygotes for nonsense, c.373C > T; p.(Gln125Ter) and novel missense variant, c.1214C > G; p.(Thr405Arg) in SLC19A2 gene. The first was diagnosed at 4 months with diabetes mellitus and severe anemia requiring transfusions. As TRMA was suspected, thiamine therapy was immediately started to prevent further transfusions and insulin therapy. His brother developed extreme anemia at 3 weeks of age while waiting for the results of the genetic test. Severe anemia in this sibling may have been prevented if thiamine had been initiated earlier.

We describe a unique case of an 8‐month‐old male child with two different and genetically unrelated entities. Haemophilia A (HA) is caused by a deficiency of functional plasma clotting fac‐tor VIII (FVIII) with X‐linked recessive traits, while inheritance of Shwachman‐Diamond syndrome (SDS) follows autosomal recessive biallelic mutations in DNA JC21 (c.183_184delTAinsCT) with exo‐crine pancreatic insufficiency, impaired hematopoiesis and leukae‐ mia predisposition. These two separate diseases combined together present a challenge to a physician regarding their combined clinical presentation and ambiguous prognosis. Hence, in this Letter we present an example of complex clarification of genetically combined haematologic cases.

Krvarenja i tromboembolije se pojavljuju kod djece i kod odraslih. Razlog tome mogu biti prirođeni ili stečeni poremećaji. Fiziologija koagulacije i fibrinolize se značajno razlikuje u novorođenčadi i manje djece, od koagulacije i fibrinolize starije djece i odraslih osoba. Sazrijevanjem i rastom djeteta te razlike nestaju. Zbog značajnih fizioloških razlika funkcije trombocita i koncentracije čimbenika koji sudjeluju u koagulaciji i fibrinolizi novorođenčad je istovremeno sklona i krvarenju i nastanku tromboze. Ova dva stanja su kod novorođenčadi međusobno usklađena. Nastanku tromboza kod novorođenčadi pogoduju središnji venski kateteri, sepsa, bolesti jetre, upala i poremećaji tjelesnih tekućina. Hemofilije su najčešće bolesti kod kojih se javljaju opsežna krvarenja. Na njih treba misliti u slučaju nenadanog krvarenja ili pozitivne obiteljske anamneze. U kasnijoj dobi na hemofiliju se mora pomisliti kod dječaka koji pojačano krvare nakon invazivnih postupaka ili traume. Profilaktičko liječenje hemofiličara je postao zlatni standard liječenja i treba započeti što ranije da se prevenira nastanak oštećenja zglobova. Djeca s poremećajima zgrušavanja krvi trebala bi biti podrobno klinički i laboratorijski obrađena da bi se moglo provesti ispravno liječenje. To značajno smanjuje mogućnost nastanka komplikacija i dugoročnih posljedica., Haemorrhagic and thrombotic events occur in both children and adults. The underlying causes are congenital or acquired disorders. The maturation and postnatal development of the human coagulation system results in significant and important differences in the coagulation and fibrinolysis of neonates and young children compared to older children and adults. Platelet function, pro- and anticoagulant protein concentrations and fibrinolytic pathway protein concentrations are developmentally regulated and generate hemostatic homeostasis that is unique to the neonatal period. At the same time, neonates have a predisposition to bleeding and develepment of thrombosis. These differences, which mostly reflect the immaturity of the neonatal haemostasis system, are functionally balanced. Central lines, fluid fluctuations, sepsis, liver dysfunction and inflammation contribute to the risk profile for thrombosis development in ill neonates. Hemophilia is the most common of the severe bleeding disorders and should be considered in the neonatal period in case of unusual bleeding or positive family history. Later, hemophilia should be suspected mainly in males because of abnormal bleeding following invasive procedures. Prophylactic treatment that is started early with clotting-factor concentrates has been shown to prevent hemophilic arthropathy and is therefore the gold standard of care for hemophilia A and B. Children with coagulation disorders should be clinically and laboratory treated according to the exact type and degree of clotting disorder and appropriate treatment should be conducted. This significantly reduces the possibility of acute complications and long-term consequences.

A mini extracorporeal photopheresis (mini-ECP) "off line" technique has been developed for use in the treatment of small children and patients with apheresis contraindications. Until now various methods have been used for buffy coat separation from whole blood. In this report we describe a protocol for mini buffy coat preparation using the automated Sepax laboratory separator for "off line" ECP treatment in a low body weight child with graft-vs-host-disease. According to our results this alternative method has been proven feasible and tolerable.

Increasing safety of allogeneic hematopoietic stem cell transplantation (allo-HSCT) has also increased the number of patients at risk for developing chronic GvHD (cGvHD). Peripheralnerve neurological manifestations and symptoms are being increasingly recognized in cGvHD, however, prospectively planned clinical studies have rarely been reported. There is a substantial lack of understanding of the mechanisms that lead to peripheral neuropathy (PN) in cGvHD and therapeutic options are profoundly limited and mostly symptomatic. Currently, myasthenia gravis, muscle cramps and PN are considered as ‘other’ complications related to cGvHD, whereas myositis and polymyositis are considered as ‘distinctive’ neurological manifestations of cGvHD. Consequently, the diagnosis of neurological cGvHD can only be established when additional ‘diagnostic’ manifestations of cGvHD are present and the neurological manifestations are still not incorporated into the scoring system.1, 2 This presents an important gap, because damage of the peripheral nervous system (PNS) in the context of cGvHD can produce severe disability, morbidity and mortality.2 In order to investigate the incidence and characteristics of PNS manifestations, with emphasis on small-fiber neuropathy (SFN), we have conducted a prospective cohort study on patients with cGvHD developed after allo-HSCT.

Within the human testis, Reinke’s crystals are found in Leydig cells but their nature and function are poorly understood. The aim of our study was to investigate the properties of Reinke’s crystals in man with the normal morphology of the testis (control group) and infertile patients diagnosed with cryptorchidism. 20 biopsies from infertile patients and six biopsies from men with regular spermatogenesis (20–30 years.) were used. Sections of the testis tissue were stained with haematoxylin and eosin and a modified Masson’s method. Specimens were observed by bright field, confocal and transmission electron microscopy (TEM). The number of Reinke’s crystals in investigated groups was determined applying stereological methods. In both groups, Reinke’s crystals were noted within the cytoplasm and nuclei of Leydig cells. Some ‘‘free’’ crystals were found within the interstitial space, outside Leydig cells. Confocal microscopy proved to be very useful in the assessment of the shape and 3D reconstruction of the crystal. TEM analysis confirmed a hexagonal form of the crystal, while crystallographic data on sections of 70–300 nm thickness provided a better insight into the organization of the crystal lattice. Stereological analysis revealed a significant increase in the number of crystals in cryptorchid testes when compared with controls. Increased number of crystals in cryptorchid specimens leads to the assumption that the prolonged exposure to higher (abdominal) temperature might stimulate enzymes involved in the synthesis of the proteins of the crystal.However, the exact molecular nature of the crystal lattice remains in both normal and cryptorchid testis obscure.

Cilj. Aktualizirati prevenciju hemoragijske bolesti novorođenčeta (HBN) ili krvarenja nastalog zbog nedostatka vitamina K u dojenačkom razdoblju sukladno novijim preporukama i praksi u svijetu. Materijal i metode. Već više od 50 godina Američka pedijatrijska akademija preporučuje intramuskularnu primjenu 0, 5 mg do 1, 0 mg vitamina K1 u novorođenačkom razdoblju, što je ponovila u svome najnovijem stajalištu od 2003. godine. U Hrvatskoj se navedena profilaksa sustavno provodi od 1994. godine nakon preporuka ad hoc skupine stručnjaka Hrvatskog pedijatrijskog društva i Hrvatskog perinatološkog društva Hrvatskog liječničkog zbora. U međuvremenu je ustanovljeno da jednokratna intramuskularna (im.) primjena vitamina K1 u novorođenačkom razdoblju ne štiti isključivo dojenu djecu od kasnog oblika hemoragijske bolesti novorođenčeta (najčešće od 2. do 13. tjedna života), koja se u većine dojenčadi manifestira kao intrakranijalno krvarenje koje može biti fatalno ili dovesti do teških neurorazvojnih posljedica. Osim toga neki roditelji su zabrinuti zbog mogućih komplikacija im. injekcije, a stručna javnost se je uznemirila nakon retrospektivnog istraživanja kojim je postavljena sumnja na povezanost između im. injekcije vitamina K1 i pojave leukoza i drugih malignoma u djece u dobi od 6 godina, koje je kasnije opovrgnuto. U nekim je zemljama dokazano da se peroralnom (po.) primjenom malih dnevnih doza vitamina K1 od 2. do 13. tjedna u isključivo dojene djece može značajno smanjiti učestalost kasnog oblika HBN. Sličan učinak je postignut i po. primjenom 1 mg vitamina K1 od 2. do 13. tjedna, ali su protivnici te metode prigovarali zbog prekomjerno visokih koncentracija vitamina K1 u plazmi dojenčadi nakon primjene jednokratne visoke doze. Uz niske dnevne doze vitamina K u nekim se zemljama primjenjuje u istom razdoblju i vitamin D 3 u dozi od 400 IJ sukladno preporukama Američke pedijatrijske akademije. Zaključak. U Hrvatskoj bi postojeće preporuke trebalo prilagoditi najnovijim preporukama prema kojima neposredno nakon rođenja sva novorođenče treba dobiti 0, 5 mg do 1, 0 mg vitamina K1 im. i nakon toga isključivo dojena i rizična novorođenčad od 8. dana do 13. tjedna života po. 25g/dan vitamina K1 i 400 IJ vitamina D 3.

Background: Chronic graft-versus-host disease (cGVHD) is a disorder that affects many organ systems in highly variable fashion occurring in approximately 50% of patients following allogeneic hematopoietic stem cell transplantation (alloHSCT). It is the major cause of non-relapse morbidity and mortality after alloHSCT in individuals otherwise cured of their hematologic diseases, inducing poor quality of life, impaired functional status, inability to work, and need for ongoing chronic care, which has also important impact to health-related costs. cGVHD Consensus Conference held in 2005 at the National Institutes of Health (NIH), USA, produced recommendations regarding cGVHD diagnosis, staging, histopathology, response criteria, biomarkers, ancillary and supportive care, and design of clinical trials. In 2014, second cGVHD NIH Consensus Conference updated these recommendations, published during 2015 as 6 papers in Biology of Blood and Marrow Transplantation (BBMT) journal. Although practitioners are generally familiar with the NIH recommendations, many barriers prevent their greater uptake in clinical practice. In order to overcome these challenges, in 2013 multidisciplinary clinic infrastructure was organized at the University Hospital Center (UHC) Zagreb, Croatia, in collaboration with the NIH leading scientists, using established cGVHD-related grading scales and measurements. Methods: Division of Hematology, UHC Zagreb, Croatia, has experience with alloHSCT since 1983, and 827 patients received alloHSCT until the end of 2014. Since the establishment of multidisciplinary cGVHD team in 2013, patients were enrolled into the Unity through Knowledge Fund (UKF) study protocol (funded by World Bank and Croatian Ministry of Science, Education and Sports) and examined by multiple subspecialists, firstly seen by hematologist, with detailed history and physical exam. Standard cGVHD scoring forms are filled according to NIH Consensus recommendations, and extensive laboratory analyses were done. Patients are seen and evaluated by other sub-specialists (Dental, Dermatology, Rehabilitation, Neurology, Ophthalmology, Gynecology, and other) with further workup as needed. Quality of life questionnaires are filled during the visit. All data are collected in a specially developed database and weekly team meetings were established. Blood and small biopsy tissue samples (skin, mouth) are stored for further research. Results: Using multidisciplinary approach since 2013, 46 (6 pediatric) cGVHD patients were assesed, median age was 41 years; range [9-71], 24 were male and 22 were female. The median time from transplant to enrollment was 20 months [2-258], from cGVHD diagnosis to enrollment 7 months [0.03-234] and from transplant to cGVHD diagnosis 10 months [2-128]. Additional 17 post-alloHSCT patients were eveluated, but without confirmation of cGVHD diagnosis. Among cGVHD patients, 31 (67%) of them received transplants from matched related donors, 27 (59%) of them had myeloablative conditioning, and 26 (57%) received peripheral blood stem cells as graft source. Thirty-five (76%) patients had previous acute GVHD, 11 (24%) had de novo cGVHD, 21 (46%) quiescent and 14 (30%) progressive onset; 41 (89%) were classified as classic and 5 (11%) as overlap; 23 (50%) patients had severe, 19 (41%) moderate, and 4 (9%) mild global cGVHD score. The most involved organs were skin (54%), eyes (50%), lungs (48%) and mouth (39%). Due to internationally peer reviewed UKF grant awarded in 2013 doctoral and postdoctoral researcher were hired, and visits of young clinicians to NIH and other cGVHD centers were realized. Several new research subprojects emerged since formation of our cGVHD team and applications to the new project calls were submitted. Also, 2 international cGVHD symposiums were organized in Zagreb, Croatia, in last 2 years stimulating education and networking. Conclusion: Implementation of NIH criteria for standardizationof cGVHD in Croatia showed remarkable results, not just improving quality of medical documentation and management of these long-terms survivors with complex and long-lasting health issues, but also facilitating further international clinical research and collaboration with cGVHD community, with potential positive impact to health-related costs and benefit to society. Disclosures Nemet: Pliva: Honoraria; Janssen: Honoraria; Celgene: Honoraria; Amgen: Honoraria; Pfizer: Honoraria; Sanofi: Honoraria.

BACKGROUND: Serious thromboembolic events connected with rFVIIa therapy in hemophilia patients are rare. Only three cases are reported in children, all of them with hemophilia A. ----- CASE PRESENTATION: We present unique case of patient with hemophilia B and high titer inhibitors to coagulation FIX, who developed severe renal damage due to thromboembolic event during rFVIIa therapy, associated with unsuspected renovascular anomalies. ----- CONCLUSION: Caution is necessary if hematuria B requires administration of rFVIIa. US color doppler renal imaging before and after drug administration should be sufficient as an early warning.

Mutations of VHL (a negative regulator of hypoxia-inducible factors) have position-dependent distinct cancer phenotypes. Only two known inherited homozygous VHL mutations exist and they cause polycythemia: Chuvash R200W and Croatian H191D. We report a second polycythemic Croatian H191D homozygote distantly related to the first propositus. Three generations of both families were genotyped for analysis of shared ancestry. Biochemical and molecular tests were performed to better define their phenotypes, with an emphasis on a comparison with Chuvash polycythemia. The VHL H191D mutation did not segregate in the family defined by the known common ancestors of the two subjects, suggesting a high prevalence in Croatians, but haplotype analysis indicated an undocumented common ancestor ∼six generations ago as the founder of this mutation. We show that erythropoietin levels in homozygous VHL H191D individuals are higher than in VHL R200W patients of similar ages, and their native erythroid progenitors, unlike Chuvash R200W, are not hypersensitive to erythropoietin. This observation contrasts with a report suggesting that polycythemia in VHL R200W and H191D homozygotes is due to the loss of JAK2 regulation from VHL R200W and H191D binding to SOCS1. In conclusion, our studies further define the hematologic phenotype of VHL H191D and provide additional evidence for phenotypic heterogeneity associated with the positional effects of VHL mutations.

Histiocitoza Langerhansovih stanica (HLS) jest bolest karakterizirana patološkim nakupljanjem i umnožavanjem stanica monocitno-makrofagnog sustava u tkivima. Bolest može zahvatiti bilo koji organski sustav. U ovoj retrospektivnoj studiji analizirani su podatci pacijenata liječenih u Zavodu za dječju hematologiju i onkologiju Klinike za pedijatriju Medicinskog fakulteta u Zagrebu kojima je potvrđena dijagnoza HLS-a u petnaestogodišnjem razdoblju od 1. 1. 1996 do 31. 12. 2010. g. Liječeno je 22-je djece oboljele od HLS-a kod koje je bilo potrebno sustavno liječenje. Izliječeno je 19-ero (86%), a umrlo je troje (14%) djece, sve troje mlađe od 2 godine s multisustavnom bolešću. Kod postavljanja dijagnoze 12-ero djece imalo je bolest samo jednog organskog sustava (55%), i to u najvećoj mjeri zahvaćen je bio koštani sustav, kod 8 bolesnika (36%). Sva su djeca liječena prema protokolima LCH-I i LCH-III. Blaže posljedice bolesti i liječenja imalo je osmero djece. Kod četvero djece zaostao je dijabetes insipidus., Langerhans’ cell histiocytosis (LCH) is a disease characterised by pathologic accumulation and proliferation of histiocytes, cells from the monocyte-macrophage system, in various tissues and organs. In this retrospective study we analyzed patients charts treated in the Department of pediatric hematology and oncology at the University Hospital Zagreb with the diagnosis of LCH. Twenty-two children were diagnosed between January 1st 1996 and December 31st 2010, and all were treated with chemotherapy. 19 patients survived (86%) and the remaining 3 (14%), all under the age of 2 with multisystem disease, died. At the time of diagnosis 12 children (55%) presented with single-system disease, the most common were bone lesions in 8 children (36%). All children were treated according to protocols LCH-I and LCH –III. Eight children had mild complications of treatment and the disease itself. Diabetes insipidus remains in 4 children.

Rezultati liječenja T-non-Hodgkin limfoma u djece

Zaustavljanje krvarenja rekombinantnim čimbenikom VIIA kod djece oboljele od solidnih intraabdominalnih tumora

The aim of this study was to assess relations among depression, anxiety and pain in children with juvenile idiopathic arthritis (JIA). Pain was measured with the visual analogue scale (VAS), and depression and anxiety with depression and anxiety subscales from the Trauma Symptom Checklist for Children (TSC-C). Pain perception was significantly correlated with depression scores.

A case report of neonate with severe haemophilia A and massive retroperitoneal haemorrhage.

The low level of serum copper in presented patient with Stiff-person syndrome, type 1 diabetes, dermatitis herpetiformis, celiac disease and microcytic anemia opens a question of the role of copper and possibly other trace elements in the development of autoimmune diseases.