Monoclonal antibody therapy of pediatric non-Hodgkin lymphomas

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

Monoclonal antibody therapy of pediatric non-Hodgkin lymphomas

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

Procjena glomerularne filtracije u djece s hemofilijom

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 <0.001), and post hoc analysis found differences between each method. Correlation analysis showed the strongest positive correlation between the bedside Schwartz equation and creatinine-cystatin C-based equation (r=0.866) among the three methods examined. A correlation between the three eGFR methods was present, but with significant differences between them. Due to the observed differences between eGFR in pediatric patients with hemophilia, further research is needed to find the optimal measurement method for eGFR. Nevertheless, we recommend implementing eGFR equations in routine clinical monitoring of pediatric patients with hemophilia.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 <0.001), a post hoc analiza pokazala je razliku između svake od navedenih metoda. Korelacijska analiza pokazala je najjače pozitivne korelacije između jednostavne jednadžbe po Schwartzu i kreatinin - cistatin C jednadžbe (r=0.866) promatrajući tri navedene jednadžbe. Korelacija između tri opisane jednadžbe za procjenu glomerularne filtracije postoji, ali sa značajnim neslaganjem. Zbog primijećenog neslaganja između procijenjene glomerularne filtracije u pacijenata s hemofilijom daljnja istraživanja su potrebna s ciljem pronalaska optimalne jednadžbe za procjenu glomerularne filtracije. Štoviše, preporučujemo uključivanje jednadžba za procjenu glomerularne filtracije u rutinsko praćenje djece oboljele od hemofilije

Resistant Hypertension

The most common causes of therapeutic failure in hypertensive control are undiscovered secondary causes of hypertension and lack of patient/doctor compliance. In about 10% of cases, it can be attributed to resistant hypertension caused by a hyperactivity of the sympathetic nervous system, condition with a high cardiovascular risk to the patient. Resistant hypertension is failure to diminish blood pressure values to <140/90 mmHg (<140/85 mmHg for diabetic patients) with a lifestyle method and prescription of least three antihypertensive drugs in optimal doses, including a diuretic, or when patients use four or more antihypertensive drugs regardless of blood pressure control. Patients with resistant hypertension are typically presented with a long-standing history of poorly controlled hypertension. Early diagnosis and adequate treatment are needed to avoid end organ damage and to prevent cardiorenovascular remodeling. Cardiorenovascular morbidity and mortality are significantly higher in resistant hypertensive population. The need for the individualization of therapy and the use of the management strategies are also given weight in the treatment of resistant hypertension patients, including optional, innovative therapies, like a renal denervation or baroreflex activation. New innovative device therapies create an additional novel pathway of blood pressure-lowering procedures and should be prescribed by a specialist hypertension clinic

Monoclonal antibody therapy of pediatric non-Hodgkin lymphomas

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

Low back pain - an unusual presentation of acute lymphoblastic leukemia in a child – a case report

INTRODUCTION/OBJECTIVES: Acute lymphoblastic leukemia is the most common malignant disease in children. Common inital symptoms are fatigue, pallor, bleeding tendency and bone pain. Laboratory findings show cytopenia with or without leukocytosis and often elevated lactate dehydrogenase. First line therapy is corticosteroids, chemotherapy and in high-risk cases with non - favorable outcome hematopoietic stem cells transplantation

Bacteriuria in Paediatric Oncology Patients: Clinical Features, Distribution and Antimicrobial Susceptibility of Bacterial Pathogens at University Hospital Centre Zagreb, Croatia over a 4-Year Period

Bacteriuria in paediatric oncology patients have not been well studied. This retrospective study analysed clinical features, distribution and antimicrobial susceptibility of bacterial pathogens cultured from urine in paediatric oncology patients over a 4-year period (2019–2022). A total of 143 episodes of bacteriuria were documented in 74 patients. Neutropenia was present in 17.5% (25/143), symptoms in 25.9% (37/143) and urinary catheter in 7.0% (10/143) episodes. Symptomatic bacteriuria episodes were statistically significantly more frequent in patients with neutropenia (p = 0.0232). The most common bacterial pathogens were Escherichia coli (n = 49; 32.2%), Klebsiella spp. (n = 34; 22.4%), Pseudomonas aeruginosa (n = 22; 14.5%) and Enterococcus spp. (n = 21; 13.8%). Extended-spectrum β-lactamases-producing (ESBL) Enterobacterales were found in 11 episodes (11/143; 7.7%) with the highest proportion among Klebsiella pneumoniae isolates (n = 7/34; 20.6%). No carbapenem-resistant Enterobacterales, multidrug-resistant P. aeruginosa or vancomycin-resistant Enterococcus spp. were found. The most important novelties are demonstrating P. aeruginosa as one of the prominent bacteriuria pathogens in this patient population, presence of ESBL isolates and carbapenem-resistant P. aeruginosa later during hospitalization highlights the need for appropriate antimicrobial treatment. However, because of the small number of symptomatic patients, further studies are needed to clarify the importance of including urine culture in the diagnostic process in patients with febrile neutropenia

Transplantacija krvotvornih matičnih stanica u djeteta s Fanconijevom anemijom – prikaz bolesnika [Hematopoietic stem cell transplantation in a child with Fanconi anemia – case report]

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