Childhood Leukemias

Leukemije dječje dobi relativno su rijetke bolesti koje zajedno čine 35% svih malignih tumora u djece. Najveći broj pripada akutnim leukemijama (AL), dok manje od 5% čine kronične leukemije. Za razliku od odraslih, najveći broj djece (75%) obolijeva od akutne limfatične leukemije (ALL), dok akutna mijeloična leukemija (AML) čini <20% svih leukemija dječje dobi. Od sredine 60-ih godina prošlog stoljeća prognoza djece s akutnom leukemijom izrazito se poboljšala. Bolesti koje su nekad bile letalne danas su izlječive i većina bolesnika s AL dugotrajno preživi bez znakova bolesti. Članak donosi pregled leukemija dječje dobi s obzirom na razdiobu, patofiziologiju, kliničku sliku, prognozu i strategije liječenja.The childhood leukemias are relatively rare diseases that collectively represent 35% of all childhood malignant tumours. The majority of them are acute leukemias (ALs), whereas less than 5% represent chronic leukemias. In contrast to adults, the majority of children (75%) suffer from acute lymphoblastic leukemia (ALL), whereas acute myeloid leukemia (AML) represents <20% of childhood leukemias. Since the 1960s, the prognosis for children with acute leukaemia has dramatically improved. Disease that were uniformly lethal are now curable and most patients have prolonged disease-free survival. This article reviews the childhood leukemias with respect to classification, pathophysiology, clinical presentation, prognosis and treatment strategies

Bazične amino kiseline sline u patogenezi karijesa

Amino acid pattern in total saliva was studied in 43 children with caries, aged 12-15 years, using the method of ion exchange cromatography. The results were compared to those obtained in a control group of 39 children without caries. In saliva from the children with caries, a significantly lower level of arginine (2 2 . 0 2 fxmol/l), and a complete lack of histidine and its derivatives were observed when compared to the control group, where the concentrations of arginine and 1-methylhistidine were 28.36 and 26.34/mmol/1, respectively. The results obtained suggested that a decreased concentration of arginine, and a lack of histidine and its derviatives might imply an increased risk of caries.Istraživan je aminokiselinski profil ukupne sline u 43 djece s karijesom u dobi od 12—15 godina metodom kromatografije na ionskom izmjenjivaču. Dobiveni rezultati uspoređeni su s rezultatima kontrolne skupine od 39 djece bez karijesa. U slini djece s karijesom nađeno je značajno manje arginina (2 2 . 0 2 fimolll) i potpun nedostatak histidina i njegovih derivata u odnosu na kontrolnu skupinu, gdje je koncentracija arginina bila 28.36 /imol/1, a 1-metilhistidinu koncentraciji od 26.34 mmol/l. Na temelju dobivenih rezultata moglo bi se zaključiti da smanjena koncentracija arginina i nedostatak histidina, kao i njegovih derivata, ukazuje na povećani rizik prema karijesu

Mogućnosti prepoznavanja straha pomoću analize dječjih crteža

The analysis of the contents of 690 children’s drawings of the dentist’s office was carried out with the aim of determining the presence of »fear-inducing« and »encouraging« elements in these drawings. The results showed that only 9% of children drew encouraging elements like flowers, pictures, decorations, etc. The presence of fear-inducing elements appea­red to depend on: 1) children’s previous experience; 2) children’s age; 3) how the topic had been presented to chidren; and 4) what elements did actually exist in the dentist’s office.Provedena je analiza sadržaja 690 dječjih crteža stomatološke ambulante s ciljem da se utvrdi zastupljenost »zastrašujućih« i »ohrabrujućih« elemenata u tim crtežima. Rezultati pokazuju kako svega 9% djece crta i ohrabrujuće elemente, kao što su cvijeće, slike, ukrasi itd. Zastupljenost zastrašujućih elemenata ovisi o: 1) ranijem iskustvu djece, 2) dobi djece, 3) načinu kako je zadatak zadan i 4) koji su elementi doista postojali u stomatološkoj ambulanti

Određivanje Au i Ag iz rude gvožđa kombinovanjem FA i ICP/AES metoda

In this paper, the combination of FA (Flame Analysis of Noble Metals - Cupellation) and ICP/AES (Atomic Emission Spectrometry with Induction Coupled Plasma) methods for determination of Au and Ag in geological samples of iron ore-magnetite was presented. Au and Ag were concentrated with Pb from PbO after desulphurisation and melting process (Pb2+→Pb). Regulus (Pb with noble metals) is then cupellated. The resulting bead-pril after cupellation was dissolved in HNO3 and then in HCl (imperial water: 2HNO3 and 6HCl). After the preparation of standard solutions and a blank test, the ICP/AES recording is performed. The obtained results were compared with those obtained by the classical method of cupellation. The advantages of ICP/AES are excellent detection limits and linear dynamic range as well as a stable and repeating signal which is particularly important for samples of iron tested due to the low content of noble metals.U ovom radu je prikazana kombinacija FA (plamena analiza plemenitih metala-kupelacija) i ICP/AES (atomska emisiona spektrometrija sa indukovanom kuplovanom plazmom) metoda za određivanje Au i Ag u geološkim uzorcima rude gvožđa-magnetit. Au i Agse koncentišu sa Pb iz PbOnakon procesa odsumporavanja i topljenja (Pb2+→Pb).Regulus (Pb sa Au i Ag)se kupelira. Dobijena perla-pril nakon kupelacije se rastvara u HNO3 a zatim u HCl(carska voda 2HNO3: 6HCl). Nakon pripreme standardnih rastvora i blank probe vrši se snimanje na ICP/AES. Dobijeni rezultati su poređeni sa rezultatima dobijenim klasičnom metodom kupelacije. Prednosti ICP/AES su odlične granice detekcije i linearni dinamički opseg kao i stabilan i ponovljiv signal što je posebno bitno za uzorke ispitivane rude gvožđa zbog malog sadržaja plemenitih metala

Promjene u aminokiselinama, proteinima i enzimima u djece s fenilketonurijom

Since children with phenylketonuria have a low incidence of caries, the aim of this study was to examine the changes occuring in the aminoacid and protein composition as well as in the composition of salivary enzymes, which might serve as biochemical indicators, representing the factors of predisposition to the development of caries. For this purpose, the analysis was carried out in the native, unstimulated, mixed salivary specimens taken from the sublingual region of children with late diagnosis of RKU (n — 16), those with early diagnosis of the disorder by means of a systematic diagnostic screeniig program for PKU using Guthrie’s test (n — 7), and a control group of children free of any disturbances of the amino-acid metabolism (n — 44). A method of qualitative chromatography on silica gel was used for separation of aminoacids, while the proportions of each individual aminoacid were determined by a densitometer. The content of total salivary proteins was assessed by the method of Lowry, and the concentration of lysozyme by the method of Prockop. Differences in particular proportions of aminoacids, and an increase in the total protein value as well as in the lysozyme activity, could be taken as the factors of predisposition or resistance to caries.Budući da djeca oboljela od fenilketonurije imaju malu incidenciju karijesa, željeli smo ispitati promjene u sastavu aminokiselina, proteina i fermenata, koje bi mogle — kao biokemijski indikatori — biti predispozicijski činioci za razvoj karijesa. U tu svrhu analizirana je nativna slina sublingvalnog područja u uzorcima djece s kasno otkrivenom fenilketonurijom (n — 16), djece s rano otkrivenom bolešću u sistematskom traganju za fenilketonurijom primjenom Guthrie testa (n — 7) te djece kontrolne skupine bez poremećaja u metabolizmu aminokiselina. Aminokiseliine su razdvajane metodom tankoslojne kromatografije na silika gelu, a proporcije pojedinih aminokiselina određene su denzitometrijski. Sadržaj ukupnih proteina sline određen je po Lowryevoj metodi u mg/dl sline. Za određivanje lizozima korištena je metoda po Prockopu. Razlike u pojedinim proporcijama aminokiselina, kao i povišeni ukupni proteini i povećana aktvnost lizozima, mogle bi se označiti kao faktor sklonosti, odnosno rezistencije prema karijesu

Promjene u aminokiselinama, proteinima i enzimima u djece s fenilketonurijom

Since children with phenylketonuria have a low incidence of caries, the aim of this study was to examine the changes occuring in the aminoacid and protein composition as well as in the composition of salivary enzymes, which might serve as biochemical indicators, representing the factors of predisposition to the development of caries. For this purpose, the analysis was carried out in the native, unstimulated, mixed salivary specimens taken from the sublingual region of children with late diagnosis of RKU (n — 16), those with early diagnosis of the disorder by means of a systematic diagnostic screeniig program for PKU using Guthrie’s test (n — 7), and a control group of children free of any disturbances of the amino-acid metabolism (n — 44). A method of qualitative chromatography on silica gel was used for separation of aminoacids, while the proportions of each individual aminoacid were determined by a densitometer. The content of total salivary proteins was assessed by the method of Lowry, and the concentration of lysozyme by the method of Prockop. Differences in particular proportions of aminoacids, and an increase in the total protein value as well as in the lysozyme activity, could be taken as the factors of predisposition or resistance to caries.Budući da djeca oboljela od fenilketonurije imaju malu incidenciju karijesa, željeli smo ispitati promjene u sastavu aminokiselina, proteina i fermenata, koje bi mogle — kao biokemijski indikatori — biti predispozicijski činioci za razvoj karijesa. U tu svrhu analizirana je nativna slina sublingvalnog područja u uzorcima djece s kasno otkrivenom fenilketonurijom (n — 16), djece s rano otkrivenom bolešću u sistematskom traganju za fenilketonurijom primjenom Guthrie testa (n — 7) te djece kontrolne skupine bez poremećaja u metabolizmu aminokiselina. Aminokiseliine su razdvajane metodom tankoslojne kromatografije na silika gelu, a proporcije pojedinih aminokiselina određene su denzitometrijski. Sadržaj ukupnih proteina sline određen je po Lowryevoj metodi u mg/dl sline. Za određivanje lizozima korištena je metoda po Prockopu. Razlike u pojedinim proporcijama aminokiselina, kao i povišeni ukupni proteini i povećana aktvnost lizozima, mogle bi se označiti kao faktor sklonosti, odnosno rezistencije prema karijesu

CD20 Positive Childhood B-non Hodgkin Lymphoma (B-NHL): Morphology, Immunophenotype and a Novel Treatment Approach: A Single Center Experience

Lymphomas represent the third most common group of cancers in childhood and adolescence, mature B non Hodgkin’s lymphoma (B-NHL) accounting for up to 60% of newly diagnosed patients. The diagnosis of specific entities of B-NHL is based on well-defined morphologic analysis, immunophenotyping, cytogenetics and molecular genetics, which determine the optimal treatment strategy. In adult population a major turning point in treatment of B-NHL has been achieved since rituximab, in combination with CHOP has improved the survival rate up to 19%. Rituximab is a chimeric monoclonal antibody that targets CD20, a transmembrane calcium channel expressed on normal and malignant B-cells that mediates cytotoxic, apoptotic and anti-proliferative effects. The effect of rituximab in pediatric population is still not well enough investigated. Based on morphology and immunophenotype of malignant cells, seven children with B-NHL in our institution were eligible for treatment with modified B-NHL-Berlin-Frankfurt-Münster (BFM)-95-based protocol with rituximab administered on day -5. The complete remission was achieved in all seven patients. Six patients are still in complete remission at least 12 months after having finished chemotherapy and one patient relapsed two months after the last cycle and subsequently died. Major adverse effects observed during treatment were prolonged B-cell depletion and myelosupression. Rituximab in combination with B-NHL-BFM-95 protocol was otherwise well tolerated and proved to be effective in children and adolescents with B-NHL. The number of our patients is too small and the follow-up of a larger group of patients will help in defining the role of rituximab in the treatment of childhood B-NHL

Određivanje koncentracije elektrolita u slini potenciometrijskom metodom

A method for concurrent determination o f potassium, total calcium and chloride concentrations by ion-selective electrodes is described in this paper. A sample o f whole saliva from the bottom o f the mouth cavity was diluted ten times with magnesium acetate solution (16 mol/ L), which- lead to calcium ionization. This method corresponded well with reference methods and may be used to determine electrolyte concentration in saliva due to its simplicity. Among three groups of subjects aged 7 - 12 years, the highest calcium concentrations were fo und in the group of subjects affected by dental caries. Concentration variability is highest in calcium concentration, whereas it is lower in other respective electrolytes.U članku je opisana metoda za istodobno određivanje koncentracije kalija, ukupnoga kalcija i klorida u slini ion selektivnim elektrodama. Uzorak pune sline s dna usne šupljine razrijeđen je 10 puta otopinom magnezijeva acetata (16 mmol/L), čime je kalcij preveden u ionizirani oblik, a istodobno je ionska jakost tako pripremljenih otopina držana stalnom (50 mmol/l). Opisana metoda pokazala je dobro slaganje s usporednim metodama i zbog svoje jednostavnosti može se rabiti za određivanje koncentracije elektrolita u slini. Rezultati koncentracije elektrolita dobiveni u trima skupinama ispitanika u dobi od 7 -12 godina pokazuju da su najviše koncentracije kalcija u skupini s karijesom. Varijabilnost koncentracije najveća je kod kalcija, a kod ostalih je mjerenih ionskih vrsta u slini manja

Electrolyte Concentration Change in Saliva after the Addition of Hydrogen and Magnesium Ions

Promjene koncentracije elektrolita i pH u slini imaju veliku ulogu u demineralizaciji zubne cakline. Slina je veoma važna u održavanju neutralnoga pH i koncentracije elektrolita potrebne za remineralizaciju cakline. U ovome radu ispitane su promjene koncentracije K+, Na+, Cl\u27 i Ca++ nastale padom pH vrijednosti sline te promjene koje su nastale nakon dodatka magnezijevih iona u slinu uz stalnu pH vrijedno- st. Mjerenja su izvedena potenciometrijski uporabom ionsko-selektivnih elektroda. Rezultati ispitivanja pokazali su da se neutralizacija sline nakon pada pH vrijednosti događa u razdoblju od pet minuta i to samim lučenjem sline. Koncentracija ioniziranoga kalcija u slini povećava se nakon dodatka magnezijevih iona pri pH vrijednosti oko 7.Changes in electrolyte concentration and pH in saliva have an important role in the enamel demineralization of teeth. Saliva is very important in maintaining neutral pH and concentration of electrolytes necessary to promote enamel remineralization. In this study we have investigated the changes of K+, Na+, Cl and Ca++ concentrations in saliva due to pH decrease and after the addition of magnesium ions. All measurements were obtained by potentiometry using ion selective electrode. The results of this study showed that significant pH decrease could be effectively improved in five minutes by saliva secretion only. Concentration of calcium ion increased in all subgroups supplemented by magnesium ion in the chewing gum, while the pH values remained about 7

Subcutaneous Panniculitis-like T-cell Lymphoma in a 19 Month-old Boy: A Case Report

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of T-cell lymphoma of CD3+CD8+ phenotype characterized by deep-seated skin nodules or plaques mimicking panniculitis, a result of neoplastic lymphocytes infiltrating the subcutaneous fatty tissue. We present a case of a 19-month year old boy with SPTCL diagnosed and successfully treated in our institution. Disease first presented with symptoms of high fever and painful erythematous nodule located below the umbilicus. Later on the infiltrates appeared on the face, legs, arms and the back of the body. As the most decisive in obtaining the diagnosis, skin biopsy showed atypical, small to medium-sized lymphatic cells infiltrating the deeper dermal layers as well as the subcutaneous adipous tissue surrounding the adipocytes. Imunohystochemical analysis showed neoplastic lymphocytes positive for CD2, CD3, CD5, CD7, CD8, Tia-1, granzyme B and perforine, and negative for CD20, CD34, TDT and CD56. No infiltration of blood vessels or epidermis was evident. Specific T-cell lymphomas protocol (EURO-LB 02) was then initiated which resulted with rapid regression of all general and local symptoms. The treatment was completed according to schedule and the child is now, 24 months after the initiation of the treatment, in complete remission