Role of Diagnostic Imaging in Chronic Recurrent Multifocal Osteomyelitis (CRMO) in Children: An Observational Study.

The aim of this single-center observational study was to analyze the applicability of various imaging studies to the diagnosis and further evaluation of patients with chronic recurrent multifocal osteomyelitis (CRMO). The analysis included the data of 10 patients with CRMO treated between 2016 and 2021. The mean ages of the patients at the first manifestation of CRMO and ultimate diagnosis were 10 years and 7 months and 11 years and 10 months, respectively. Conventional radiography demonstrated focal loss of bone density in only 30% of the patients. Computed tomography showed disseminated foci with non-homogeneous osteolytic/osteosclerotic structure, with a massive loss of cortical layer and strong periosteal reaction. On magnetic resonance imaging (MRI), most patients presented with multifocal hypodense areas on T1-weighted images, with the enhancement of signal on T-weighted and STIR sequences. The duration of follow-up varied between 3 months and 3 years. In 40% of the patients, both clinical symptoms and the abnormalities seen on MRI resolved completely, whereas another 50% showed partial regression of clinical and radiological manifestations. MRI findings, co-existing with characteristic clinical manifestations, play a pivotal role in establishing the ultimate diagnosis of CRMO. MRI can also be used to monitor the outcomes of treatment in CRMO patients.

Tumour of the orbit and pterygopalatine fossa: delayed recognition of possible IgG4-related disease.

INTRODUCTION: IgG4-related disease (IgG4-RD) is a systemic fibrotic-inflammatory disease characterised by elevated serum concentration of IgG4 and tissue infiltration by plasma cells. IgG4-RD is a newly recognised fibro-inflammatory condition, characterised by organ mass lesions, special histopathological appearance, and - often but not always - elevated serum IgG4 concentrations. IgG4-RD is a separate, clinically distinct disease entity, but, due to its heterogeneous manifestation, it is a subject of interest of physicians of various specialties. Histopathological examination is the gold standard in the diagnosis. CASE REPORT: In the paper we discuss the case of a 13-year-old patient who had been diagnosed with fully symptomatic IgG4-RD nine years after initial manifestation. Although IgG4-RD is diagnosed markedly more often in adults than in children, in this case report we would like to emphasise that the disease may also occur in paediatric patients, and it constitutes both a diagnostic and therapeutic challenge in this age group. CONCLUSIONS: IgG4-RD is a poorly recognised disease, especially in the paediatric population. To the best of our knowledge, case reports on IgG4-RD in paediatric patients available in the literature are sparse. The non-specific and heterogeneous manifestation may hinder the diagnostic process, and in some cases the disease is diagnosed accidentally, especially when it is asymptomatic. Since 2015, the first-line treatment in IgG4-RD has been glucocorticoids; however, combination therapies should not be underestimated as another method to achieve permanent remission.

Sonographic Image of Solitary Kidney in Wilms Tumour Survivors.

BACKGROUND/AIMS: This study presents an analysis of the sonographic and laboratory parameters of solitary kidney in Wilms tumour survivors (TWs) and compares these parameters with those of healthy individuals. METHODS: Fifty-three TWs who completed treatment for Wilms tumour and 44 healthy individuals were enrolled. The study protocol consisted of completing a medical history, sonographic examination of the solitary kidney, estimation of glomerular filtration rate (eGFR) by the Schwartz or MDRD formulas, albumin urine excretion and BP measurement. RESULTS: Sonographic signs of kidney damage were observed in 22 (41,5%) TWs. The most frequently detected abnormalities are hyperechoic rings around renal pyramids (28,3% TWs). Hypertrophy of the solitary kidney occurred in 71,7% of cases. The mean volume of the solitary kidney was 77% of the sum of the two kidney volumes in the control group. The median eGFR in the TWs group was 117 with 25Q-105,5, 75Q-130 ml/min/1,73 m2 vs 131,8 with 25Q-124, 75Q-140 ml/min/1,73 m2 in the control group (p=0,000). Six TWs (11,3%) had a value of eGFR below 90 ml/min/1,73 m2. Increased urine albumin excretion (> 30 mg/g) was observed in 7 TWs (13,2%) and in 3 (6,8%) individuals in the control group. CONCLUSION: Ultrasonographic abnormalities in solitary kidney of TWs are frequent. The most frequently detected abnormalities are hyperechoic rings around renal pyramids. Sonographic examination of TWs ought to be performed not only to detect tumour recurrence but also to assess the signs of kidney damage and their progression.

Evaluation of the frequency of childhood hospitalization due to thermal injuries in the Provincial Polyclinical Hospital in Torun, Poland, 2007-2011.

INTRODUCTION: Burns constitute the most common and severe injuries suffered in childhood. OBJECTIVE: The study was aimed at providing a retrospective analysis of the etiology, location, extent and depth of burns, as well as treatment methods and length of hospital stay. MATERIAL AND METHODS: A retrospective analysis of 508 cases of children treated due to a thermal injury between 1 January 2007 31 December 2011 was conducted at the Department of Paediatrics, Urology and Paediatric Surgery, Children's Hospital, L. Rydygier Provincial Hospital in Torun, Poland. RESULTS: The sample group included more boys (58.9%) than girls (41.1%). The most numerous group comprised children aged 1-2 years (44.5%). Burns were largely suffered at home (91.9%). Injuries were largely caused by thermal burns (99.2%). Half of the children sustained injury to one body area (51.4%), while every third sufferer (37.9%) was affected by burns to body parts prone to trigger shock. Burns up to 5% of the Total Body Surface Area (TBSA) were suffered by half (51.2%) of the children. Most of the patients underwent conservative treatment (89.4%). CONCLUSIONS: Burns were mostly suffered by children at 1-2 years of age, with boys prevailing over girls. Injuries were largely suffered at the child's family home, in the afternoon or evening, while the child was in the care of the parents. Scalds, caused by hot liquid, constituted the most frequent type of injury. The most numerous group of affected children comprised burns to limb areas, and thorax with limbs, with the TBSA of up to 5%. The great majority of the patients underwent conservative treatment, with a hospitalization period of up to 3 days.

Clinical factors in relapses of Wilms' tumor--results for the Polish Pediatric Solid Tumors Study Group.

BACKGROUND: The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. MATERIAL AND METHODS: Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. RESULTS: Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p=0.256) or gender (p=0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences (p=0.703). Those who died due to disease progression comprised six out of 26 patients with a first recurrence (four HR, two INT), and seven out of eight with a second recurrence (one HR, six INT). CONCLUSIONS: The prognosis after relapse in initially metastatic patients did not differ from that in patients who had primarily localized disease. The pathology variants probably had more significance.

Healthcare-associated infections in a neonatal intensive care unit.

INTRODUCTION: Healthcare-associated infection is a common problem in patients from neonatal intensive care units and it is one of the leading causes of death in this group of patients. Healthcare-associated infections are associated with increases in mortality, morbidity, and prolonged length of hospital stay. The aim of the study was to assess the incidence, clinical presentation, mortality and aetiology of healthcare-associated infections in newborns in a neonatal intensive care unit between 2005 and 2010. MATERIAL AND METHODS: The research involved documentation of 2610 neonates hospitalized in this period in the Neonatal Intensive Care Unit, Dr Jan Biziel University Hospital No. 2 in Bydgoszcz. The incidence, clinical presentation, mortality and causative factors of healthcare-associated infections were assessed. RESULTS: The prevalence of healthcare-associated infections was 7.32%. The most frequent healthcare-associated infections were bloodstream infection (65.4%) and urinary tract infection (22.5%). The mortality rate was 2.1%. The most frequent pathogens were coagulase-negative staphylococci (36.1%) and Klebsiella pneumoniae (29.3%). CONCLUSIONS: The rate of healthcare-associated bloodstream infections in the analysed department is low, taking into consideration the specificity of the department. There is a necessity to establish convenient definitions of various kinds of healthcare-associated infecions in neonates, especially those born preterm.

Renal function and low-molecular-weight proteins (cystatin C, ß2-microglobulin, neutrophil gelatinase-associated lipocalin) in child and young adult cancer survivors.

BACKGROUND: We sought to verify the hypothesis that children and young adults with cancer who have completed treatment differ according to the type and degree of renal damage. PROCEDURE: This study included 144 children and young adults (73 female) who had completed treatment for leukemias and lymphomas (group L, n=45), Wilms tumor (group W, n=52) and other solid tumors (group S, n=47). The following parameters were evaluated: serum concentrations of creatinine, cystatin C, ß2-microglobulin, neutrophil gelatinase-associated lipocalin and urine excretion of albumin, and urinalysis with sediment. Glomerular filtration rate (eGFR) was estimated using the classic Schwartz (eGFRSch), Schwartz redux (eGFRSchred), and Filler (eGFRFiller) formulas and with the new Schwartz equation for patients with chronic kidney disease (eGFRSchCKD). RESULTS: Group S had the lowest eGFRSchCKD and eGFRFiller, the highest serum cystatin C and the highest albumin excretion compared with groups L and W. Groups S and W had lower eGFRSch and eGFRSchred and higher serum ß2-microglobulin and neutrophil gelatinase-associated lipocalin compared with group L. Group W had lower eGFRSchCKD than group L. CONCLUSIONS: Children and young adults with cancer who have completed treatment differ in the type and degree of renal damage they sustain.

[Characteristics of extracranial malignant germ cell tumours in two age groups of children (0-10 and 10-18 years). Multicentre experiences]. / Charakterystyka pozaczaszkowych zltosliwych guzów germinalnych u dzieci w dwóch grupach wiekowych 0-10 lat i 11-18 lat. Badania wieloosrodkowe.

UNLABELLED: In order to assess if any differences exist in children germ cell tumours depending on age, we compared some features of germ cell tumours in two age groups:younger than 10 and between 11 and 18 years. MATERIAL AND METHODS: Data of 146 patients with germ cell tumours treated in 15 Polish paediatric oncology departments between 1995 and 2005 were evaluated. They were divided into two groups: 76 children 0-10 years old (group I) and 70 patients 11-18 years old (group II). Tumour morphology, sex of patients, primary tumour and metastases localization, disease stage, biochemical markers, treatment response, disease relapse and long survival were analyzed. Every patient was treated according to the TGM 95 protocol. RESULTS: In group 1, 67 tumours were assessed histologically. 64%t tumours had homogenous structure with yolk sac tumour in predominance and 36% were mixed. Yolk sac tumour (YST) or teratoma as components of mixed tumours were the most commonly found. In older group 64 tumours were examined, 41% were homogenous, and seminoma/dysgerminoma predominated. In 59% mixed tumours the most common components were YST embryonal carcinoma and teratoma. The most common primary site in group I was the sacrococcygeal region while in group II - the gonads. Disseminated disease was recognized mostly in older children. Among two evaluated serum markers, AFP was increased mostly in younger patients (76% vs 44%), and 3HCG in older group (40% vs 9%). Treatment response was comparable in both groups. Two relapses were observed in each group. Poor outcome was noted in 17/140 analyzed patients: 9 (12%) in group I and 8 (11%) in group II. In 12 of patients with poor outcome the cause of death was progression and in 5 of them - treatment complications. CONCLUSIONS: 1. Germ cell tumours in younger and older children differ in histology, primary localization and serum level of biochemical markers. 2. In older patients germ cell tumours are recognized more frequently in advanced clinical stages. 3. Treatment response was comparable in both groups. 4. There is a need to analyze the intensity of chemotherapy to precise the adequate risk groups according to primary treatment response.

Activation of NF-ĸB in leukemic cells in response to initial prednisone therapy in children with acute lymphoblastic leukaemia: relation to other prognostic factors.

Nuclear factor ĸB (NF-ĸB) is a transcription regulator of proliferation and cell death. Increased activation of NF-ĸB may be responsible for treatment failure in children with acute lymphoblastic leukaemia (ALL). This study aimed to assess changes in NF-ĸB activation in peripheral blood mononuclear cells prior to and after 6 and 12 h of prednisone administration in relation to age, initial WBC count at diagnosis and early treatment response in childhood ALL. The study comprised 55 children with de novo ALL. Cells were stained with mouse anti-NF-ĸB (p65) antibody followed by goat anti-mouse antibody conjugated with FITC and measured by laser scanning cytometer. The nuclear/cytoplasmic (N/C) ratio of NF-ĸB reflecting activation of NF-ĸB was decreased 12 h after treatment in the standard risk group patients, whereas it remained statistically unchanged in the non-standard risk group patients. Changes in the N/C ratio of NF-ĸB were not associated with age and early treatment response; however, in children with an initial WBC count higher than 20 000/µl at diagnosis, this ratio was increased after 6 and 12 h from prednisone administration. The association of higher activation of NF-ĸB with an elevated initial WBC count suggests that activation of NF-ĸB may be responsible for treatment failure in children with ALL.

Thymic carcinoma in children: a report from the Polish Pediatric Rare Tumors Study.

BACKGROUND: Invasive thymomas and thymic carcinomas are rare tumors jointly accounting between 0.2% and 1.5% of malignancies in adults. They are usually at an advanced stage when diagnosed and have both high recurrence and poor survival rates. In this report, the aim is to explore our experience in the treatment of thymic carcinomas in Polish children. PROCEDURE: The clinical data of nine children with thymic carcinomas, treated between 1992 and 2008 in the Polish oncological and surgical centers was retrospectively analyzed. RESULTS: In five cases, presenting symptoms resulted from the compression of the respiratory ways by the mediastinal tumor. In two children paraneoplastic autoimmune syndromes were associated with thymic carcinoma. In accordance with the Masaoka classification, two patients had stage II, five had stage III, and two had stage IV of the disease. Diagnostic biopsy of mediastinal tumor was performed on eight patients and one underwent complete primary resection and subsequently received radiotherapy; he has passed 11 years since the conclusion of therapy. Six patients received multi-drug chemotherapy with or without steroids. Delayed surgery was performed in four children (R0-2, R1-1, and R2-1). After complete resection, one child received chemotherapy. In three patients, chemotherapy and radiotherapy was administered. Seven patients died, including six due to progression of the disease with the other as a result of complications following chemotherapy; only two patients classed at stage II remain alive. CONCLUSIONS: Most thymic tumors in pediatric patients are inoperable at diagnosis, which results in poor prognosis. Improved chemotherapy approaches are needed.

Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.

BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. RESULTS: Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.

Health-related quality of life among paediatric survivors of primary brain tumours and acute leukaemia.

PURPOSE: Comparative assessment of the HRQL of paediatric survivors of brain tumours (BT) and of acute leukaemia against a population of their healthy peers. METHODS: The study consisted of patients who had completed treatment for BT (n=36) or acute leukaemia (n=35) and were aged between 8 and 19. Healthy children (n=60) were selected from among pupils of schools. HRQL was evaluated directly and indirectly on the basis of the Polish language version of the PedsQLTM 4.0 Generic Core scales. The influence of selected factors (sex, age, time from the end of treatment and type of treatment) on the HRQL result was analysed. RESULTS: In all the aspects analysed (total, physical, psychosocial, emotional, social and school functioning), the HRQL of BT and leukaemia survivors was significantly lower in comparison to their healthy peers. The HRQL of patients after BT treatment was also significantly lower than that of the survivors of leukaemia. The parent-proxy reported HRQL was consistent with the children's selfassessment. Patients treated with radiotherapy presented a significantly lower evaluation of life quality in the physical sphere. CONCLUSIONS: Evaluation of HRQL should be treated as an additional independent parameter in an assessment of the long-term results of oncological treatment.

Simultaneous assessment of p53 and MDM2 expression in leukemic cells in response to initial prednisone therapy in children with acute lymphoblastic leukemia.

Ineffective apoptosis is one of main causes of a treatment failure in childhood acute lymphoblastic leukemia (ALL). p53 plays a crucial role in triggering apoptosis of ALL in response to prednisone treatment. MDM2 is the endogenous inhibitor of apoptosis that downregulates the functional activity of p53 protein. This study is aimed to evaluate changes in MDM2 and p53 expression in peripheral blood mononuclear cells collected from children with ALL prior to and after 6 and 12 h of prednisone administration in relation to early treatment response. The study comprised 35 children with newly diagnosed ALL, subdivided into good (n = 24) and poor (n = 11) early treatment responders. MDM2 - associated APC fluorescence and p53 - associated FITC fluorescence were measured by the laser scanning cytometer. In the group of poor responders, p53 and MDM2 fluorescence were significantly higher than in the group of good responders. In the group of good early treatment responders, a statistically significant rise of p53 fluorescence measured in the nucleus and in the cytoplasm 12 h after prednisone administration as well as increase in MDM2 fluorescence measured in the cytoplasm 6 and 12 h after prednisone administration were seen. These data suggest that pretreatment overexpression of MDM2 protein may contribute to poor early treatment response.

[Pain in hospitalized teenagers suffering from neoplastic disease. Preliminary report]. / Ból w chorobie nowotworowej u hospitalizowanej mlodziezy--doniesienie wstepne.

INTRODUCTION: The aim of this study was to perform a quantitative and qualitative assessment of pain in hospitalised teenagers suffering from neoplastic disease. Pain is a regular finding in patients with neoplasms and is classified according to its location, intensity, and type (character). Pain is a kind of stress that triggers mechanisms of psychological coping, interferes with activities of daily living, impairs social interactions, and adversely affects the psyche. The aim of this study was to examine the clinical and psychosocial context of pain in teenagers suffering from neoplastic disease. MATERIAL AND METHODS: We examined 40 patients with neoplasms aged 12 to 20 years. We recorded the location, duration, intensity, and character of pain, the impact of pain on activities of daily living, and ways of coping with pain. The following scales were used: visual analog scale (VAS), numerical rating scale (NRS), activities of daily living scale, pain questionnaire, pain coping questionnaire. RESULTS: The results were analyzed statistically using quantitative, percentage, and rank tests. On the day of the examination, the patients reported pain in the lower limbs (27.5%), head (25%), and chest (17.5%). The intensity of pain was 5.75 on the VAS scale. Pain usually interfered with learning (4.22 on the 0-10 scale) and had a negative effect on mood (5.9 on the 0-10 scale). In describing their pain, the patients used words of the sensory (piercing, pulsating, shooting, stabbing) and emotional (tormenting, troublesome, disgusting) categories. The most common ways of coping with pain included: wishing that the pain disappeared (95% of children), telling parents about the pain (95%), asking for a drug (92.5%), and going to sleep (72.5%). The general health of the patients was established on the basis of the Karnofsky scale index which showed that 57.5% of them were in the upper and 42.5% were in middle categories of health. CONCLUSIONS: All patients experienced pain which was chiefly the side-effect of chemotherapy and the outcome of the disease. Pain interfered to a minor degree (mean 3.42) with activities of daily living. The patients did not remain passive with their pain and made efforts in the cognitive, emotional, and voluntary spheres to reduce the intensity of pain. A pain assessment and management sheet should be devised for patients treated at pediatric oncology centers.

[Results of immunosupressive therapy in children with severe aplastic anaemia. Report of the Polish Paediatric Haematology Group]. / Wyniki leczenia ciezkiej postaci anemii aplastycznej u dzieci. Raport Polskiej pediatrycznej grupy do spraw nienowotworowych chorób ukladu krwiotwórczego.

INTRODUCTION: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When there is no donor available, combined immunosuppressive therapy is given. AIM: evaluation of results of immunosupressive therapy in children with severe aplastic anaemia. MATERIAL AND METHODS: SAA was diagnosed in 105 children (42 girls, 73 boys), aged 2-18 years, in the eleven haematological centres in Poland, between 1993-2007. All patients received the Severe Aplastic Anaemia Working Party of the EBMT protocol which included: antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone. Granulocyto- or granulocytomacrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84 or 112 and 180 of the therapy. RESULTS: complete remission occurred in 53 patients (51.5%), partial remission in 27 (24.7%), no response was obtained in 25 children (23.8%) on day 180, of the therapy. Period of observation was from 12 months to 12.5 years. During this time relapse occurred in 10 patients (9.5%). We observed 22 deaths: 8 early, during the first 3 months of IS and 14 after the first 3 months of immunosuppresive therapy (IS). At present 70 children (66.6%) are in first remission with lasts from 12 months to 12.5 years. The survival at 12.5-years is 78.6%. During the 12.5 years of follow-up we had two cases with a late clonal complication (PNH and MDS). Transformation to acute nonlymphoblastic leukaemia was observed in two of our patients. CONCLUSIONS: 1. Immunosuppresive therapy (IS) in children with SAA, without bone marrow family donors, is more effective after introduction of combined IS (12.5 years survival in this study was 80% for children with very severe aplastic anaemia (v SAA). 2. In our studies among the children followed up after IS therapy, there were: 1 case of periodic nocturnal haemoglobinuria (PNH), 1 case of myelodysplastic syndrome (MDS) and 2 cases of myeloid leukaemia (probability of incidence was 3.8%).

Predictive value of multidrug resistance proteins and cellular drug resistance in childhood relapsed acute lymphoblastic leukemia.

PURPOSE: Cellular resistance in childhood acute leukemias might be related to profile and function of multidrug resistance proteins and apoptosis regulating proteins. The aims of the study were: (1) analysis of expression of MRP1, PGP1, LRP, BCL-2 and p53 proteins; (2) correlation with ex vivo drug resistance, and (3) analysis of their prognostic impact on clinical outcome in childhood acute lymphoblastic (ALL) and acute myeloid (AML) leukemia. METHODS: Total number of 787 children diagnosed for initial ALL (n = 527), relapsed ALL (n = 104), initial AML (n = 133) and relapsed AML (n = 23) were included into the study. Mean follow-up period was 3.5 years. Drug resistance for up to 30 anticancer agents was performed by the MTT assay. Expression of all proteins was tested by flow cytometry. RESULTS: Both initial AML and relapsed ALL samples showed higher drug resistance than initial ALL samples. No significant differences were found in drug resistance between initial and relapsed AML samples. The presence of multidrug resistance and apoptosis proteins had no impact on pDFS in iALL and iAML, however strong trend towards adverse prognostic impact of MRP1, PGP and LRP on pDFS in rALL was observed. The same trend was observed for each of analyzed co-expressions of tested multidrug resistance proteins. CONCLUSIONS: The phenomenon of cellular drug resistance in childhood acute leukemias is multifactorial and plays an important role in response to therapy. Expression of MRP1, PGP and LRP proteins, as well as their co-expression play possible role in childhood relapsed ALL.

[Thymoma and thymic carcinoma--review of literature and clinical characteristics based on the Polish Paediatric Solid Tumours Study Group experience]. / Grasiczaki i raki grasicy--przeglad pismiennictwa i charakterystyka kliniczna w oparciu o dane Polskiej Pediatrycznej Grupy Guzów Litych.

Thymomas and thymic carcinomas are rare neoplasms derived from the epithelial tissue of thymus, very infrequently developing in young adults and children. The estimation of thymomas' invasiveness has been the matter of discussion for many years reflected by numerous clinical and histological classifications. In 1999 the WHO classification was created, joining all the most important issues present in previously used systems. It is believed that histological structure is the most important prognostic factor in thymic carcinomas while in less aggressive types of thymomas the clinical stages influence the outcome. Staging of thymomas is most commonly based on the Masoka classification. Independent evaluation of the stage and histological aggressiveness are necessary to predict the clinical course and outcome in thymomas. Thus the term 'malignant thymoma' has been replaced by 'invasive thymoma' in clinical practice. The treatment strategy depends on the clinical stages of thymoma. Complete resection of the tumour is the treatment of choice with supplementing radiotherapy in more advanced clinical stages. Chemotherapy in invasive thymomas has been reported to play an increasingly important role as induction, supplementing and palliative therapy. It has been proved that combined treatment improves the outcome in invasive thymomas, especially in thymic carcinomas. This paper reviews the literature data concerning the histology, clinical issues and treatment of thymomas and thymic carcinomas. The clinical data on nine children with thymic carcinomas treated between 1992 and 2006 in the Polish oncological and surgical centres were also analysed and presented. Based on multicentre data we were able to conclude the following: 1. Thymic carcinomas in children are very rare and that is why early diagnosis is often difficult. 2. At diagnosis most cases are already inoperable, which results in poorer prognosis. 3. Complex adjuvant chemo- and radiotherapy in childhood thymic carcinomas seem to prolong overall survival. 4. Further detailed analysis in all the cases of thymic carcinomas in children is recommended in order to estimate the optimal strategy of treatment.

The role of radioisotopic methods in imaging of intervertebral disc inflammation in children.

The authors present the case of a 17-year-old girl, with pain over lumbar spine area, treated by paediatricians and rehabilitation specialists, discussing diagnostic imaging and laboratory examinations together with clinical observations. Spondylodiscitis was diagnosed after bone scintigraphy with 99mTc-MDP, the course of disease was monitored by immunoscintigraphy amongst other techniques.

[Imatinib mesylate in treatment of childhood chronic myeloid leukaemia. Preliminary report]. / Zastosowanie imatinibu w terapii przewleklej bialaczki szpikowej u dzieci. Doniesienie wstepne.

UNLABELLED: THE AIM of this preliminary study was to estimate the efficacy and adverse events of imatinib, a selective tyrosine kinase inhibitor, in children with chronic myeloid leukaemia. MATERIAL AND METHODS: Six children aged 7-12 with follow-up from 5.5 to 25.5 months were enrolled into the study. Imatinib was administered in daily dose 340 mg/m(2) (maximum 400 mg) and was continued for a period from 2.5 to 14.5 months until haematopoietic stem cell transplantation. RESULTS: Complete haematological remission was achieved in 5 out of 6 patients. Complete and major cytogenetic remission was achieved in 3 and 2 children, respectively. Molecular remission was achieved in 3 children with complete cytogenetic remission. One child with chronic myeloid leukaemia diagnosed at blast crisis demonstrated resistance to imatinib therapy. During imatinib therapy myalgia, ostealgia and mild myelosuppression were observed. CONCLUSIONS: Imatinib seems to be an efficient and well tolerated drug in children with chronic myeloid leukaemia. However, its use in advanced leukaemia may be ineffective. Therefore, further research on the efficacy of imatinib should be carried out as well as the most effective dosage needs to he worked out.

[Results of immunosuppressive therapy in children with severe aplastic anaemia. Report by the Polish Paediatric Leukaemia and Lymphoma Study Group]. / Efekty leczenia immunosupresyjnego ciezkiej postaci anemii aplastycznej u dzieci. Raport polskiej pediatrycznej grupy ds. leczenia bialaczek i chloniaków.

INTRODUCTION: Bone marrow transplantation from HLA identical family donors is the treatment of choice for children with severe aplastic anaemia (SAA). When no donor is available, combined immunosuppressive therapy is given. AIM: Evaluation of results of immunosuppressive therapy in children with severe aplastic anaemia. MATERIAL AND METHODS: SAA was diagnosed in 85 children (31 girls, 54 boys) aged 2-17.5 years in the eleven centres of the Polish Paediatric Leukaemia and Lymphoma Study Group (PPLLSG) in Poland between 1993-2003 years. All patients received protocol of the Severe Aplastic Anaemia Working Party of the Europe Bone Marrow Transplant (EBMT): antilymphocyte globulin or antithymocyte globulin, cyclosporin A, prednisolone and granulocyto- or granulocyto-macrophagic-cell stimulation factor was additionally administered during deep neutropenia. Haematological response was evaluated on day 84, 112 or 180 of the therapy. RESULTS: complete remission occurred in 43 patients (50.5%), partial remission in 22 (25.4%), no response was obtained in 20 children (23.7%) in 180 day of the therapy. Period of observation was from 12 months to 10.5 years. During this time relapse occurred in 6 patients (7%). We observed 16 deaths: 7 early during the first 3 months of immunosuppressive therapy (IS) and 9 after the first 3 months of IS. CONCLUSION: the actual survival at 10-years, after immunosuppressive therapy is 81.2% in our group. Transformation to leukaemia or myelodysplastic syndrome (MDS) was not observed in any of our patients. We observed one case with paroxysmal nocturnal haemoglobinuria (PNH).