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Ankylosing spondylitis (AS) is a chronic and progressive systemic disease characterized by the occurrence of inflammation and later ossification in the sacroiliac joints, spine joints, peripheral joints and tendons. It causes pain, swelling, limitation of mobility and partial or complete stiffness of the spine joints. AS is the second most common inflammatory disease of the joints right after rheumatoid arthritis. In most patients, the first symptoms appear between 15 and 40 years of life. In 90% of patients, the presence of HLA-B27 antigen is found. However, not everyone with this antigen will be affected by AS. This disease is 2-3 times more likely to affect men than women.

Background of Inflammatory diseases of the intestines affect more and more people, this is caused not only by genetic determinants but also by environmental conditions and lifestyle. Lack of physical activity may affect the symptoms of the disease and increase their intensity, therefore during the remission of the disease it is recommended to use training and physiotherapeutic techniques to reduce the symptoms, such as pain, fatigue or reduced quality of life. The below systematic review shows the impact of individual physical trainings and physiotherapeutic techniques on symptoms resulting from inflammatory bowel diseases.

Type 2 diabetes belongs to the group of civilization diseases of the 21st century. In Poland, over 3 million people suffer from diabetes, including 1 million being undiagnosed. In the initial stage, this disease does not hinder functioning in everyday life, often people get to know about being affected by this disease during other diagnostic tests related to another illness. As diabetes develops, there may be numerous complications, including: retinopathy, nephropathy, neuropathy, cerebrovascular disease, ischemic heart disease, peripheral artery disease. Untreated type 2 diabetes can lead to death, so it is very important to start treatment quickly. The aim of therapy is to balance carbohydrate and lipid metabolism, achieve optimal blood pressure, proper body weight, and prevent complications. This can be achieved by combining a properly selected diet, physical activity and pharmacotherapy.

Although in the age of media writing is experiencing a kind of renaissance, and young people cannot imagine everyday life without texts, emails, online discussion forums or virtual instant messaging, writing as part of institutionalized teaching evokes rather negative associations among many learners. Therefore, based on the example of the Polish-German online tandem-tridem project, an attempt will be made in this article to answer the following questions: How can one draw on the positive, out-of-school experiences of learners with (foreign) writing in foreign language classes? How can they be used in the institutionalized process of teaching and learning foreign languages while developing writing skills? What advantages and dangers are associated with the mentioned activities. [ABSTRACT FROM AUTHOR]

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Nephropathy is one of the major vascular complications present in adults with diabetes mellitus type 1 (DM1) and is the most common cause of chronic kidney disease in adults. Currently, in everyday medical practice, microalbminuria is used as a marker to identify individuals at risk of nephropathy, however it only occasionally may be observed during the first 5 years from the diagnosis of DM1. Despite of intensive research in the field of diabetic nephropathy development, its pathomechanism is still not fully understood. In the presented work we describe the correlation between N-acetyl-β-D-glucosaminidase, cistatin C and the development of microalbuminuria. Also, we attempted to explain therelations between the degree of metabolic compliance (HbA1c), the level of vascular endothelial growth factor (VEGF), tumor necrosis factor (TNF-β), interleukin 6, 12, 10 (IL-6, IL-12, IL-10) and the development of diabetic nephropathy in children. Among the analyzed immunologic markers, those prognostic for occurrence of microalbuminuria and for development of pathologic changes in kidneys in the course of DM1 in children were pointed out. Plausibly, identification of early markers of the developing diabetic nephropathy might contribute to better understanding of its pathogenesis and also to research of novel therapeutic options aimed at improvement of quality of life of DM1 patients by means of slowdown or even avoidance of the development of late vascular complications. [ABSTRACT FROM AUTHOR]

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Copyright of Pediatric Endocrinology, Diabetes & Metabolism is the property of Termedia Publishing House and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The authors present a case of 14-years old boy with purpura vera who developed cough and dyspnoea after 3 months of hydroxyurea therapy. His treatment for asthma bronchiale with nebulised ?-2 mimetic, steroide was unsuccessful. Chest HRCT scans, DLCO and spirometry results were normal. Perfusion scintigraphy showed extensive perfusion defects. High level of D-dimer (>2000 mg/ml) confirmed pulmonary embolism. Anticoagulant therapy with heparin and fibrinolytic agent (alteplase) was used with success. The patient was also shown to be deficient in protein S. The demonstrated case proves that pulmonary embolism should be considered during diagnosing as a discriminating factor in addition to such pathological respiratory symptoms as dyspnoea and cough, especially in patients with high-risk factors (hypercoagulable state, venous thrombosis, central venous catheters). [ABSTRACT FROM AUTHOR]

Vein thrombosis is a rare disorder diagnosed in childhood. The principal causes of this illness are inborn thrombophilias, essential thrombocytosis, neoplasms, autoimmunologic diseases and pathologic reaction to certain drugs. Budd-Chiari syndrome, a hepatic vein occlusion is an atypical manifestation of vein thrombosis. Due to the abundant symptomatology, interdisciplinary diagnostic procedures and serious prognosis the Budd-Chiari syndrome is a challenge for contemporary medicine. We present a 16 years old girl with essential thrombocytosis, the complicated by hepatic vein thrombosis.

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.

The presented programme "Health for Pomeranians" is based on a number of legal acts and the agreement between the authorities of the Region and the Medical University of Gdansk. The main objective of its authors was to improve the three-stage medical care of mother and child. Particular emphasis is placed on improving the preventive care of the pregnant woman and infant, and child who is in the developmental age, presenting the schedule and the range of recommended tests in relation to the patient's age. After an analysis of epidemiological data, the current ambulatory care and number of beds for patients in the Pomeranian region, the needs for the years 2009-2013 according to professional medical, instrumentation and the proposed changes in the diagnostic-therapeutic profile of outpatient clinic and hospitals were presented. In terms of outpatient care as the most important change was the proposal to create a pediatric clinic for consultation within the province, while in the hospital use the expansion of highly specialized care, particularly intensive care unit and rehabilitation.

Approximately 60 children aged 0-18 years are diagnosed of NBL each year in Poland. About 60% of all patients suffering from NBL have a chance for durable cure. Unfortunately the prognosis for patients within the high-risk group accounting for more than 50% of all NBL patients remains poor despite the introduction of more intensive chemotherapy regimens with radical surgery procedures and megachemotherapy with subsequent stem cell transplantation. Only one third of patients in this group can be cured. To improve the treatment results of the high-risk patient group and to decrease the rate of therapy related side effects current European treatment protocols have been introduced systematically in Poland. In February 2009 information about 389 patients (age 0.1-16.5 years) diagnosed between 2001 and 2008 were obtained. Results of therapy of 319 patients who started treatment from 2001 to 2007 were analyzed. Between 104 infants and 215 children over 1 year of age, stage 4 of disease was found in 25% and 54.5%, respectively. In this period additionally to European treatment protocols, two another protocols were used. Satisfactory treatment results were obtained in 104 infants (5-year event free survival /EFS/=82.6%), irrespective of the type of treatment protocol. Over 5-year EFS for children over 1 year of age in 1, 2 and 3 stage of disease was: 100%, 86.3% and 64.5%, respectively. On the contrary, 107 patients with 4 stage of disease achieved the 5-year EFS of 27% only. Treatment results obtained in patients treated according to the European HR-NBL-1/ESIOP protocol were better than for patients treated according to other treatment protocols (5-year EFS: 31.1% and 16.4%, respectively), but difference between these groups was not significant. Between 2001 and 2007 data reporting increased to 81% from 19% noted earlier. Unfortunately, results of treatment for children over 1 year of age remain still unsatisfactory. That is why there is a need of improvement of modern, unified treatment realization as well as better data reporting. For realization of these aims adequate financial support is essential.

Four consecutive intensive unified regimens (BFM-AML-83, PGP-AML 94, PGP-AML 98 AML-BFM 2004 Interim) for acute myelocytic leukemia (AML) have been conducted by the Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) since 1983. The last one, introduced four years ago is still active, and only preliminary result may be presented. There were 726 children with AML diagnosed (226, 102, 247 and 151 in the I, II , III and IV periods, respectively), and 603 of them were eligible for evaluation (208, 83, 195 and 117, respectively). Complete remission rates were: 71.4%, 67.5%, 81.4% and 87% in consecutive periods, respectively. Five-year overall survival (OS) and event-free survival (EFS) rates were: 33% and 32% for PGP-AML 83 regimen, 38% and 36% for PGP-AML 94 regimen, and 53% and 46% for PGP-AML 98 regimen, respectively. For AML-BFM Interim 2004 the 3-year OS and EFS were 57% and 57%, respectively. Despite continuous improvement of the treatment results, the number of failures have remained too high, but the pattern have changed in the following way: Early deaths (from diagnosis to 15 day of treatment) decreased only in the fourth period to 3%. "Aplasia deaths" (between day 15 and 42) decreased gradually from 16% in the first period to 1.5% and 2.2% in the third and in the fourth period, respectively. Deaths in remission decreased from 10% in first and second period to 3.5% at present. Number of non responders increased between first and second period from 6% to 18%, later decreased to 8.2% at present. These trends e.g. decrease of early death and treatment related mortality reflect both the better efficacy of antileukemic treatment and the improvement of supportive care.

Currently over 90% of children and adolescents with Hodgkin's disease (HD) can be cured thanks to use of multidrug chemotherapy (CT) combined with involved-field radiotherapy (IF-RT). However, the intensive treatment may increase the risk of late complications which may impair the patients' quality of life. In order to decrease the incidence of late complications the protocol with limited use of IF-RT was introduced in centers of Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG). This study presents the treatment results of patients treated with CT only in comparison with the therapy results of children treated with CT and IF-RT. From 1997 to 2006, 634 children (age: 2-22,5 years) with HD were treated in 14 oncological centers of PPLLSG. Majority of patients received CT (3-8 cycles of MVPP/B-DOPA) combined with IF-RT. In 45 patients with IA-IIA stages presenting favorable risk factors (small mediastinal tumor, peripheral nodular mass of a maximum diameter < 6 cm, involvement of less than three nodular regions, ESR < 50 mm after 1 h, histologic type other than lymphocyte depletion and very good treatment response assessed after 3 CT cycles) IF-RT was omitted. Among 634 children first complete remission (RC) was not achieved in 2.4% of patients. Relapses occurred in 24 children (3.9%). The rates of 5-year overall survival (OS), relapse-free survival (RFS) and event-free survival (EFS) were 97%, 96% i 92%, respectively. All patients treated with CT only remain in first CR. All serious late complications (including 7 second neoplasms) occurred in patients treated with CT combined with RT. Seven children died because of severe complications, among them two in first CR (aplastic anemia, sepsis). Our results show that the use of CT only in precisely selected group of patients with HD do not impair the treatment results and may decrease the risk of late life threatening complications. Treatment response assessment with the use of PET may in future increase the number of patients treated without RT and limit the need of the use of invasive diagnostic methods in patients with residual mass.

Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy. Due to new therapeutic schedules and cooperation between oncological centers it is curable in more than 80% of affected children. For the optimalization of the therapy there is necessary to assess the risk criteria that have influence on the treatment results in the certain groups of patients. Hyperleukocytosis and the age (less than 1 year and more than 10 years) are known as unfavorable risk factors. The study was designed to assess the long-term treatment results in children with ALL and the initial leukocytosis over 50 000/mm3 with the use of the modified "New York" protocols. We present the treatment results of 340 children with ALL treated in nine centers of Polish Pediatric Leukemia/Lymphoma Study Group (PPLLSG) according to three consecutive versions of modified "New York" protocol (group I, II, and III) between 1987 and 2003. Within the analyzed groups the first complete remission (I CR) was achieved in 91%, 95% and 96% of the patients, respectively. Relapses occurred in 37%, 21.5% and 26% of the patients and 3.7%, 1.8% and 5.7% of children died in the I CR due to complications, in the I, II and III therapeutic group, respectively. Obtained 5-and 10-year event-free survival (EFS) were 56% and 53.5% for the group I and 73% and 73% for the group II. Five-year EFS for the group III was 67%. The implementation of the New York protocol in 1987 and New York I in 1997 has improved the treatment results in children with ALL and initial leukocytosis over 50 000/mm3. Protocol New York II did not further improve the treatment results. Among analyzed parameters (age, gender, the initial leukocytosis, the blast cells immunophenotype) only age had the statistical significance. The implementation of modified "New York" protocols has improved the treatment results in children with ALL and initial leukocytosis over 50 000/mm3 compared to previous results.

Introduction: The natural history of type 1 diabetes is concerned with the appearance of autoantibodies against antigens of pancreatic beta cells. The last decade revealed some evidence of the participation of T regulatory lymphocytes - cells which suppress immune response - in the pathogenesis of type 1 diabetes and prediabetes., Aim of the Study: was the assessment of T regulatory cells in the blood of children at risk for developing type 1 the diabetes mellitus., Material and Methods: 85 subjects, siblings of children with type 1 diabetes, were enrolled into the study. The presence of anti-GAD65 antibodies was assessed. With the use of flow cytometry the following cell subpopulations were noted: CD4+, CD4+CD25high and CD4+CD25highCD127low with the coexpression of: CD28, CD45RO, CD54, CD62L and CD134 molecules., Results: We did not observe any differences in white blood cell count, lymphocyte (including CD4+) count and the percentage between the examined and control groups. We noted higher percentages of T regulatory cells: CD4+CD25high, CD4+CD127low and CD4+CD25highCD127low in children with the presence of anti-GAD65 antibodies as compared to the control children., Conclusion: Higher percentages of T regulatory cells in the blood of children with the presence of anti-GAD65 antibodies may suggest an intensive regulatory response present in patients at risk for developing type 1 diabetes.

The aim of the paper is to present the initial results of molecular examination which was started in 2006 for children with acute myeloid leukemia. Better knowledge of biology of this disease, can result in establishing of new risk factors what allows more precise patient stratification to different therapeutic groups. Study was obtained patients until to 18 years of age treated according to AML-BFM 2004 INTERIM protocol in 14 centers of the Polish Pediatric Leukemia/Lymphoma Study Group. Mononuclear cells were collected from bone marrow on time points established according to the AML-BFM 2004 INTERIM protocol. Collected cells were isolated on Ficoll gradient, and RNA and DNA were isolated using TRIZOL reagent. To synthesize cDNA an amount of 1 mg of total RNA was used. To perform quantitative RT-PCR and RQ-PCR reactions 4 fusion gene transcripts (AML1-ETO, CBFb-MYH11, PML-RARA /subtype bcrl and bcr3/) were used according to the protocol established by Europe Against Cancer Program. An expression of WT1 gene was tested additionally. An analysis of ABL control gene was used to normalize of achieved results. Determination of duplication of FLT3 gene in DNA sample was performed with starters complementary to JM region. Genotyping was performed in 75 patients with acute myeloid leukemia so far. AML1-ETO fusion gene transcript was found in 14 patients (19%). PML-RARA (subtype bcr3) and CBFB-MYH11 gene transcripts were detected in 3 (4%) and 3 (4%) patients, respectively. Duplication of FLT3 gene was found in 4 (5.3%) cases. Between 67 tested children over expression of WT1 was present in 51 patients (76%). Analysis of MRD level in subsequent time points showed systematic decrease of number of fusion gene transcript copies and gene WT1 expression. To establish the rate of molecular marker presence in AML in children and the influence of the presence of MRD on the treatment results as well, the study has to be conducted on a larger group of patients with longer follow-up.

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%).

Unlabelled: The occurrence of a second tumour is a severe complication of neoplastic disease and its treatment, and it reduces the patient's chances to survive. The aim of the study was to assess the frequency of a second neoplasm and its clinical course in children treated in Gdańsk in the years 1992-2007., Patients and Methods: There were 420 children and young adults included in the study. They were treated for malignant tumours in this period in the Department of Paediatrics, Haematology, Oncology and Endocrinology Medical Academy of Gdańsk. The medical records of these patients were analysed., Results: The second neoplasm was diagnosed in 9 patients, aged 9 to 23 years. They were treated for nephroblastoma - 3 cases, soft tissue sarcoma - 2, Ewing's sarcoma - 1, medulloblastoma - 1, retinoblastoma - 1 and neuroblastoma - 1 case. The second neoplasms were: acute non lymphoblastic leukaemia - 2, soft tissue sarcoma - 2, osteosarcoma - 2, chondrosarcoma - 1, renal cell carcinoma - 1 and glioblastoma multiforme - 1 case. Time between the first and second diagnosis was from 3 and 11/12 to 19 years. Treatment failed in 5 out of 9 children treated for osteosarcoma (2/2), chondrosarcoma (1/1), soft tissue sarcoma (1/2) and acute non lymphoblastic leukaemia (1/2). These patients died of progression of neoplastic disease during 2 to 20 months after the diagnosis of the second tumour., Conclusions: The diagnosis of the second tumour worsens the prognosis. It is difficult to define the factors that predispose to the second neoplasm. In 5 cases the second neoplasm occurred in the region which was previously irradiated.

The role of angiogenesis in the growth of neoplasms and the factors engaged in the regulation of this process are discussed. In this paper the knowledge concerning the role of angiogenesis in the diagnosis, prognosis and treatment of malignancies is presented. The types of antiangiogenic agents used at present in oncological treatment, the first clinical experiences and the results of preclinical and clinical studies are presented. Special attention is paid to the new form of chemotherapy used in metronomic schedules and aimed at the inhibition of pathological formation of blood vessels in malignant tissues. The first clinical studies on metronomic chemotherapy in children, as well as the trials of combined antiangiogenic therapy and metronomic chemotherapy are presented.

Unlabelled: The aim of the study was to analyze the prognostic factors for the outcome in childhood head/neck soft tissue sarcomas (STS) treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk between 1992 and 2007., Material and Methods: Among 23 children with STS in ten it was located in non-parameningeal region, in eight it was in the parameningeal region, and in five in the orbit. Patients were qualified to particular disease stages according to the current diagnostic and therapeutic protocols (Cooperative Weichtel-sarkom Studie-CWS). The qualifications were based on the results of radiologic examinations (ultrasonography, computed tomography and magnetic resonance of the head and neck region, computed tomography of the chest and abdomen and bone scintigraphy) and also on myelogram smears and cerebrospinal fluid investigation., Results: The longest history concerned the parameningeal (8 months), the shortest the orbital soft tissue sarcomas. Six out of ten patients with non-parameningeal STS were referred to the oncologist with a delay of 6.5 months. This was due to the initially false histopathological diagnosis which excluded the neoplastic process. 82.6% of tumours were diagnosed in advanced, inoperable stages. In 16 children rhabdomyosarcomas (including embryonal subtype - RME in 10, alveolar - RMA in five and undifferentiated in one); in seven non-rhabdomyosarcomas (non-RMS) were found. Good response to chemo- and radiotherapy was observed in 60% of children, mainly with RME. Nine children (mainly with non-parameningeal STS) relapsed. 15 patients are alive (including all with orbital, 6/10 with non-parameningeal and 4/8 with parameningeal STS). Eight children died of disease progression., Conclusions: 1. Poor outcome in our patients with non-parameningeal head/neck STS results from false initial diagnosis cansing a delay in referring them to the oncologist. It is essential to give more training to the histopathologists about neoplasms in children. 2. Because complete tumour resection in parameningeal STS is rarely feasible, the prognosis in this group is uncertain despite intense chemo- and radiotherapy. 3. The prognosis in orbital STS is usually good; however, they need mutilating surgery in selected cases not responding to therapy.

Chronic neutropenia is a decrease in circulating neutrophils in the peripheral blood lasting over 6 months. Values need to be refered with the age and race. In children aged 2 weeks to 12 months reffered values are above 1000/03BCL. There are congenital and aquired reasons of neutropenia in infancy. The most common type of chronic neutropenia in infants is chronic, benign neutropenia (AIN). Authors present ten infants between three and six months with chronic, benign neutropenia. The reason of ordering laboratory tests at outpatient clinic were benign upper respiratory tract infections (four cases), pallor (four cases) and on parental demand (one case). In one infant neutropenia was observed during treatment of pneumonia at a district hospital.

Unlabelled: Neurofibromatosis type I (NF1) is one of the most common genetic disorders in man, predisposing to benign and malignant tumours. The most common malignancies comprise nervous system tumours, less frequently soft tissue sarcomas (STS) and leukaemia - myelodysplasia syndrome. Herein we report five cases of STS diagnosed in children affected with NF1 (3 girls and 2 boys, age: 8 months -17 years). Neurogenic tumours were diagnosed in three children (malignant peripheral nerve-sheath tumour in two and malignant triton tumour in one), while soft tissue sarcomas of rhabdomyosarcoma origin were found in two patients. In four cases the primary tumours were highly locally advanced, unresectable and located in pelvis minor. All patients received protocols for soft STS: CWS-91, 96 and 2002. The paper presents the clinical symptomatology, the course and outcome in children with NF1 affected with STS., Conclusion: basing on our own observations STS in NF1 seems to have poor prognosis in spite of combined therapy. Since early diagnosis is essential, children with NF1 should remain under the care of the oncologist.

The Aim of the Study: was to evaluate age and height at the diagnosis of childhood growth hormone deficiency in the group of children who were qualified to growth hormone (GH) treatment in pediatric endocrinology department at Medical University of Gdańsk in period 1994-2004., Material and Methods: The analysis comprised 170 GH-deficient children (111 boys) in whom GH treatment was indicated in our department in the period 1995-2004 yr., Results: At diagnosis the mean age was 11,1+/-3,2 yr., mean height -3,6+/-1,0 SDS. Mean age in males was significantly higher, than in females, but there was no statistically significant difference between them. 25% of patients were pubertal. 44% of children lived in rural area, 56% in urban area and the age of diagnosis was similar in both groups. Children from rural area were, however, significantly shorter than those from urban area. 25.3% of patients started with GH treatment in the period 1995-2000 yr. and 74.7% after year 2000. Children who started with GH treatment before year 2000 were younger (9,6+/-3,6 yr.) than those after year 2000 (11.6+/-2.9 yr.). They were also significantly shorter (-4.2+/-1.1 SDS) than patients diagnosed after year 2000 (-3.4+/-0.9 SDS). Height in 67% of patients diagnosed before year 2000 and 36% diagnosed after year 2000 was below -3.5 SDS., Conclusions: Children with GH deficiency were referred to our department relatively late and the age of the diagnosis did not decrease during the period 1995-2004. The deep height deficiency observed in children at the diagnosis of GH deficiency decreased after year 2000 but it is still a problem in patients from rural area. General practitioners should earlier identify children with height deficiency and refer them to pediatric endocrinologists.

Unlabelled: Between the years 2002 and 2006 in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, six patients with neuroblastoma were qualified for the high risk group. Aim of the study was the evaluation of treatment results after using new methods of therapy., Material and Methods: Intensive multimodal therapy according to the new European protocol HR-NBL-1/ESIOP, was implemented in 6 children. In two cases the high risk group qualification was delayed. Mean time of observation was 2 years 4 months., Results: After the end of therapy, three of six children remain in complete remission. The histopathology of the tumour was assessed according to Shimada classification. Number of NMYC copies was estimated by the FISH method. One patient died of infection after high dose chemotherapy. Relapses occurred in two patients. One of them died of progression and the other is alive, in the third relapse, with progression of disease., Conclusions: The preliminary analysis shows good results of the new protocol in half of the patients. Further additional cooperative studies with bigger groups of patients are required.