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Background: Studies have shown the risk factors for COVID-19 severity in children, including comorbidities, but information on the infection course in children with life-limiting conditions is sparse., Aim: To describe the effect of COVID-19 on pediatric patients receiving palliative care due to life-limiting conditions., Design: We conducted retrospective cohort study. The WHO Clinical Progression Scale was used to measure COVID-19 severity., Setting/participants: Seven of the 24 invited pediatric palliative care centers participated in this study. We analyzed the medical records of children under palliative care with confirmed COVID-19 (January 2020-April 2022)., Results: Records of 60 patients with COVID-19 aged 0.24 to 21.6 years (mean (SD); 9.8 (6.6)) were collected. The largest group of patients with COVID-19 was children with congenital malformations and chromosomal abnormalities (42%); the most common manifestation was fever (85%). Bacterial coinfection was confirmed in 17 (28%) children. Fifteen (25%) children required hospitalization, including four admitted to the Intensive Care Unit. Mild COVID-19 was identified in 44 (73%) children, moderate in 11 (18%), severe in 3 (5%), and death in 2 (3%). Six of the 20 eligible children were vaccinated against SARS-CoV-2, followed by 16 mothers and fathers., Conclusion: In the study population initial presentation of COVID-19 was predominantly a mild; however, the small sample size precluded definitive conclusions. For children under palliative care, we should identify if they have an advance care plan for COVID-19, such as desires for intensive care support. Further studies are needed to define the short and long-term effects of COVID-19 in children with life-limiting conditions., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. Data sharingThe data and analytical materials related to this study were maintained and managed according to organizational guidelines and ethical regulations. This information will not be made publicly available in the interest of patient confidentiality and anonymity. Requests for further information were directed to the corresponding author.

Introduction: Wady letalne prowadzą do wewnątrzmacicznego zgonu płodu lub dziecka bezpośrednio po urodzeniu lub we wczesnym okresie niemowlęcym, bez względu na zastosowane leczenie. W przypadku wad letalnych nie ma możliwości skutecznej pomocy dziecku, mimo postępu mi zeadsytcoysnoyw ania najnowocześniejszej aparatury lub terapii. Rodzice, którzy decydują się na urodzenie dziecka z wadą letalną mogą być objęci perinatalną opieką hospicyjną, która ma charakter kompleksowy. Polega ona na wsparciu ciężarnej w okresie przygotowania do porodu, w czasie porodu i po porodzie oraz na wsparciu jej rodziny oraz obejmuje udzielenie rodzicom pełnej informacji o chorobie ich dziecka. Opieka nad dzieckiem po urodzeniu jest nastawiona na ochronę przed uporczywą terapią i zapewnienie dziecku opieki paliatywnej., Cel: Wykazanie znaczenia perinatalnej opieki paliatywnej dla kobiet w ciąży, u których wyniki badań prenatalnych wskazywały na ciężkie zaburzenie rozwojowe u płodu o potencjalnie letalnym rokowaniu oraz przedstawienie schematu postępowania według modelu wewnątrzszpitalnego hospicjum perinatalnego., Materiał I Metody: Analizą retrospektywną objęto dokumentację 67 pacjentek skierowanych do Programu RAZEM we Wrocławiu w latach 2014-2018 z powodu nieprawidłowych wyników badań prenatalnych (ultrasonograficznych lub/i genetycznych), które wskazywały na ciężkie zaburzenie rozwojowe u płodu o potencjalnie letalnym rokowaniu. Dokonanoanalizy danych socjodemograficznych, danych klinicznych rozpoznania choroby u płodu, przebiegu ciąży i porodu, trybu postępowania w okresie prenatalnym, podczas porodu i po urodzeniu się dziecka., Wyniki: Do Programu zostało skierowanych 67 kobiet w wieku 20-43 lat (średnio 31,2), które zgłaszały się w okresie od 15 do 39 tygodnia ciąży (średnio w 25. tygodniu ciąży). Do opieki paliatywnej zakwalifikowano 57 kobiet, czyli 85% skierowanych do programu. Opiekę paliatywną kontynuowano u 51 pacjentek, ponieważ 6 kobiet w trakcie procesu diagnostycznego zdecydowało się na zakończenie ciąży (10,5%). Najczęstszymi zaburzeniami u płodów były aberracje chromosomowe, wady OUN i wady nerek. W 95% przypadków doszło do obumarcia wewnątrzmacicznego płodu lub śmierci noworodka., Wnioski: Perinatalna opieka paliatywna jest niezbędną formą opieki dla kobiet w ciąży, u których wyniki badań prenatalnych wskazują na ciężkie zaburzenie rozwojowe u płodu o potencjalnie letalnym rokowaniu. Model wewnątrzszpitalny hospicjum perinatalnego jest korzystną formą opieki, zapewnia jej spójność i dobrą komunikację w zespole, co wpływa na dobrą jakość opieki., Introduction: Lethal defects lead to the intrauterine death of the fetus or the passing away of the child immediately after birth or in early infancy, regardless of the treatment used. In the case of lethal defects, it is not possible to effectively help the child, despite using the most modern equipment or medicines in the treatment or the progress made by medicine. Parents, who decide to continue the pregnancy, although the fetus has a lethal defect that cannot be cured, may be covered by perinatal hospice care, which is comprehensive and consists in supporting the pregnant woman during the prenatal time, during delivery and after delivery and support of her family, giving full information to the parents about their child's illness. Childcare after birth is focused on protecting the infant from persistent therapy and providing him with appropriate conditions., Aim: To demonstrate the role of perinatal palliative care for pregnant women in whom the results of prenatal tests pointed to a severe developmental disorder in the fetus with a potentially lethal prognosis, and to present a pattern of behavior for their hospitalization in the perinatal hospice., Materials and Methods: The retrospective analysis included documentation of 67 patients referred to the RAZEM (TOGETHER) Program in Wrocław in 2014-2018 due to abnormal results of (ultrasound and / or genetic) prenatal tests, which indicated a serious developmental disorder in the fetus with potentially lethal prognosis. Analysis was conducted of sociodemographic data, clinical data on fetal diagnosis, pregnancy and delivery, the procedure for prenatal delivery and postnatal birth., Results: 67 women aged 20-43 years (mean 31.2) were referred to the RAZEM Program. Out of these, 57 women were enrolled for palliative care, which accounted for 85% of those referred to the program. Palliative care was continued in 51 patients, because 6 women decided to terminate their pregnancy during the diagnostic process (10.5%). The most common abnormalities in the fetuses were chromosomal aberrations, CNS defects and kidney defects. In 95% of the cases, intrauterine fetal death or neonatal death occurred., Conclusions: Perinatal palliative care is an indispensable form of care for pregnant women in whom the results of prenatal tests indicate a serious developmental disorder in the fetus with potentially lethal prognosis. The in-hospital model of a perinatal hospice is a beneficial form of care, as it ensures consistency and good communication in the team, which favourably affects its quality., (© 2019 Agnieszka Jalowska, Joanna Krzeszowiak, Agnieszka Stembalska, Krzysztof Szmyd, Mariusz Zimmer, Gizela Jagielska, Małgorzata Raś, Agnieszka Pasławska, Agnieszka Szafrańska, Dorota Paluszyńska, Tomasz Fuchs, Karolina Pesz, Maria Sąsiadek, Barbara Królak-Olejnik, Robert Śmigiel, published by Sciendo.)

Genetic disorders are the leading cause of infant morbidity and mortality. Due to the large number of genetic diseases, molecular and phenotype heterogeneity and often severe course, these diseases remain undiagnosed. In infants with a suspected acute monogenic disease, rapid whole-exome sequencing (R-WES) can be successfully performed. R-WES (singletons) was performed in 18 unrelated infants with a severe and/or progressing disease with the suspicion of genetic origin hospitalized in an Intensive Care Unit (ICU). Blood samples were also collected from the parents. The results from the R-WES were available after 5-14 days. A conclusive genetic diagnosis was obtained in 13 children, corresponding to an overall diagnostic yield of 72.2%. For nine patients, R-WES was used as a first-tier test. Eight patients were diagnosed with inborn errors of metabolism, mainly mitochondrial diseases. In two patients, the disease was possibly caused by variants in genes which so far have not been associated with human disease ( NARS 1 and DCAF5 ). R-WES proved to be an effective diagnostic tool for critically ill infants in ICUs suspected of having a genetic disorder. It also should be considered as a first-tier test after precise clinical description. The quickly obtained diagnosis impacts patient's medical management, and families can receive genetic counseling., Competing Interests: The authors declare no conflict of interest.

Objective: Lethal defects lead to the intrauterine death of the fetus or the passing away of the child immediately after birth or in early infancy, regardless of the treatment used. In the case of lethal defects, it is not possible to effectively help the child, despite using the most modern equipment or medicines in the treatment or the progress made by medicine. Parents, who decide to continue the pregnancy, although the fetus has a lethal defect that cannot be cured, may be covered by perinatal hospice care, which is comprehensive and consists in supporting the pregnant woman during the prenatal time, during delivery and after delivery and support of her family, giving full information to the parents about their child's illness. Childcare after birth is focused on protecting the infant from persistent therapy and providing him with appropriate conditions. Aim: To demonstrate the role of perinatal palliative care for pregnant women in whom the results of prenatal tests pointed to a severe developmental disorder in the fetus with a potentially lethal prognosis, and to present a pattern of behavior for their hospitalization in the perinatal hospice., Patients and Methods: Materials and methods: The retrospective analysis included documentation of 67 patients referred to the RAZEM (TOGETHER) Program in Wrocław in 2014-2018 due to abnormal results of (ultrasound and / or genetic) prenatal tests, which indicated a serious developmental disorder in the fetus with potentially lethal prognosis. Analysis was conducted of sociodemographic data, clinical data on fetal diagnosis, pregnancy and delivery, the procedure for prenatal delivery and postnatal birth., Results: Results: 67 women aged 20-43 years (mean 31.2) were referred to the RAZEM Program. Out of these, 57 women were enrolled for palliative care, which accounted for 85% of those referred to the program. Palliative care was continued in 51 patients, because 6 women decided to terminate their pregnancy during the diagnostic process (10.5%). The most common abnormalities in the fetuses were chromosomal aberrations, CNS defects and kidney defects. In 95% of the cases, intrauterine fetal death or neonatal death occurred., Conclusion: Conclusions: Perinatal palliative care is an indispensable form of care for pregnant women in whom the results of prenatal tests indicate a serious developmental disorder in the fetus with potentially lethal prognosis. The in-hospital model of a perinatal hospice is a beneficial form of care, as it ensures consistency and good communication in the team, which favourably affects its quality.

Background: It has recently been suggested that the adenosine A 2A receptor plays a role in several animal models of depression. Additionally, A 2A antagonists have reversed behavioral deficits and exhibited a profile similar to classical antidepressants., Methods: In the present study, imidazo- and pyrimido[2,1-f]purinedione derivatives (KD 66, KD 167, KD 206) with affinity to A 2A receptors but poor A 1 affinity were evaluated for their antidepressant- and anxiolytic-like activity. The activity of these derivatives was tested using a tail suspension and forced swim test, two widely-used behavioral paradigms for the evaluation of antidepressant-like activity. In turn, the anxiolytic activity was evaluated using the four-plate test., Results: The results showed the antidepressant-like activity of pyrimido- and imidazopurinedione derivatives (i.e. KD 66, KD 167 and KD 206) in acute and chronic behavioral tests in mice. KD 66 revealed an anxiolytic-like effect, while KD 167 increased anxiety behaviors. KD 206 had no effect on anxiety. Furthermore, none of the tested compounds increased locomotor activity., Conclusion: Available data support the proposition that the examined compounds with adenosine A 2A receptor affinity may be an interesting target for the development of antidepressant and/or anxiolytic agents., (Copyright © 2016 Institute of Pharmacology, Polish Academy of Sciences. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.)

Background: The majority of antineoplastic agents have been found to show mutagenic, teratogenic and carcinogenic potential. Biological effects of long-term contact with low levels of cytostatic drugs are not well known. The results of many studies are ambiguous, which might be associated with varying levels of exposure in different hospitals., Material and Methods: About 100 physicians and nurses employed in the Department of Pediatric Bone Marrow Transplantation, Hematology and Oncology, Wroclaw Medical University, are exposed to cytostatic drugs. Over the last 15 years, nine cases of cancer occured, which may result from occupational exposure to antineoplastic agents. The most severely affected professional group are physicians, among whom 5 cases of cancer were identified., Conclusions: Clinical situation of the Department requires an immediate action, including genetic studies, which are already under way. Moreover, all of the safety rules applicable when working with antineoplastic agents should be applied.

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.

Oesophageal atresia is a congenital defect of alimentary tract concerning the interruption of oesophagus with or without connection with the trachea. Its incidence is 1:3000-3500 of live-born. Associated anomalies including genetic disorders occur in 50% of patients. Edwards syndrome which is trisomy of chromosome 18 with poor prognosis. The incidence of Edwards syndrome is 1:5000 of live-born. About 5% of these children live more than 1 year. The aim of this article is a retrospective analysis of the course of treatment of newborn with oesophageal atresia and Edwards syndrome and making of therapeutic decision. The authors from different medical specializations: clinical genetics, paediatric surgery, paediatrics and neonatology, paediatric intensive care and palliative medicine, have undertaken a discussion regarding surgical treatment of children with oesophageal atresia and chromosomal, lethal syndrome.

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%).

Aims: The aim of the study was the analysis of risk factors of therapeutic failures in children with malignant germ cell tumours treated within the multicentre programme of PPGGL from 1999--2006., Materials and Methods: The investigated group included 18 (14.3%) patients, of 123 who have finished the treatment of malignant germ cell tumour, in whom no remission was obtained or relapse occurred. All the patients were treated according to the TGM 95 programme. Both clinical and morphological data of the group have been analysed., Results: Among 18 patients with therapeutic failures 12 died. Two patients from the high risk group died of complications of the treatment--sepsis during neutropenia after chemotherapy and one after haemorrhage to the central nervous system. The other 9 died from progression of malignancy, 6 of them belonged to the high risk group. 10 (82%) of 12 patients who died had extragonadal location and in 11 (92%) the tumour was in stage III or IV of the disease. The most frequent histology in this group was mixed germ cell tumour with component of yolk sac tumour or carcinoma embrionale. 92% patients had elevated AFP, in 4 it was above 15000 ng/ml. In 11 (92%) patients primary chemoresistance was observed, and radical surgery was not possible for the reason of advanced stage of the disease. In 6 patients relapse occurred. In 3 patients testis was the primary location (I and II stage), in 3 patients the tumour was localized in the sacrococcygeal region (III and IV stage). All the patients are alive in remission after second line therapy, with 78 months (median) of follow-up., Conclusions: 1. The main risk factor for therapeutic failures in malignant germ cell tumours was primary chemoresistance in inoperable tumours of the sacrococcygeal region. 2. The mortality of treatment complications was low. 3. The relapse of cancer was not a risk factor for therapeutic failure due to the high probability of second remission 4. Therapeutic failures are mainly observed in patients with mixed germ cell tumour with components of yolk sac tumour or carcinoma embrionale. 5. Tumour chemoresistance should be considered an essential factor in identifying high risk patients.

Aim of the Study: Measurement of c-myb expression in leukaemia cells in children and in normal cells of healthy controls., Material and Methods: 37 patients, 23 boys and 14 girls with acute leukaemia, aged 1-17 years, were included in the study (32 with acute lymphoblastic leukaemia and 5 with acute myeloblasts leukaemia) Control group consisted of 17 healthy children, 8 boys and 9 girls, 4-18 years old. After the isolation of mononuclear cells from bone marrow I peripheral blood mRNA was isolated, then with the use of reverse transcriptase cDNA was synthesized. The level of expression of c-myb was analyzed with polymerase chain reaction method. The final result was analyzed as the ratio between fluorescence of c-myb gene and the control gene., Results: Mean level of expression of c-myb gene in leukaemia cells was statistically significantly higher than in the control group (0.71+/-0.53 vs. 0.51+/-0.22; p=0.05), as well as c-myb level between leukaemia cells in relapse cases and controls (0.83+/-0.23 vs. 0.51+/-0.22; p=0.01). There was no difference between c-myb expression in different diagnosis. The comparison of c-myb expression in good and poor response group was not statistically significant (p=0.33)., Conclusions: Possible influence of increased expression of c-myb gene in the promotion of leukaemia was found. The role of c-myb expression as a prognostic factor in acute leukaemias of children was not confirmed.

Unlabelled: Approximately 2% of of all malignant tumours in boys are localised in the testis. Among them 80% are germ cell tumours with the malignant elements of yolk sac tumour. AIM of the study was evaluation of the efficacy of malignant testicular tumour treatment programme in children., Material and Methods: Since 1998 31 boys aged 1 month to 18 years (median 14 years) with malignant testicular tumours were enrolled in the multicentre trial. Patomorphologically clear yolk sac tumour (33%) and mixed germ cell tumour (42%) with the majority of yolk sac tumour component or carcinoma embryonale, occurred most often. Alfa-feto-protein was increased in 63% and choriogonadotropin in 26 patients. 61% patients had local clinical stage and the tumour was localized in the testis. In 39% patients tumour exceeded the testis margin. 4 patients were excluded from analysis as 3 are actually treated and 1 died on the second day of admittance to hospital. All patients received TGM 95 regimen (Tumeurs Germinales Malignes). Surgery (orchidectomy) was applied in 27 boys, 26 were primary (81% complete), 3 secondary (100% complete). 33% received no chemotherapy after surgery, in 41% VBP protocol (vinblastine, bleomycin, cisplatin) was given and in 26%o VIP protocol (ethoposide, ifosphamide, cisplatin). Two patients received also ABK (adriamycine, bleomycin, carboplatin)., Results: Among 26 children with germ cell tumours, 25 (96%) are alive, 23 (88%) are in first remission after completion of treatment. One child died due to central nervous system metastases. 2 children had local recurrence treated with chemotherapy or surgery with good result. Median follow-up is 45 months., Conclusions: TGM regimen is highly efficient in the treatment of malignant testicular tumours. Problems occur in cases of disseminated disease.

Unlabelled: Approximately 1% of all malignant tumours among children are localized in the ovary. The majority belongs to germ cell tumours and occurs in the peripubertal period. AIM of the study was the evaluation of the efficacy of malignant ovarian germ cell tumour treatment programme in children., Material and Methods: Since 1998, 40 girls with malignant ovarian tumours were enrolled in the multicentre trial. Mixed germ cell tumours with yolk sac elements and dysgerminoma occurred the most often. Alfa-fetoprotein (AFP) was increased in almost one half of patients. Tumour exceeded the ovary margin in more than half the patients and 25% were qualified as high risk group. 38 children completed the treatment. All but one patient with neuroblastoma received TGM protocol (Tumeurs Germinates Malignes). A VBP regimen (vinblastine, bleomycin, cisplatin) was applied in 19 girls, VIP regimen (etoposide, ifosfamide, cisplatin) in 16, two received no chemotherapy. Due to delayed remission after first-line chemotherapy it was prolonged with ABK (adriamycine, bleomycine, carboplatin) in 3 patients, 1 megachemotherapy regimen with autologous bone marrow transplantation was realized, one patient received a 1.5 year long oral chemotherapy. All the children underwent surgery, 34 primary (56% complete), 12 secondary (75% complete). 8 children were operated twice., Results: Among 34 children with germ cell tumours and 3 with sex cord tumours who completed the treatment all are alive in the first remission. 1 child with neuroblastoma localised in the ovary died due to recurrence. A median follow-up period was 42 months., Conclusions: The TGM protocol appears to be highly efficient in treatment of germ cell tumours even in advanced stages.

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).

Unlabelled: Germ cell tumors constitute about 3% of all pediatric malignancies. Since 1998 the multicenter trial was initiated in Poland., Material and Methods: 95 children (aged from 1 month to 17 years--mean 9.2 years) were registered. There were 38 boys and 57 girls. Diagnosis was made on histopathological examination in 88% patients (pts) and in 12% was established on imaging and biochemical findings (elevated AFP). Mixed germ cell tumor and yolk sac tumor prevelaged. AFP was elevated in 72% pts; in 26% it was over 15.000. Primary tumor was localized in gonads (59%) and in sacrococcygeal region (30%). Following disease stages were identified: I and II--41% pts, III--34%, IV--25%. All patients were treated according to French TGM'95 protocol. 43 belonged to high risk and 52 to standard risk group. 77 children completed therapy, 15 continue treatment and 3 were lost from follow-up., Results: Among children who were off therapy, 70 (91%) are alive in a complete remission (second remission in 3 cases). Survival in high risk group is 89%, while in standard risk group is 93%. Median time of follow-up is 31 months from the beginning of treatment and 25 months after completion of therapy. 7 children died; all had progressive disease., Conclusion: The outcome of malignant germ cell tumors treatment in Poland is favourable and comparable to results showed by other study groups in the world.

Between 1995 and 2001 echo-cardiography was performed in 244 children (128 boys, 116 girls) with acute lymphoblastic leukaemia (ALL) before the beginning of therapy with anthracyclines (medium 5.4 days after the diagnosis). The mean age at diagnosis was 5.4 years (range 9 months to 17.7 years). 189 children (97 boys and 92 girls) were included into the standard and medium risk groups and 55 (31 boys and 24 girls) into the high risk group. 29% of ALL children had disturbances in ECG. Changes in the thickness of the intraventricular septum (%IVSTh) and left ventricular posterior wall (%LVPWTh) were statistically lower, especially in children under 7 years of age. Some children showed lowering of shortening fraction (%FS - 8.6%), ejection fraction (%EF - 10.2%) and corrected velocity of fibber-shortening (Vcfc - 25.8%). Children with decreased shortening fraction (%FS) had left ventricular posterior wall thickness (%LVPWTh) impairment. Changes in diastolic function indicate impaired relaxation and compliance of the left ventricle. Decreased peak early filling velocity (E) was found. There were also longer deceleration time (EDecT) and decreased deceleration from peak E velocity (E/Dec) and longer isovolumetric relaxation time in children in standard and medium risk groups. Shorter acceleration time (EAccT) was seen in the high risk group. Evaluation of cardiac function before anthracycline chemotherapy will allow to select patients with pre-existing cardiac impairment for whom cardioprotective treatment is absolutely necessary.

In the Department of Paediatric Hematology and Oncology (University School of Medicine in Wrocław) 25 patients, 1 to 19 years old, 8 boys and 17 girls, suffering from germ cell tumors were treated from June 1989 to August 1998. In the course of the treatment both surgery, chemotherapy and radiotherapy were used. The level of oncological markers: alpha-fetoprotein (AFP), lactic dehydrogenase (LDH), carcinoembryonal antigen (CEA), beta-choriongonadotropin (beta-HCG) and CA 125 was examined at the beginning, during the therapy, and at the end of the treatment. Increased levels of oncological markers were found: AFP in 17 children, LDH in 10 children, CEA in 3 children, beta-HCG in 3 children and CA 125 in 2 children. In all cases the level of AFP was decreasing during the therapy. Normal levels of AFP and LDH were observed in children with complete remission. The use of AFP in diagnostics and monitoring of germ cell tumors was proved.