The objective of the study was to analyse levels IL-12 and to relate the findings to the clinical course of type 1 diabetes mellitus (DM1). We examined a group of 102 children with DM1 and 39 healthy children (as the control). All the... more
The objective of the study was to analyse levels IL-12 and to relate the findings to the clinical course of type 1 diabetes mellitus (DM1). We examined a group of 102 children with DM1 and 39 healthy children (as the control). All the children with DM1 had their daily urine albumin excretion, HbA1c, C-peptide measured, 24hrs blood pressure monitoring and ophthalmologic examination. In accordance to the ophthalmologic examination and level IL-12 in the serum the diabetic children were divided into 3 groups: group A: IL-12>0 pg/ml; group B: IL-12=0 pg/ml; group C: IL-12=0 pg/ml and IL12>0 pg/ml. Serum levels of IL-12 and TNFalpha were measured by the immunoenzymatic ELISA method, Quan-tikine High Sensitivity Human by R&D Systems (USA). Children of group A were characterized by significantly high level of IL-12 and by the absence of TNFalpha as compared with the children of group B, who had undetectable IL-12 along with high TNFalpha level. Additionally, children of group A had significantly lower urine albumin excretion and had only developed retinopathy. However, the children of group B not only had retinopathy, nephropathy but also arterial hypertension. The patients of group A were also analysed against the children of group C, who were characterized by high IL-12 level and some of them had also detectable TNFalpha, but without retinopathy and nephropathy. The results of our study imply the existence of balance between IL-12 and TNFalpha in type 1 DM children, which seems to warrant the stage of disease without diabetic complications. However, the IL-12 domination tends to prevent or delay nephropathy development but does not protect from retinopathy.
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The aim of the research work is the evaluation of auditory function in children with rare, genetically determined Brachmann-de Lange syndrome. Test material came from 18 children (7 girls and 11 boys) between 11 months and 18 years of age... more
The aim of the research work is the evaluation of auditory function in children with rare, genetically determined Brachmann-de Lange syndrome. Test material came from 18 children (7 girls and 11 boys) between 11 months and 18 years of age with Brachmann-de Lange syndrome who have been diagnosed and treated at ENT Department and Department of Paediatrics, Haematology, Oncology and Endocrinology Medical University of Gdansk with support of Cornelia de Lange Association - Poland. In all children examinations of brainstem auditory evoked potential have been carried out as well as tympanometric examination in case of finding hearing loss. All these examinations were carried out in ENT Department of Medical University of Gdansk, using Racia-Alvar Centor C apparatus and Madsen-Zodiak 901. 9 (50%) of patients demonstrated hearing loss. In 3 (16.7%) cases the conduction hearing loss was connected with the chronic diseases of middle ear which required medical treatment. In remaining 6 (33.3%) cases due to sensorineural hearing loss children had hearing aids applied and underwent rehabilitation. The results indicate that all children with Brachmann-de Lange syndrome should undergo examinations of brainstem auditory evoked potential. It enables to detect hypoacusis and initiate proper treatment. Lack of the opportunity of having a hearing aid applied and further rehabilitation deepens the social isolation of the little patients and inhibits their natural progress in communication. In such cases mental retardation may subsequently occur.
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Adolescent, Poland, Humans, Child, Hearing aids, and 6 moreFemale, Male, Infant, Cornelia de Lange Syndrome, Audiometry, and Child preschool
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... 1985, Rubin & Nelson 1990). Increased serum levels of sIL-2Rα have been found in a variety of autoimmune, inflammatory and infectious diseases as well as in acquired immunodeficiency disorders (Naveau et al. 1999,... more
... 1985, Rubin & Nelson 1990). Increased serum levels of sIL-2Rα have been found in a variety of autoimmune, inflammatory and infectious diseases as well as in acquired immunodeficiency disorders (Naveau et al. 1999, Peteiro-Cartelle & Alvarez-Jorge 1999, Shi et al. 1999). ...
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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... more
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. 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. 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. 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.
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Treatment Outcome, Adolescent, Humans, Child, Female, and 4 moreMale, Sarcoma, Prognosis, and Disease Progression
The aim of our study was to determine an association between the -174G>C IL-6 polymorphism (rs1800795) and occurrence of retinopathy and nephropathy in type 1 diabetes mellitus (T1DM)... more
The aim of our study was to determine an association between the -174G>C IL-6 polymorphism (rs1800795) and occurrence of retinopathy and nephropathy in type 1 diabetes mellitus (T1DM) patients. Two hundred ten children/adolescents with long-standing T1DM (16.5 +/- 3.8 y; with diabetes duration of 8.4 +/- 3.0 y) were enrolled into the study. A group of 170 healthy young (16.9 +/- 5.2 y) sex-matched volunteers was qualified as the control. The IL-6 polymorphism was genotyped with the PCR-RFLP method. Serum and urine IL-6 concentrations were measured by the ultra-sensitive ELISA tests. The -174GG genotype was under represented in the diabetic patients compared with the controls. Patients with this genotype were free from nephropathy and retinopathy. The group of -174GG carriers was characterized by the highest urine IL-6 concentrations in relation to other genotypes. In the multivariate logistic regression analysis adjusted for age, duration of the disease, age of disease onset, HbA1c, and albumin excretion rate, the -174GG genotype was the only independent variable that significantly decreased the risk of jointly analyzed retinopathy and nephropathy [OR = 0.65; 95% CI = 0.52-0.82; p = 0.0003]. We propose that the -174GG patients are protected from late diabetic complications by different IL-6 dependent mechanisms.
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THE AIM of the study was to evaluate the incidence of pulmonary complications in children cured from Hodgkin's... more
THE AIM of the study was to evaluate the incidence of pulmonary complications in children cured from Hodgkin's disease (HD). 42 children with HD were treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, between 1994 and 2004. Stages of HD: II--26 children, III--10, IV--6; general symptoms (group B) were present in 50% of patients. Mediastinal involvement was found in 33 children, lung parenchyma infiltration in seven and bronchi involvement in one. In 1/3 of these patients the localization of HD within the chest was massive and symptomatic with signs of the superior caval vein symptoms, cough, dyspnea and cardiac tamponade. The treatment was conducted according to the schemes of the Polish Paediatric Leukaemia /Lymphoma Study Group. Eleven patients required therapy modification including six, in whom the intense line II chemotherapy Salvage 95 was introduced. 29 patients received chest irradiation with doses between J 75 and 36.5 Gy. Pulmonary function was evaluated from the results of clinical examination, 1 chest radiography (CXR), computed tomography, spirometry and lung scintigraphy. Pulmonary complications occurring as fatigue and diminished physical effort tolerance was observed in only two children. Some of the remaining 40 patients demonstrated asymptomatic abnormalities in the analysed tests. Abnormalities in CXR (upper mediastinal fibrosis, postoperational changes within the diaphragm and pneumonitis) were found in six children, minor ventilation problems in spirometry--in 12 and decreased lung perfusion in five. The scintigraphic signs of lung embolisation were not observed in our material. Most of the pulmonary complications occurred in children with enlarged lymph notes located within the chest, especially these with bulky disease presenting with cardio-pulmonary symptoms. In this group of patients the chest irradiation was performed in all except four children, three patients were also administered aggressive salvage chemotherapy. The pulmonary complications in children after completed therapy of HD are not common and mainly asymptomatic and occur predominantly in patients with massive mediastinal and/or lung involvement at diagnosis. The issue needs further evaluation of a more numerous group of HD survivors and a longer follow-up.
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Management, Pediatrics, Adolescent, Poland, Humans, and 7 moreChild, Female, Male, Infant, Retrospective Studies, Prognosis, and Child preschool
ABSTRACT
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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... more
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.
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About 7% of all childhood cancers comprise non-Hodgkin's lymphoma (NHL). NHL... more
About 7% of all childhood cancers comprise non-Hodgkin's lymphoma (NHL). NHL are heterogenous group of neoplasms deriving from lymphatic system (cell B and T). B-cell NHL characterize by high malignancy, but coincidentally good reaction for treatment. In about 20% primary tumour is localised within head and neck, and nasopharynx lymphomas comprise 10%. This location maintains the biggest diagnostic and therapeutic difficulties because of tumours of this site proliferate in the region of frequent infections postponing a proper diagnosis and the local control after complete treatment is difficult. The authors analyse clinical symptoms before diagnosis, the incidence of nasopharynx lymphomas, histopathological type of neoplasm, clinical stage, results of treatment. The study includes 97 patients who were treated because of lymphomas between 1993-2002. The character of clinical symptoms and their duration, histopathological type of lymphomas, results of treatment were analysed. The primary nasopharynx location was assessed in 9 patients (9.3%). Sex: 7 boys, 2 girls, age: 2-17 years. The duration which elapsed from initial clinical symptoms to diagnosis was 2-10 weeks. The histopathological assessment in 6 children was Burkitt lymphoma and in 3 children--Burkitt-like lymphoma. Metastases: CNS--1 patient, bone marrow--1 patient, abdomen--1 patient. Treatment was performed according to LMB-89 protocol. First complete remission--7 patients; second complete remission--2 patients. Lymphomas of nasopharynx cause diagnostic problems because of their early stage pseudo-inflammatory manifestation. Special attention should be paid to perform imaging studies (MRI/CT), which are useful in the reaching the proper diagnosis. The radiologic evaluation of primary lesion is still difficult. In the doubtful cases, the surgery and histopathological examination are necessary.
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Reactive oxygen species (ROS) play a role in cancerogenesis processing and damage tissues. Furthermore, oncological treatment may impair proper function of the gut barrier. The aim of this study was to measure intestinal permeability in... more
Reactive oxygen species (ROS) play a role in cancerogenesis processing and damage tissues. Furthermore, oncological treatment may impair proper function of the gut barrier. The aim of this study was to measure intestinal permeability in children in clinical remission for solid tumours and to search for a possible relationship between free radicals and the intestinal barrier. No such investigation in children has been reported so far. The prospective study consisted of 19 paediatric patients with cancer after completion of chemotherapy. 32 healthy children from the outpatients clinics were recruited for measurement of intestinal permeability and antioxidant barrier as a control group. Intestinal permeability was assessed by measurement of urinary lactulose and mannitol after oral challenge. Antioxidant enzymes: superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) in erythrocytes were assessed. Ischemia modified albumin (IMA) concentration was measured in serum. Cancer patients excreted less mannitol and more lactulose versus controls. The ratio of lactulose to mannitol was significantly higher in oncological children vs control (mean 0.188 and 0.0453, respectively, p=0.0006,). Significantly higher IMA level in the oncological group vs control was noted (mean 123.8 and 87.3 U/ml, respectively, p=0.0037). No correlation between intestinal permeability and oxidative stress barrier was found. Our data shows that intestinal barrier is damaged in paediatric cancer patients after chemotherapy. IMA is believed to play a protective role in the defence against tissue damage. No correlation was found between these two barriers.
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Oxidative Stress, Adolescent, Antioxidants, Prospective studies, Humans, and 17 moreChild, Permeability, Female, Male, Young Adult, Glutathione Peroxidase, Superoxide Dismutase, Intestinal absorption, Neoplasms, Erythrocytes, Antineoplastic Agents, Biochemistry and cell biology, Mannitol, Case Control Studies, Lactulose, Serum albumin, and Child preschool
The aim of the work is to assess the correlation between HbA1c, VEGF, TNF-alpha, IL-6 and the development of diabetic retinopathy in children diagnosed with diabetes mellitus type 1. One hundred fourty nine children with long-standing... more
The aim of the work is to assess the correlation between HbA1c, VEGF, TNF-alpha, IL-6 and the development of diabetic retinopathy in children diagnosed with diabetes mellitus type 1. One hundred fourty nine children with long-standing diabetes mellitus type 1 were enrolled in the study. The patients were divided into two subgroups: children with retinopathy (Group 1; n=28) and children without retinopathy (Group 2, n=121). The control group consisted of 62 healthy children. All the examined children had VEGF, TNF-alpha and IL-6 measured in the blood serum using highly sensitive ELISA tests. Statistically significant higher blood serum levels of HbA1c, VEGF, TNF-alpha and IL-6 were found in the group 1 in comparison with the group 2. Children of the group 2 in relation to healthy controls showed statistically significant higher blood serum levels of VEGF, TNF-alpha and IL-6. Elevated levels of VEGF, TNF-alpha, IL-6, HbA1c in blood serum of children with diabetes mellitus type 1 detected before development of overt signs of diabetic retinopathy might indicate the possibility of the occurrence of changes in the eye shortly.
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Neoangiogenesis is a complex process, in which crucial role seems to be played by Vascular Endothelial Growth Factor (VEGF), Tumor Necrosis Factor-alpha (TNF-alpha) and Interleukin-12 (IL-12). Therefore it appeared to be worth of analysis... more
Neoangiogenesis is a complex process, in which crucial role seems to be played by Vascular Endothelial Growth Factor (VEGF), Tumor Necrosis Factor-alpha (TNF-alpha) and Interleukin-12 (IL-12). Therefore it appeared to be worth of analysis to investigate the relation between IL-12, VEGF, TNF-alpha and the clinical course of the disease in children with Diabetes Mellitus type 1 (DM1). One hundred and twenty six children in age 14.9+/-3.2 years diagnosed with DM1 from the Department of Paediatrics, Haematology, Oncology and Endocrinology of the Medical University of Gdańsk were enrolled in the study along with 54 healthy children (as the control). All the children had their daily urine albumin secretion, HbAlc, C-peptide measured; 24 hrs blood pressure monitoring and ophthalmologic examination. Additionally, all of them had serum IL-12, VEGF, TNF-alpha measured using highly-sensitive ELISA tests (Quantikine High Sensitivity Human by R&D Systems, Minneapolis, Minn., USA). The children were divided into 2 groups: with retinopathy and without retinopathy. Between the groups statistically significant differences in age, duration of the disease, HbAlc serum level, C-reactive protein, daily albumin urine secretion and the systolic and diastolic blood pressure were found. Besides, statistically significant higher levels of VEGF, TNF-alpha and IL-12 were found in the group with retinopathy in comparison without retinopathy and healthy control group. Our results suggest that VEGF, TNF-alpha and IL-12 are engaged in neoangiogenesis regulation of diabetic retinopathy children.
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... sIL-2R (Zhang et al. 2006), while not all malignant solid tumours are. There are, how-ever, reports on the diagnostic utility of serum sIL-2R in adult patients wit STS (Ruka et al. 2001; Rutkowski et al. 2002). It is suggested that ...
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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... more
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.
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Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular... more
Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms. The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients). Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression. The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.
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Research Interests:
Adolescent, Humans, Child, Blood Pressure, VEGF, and 15 moreDiabetic Retinopathy, Female, Male, Statistical Significance, Clinical Sciences, IL, C reactive protein, Blood Pressure Monitoring, Tumor necrosis factor-alpha, Group, Biological markers, Type 2 Diabetes Mellitus, High Sensitivity, Vascular Endothelial Growth Factor, and Interleukin
Opsoclonus-myoclonus-ataxia (OMA) syndrome is the most common paraneoplastic neurological syndrome in childhood. We reviewed the literature and reported on clinical and pathological characteristics of four children with OMA and peripheral... more
Opsoclonus-myoclonus-ataxia (OMA) syndrome is the most common paraneoplastic neurological syndrome in childhood. We reviewed the literature and reported on clinical and pathological characteristics of four children with OMA and peripheral neuroblastic tumours. In two of the children the onset of neurological symptoms was connected with a vaccination and in one with viral infection. The suprarenal gland was the primary localization of the tumour in 3 of the children and in one the tumour was located in the retroperitoneal area. All cases were in stage II or III of the disease, with no metastases or MYCN amplification. The group included two ganglioneuroblastomas, one ganglioneuroma and one differentiating neuroblastoma. The tumours were characterized by the presence of lymphocytic infiltrations with lymphadenoplasia. Immunohistochemical analysis of inflammatory infiltrations revealed mixed type populations of lymphocytes with prevalence of the cytotoxic type (CD8 and CD56-positive cells). The participation of dendritic cells and macrophages was also detected. All patients were treated by surgery alone or with adjuvant chemotherapy with a positive outcome. In 3 cases persistent neurological disorders were observed with exacerbations during infections. In some patients the onset of OMA is related to vaccination or infection. Children with OMA and neuroblastoma despite a good oncological prognosis often present permanent neurological and developmental deficits. The inflammatory infiltrations within the tumours are combined, with predominant participation of cytotoxic cells.
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The aim of the study was to assess the incidence of relapse and outcome of treatment in children with nephroblastoma relapse. In the years 1989-2002 at our centre 67 children were treated for nephroblastoma. Patients were divided into two... more
The aim of the study was to assess the incidence of relapse and outcome of treatment in children with nephroblastoma relapse. In the years 1989-2002 at our centre 67 children were treated for nephroblastoma. Patients were divided into two subgroups. Group I comprised 25 children treated in accordance with the SIOP-9 programme, whilst group II consisted of 42 patients following the SIOP 93-01 protocol. In group I, five children had relapse diagnosed: local in 2, pulmonary in 2 and cerebral in one case. Two of the cases were diagnosed during the treatment, and the other 3 after 1 to 19 months from the completion of treatment. In group II relapse was diagnosed in 4 children: local in 2 and pulmonary in 2 cases. One case was diagnosed while on treatment, and the other 3 were diagnosed 1,2 and 4 months after completion of treatment. Among the children who followed the SIOP-9 programme three patients died, while among those following SIOP 93-01 two patients died. The prognosis in patients who relapsed remains uncertain. The histological type of tumour and the moment of relapse occurrence are significant prognostic factors.
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Extensive diagnostic and therapeutic dilemmas appear in children With primary malignant neoplasms located in the minor pelvis. To evaluate the clinical symptoms, disease course and the results of treatment in patients with malignant... more
Extensive diagnostic and therapeutic dilemmas appear in children With primary malignant neoplasms located in the minor pelvis. To evaluate the clinical symptoms, disease course and the results of treatment in patients with malignant pelvic neoplasms. The study included 31 children (13 boys and 18 girls; aged 2 months to 16 years; mean age -- 8 years) treated in the Departments of Paediatric Oncology and Haematology in Gdansk and Lublin during the period of 1992-2003. The group comprised 17 patients with soft tissue sarcomas (MTM) (55%), 12 with germinal tumours (TGM) (39%) and tow. with neuroblastoma (NBL) (6%). The great majority of children (90%) presented with highly advanced disease (stages III + IV -- in 28 out of 31 patients). with data analysis we were able to distinguish two categories of patients with different prognosis: with MTM and TGM. Most of he MTM tumours (11/17 - 65%) were localized in the urinary tract, the remaining six developed within pelvic muscles. Ten out of twelve TGM tumours (83%) were located in the ovaries. Radical tumour resection, especially primary resection, was shown to play the key role in both groups. Among TGM patients it was performed in 75% while in MTM patients -- in only 12%. All of these patients entered clinical remission and remain disease free. After adjuvant chemo- and/or radiotherapy secondary tumour resection was done in 17% of TGM and 41% of MTM patients. in patients, who were not able to undergo radical tumour resection (mainly MTM patients), the disease progressed and led to death.
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beta 2-Microglobulin (beta 2-M) measurement acts as an important diagnostic tool of some lymphoproliferative disorders in adults. Its significance as a marker of disease activity in pediatric malignancies has not been evaluated yet. The... more
beta 2-Microglobulin (beta 2-M) measurement acts as an important diagnostic tool of some lymphoproliferative disorders in adults. Its significance as a marker of disease activity in pediatric malignancies has not been evaluated yet. The aim of this study was to determine the clinical significance of the pre-treatment serum levels of beta 2-M in children suffering from neoplastic disease. The study was conducted in 61 children (28 girls and 33 boys) aged from 2.0 to 16.9 years at the time of disease diagnosis. The study group consisted of 6 common types of childhood malignancies: acute lymphoblastic leukemia (ALL--22 patients), acute nonlymphoblastic leukemia (ANLL--3), Hodgkin's disease (HD--12), non-Hodgkin's lymphomas (NHL--6) and malignant solid tumors: Wilms' tumor (Tu Wilms--10) and soft tissue sarcomas (SA--8). The control group consisted of 30 healthy children. Different behavior of beta 2-Microglobulin levels depending on histological type of the neoplasm was observed. The pre-treatment levels of analyzed marker in children with lymphoproliferative disorders (acute leukemias and lymphomas) were significantly elevated as compared to the levels in the healthy controls (median: 2.90 vs. 2.05 mg/L, p < 0.001). On the contrary, in patients with malignant solid tumors (Wilms tumor and soft tissue sarcomas) the median levels of beta 2-M at diagnosis were within normal range. Elevated values of serum beta 2-M were stated in the majority (67%) of patients with lymphoproliferative disorders and in 39% of patients with malignant solid tumors. beta 2-Microglobulin may serve as an additional diagnostic marker in children with leukemias and lymphomas. Measurement of serum beta 2-M seems to have no clinical value in the diagnostics of pediatric malignant solid tumors.
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The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis... more
The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease. The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh). Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopathological type of HD predominated. Poor response to standard treatment was observed in five children. One patient received additional cycles of chemotherapy MVPP/B-DOPA, four children were administered the 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd therapy lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation. Radiotherapy was given to 13 children. 13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years. In two of them late complications affecting hormonal status, cardio-pulmonary disorders and chronic B and C hepatitis were observed. Two children died including one admitted in a very severe condition, after long-lasting medical history who died of neutropenia-related sepsis. The second boy died 12 months after stem cell transplantation because of a second neoplasm--acute myeloblastic leukaemia. Chemo- and radiotherapy implemented according to protocols approved by the PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis made at earlier stages would result in giving less aggressive therapy, connected with a lower risk of durable late complications.
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... sIL-2R (Zhang et al. 2006), while not all malignant solid tumours are. There are, how-ever, reports on the diagnostic utility of serum sIL-2R in adult patients wit STS (Ruka et al. 2001; Rutkowski et al. 2002). It is suggested that ...
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Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias.... more
Neurofibromatosis type 1 (NF1) is a frequent genetic disorder of autosomal-dominant pattern. The incidence is about 1 per 3000 live births. Patients with NF1 are predisposed to malignancies including soft tissue sarcomas and leukaemias. The aim of the study was assessment of the most frequent symptoms on the basis of long term observation of children with NF1 and presentation of implemented diagnostic and therapeutic procedures. In our department there are 149 children (71 boys and 78 girls) aged from 7 months to 18 yrs with diagnosed or suspected NF1. Each child is carefully followed up every 6 months on outpatient basis. Paediatric, neurological and opthalmological examinations are performed during the first visit and in cases of any new symptoms. Number of Lisch nodules, vision field, audiogram, dermatological evaluation of skin abnormalities as well as orthopaedic examination are also investigated. In any case of NF1 without neurological symptoms, MRI of the brain and spine is carried out every 2 years. Moreover, each child is consulted in the Genetic Clinic. Cafe-au-lait spots were observed in all 149 children, freckling of the armpits in 40, peripheral neurofibromas in 30, Lisch nodules in 2 patients. Secondary symptoms and complications such as mental retardation (9 cases) and epilepsy (10 cases), cognitive disorders and learning disabilities (21), abnormalities in MRI examination (53), benign or malignant CNS tumours (9), scoliosis (99) were diagnosed. In 5 patients malignant neoplasms occurred (3.4%) including: RMS--2 cases, Triton tumour--1 case, MPNST--1 case. Two children died of disease progression, one of treatment complications (sepsis) and two children are alive. 1. Patients with NF1 need regular specialist medical care. 2. Continuous education of the families with this disease is necessary. 3. Diagnostic and therapeutic procedures recommended for patients with NF1 need to be implemented at different levels of health care.
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Despite that numerous investigations on the nature of diabetic microangiopathy were carried out, its pathomechanism remains unclear. The aim of the study was to analyze the relation between early diabetic microangiopathy and the... more
Despite that numerous investigations on the nature of diabetic microangiopathy were carried out, its pathomechanism remains unclear. The aim of the study was to analyze the relation between early diabetic microangiopathy and the proinflammatory cytokines, NAG and its A and B isoforms in blood and urine in children diagnosed with diabetes mellitus type 1. The study was carried out on the group of 56 children with diabetes mellitus 1 (age 13.6+/-3.74) and 35 healthy children selected as the controls. All the patients had 24 hrs albuminuria, HbA1c, C-peptide as well as the NAG enzyme and its A and B isoforms serum and urine activities measured. Additionally, all the children had TNF-a and IL6 level in serum measured. Each patient had 24 hrs blood pressure monitored and underwent ophthalmologic examination. Children with long-standing diabetes mellitus and retinopathy (group 1, n=15) were older and were characterized by a statistically significant longer duration of the disease and higher HbA1c level in comparison with the patients who presented with no sign of diabetic retinopathy (group 2, n=41). In the group 1 statistically significant higher TNF-alpha serum level (p=0.01), NAG (p=0.002) and its isoforms A (p=0.007) and B (p=0.001) urine activities were measured in relation to the group 2. Additionally the level of IL-6 and NAG and its isoforms A and B serum activities were higher in group 1 than in group 2, however the differences were of no statistical significance. Moreover the children from group 2 in comparison with the healthy controls showed statistically significant higher TNF-alpha serum activity (p=0.016) and NAG (p<0.001) and its A (p<0.001) and B (p<0.001) isoforms both serum and urine activities. The occurrence of the detectable serum TNF-alpha activity in children with diabetes mellitus type 1 showing no sign of diabetic retinopathy and nephropathy and no microalbuminuria with the concomitant increase of NAG and its isoforms serum and urine activities might point toward prompt occurrence of these changes in the eye and the kidneys.
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The X-linked anhydrotic ectodermal dysplasia is a rare disease in which defects in development of ectodermal derivatives are observed. This syndrome is clinically characterized by total or partial anodontia, characteristic physionomy and... more
The X-linked anhydrotic ectodermal dysplasia is a rare disease in which defects in development of ectodermal derivatives are observed. This syndrome is clinically characterized by total or partial anodontia, characteristic physionomy and absent or reduced sweating. Recurrent fever was a clue to the disease diagnosis in 5 month old infant. The diagnosis was confirmed by the mutation of EDA exon 9.
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Membrane Proteins, Humans, Mutation, Male, Infant, and 3 moreFever, Anodontia, and Ectodermal Dysplasia
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Treatment, Adolescent, Poland, Humans, Child, and 7 moreFemale, Male, Infant, Incidence, Newborn Infant, Retrospective Studies, and Child preschool
Hereditary spherocytosis is the most frequently occurring haemolytic anaemia. Some patients manifest clinical signs during the neonatal period and require transfusion. A case of hereditary spherocytosis in neonate is presented. The use of... more
Hereditary spherocytosis is the most frequently occurring haemolytic anaemia. Some patients manifest clinical signs during the neonatal period and require transfusion. A case of hereditary spherocytosis in neonate is presented. The use of rhEPO during the first months may be an alternative therapy to red cell transfusion.
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The aim of the work is to assess the correlation between HbA1c, VEGF, TNF-alpha, IL-6 and the development of diabetic retinopathy in children diagnosed with diabetes mellitus type 1. One hundred fourty nine children with long-standing... more
The aim of the work is to assess the correlation between HbA1c, VEGF, TNF-alpha, IL-6 and the development of diabetic retinopathy in children diagnosed with diabetes mellitus type 1. One hundred fourty nine children with long-standing diabetes mellitus type 1 were enrolled in the study. The patients were divided into two subgroups: children with retinopathy (Group 1; n=28) and children without retinopathy (Group 2, n=121). The control group consisted of 62 healthy children. All the examined children had VEGF, TNF-alpha and IL-6 measured in the blood serum using highly sensitive ELISA tests. Statistically significant higher blood serum levels of HbA1c, VEGF, TNF-alpha and IL-6 were found in the group 1 in comparison with the group 2. Children of the group 2 in relation to healthy controls showed statistically significant higher blood serum levels of VEGF, TNF-alpha and IL-6. Elevated levels of VEGF, TNF-alpha, IL-6, HbA1c in blood serum of children with diabetes mellitus type 1 detected before development of overt signs of diabetic retinopathy might indicate the possibility of the occurrence of changes in the eye shortly.
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The objective of the study was to analyse levels IL-12 and to relate the findings to the clinical course of type 1 diabetes mellitus (DM1). Material and methods: We examined a group of 102 children with DM1 and 39 healthy children (as the... more
The objective of the study was to analyse levels IL-12 and to relate the findings to the clinical course of type 1 diabetes mellitus (DM1). Material and methods: We examined a group of 102 children with DM1 and 39 healthy children (as the control). All the children with DM1 had their daily urine albumin excretion, HbA1c, C-peptide measured, 24hrs blood pressure monitoring and ophthalmologic examination. In accordance to the ophthalmologic examination and level IL-12 in the serum the diabetic children were divided into 3 groups: group A: IL-12>0 pg/ml; group B: IL-12=0 pg/ml; group C: IL-12=0 pg/ml and IL12>0 pg/ml. Serum levels of IL-12 and TNFa were measured by the immunoenzymatic ELISA method, Quantikine High Sensitivity Human by R&D Systems (USA). Results: Children of group A were characterized by significantly high level of IL-12 and by the absence of TNFa as compared with the children of group B, who had undetectable IL-12 along with high TNFa level. Additionally, childre...
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The intrauterine toxoplasma gondii infection is linked with a high risk of central nervous system/CNS/damage in the fetus. Why, despite the wide knowledge of this neurodegenerative disease prophylaxis and therapy, do we still meet... more
The intrauterine toxoplasma gondii infection is linked with a high risk of central nervous system/CNS/damage in the fetus. Why, despite the wide knowledge of this neurodegenerative disease prophylaxis and therapy, do we still meet neonates with the CNS damage? Authors present two cases of congenital toxoplasmosis with the CNS involvement. One of the cases was identified as the internal hydrocephalus during a prenatal ultrasound examination but no further diagnostics tests were undertaken. The congenital toxoplasmosis and severe CNS damage was diagnosed post delivery. In the other case some clinical symptoms of the CNS infection appeared in the neonatal period. Now this patient is eight months old and presenting mild motor developmental delays. 1. There are many clinical symptoms of the congenital toxoplasmosis, that can occur in the prenatal/case 1/and postnatal/case 2/period. 2. No serologic tests for toxoplasmosis were performed in mothers during pregnancy, what delays treatment a...
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The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in... more
The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease. The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh). Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopatholog...