Publications by authors named "Vaan G"

People with sensory impairments combined with intellectual disabilities show behaviours that are similar to Autism Spectrum Disorder (ASD). The instrument Observation of Autism in people with Sensory and Intellectual Disabilities (OASID) was developed to diagnose ASD in this target group. The current study focuses on the psychometric properties of OASID.

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Background: It is difficult to diagnose autism spectrum disorder (ASD) in people with a combination of intellectual and sensory disabilities because of overlap in behaviour. The ASD typical behaviours of people with combined intellectual and sensory disabilities are often caused by their disabilities and not by ASD. Current diagnostic tools are inadequate to differentiate between people with and without ASD when they have these combined disabilities, because tools lack norms for this population or are subjective, indirect or unable to adapt to the variety of disabilities that these people may have.

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Background: Complicating malignant hematopoietic proliferations might severely hamper the course of acute lymphoblastic leukemia (ALL) in patients with an otherwise good prognosis. It is important to distinguish whether such neoplastic proliferations represent ALL relapses or secondary treatment-related malignancies.

Procedure: We present an 11-year-old girl with precursor-B-ALL in whom maintenance treatment was complicated by an isolated ALL relapse in the brain, nodular lymphoproliferations in the liver, and an isolated myelo-monocytic leukemia cutis.

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In The Netherlands from July 1988 to October 1991, children (0 to 16 years of age) with de novo acute lymphoblastic leukemia (ALL) were treated according to protocol ALL-7 of the Dutch Childhood Leukemia Study Group (DCLSG). In this protocol, chemotherapy and treatment stratification were identical to the ALL-BFM-86 protocol (Reiter et al, Blood 84:3122, 1994), but cranial irradiation was restricted to patients with initial central nervous system (CNS) involvement. Patients were stratified into 3 risk groups, based on leukemia cell mass and response to initial treatment: standard-risk group (SRG), risk group (RG), and experimental group (EG).

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Purpose: Here we report the results of a nationwide cooperative study in the Netherlands on acute lymphoblastic leukemia (ALL) in children. The aim of the study was to improve the cure rate and to minimize side effects in a group of non-high-risk ALL patients, especially with regard to the CNS. A second aim was to study potential prognostic factors.

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Unlabelled: We describe a patient with myelodysplastic syndrome with monosomy 7 presenting with a T-cell defect. He suffered from infections from the age of 10 years, when a CD4 deficiency and impaired lymphoproliferative responses in vitro were found. The only symptom of a myelodysplastic syndrome at that time was thrombocytopenia with giant platelets.

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Noonan's syndrome (NS) is a syndrome with multiple congenital anomalies, characterized by craniofacial anomalies, congenital heart disease, skeletal and genital abnormalities, and mild mental retardation. Chromosomal abnormalities have been found in only a few cases. The combination of NS and acute leukemia has been reported in only three cases.

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Late events and side effects are reported in 392 children cured of leukemia. They originated from 1193 consecutively newly diagnosed children between 1972 and 1982, in first continuous complete remission for at least 6 y after diagnosis, and were treated according to Dutch Childhood Leukemia Study Group protocols (70%) or institutional protocols (30%), all including cranial irradiation for CNS prophylaxis. Data on late events (relapses, death in complete remission, and second malignancies) were collected prospectively after treatment; late side effects were retrospectively collected by a questionnaire, completed by the responsible pediatrician.

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Juvenile xanthogranuloma (JXG) is a rare benign disease of the skin. It is seen in combination with juvenile chronic myelo-monocytic leukemia (JCML) and/or neurofibromatosis type 1 (NF1). The association with acute lymphoblastic leukemia is hardly mentioned in the literature.

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The light chain ratios and the concentrations of immunoglobulin G (IgG), IgA, and IgM were measured before, during, and after antileukemic therapy in 10 patients with common acute lymphoblastic leukemia. The concentrations of IgG, IgA, and IgM decreased substantially during treatment but recovered slowly after cessation of the therapy. The light chain ratios were not systematically affected, but at diagnosis the kappa/lambda ratios of total serum Igs, IgG, and in particular IgM were somewhat lower in the patient group compared with an age-matched reference group.

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We report on a mother who had been treated for a ganglioneuroblastoma and her daughter who had a long segment aganglionosis. Review of the relevant literature and recent molecular findings warrant the conclusion that most likely, there is a causal relation between these two neurocristopathies.

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Primary antiphospholipid syndrome or PAPS is characterised by antiphospholipid antibodies and arterial and/or venous thromboses. Numerous other clinical features have been shown to be related to this syndrome. Chorea is a well known but rare phenomenon in systemic lupus erythematosus; it has been shown to be strongly related to the presence of antiphospholipid antibodies.

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A girl with acute non-lymphoblastic leukaemia was treated with immunosuppressive chemotherapy. After cessation of therapy she had three consecutive episodes of infection due to Streptococcus pneumoniae from which she recovered and was shown to have developed a combined deficiency of both IgG2 and IgG4. The patient eventually relapsed and died 3 years after the initial diagnosis.

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The frequency of naevocytic naevi (moles) in patients with childhood haematologic malignancies was studied. All patients had received multiple chemotherapy. The majority had also received cranial irradiation as part of their central nervous system leukaemia/lymphoma prophylaxis.

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Few details exist on the course of mumps during cytostatic treatment. We therefore describe our observations on the course of mumps seen between 1974 and 1988 in eight children suffering from acute lymphocytic leukaemia (ALL). Our data suggest that in malignant disease the course is rarely severe and that the infection often remains subclinical, as in healthy children.

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We describe a patient who developed pyoderma gangrenosum during the remission phase of acute myeloid leukaemia whilst receiving maintenance therapy with methotrexate and 6-mercaptopurine. The spontaneous resolution of these skin lesions following discontinuation of chemotherapy suggests that these drugs may be of major significance in the aetiology of pyoderma gangrenosum. Nevertheless, 27 months later, a relapse of the leukaemia followed.

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Methotrexate (MTX) and 6-mercaptopurine (6MP) have been used since 30 years in the maintenance treatment of acute lymphoblastic leukemia (ALL) of childhood. A synergistic effect of this combination was demonstrated in mouse and childhood leukemia. In this article an overview is given of our investigations, concerning the biochemical basis of this synergism.

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We report the association of myasthenia gravis (MG) and autoimmune thrombocytopenic purpura (AITP) in a 13-year-old girl. The co-existence of these autoimmune diseases is rare in adults and, to our knowledge, never described in children. In our patient thymectomy had a therapeutic effect on both MG and AITP, suggesting a altered T-cell function as a pathogenic factor of major importance in both affections.

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The pretreatment distribution of DNA content was determined in the leukemic blasts of 114 children with standard risk acute lymphocytic leukemia. The patients were admitted to four different centers for pediatric oncology, participating in a national study ALL-V. In 39 of 107 evaluable patients (36.

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The effects of methotrexate (MTX) on cytotoxicity (trypan blue exclusion and soft agar clonal growth), cell cycle perturbation, and purine and pyrimidine ribonucleotide and deoxyribonucleotide pools have been studied in MOLT-4 malignant T-lymphoblasts. Two concentrations of MTX, 0.02 microM and 0.

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In the posttherapeutic bone marrow of a group of 30 children with acute lymphoblastic leukemia (ALL), small numbers of a particular lymphoid cell with a comparatively large size and large dark nucleus were found. This cell was called the "posttherapeutic lymphoid cell." This type of cell is easily distinguishable in the May-Grünwald-Giemsa-stained smears as well as in semi- and ultrathin Epon sections.

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