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Biomedical subjects

M H van Weel-Sipman

Publications and source records attributed to M H van Weel-Sipman.

12 recordsLinked to original sources
PubMed2003

Eye shielding during total body irradiation for bone marrow transplantation in children transplanted for a hematological disorder: risks and benefits.

This is a retrospective analysis of 188 children who underwent total body irradiation (TBI) in one or two fractions before bone marrow transplantation (BMT) for a hematological disorder. While 139 children had eye shielding during TBI to decrease cataract formation, 49 did not. The blocks used for shielding caused cylindrical areas of decreased dose intensity in the brain. The aim of the study was to determine if there was an increased risk of relapse in the eyes or in the CNS after shielding of the eyes. The probability and severity of cataract formation with and without shielding were also evaluated. None of the 49 children without shielding had a relapse in their eyes or in the CNS after BMT. Of the children with shielding, none had a relapse in the eyes but two of the 139 (1.4%) had a CNS relapse. The incidence of cataracts without shielding was 90% (19 of 21 evaluable patients), while with shielding it was 31% (20 of 64). Severe cataracts were present in eight of 21 (38%) patients without and two of 64 (3%) patients with shielding. The probability of staying cataract free for at least five years was 0.77 with and 0.33 without shielding, at 8 years it was 0.53 and 0.24 respectively. The relative risk of developing a cataract without shielding vs shielding was three (95% CI=1.5; 5.9). It appears that the incidence of relapse in the eyes and CNS is not increased when the eyes are shielded during TBI. Shielding increased the latency time of cataract formation and decreased the severity of cataracts.

Adolescent↗
PubMed2002

[Favorable current prognosis after HLA-identical bone marrow transplantation for children with required severe aplastic anemia; evaluation of 30 years of bone marrow transplantation at the Leiden University Medical Center].

OBJECTIVE: To evaluate the results of 30 years of allogeneic HLA-identical bone marrow transplantation (BMT) as the treatment for children with acquired severe aplastic anaemia. DESIGN: Retrospective, descriptive. METHOD: Of all patients who underwent an HLA-identical sibling-donor BMT for severe aplastic anaemia at the Department of Paediatrics, Leiden University Medical Center, in the period 1971-2000, and had a follow-up period of at least 1 year, the medical data were reviewed. The patients were split into 2 groups: patients transplanted before 1989 (n = 24), and patients who had their BMT from 1989 onwards (n = 20). This was due to a change in the treatment policy, namely a reduction in the period between diagnosis and BMT, resulting in fewer blood transfusions as well as changes in the prophylaxis against graft-versus-host disease (GvHD) from 1989 onwards (combination therapy using methotrexate and cyclosporin). RESULTS: There was an increase in the 1-year actuarial survival rate from 67% in the period before 1989 to 90% thereafter. The incidence of GvHD has significantly decreased since the introduction, in 1989, of the combination therapy using methotrexate and cyclosporin, with only 1/20 patients suffering from acute GvHD versus 13/24 prior to 1989 (p = 0.002). No patients acquired chronic GvHD after 1989, whereas before 1989, 10 patients had acquired this (p = 0.001). CONCLUSION: The prognosis of allogeneic HLA-identical sibling transplantation for paediatric patients with severe aplastic anaemia has considerably improved over the last 30 years due to improved supportive care, a significant decrease in GvHD and a shorter period between diagnosis and BMT, with the result that less blood transfusions have been required and less sensitisation has occurred. The long-term survival chance has increased to 90%.

Anemia, Aplastic↗
PubMed2002

Value of panoramic radiographs in paediatric pre-bone marrow transplantation oral evaluation.

Panoramic radiographs (orthopantomogram [OPT]) are, beside clinical examination, helpful in detecting possible dental foci of infections before bone marrow transplantation (BMT). The value of an OPT in paediatric BMT-recipients was assessed by the results of dental evaluation, consisting of an OPT and a consultation by an oral surgeon, in 161 children between 1987 and 1999. Eleven out of 161 children had at least one oral focus of infection that required treatment before myeloablative therapy. In seven children the foci were detectable both clinically and radiographically and in two children the foci were visible only clinically. Only two children had a focus that was only detectable by means of radiographs. In both patients it concerned erupting third molars. In conclusion, we recommend an OPT only when the child has clinically obvious abnormalities. In addition, in older children an OPT will be indicated to evaluate the third molars.

Adolescent↗
PubMed2000

An infant with severe combined immunodeficiency syndrome, an alpha-thalassemia trait and renal Fanconi syndrome.

We describe an infant with severe combined immunodeficiency syndrome and an alpha-thalassemia trait who developed a renal Fanconi syndrome after his first stem cell transplantation. This syndrome consists of a generalized failure of proximal tubular reabsorption, which leads to a large number of metabolic disturbances. The etiology varies from inherited causes, including an idiopathic form, to acquired causes such as intoxications, immunological disorders and hemoglobinopathies. In this case report we discuss possible explanations of the Fanconi syndrome in our patient.

Consanguinity↗
PubMed1990

Late effects of total body irradiation and cytostatic preparative regimen for bone marrow transplantation in children with hematological malignancies.

Twenty-seven children, surviving disease-free for more than 1 year after allogeneic bone marrow transplantation (BMT) for hematological malignancy were evaluated for the long-term effects on endocrine function, sexual development, physical growth, appearance of ocular cataract and psychological sequelae. The growth rate was not decelerated in the prepubertal period in children not affected by chronic graft-versus-host (GVH) disease and without previous cranial irradiation. Development of sexual characteristics was delayed in 4 relevant cases. Thyroid function was not adversely affected, gonadal function was impaired in girls, transplanted after menarche, ocular cataract developed in all cases, irradiated without shielding of the eyes after 4 years. Psychologically, children after BMT had an advantageous social development.

Adolescent↗
PubMed1988

Leukemia relapse in host cells 5 years after bone marrow transplantation for acute non-lymphocytic leukemia in first complete remission.

A case of leukemia relapse in a 15-year-old girl occurring 5 years after successful allogeneic bone marrow transplantation for acute non-lymphocytic leukemia is reported. Laboratory findings demonstrated that this relapse was in host cells and had the same phenotype as the original leukemia. Incomplete lymphoid chimerism after bone marrow transplantation might have resulted in an inappropriate graft-versus-leukemia effect.

Adolescent↗
PubMed1987

[Acute leukemia in Fanconi's anemia. Report of a patient and review of the literature].

A case is reported of a 7 years old boy with Fanconi's Anemia (FA). The aplastic anemia has been treated with androgens. Six years after the confirmation of the diagnosis FA a (pre-)leukemia occurred. Because of the known complications of cytostatics in FA and the bad prognosis, the leukemia has not been treated. The boy died 4 months later. An overview of the literature shows 31 patients with acute non-lymphocytic leukemia in FA, of which the clinical course, chromosomal investigations and therapy are discussed.

Acute Disease↗
PubMed1980

Colony forming units in culture in childhood aplastic anemia.

The results of the determination of the numbers of colony forming units in culture of the bone marrow of 17 children with aplastic anaemia before and after bone marrow transplantation, of 4 children with antilymphocyte globulin and of 16 children treated conventionally are presented. In the aplastic phase the number of C.F.U.-C. was very low to zero. After successful transplantation the number of bone marrow C.F.U.-C. rose but did not become normal. After antilymphocyte globulin and conventional therapy the number of bone marrow C.F.U.-C. remained low, even when a restoration of the haematological values in the peripheral blood took place.

Anemia, Aplastic↗