[Good primary care can give support and help to severely ill/dying children and their families].
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to S Garwicz.
Explore the source record for details and available documents.
Hepatocellular carcinoma and obliterated hepatic bile duct were found at postmortem examination in a 4-year-old girl with arteriohepatic dysplasia (Alagille's syndrome). AFP level was extremely high. Liver cirrhosis was present on percutaneous needle biopsy 9 months before she succumbed in progressive liver failure. Episodes of repeated gastrointestinal, life-threatening hemorrhages occurred during the last 6 months of her life. Histopathologic findings of the eyes were documented at autopsy.
In this population-based study, 808 children aged 1-15 years from Denmark, Finland, Iceland, Norway and Sweden, were diagnosed between July 1981 and June 1986 as suffering from non-B-cell acute lymphoblastic leukemia (ALL). The total population was 4.5 million children. Remission was achieved in 770/808 of the patients (95%). No sex difference in the remission rate was observed. The event free survival (EFS) at 102 months was 0.47 for males and 0.62 for females (p less than 0.001). There was no difference in EFS between males and females with standard-risk (0.58 and 0.60) or intermediate-risk (0.47 and 0.60) ALL, respectively. The EFS for females with high-risk ALL (0.68) was superior to that of males with high-risk ALL (0.31). Cox multivariant analysis showed that white blood cell count, sex, age and thrombocyte count were significant prognostic factors in all children. The intensified treatment according to the prognostic factors used in this study led to equal EFS for females with ALL from all risk groups. Males with high-risk ALL, however, did not benefit from the intensified treatment.
The breakpoints in chromosome 22 were determined in five children with Philadelphia-positive chronic myeloid leukemia. All had rearrangements within the major breakpoint cluster region (M-bcr). Four patients had breakpoints in the 5' region of M-bcr (zones 1-3), whereas one had a rearrangement in the 3' region (zone 4). The patient with the 3' rearrangement was the only one to develop a lymphoid blast crisis; he also had a substantially longer survival (102 months) than the others (11-54 months).
Among a cohort of 981 children who were followed up 4.3-26.5 years after cessation of antileukemic therapy, eight patients in remission of acute lymphoblastic leukemia (ALL) developed a distinctively new malignant disease. The second malignant neoplasms (SMN) included brain tumors, basal cell carcinomas, thyroid cancer, leiomyosarcoma and finally rhabdomyosarcoma in a patient who also had suffered from Hodgkin's disease while still on antileukemic treatment. Cranial radiation had been given to 58.4% of the patients in the study group, which consisted of 895 ALL patients who had completed various chemotherapy protocols. With one exception, the SMN appeared after 7.5-16.5 years at a location previously exposed to radiotherapy (RT). The estimated cumulative risk of SMN appearing within 20 years after diagnosis was 2.9%, and the corresponding risk for cases with RT was 8.1% compared to 0.3% for those without (p = 0.05). In a Cox regression analysis, the incidence rate ratio of SMN between patients with and without RT was 6.7 (95% CI = 0.8, 57.7). Based on age-, year- and sex-specific cancer incidence figures for Norway, the overall standardized incidence rate ratio (SIR) of SMN after treatment for ALL was 5.9 (95% CI = 2.2, 12.9). The number of brain tumors among patients who had received cranial radiation was nearly 27 times greater than expected, whereas no such tumors were seen after chemotherapy. Individuals treated for childhood ALL are at increased risk of a new malignancy, and this seems mainly to be associated with previous irradiation.
Quantification of platelet-bound immunoglobulin is widely used in the evaluation of thrombocytopenia. Several methods have been devised among which labelled ligand-binding assays seem to be most appropriate. In series of adult patients such assays have been shown to be superior in separating immune-thrombocytopenia from thrombocytopenia of non-immune causes. We studied 62 children with thrombocytopenia of various causes, using radiolabelled protein A as a ligand to measure platelet-surface bound IgG. The test was highly sensitive (93%) in detecting immune-thrombocytopenia. The specificity, however, was only 57%, which is less than in published studies of adults. In a number of cases presumed to be non-immune-thrombocytopenia, notably a few patients with leukaemia and bone marrow aplasia, we found increased amounts of platelet surface-bound IgG. The significance of this finding is not clear. An indirect assay measuring platelet-specific IgG in plasma was less sensitive (46%) but highly specific for immune-thrombocytopenia (89%). The measurements of platelet-surface-bound IgG and platelet-specific IgG in plasma are of limited diagnostic value in childhood thrombocytopenia but are useful in following the treatment in chronic ITP.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
The rapid development of chemotherapy and radiotherapy during the last decade makes an increasing demand for a reliable classification of malignant non-Hodgkin's lymphomas. This is especially important in children since in this age group the lymphomas show a much worse prognosis than in adults. An attempt at a modern classification is offered by the so-called Kiel classification. 38 children with non-Hodgkin's lymphomas previously classified according to Rappaport were re-evaluated according to the Kiel system. Reclassification was technically feasible in 26 patients. There was good agreement on typing between the two independently working cytopathologists. 3 patients proved to be cases of histiocytic medullary reticulosis. Among the remaining patients, a much larger variety of histological subgroups was seen than in the one published paediatric series of Lennert. 17 patients had high-grade malignant lymphomas with lymphoblastic lymphomas predominating. Some clinical correlations not detectable with the Rappaport classification were found using the Kiel system. 6 patients were judged as having low-grade malignant lymphomas but in this group the survival was poorer than expected and 1 patient showed leukaemic transformation. The Kiel classification makes high demands for adequate surgical techniques and preparatory routines. It seems to represent a step forward in the classification of non-Hodgkin's lymphomas but much more experience is needed, especially in children, in order to evaluate its role as a guide to differentiated therapy.
Explore the source record for details and available documents.
Echocardiography was used to evaluate left ventricular function in 8 children treated with adriamycin for malignant disease. Preejection period (PEP), left ventricular ejection time (LVET) and percent change in left ventricular internal dimension with systole (delta LVID) were measured before, during and immediately after 22 injections of adriamycin as well as 14 injections of other cytotoxic drugs and physiologic saline. No immediate effects on left ventricular function could be discerned. When functional parameters were evaluated longitudinally in patients with relatively higher cumulative doses of adriamycin, percent change in left ventricular internal dimension with systole showed some tendency to decrease, while the other parameters remained essentially unchanged.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Cerebral cortical calcification identical to that of the Sturge-Weber syndrome was observed in two children. In one child the calcification appeared after intrathecal administration of methotrexate and skull irradiation because of leukemia involving the central nervous system. In the other child, who had coeliac disease and epilepsy, the calcification appeared after treatment with anticonvulsants. This treatment was also contributing to the development of profound megaloblastic anemia. The unspecificity of the Sturge-Weber calcification is stressed and the hypothesis is put forward that the calcification may be secondary to folic acid deficiency interfering with the matabolism in the central nervous system.
Explore the source record for details and available documents.
It is proposed that atypical spherocytosis is a result of severe spherocytosis combined with splenic dysfunction. The spleen exercises a "conditioning" effect on the red blood-cell but is insufficient in the "culling" and "pitting" functions. As a result, a large population of severely damaged erythrocytes is circulating in the peripheral blood giving rise to atypical autohaemolysis.
Massive leukaemic involvement of the intestine appeared in a 9-year-old girl with acute lymphoblastic leukaemia. The unusual feature in this case was that the gut involvement occurred during complete haematological remission. Surgical and subsequent radiological treatment completely eradicated the engagement and at autopsy 9 months later there were no signs of the intestinal involvement.
Out of 12 children with acute lymphoblastic leukaemia treated with craniospinal irradiation during primary haematologic remission, 8 developed a postirradiation syndrome characterized by fever and tiredness. The symptoms lasted 1-2 weeks and subsided spontaneously. Longitudinal EEG studies revealed no acute disturbances during the irradiation therapy but in all cases studied, moderate to severe diffuse general slowing developed during the postirradiation syndrome. Complete normalization of the EEG occurred in all children at follow-up. It is concluded that the described EEG abnormalities constitute an integral part of the postirradiation syndrome.