The total rate of thrombotic complications in children with TCL and malignancy is high. A substantial proportion of thrombotic complications can be clinically silent. The use of a heparin lock once a week seems far from effective in preventing thrombotic events in patients with TCL. The TCL life span has no influence on the rate of thrombotic events.
Optimum management of hip displacement in children with cerebral palsy (CP) is facilitated by an approach that focuses on anticipatory and preventive measures. Hip surveillance programs for children with CP were developed at the beginning of the new millennium, with the purpose of identifying hip displacement sufficiently early to permit a choice of effective management options. In the early years, hip surveillance was guided by epidemiological analysis of population-based studies of prevalence. In Australia, a National Hip Surveillance in CP Working Group was first convened in 2005. This resulted in a 2008 Consensus Statement of recommendations published and endorsed by Australasian Academy of Cerebral Palsy and Developmental Medicine (AusACPDM). The group undertook that the recommendations should be reviewed every 5 years to ensure currency and congruency with the emerging evidence base. As new evidence became available, hip surveillance guidelines developed, with the most recent 2020 Australian Hip Surveillance Guidelines endorsed by the AusACPDM. Implementing comprehensive hip surveillance programs has now been shown to improve the natural history of hip dislocations and improve quality of life. Standardised hip surveillance programs can also facilitate planning for multicentre research through harmonisation of data collection. This, in turn, can help with the identification of robust new evidence that is based on large cohort or population studies. Here a review of evidence informing the updated 2020 Hip Surveillance Guidelines is presented.
Several reports indicate that lungs are the extralymphatic site most commonly affected in patients with Hodgkin lymphoma; however, the data in children are rather limited. This retrospective study aimed to assess the frequency, clinical picture, and the impact on prognosis in children with pulmonary Hodgkin lymphoma, who were diagnosed and treated in a single center during a 10-year period. Pulmonary lesions related to HL: nodules and parenchymal infiltrates with cavitations were found in 3 of 32 (9.4%) patients; in 2 cases these were found as the concomitant manifestation whereas in 1 case as the solitary form (Primary Pulmonary Hodgkin Lymphoma). B-DOPA and MVPP chemotherapy combined with mediastinal and pulmonary irradiation resulted in sustained remissions in all 3 patients, lasting 3, 7, and 64 months, respectively. Lung involvement occurs in up to 10% of children with Hodgkin lymphoma. Primary pulmonary Hodgkin lymphoma is a rare and atypical form of Hodgkin lymphoma; thus is associated with delayed diagnosis which does not seem to affect prognosis. It should be suspected in a child with non-resolving pneumonia and pulmonary parenchymal infiltrates with cavitations.
Defect in function of tumor suppressor genes may lead to initiation/progression of leukemias. RB1, CDKN2A and TP53 gene alterations are found in acute lymphoblastic leukemia (ALL) in children. Data showing a contribution of these alterations to the pathomechanism of leukemias are contradictory and their impact on a disease course still remains undefined. The main aim of the study was to identify and the characterize of RB1, CDKN2A and TP53 allele loss in ALL children patients at diagnosis. 46 children with de novo ALL were examined. Fluorescent in situ hybridization was performed on bone marrow smear preparations. We demonstrated that at least one of three investigated deletions occurred statistically more frequently in T-lineage leukemia patients (p = 0.044); this was the most frequent in respect to RB1 gene (p = 0.054). Additionally, at least one of the examined deletions was observed statistically more frequently in patients with WBC above 20 000/µl (p = 0.043), this was the most frequent for CDKN2A gene (p = 0.066). Presented results seem to give an evidence that deletions of RB1 and CDKN2A genes may contribute to the development of hyperleukocytic type of T-lineage ALL in children, nevertheless this observation needs further investigations.
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