The results of a longitudinal study conducted with children undergoing treatment for acute lymphoblastic leukaemia (ALL) and their families in Brisbane, Australia, indicate that the emotional impact of one of the protocol drugs, dexamethasone, is acutely distressing. The findings presented cover the second interviews with the parents and children of the first 11 ALL families to have completed the re-consolidation stage of treatment. The results indicate that the negative impact of this drug is particularly severe during the reconsolidation stage, when families are exhausted with coping with the intensity of treatment. Thus, the administration of dexamethasone is a critical point in the pathway of care for children with ALL. The emotional consequences of the drug are profoundly disturbing, not only for the child, but for the whole family. The findings indicate that the period when dexamethasone is being administered is an important time for providing families with emotional support and information about likely sequelae of treatment. Because of guilt and self-doubt parents will not necessarily seek help, even if it is greatly needed. Recommendations are provided as to possible ways of reducing the distressing impact of the administration of this pharmaceutical intervention.
This is a novel case report of alloimmune neonatal neutropenia (ANN) linked to the neutrophil antibody anti-HNA-4a (MART). Since its discovery, the HNA-4a antigen has never been associated with any clinical neutropenia. A first-born neonate with respiratory distress was found to be severely neutropenic, because of ANN. The broad reactivity of the antibody together with its capture by CD11b and CD18 in monoclonal antibody immobilization of granulocyte antigen test suggested HNA-4a specificity. DNA sequencing confirmed that the father is HNA-4a-positive and that the mother is HNA-4a-negative, supporting the diagnosis of ANN linked to MART.
Iron deficiency is prevalent and an important determinant of child health. Precise and accurate diagnosis remains challenging. Iron supplementation is required for premature and low-birthweight infants. Oral iron salts remain the recommended treatment of choice in most instances.
H aem olytic-u raem ic syndrome (HUS) is characterised by the triad of microangiopathic haemolytic anaemia, thrombocytopenia and acute renal insufficiency, and is a major cause of acute renal failure in childhood.' HUS has been broadly classified into two forms: the typical or epidemic form, also known as diarrhoeaassociated (D+) HUS, and the atypical or sporadic form, diarrhoea-negative (D-) HUS.2,3 Most children with D+ HUS recover spontaneously, whereas those with D-HUS have a poorer prognosis.v' Acute mortality ranges from 5% to 10%.4,5We conducted a retrospective survey of patients with HUS in the Brisbane area over a 16-year period. Our aim was to review the features and management of HUS and to determine whether features that differed between D+ and D-HUS were predictive of clinical course and development of chronic renal failure.
Methods
AbstractObjective: To review the clinical course of haemolytic-uraemic syndrome (HUS) in children admitted to Brisbane children's hospitals between April 1979 and October 1995. Design: Retrospective case survey. Setting: Royal Children's Hospital and Mater Misericordiae Children's Hospital (the two major children's hospitals in Brisbane).Subjects: All children hospitalised for HUS. Outcome measures: Clinical and laboratory features on presentation (including typical [diarrhoea-positive, D+] or atypical [diarrhoea-negative, D-] presentation), clinical course, treatment and features on subsequent outpatient follow-up (1, 3, 6 and 12 months later), renal outcome on long term follow-up (3-16 years later).Results: 55 children (aged 2 months to 13 years) were hospitalised for HUS, but no epidemic was detected. Seven children (13%) had D-presentations, including three (5%) with T-activation caused by pneumococcal pneumonia. Thrombocytopenia was more severe and prolonged in D-patients (P
This study compared the prognosis of patients treated for aplastic anaemia at Great Ormond Street Hospital for Children from 1973-88 (group A; n = 38) with a more recent cohort from 1989-96 (group B; n = 37). The two groups were similar in terms of clinical history, age, and severity of aplasia. The main treatment diVerences included the use of androgen treatment in group A (21 of 38 patients) but not in group B, and the addition of cyclosporin A to immunosuppressive treatment for 14 patients in group B. Actuarial survival at eight years' follow up was significantly better for group B (84%; 95% CI, 68% to 93%) than for group A (45%; 95% CI, 30% to 60%), because of improved outcome for both immunosuppressive treatment (86% v 39%) and bone marrow transplantation (93% v 56%). There was no evidence for late clonal disorders or secondary malignancies in survivors in either group. The prognosis for aplastic anaemia has improved greatly in recent years so that over 80% of children are long term survivors. (Arch Dis Child 1999;80:158-162)
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