J. Kernahan

The long-term effects of treatment on the dental condition of children surviving malignant disease

Fifty‐two long‐term survivors of childhood leukemia or solid tumors had a clinical dental examination along with 49 of their siblings. The 52, with an additional 30 examined in a previous study, were studied radiologically with a panoramic tomogram. All children with leukemia had received chemotherapy for 2 or 3 years and irradiation on standard protocols and the solid tumor group had received chemotherapy for 6 to 24 months. There was no difference between siblings and patients for dental caries, gingivitis, and oral hygiene, mouth opening, overjet, and overbite. More solid‐tumor patients had abnormal occlusion (P < 0.02) and those with abnormalities tended to have been treated at an earlier age. Enamel opacites and hypoplasia were more common in patients than siblings and in the leukemia than in the solid tumor group. Sixty‐five percent of the children had abnormalities on radiologic examination including failure of the tooth to develop, small crown, hypoplasia of the crown, and abnormal root development. In most cases the radiologic abnormality could be correlated in time with the patients treatment and a knowledge of the normal time of tooth development. Three teeth extracted during the course of the study were examined histologically and these showed prominent incremental lines which could be correlated in time with vincristine treatment.

Development of a measure to assess the perceived illness experience after treatment for cancer.

The development of a scale to measure perceived illness experience in young people with cancer is described. Areas of concern were first identified through semistructured interviews conducted with children and adolescents. As a result, 78 items were generated to cover the main areas identified (physical appearance, interference with activity, peer rejection, integration in school, manipulation, parental behaviour, disclosure, preoccupation with illness, and impact of treatment). These items were rated (on five point scales) by 41 patients (mean age 14.6 years) and 35 of their parents. Measures of physical functioning (symptoms, functional disability, and restrictions) and psychological functioning (symptoms) were included for validation purposes. Test-retest reliability was calculated on the basis of ratings made by a subsample of parents on two separate occasions. A 34 item scale was constructed with four items in each of the areas identified above, except for physical appearance (n = 2). The scale has adequate internal reliability and validity. There were significant correlations between parents and their children on all subscales except for illness disclosure and impact of treatment, suggesting that parents may be less reliable informants for their children in these contexts. The scale has potential use in clinical contexts, in evaluating the psychosocial impact of different treatment regimens, and as an outcome measure in intervention work.

Attentional ability among survivors of leukaemia.

Attentional ability in 19 survivors of acute lymphoblastic leukaemia and 19 sibling controls was assessed using a neuropsychological model of attention. Analysis revealed that children who had received treatment for leukaemia exhibited significantly poorer performance on measures of the “focus encode” and “focus execute” elements of attention and on measures of the ability to respond to external cues and feedback. No significant differences in performance were found for measures of sustained attention and the ability to shift attention. These results indicate that children who have received treatment for leukaemia may experience highly specific attentional deficits that could have an impact on academic performance, particularly mathematical and reading skills. It is suggested that this underlying attentional deficit might be the source of the neuropsychological sequelae associated with the disease. Future attempts at remediation should incorporate activities specifically designed to ameliorate focusing difficulties.

Natural killer cell activity in childhood acute lymphoblastic leukaemia in remission

Summary. Fifteen children with acute lymphoblastic leukaemia (ALL) in remission receiving maintenance chemotherapy and 12 ALL patients off treatment and in remission were tested for natural killer (NK) cell activity in vitro. Compared with a control population the children with ALL receiving maintenance chemotherapy had low levels of NK cell activity. This effect was not due to a specific reduction in NK cell numbers since proportions of mononuclear cells detected by the monoclonal antibodies HNK‐1 (Leu‐7) and Leu‐11a were normal. Furthermore NK cell activity in patients could only be partially increased by pre‐incubation of effector cells with interferon (αIFN). These studies confirm the lack of NK cell activity in children with ALL and show that this phenomenon is directly related to functional NK cell impairment. Our study has further shown that this effect is transient since ALL patients off treatment and in remission showed normal levels and augmentation of NK cell activity.

Dental health of survivors of malignant disease.

A full dental examination on 64 children aged from 3 to 20 years who were in long term remission from malignant disease showed normal facial growth, caries incidence, and periodontal indices. There was increased incidence of hypodontia and hypoplasia which in some could be ascribed to the original disease or its treatment.

Virus infections in children with acute lymphoblastic leukaemia.

The pattern of virus isolation and illness was studied in 64 children with acute lymphoblastic leukaemia (ALL) during periods of apparent infection and when the children were well. The virus isolation rate of 2.2 viruses per child a year is similar to that previously found in normal children. In only 32% of children with symptoms were viruses found and 14.5% had viruses isolated when asymptomatic. The children with ALL appear to be more vulnerable to multiple virus infections and to excrete the virus for longer periods. This may be due to failure of production of both local and systemic antibodies. The failure in the past to recognise the true importance of virus infections in ALL may have been due to inadequate diagnostic techniques.

Memory after treatment for acute lymphoblastic leukaemia.

Long term survivors of acute lymphoblastic leukaemia (ALL) often experience cognitive difficulties, which may be related to impairment of memory function. Memory ability has been studied in a group of survivors of ALL along with sibling controls and in children who have received treatment for other forms of cancer. Children in the ALL group were found to have significant deficits in memory function in tasks which required the application of strategic planning behaviour. These deficits are potentially remediable by educational strategies.

Role of infection in the death of children with acute lymphoblastic leukaemia

Twenty-four consecutive deaths from a total of 70 children receiving treatment for acute lymphoblastic leukaemia (ALL) have been reviewed. An attempt has been made to ascribe the cause of death to either infection, haemorrhage, the leukaemia itself, or a combination of these factors. No child was free of infection at death. Infection, with or without haemorrhage, was responsible for the deaths of all 15 children whose leukaemia had not relapsed. Although infection was present at death in all 9 children whose leukaemia had relapsed, the leukaemia process itself was also a major contributing factor. Viruses were associated with death in many of the children and may be emerging as important pathogens in children with ALL. Familiarity with a protocol may be an important factor in the prevention of fatal infections in such children. Centralization of treatment is necessary if this expertise is to be acquired.

Relapse of acute lymphoblastic leukaemia 14 years after presentation : use of molecular techniques to confirm true re-emergence

SUMMARY. Late relapse after successful treatment of acute lymphoblastic leukaemia (ALL) in children is well recognized but rare. It is often uncertain whether this represents a true relapse of the original disease or a second malignancy. We present the case of a patient who relapsed 14 years after the original diagnosis of childhood ALL in whom both the orignal leukaemic cells and those taken at relapse had an identical T cell receptor gamma (TCRG) gene rearrangement. This analysis confirms that this relapse is a true re‐emergence of the patients original disease. The term ‘cure’ should be used with caution in childhood ALL, even after long periods in continuous remission.

Chronic myelomonocytic leukemia with t(13;14) in a child

Bone marrow cells from a child with chronic myelomonocytic leukemia showed an acquired, non-Robertsonian translocation between chromosomes 13 and 14, t(13;14)(q12.?2;q32.?3). This rearrangement has not previously been reported in childhood myeloproliferative or myelodysplastic disorders.