Man with cherubism

Cherubism was diagnosed in a male when he was 6 years old. Cherubism is a benign, bilateral, painless lesion. It is commonly located in the mandible but in a minority of patients also in the maxilla. Cherubism is a familial condition with an autosomal dominant pattern of inheritance. At least one member of the family of the patient described had cherubism. The disease becomes manifest during early childhood and progresses until puberty when it spontaneously regresses. In the majority of patients no treatment is required. However, in the patient presented surgical procedures and odontological corrections were necessary. Due to a flare-up of the disease at the age of 22 years, the patient was treated with calcitonin for 1 year followed by bisphosphonates. During these treatments the disease symptoms diminished.     

Isolation of a Theileria species from Eland (Taurotragus oryx) infective for cattle.

Theileria infections were induced in cattle by feeding ticks on them from 3 sources: (a) adult rhipicephalid ticks obtained from the vegetation in a paddock containing an eland EAO at the Animal Orphanage, Nairobi National Park, Kenya, (b) Rhipicephalus appendiculatus adults fed as nymphs on the same eland, (c) R. pulchellus adults fed as nymphs on an eland W 68 captured in the Machakos district of Kenya. Both eland were harbouring Theileria parasites at the time nymphal ticks were fed. Mild infections were produced when adult ticks from these 3 batches were applied to cattle associated with low numbers of schizonts and piroplasms. The indirect fluorescent antibody test demonstrated that cattle recovered from infections resulting from the above 3 tick batches from eland W 68 and EAO produced antibodies which reacted with schizont antigen of the Theileria species (eland) and Theileria species (Githunguri) which had been isolated from cattle and not to antigens of other Theileria species used. The cattle recovered from the Theileria species (eland) were fully susceptible to a lethal challenge of a T. parva (Muguga) stabilate. It was concluded that the Theileria species (eland) and Theileria species (Githunguri) may be closely related and could represent a new species of Theileria infective to cattle.     

Health-related quality of life problems of children aged 8–11 years with a chronic disease

In paediatric research, Health-Related Quality-of-Life (HRQoL) has received increasing recognition as an important health outcome. This study aimed to investigate the nature and prevalence of HRQoL problems in children with different chronic diseases. Data were available on 318 children aged 8–11 years with different diseases: congenital heart disease (n?=?50); coeliac disease (n?=?105); asthma (n?=?32); cancer (n?=?23); juvenile chronic arthritis (n?=?45); children with capillary haemangioma (n?=?25) and severe meningococcal disease (n?=?38). They all answered a validated generic instrument [TNO-AZL Children's Quality of life questionnaire] (TACQoL), in the outpatient clinic or at home. Analyses of variance were performed to investigate differences in mean scores for children with chronic conditions in comparison to healthy children. Prevalence of children at risk for substantial HRQoL problems was based on the 25th percentile in the norm population. In comparison to healthy children, only a small number of differences were found in mean scores of children studied. In contrast, prevalence of HRQoL problems in children with chronic diseases was higher in several domains. It is concluded that using an indicator variable of the norm 25th percentile seems important in identifying at-risk children with chronic disease.     

Health related quality of life in parents of six to eight year old children with Down syndrome

Raising a child with Down syndrome (DS) has been found to be associated with lowered health related quality of life (HRQoL) in the domains cognitive functioning, social functioning, daily activities and vitality. We aimed to explore which socio-demographics, child functioning and psychosocial variables were related to these HRQoL domains in parents of children with DS. Parents of 98 children with DS completed the TNO-AZL adult quality of life questionnaire (TAAQOL) and a questionnaire assessing socio-demographic, child functioning and psychosocial predictors. Using multiple linear regression analyses for each category of predictors, we selected relevant predictors for the final models. The final multiple linear regression models revealed that cognitive functioning was best predicted by the sleep of the child (β = .29, p < .01) and by the parent having given up a hobby (β = ?.29, p < .01), social functioning by the quality of the partner relation (β = .34, p < .001), daily activities by the parent having to care for an ill friend or family member (β = ?.31, p < .01), and vitality by the parent having enough personal time (β = .32, p < .01). Overall, psychosocial variables rather than socio-demographics or child functioning showed most consistent and powerful relations to the HRQoL domains of cognitive functioning, social functioning, daily activities and vitality. These psychosocial variables mainly related to social support and time pressure. Systematic screening of parents to detect problems timely, and interventions targeting the supportive network and the demands in time are recommended.     

Anxiety and Depression in Mothers and Fathers of a Chronically Ill Child

We aimed to determine the levels of anxiety and depression in mothers and fathers of a chronically ill child (0–18 years) and to study which parental and child variables are associated with anxiety and depression. In a cross-sectional design, anxiety and depression were assessed with the Hospital Anxiety and Depression Scale. Scores were compared to a Dutch reference group by analysis of variance and logistic regression analysis. Linear regression analyses were performed to examine which variables were associated with anxiety and depression. Mothers of a chronically ill child (n = 566) scored significantly higher than the reference group (p < .001) on anxiety (Mean 5.9 vs 4.8) and depression (Mean 4.5 vs 3.1). Fathers (n = 123) had higher depression scores (Mean 4.5 vs 3.6; p < .05), but fathers’ anxiety scores were comparable to the reference group. The percentages of mothers in the clinical range of anxiety (31.8 vs 20.7 %, OR 2.03, 95 % CI 1.46–2.83) and depression (23.0 vs 12.0 %, OR 2.76, 95 % CI 1.84–4.13) were higher (p < .001) than in the reference group. No differences were found for fathers in the clinical range for anxiety and depression. Practical problems in daily life (a: β = .33, d: β = .25) and parenting stress (a: β = .30, d: β = .32) showed the strongest association with anxiety and depression for parents as a group. Illness-related characteristics of the child were not related. Parents of a chronically ill child, especially mothers, reported high levels of anxiety and depression. Awareness about parental anxiety and depression in pediatrics is important as well as targeted interventions.     

Quality of life and behavioral functioning in Dutch pediatric patients with hereditary spherocytosis

The objective of this study was to evaluate health-related quality of life (HRQoL) and behavioral functioning in pediatric patients with hereditary spherocytosis (HS). A cross-sectional study was conducted in 132 Dutch children and adolescents with HS and aged 8–18 years of whom 48 underwent splenectomy prior to the study. HRQoL was assessed using the KIDSCREEN-27, and behavioral functioning was evaluated using the strength and difficulties questionnaire (SDQ). Scores of pediatric patients with HS were compared to a Dutch norm population. Additionally, the effects of three factors were assessed: fatigue, self-image, and parents’ perceived vulnerability (measured with the checklist individual strength, the self-perception profile for children and adolescents, and the child vulnerability scale). Both unsplenectomised and splenectomised pediatric patients reported lower HRQoL on the domain physical well-being (KIDSCREEN-27) compared to Dutch peers. For behavioral functioning, parents of both groups reported more emotional problems (SDQ) compared to the norm population. Pediatric patients with lower scores on physical well-being experienced more fatigue. The patients’ perceived social acceptance and parents’ perceived vulnerability appeared as determinants of emotional problems. Conclusion: Pediatric patients in the current study generally report few complaints, and the results suggest that these patients overall have a strong ability to cope with HS. Despite these few complaints, fatigue and parents’ perceived vulnerability seem to be important determinants for lower HRQoL and more emotional problems. Therefore, screening on these factors could serve as an addition to the treatment of HS, to help pediatric patients who are at risk for lower HRQoL or more emotional problems.