Application of UKHCDO 2004 guidelines in type 1 von Willebrand Disease ‐ a single centre paediatric experience of the implications of altered or removed diagnosis

Summary. Diagnosis of type I von Willebrand Disease (VWD) can be challenging. In 2004, the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) proposed more stringent diagnostic criteria to replace the 1995 guidelines. To determine the true number of cases of type 1 VWD in a single paediatric centre, the 2004 UKHCDO Guideline for the diagnosis of VWD was used to evaluate 114 patients on our type 1 VWD register. Clinical and laboratory data were collected and analysed to see whether they met the criteria for type 1 VWD. Only 8% remained on the type 1 VWD register. 18% have been classified as ‘possible type 1 VWD’. Twenty five surgical procedures have since been performed on patients from the group in which the diagnosis was removed without any haemostatic support or bleeding complications. Reaction to the removal of the VWD diagnosis or delivery of an alternative diagnosis was positive for most patients and families. This study is the first to assess the impact of the 2004 UKHCDO Guidelines on the diagnosis of VWD. It provides evidence that the prevalence of type 1 VWD may actually be closer to that of haemophilia instead of the previously reported 1–3% of the general population. We propose that all centres should review their patients with a diagnosis of VWD to revalidate this disease that claims to be our most common inherited bleeding disorder.     

Rising Charges and Costs for Pediatric Catheter Ablation

Pediatric Ablation Charges and Costs Are Rising. Introduction: Catheter ablation has been shown to be effective for pediatric tachyarrhythmias, but the associated charges and costs have not been described in the recent era. Understanding such contemporary trends may identify ways to keep an effective therapy affordable while optimizing clinical outcomes. Methods: We used the 1997–2009 Kids’ Inpatient Databases to examine trends in charges and costs for pediatric catheter ablation and identify determinants of temporal changes. Results: There were 7,130 discharges for catheter ablation in the sample. Mean age at ablation was 12.1 ± 0.2 years. Patients with congenital heart disease (CHD) made up 10% of the sample. Complications occurred in 8% of discharges. Mean total charges rose 219% above inflation (from $23,798 ± 1,072 in 1997 to $75,831 ± 2,065 in 2009). From 2003 to 2009, costs rose 25% (from $20,459 ± 780 in 2003 to $25,628 ± 992 in 2009). Charges for ablation increased markedly relative to surgical procedures, but with a similar slope to other catheter‐based interventions. Multivariable analysis revealed that year (P < 0.0001), payer (P = 0.0002), CHD (P < 0.0001), valvular heart disease (P = 0.0004), cardiomyopathy (P = 0.0009), hospital region (P < 0.0001), length of stay (P < 0.0001), and complications (P < 0.0001) predicted increased charges. The same factors also predicted increased costs. Charges and costs varied considerably by region, particularly for high‐volume centers (P < 0.0001). Conclusions: Charges and costs for pediatric catheter ablation increased relative to other procedures and significantly outstripped inflation. Further study of complications, length of stay, and regional differences may help control rising costs while maintaining quality of care. (J Cardiovasc Electrophysiol, Vol. 24, pp. 162‐169, February 2013)     

Prevalence and aetiology of neurological impairment in extremely low birthweight infants

Objective : To determine the prevalence and perinatal predictors of cerebral palsy, intellectual impairment, visual impairment and deafness in a cohort of extremely low birthweight (ELBW) infants at two years of age.
Methodology : The study population comprised 199 of the 224 (89%) ELBW infants managed at the Mater's Mothers Hospital, Brisbane, between July 1977 and February 1990 and who survived to two years. The prevalence of cerebral palsy, intellectual impairment, blindness and deafness was measured by clinical, psychometric and audiological assessment and the association with 24 risk factors examined.
Results : Cerebral palsy occurred in 20 children (10%). Risk of cerebral palsy was associated with ventricular dilatation, intraventricular haemorrhage, necrotizing enterocolitis and multiple birth, though only ventricular dilatation (OR 4.41; 95% Cl 1.32-14.8) remained significant in the adjusted analysis. Intellectual impairment occurred in 20 children (10%) and was independently associated with ventricular dilatation (OR 15.0; 95% Cl 2.2-102.8), ventilation F _iO₂ >80% (OR 3.4; 95% Cl 1.01-11.5), vaginal delivery (OR 3.5; 95% Cl 1.09-11.4) and male sex (OR 6.1; 95% Cl 1.67-22.3). No perinatal predictor was statistically associated with risk of deafness. Retinopathy of prematurity (OR 36.9; 95% Cl 2.8-495.5) was associated with risk of later visual impairment.
Conclusions : Intellectual impairment was associated with a broad range of perinatal variables. Cerebral palsy was associated with fewer variables, all of which were also associated with intellectual impairment. Neurologic injury was associated with male sex and multiple birth, which are not biological insults themselves, but may be markers of susceptibility to injury.     

Dietary manipulation in childhood atopic dermatitis

The dietary habits of 73 children seen consecutively in a dermatology out-patient department were studied to ascertain the nature and frequency of dietary manipulation and to assess any potential hazards. Although most children had only mild flexural dermatitis, 71% had had significant alterations made to their diet before attendance at hospital. Almost all diets were unsupervised and only six children had seen a dietitian. Over half had been started on diets before any professional advice had been sought. Information on diet had come from media, books or magazines (51%), friends (32%), general practitioners (27%) and district nurses or health visitors (18%). The major dietary alterations included the exclusion of eggs, dairy products and food additives, and the use of soya and goat's milk. Under 10% of patients claimed benefit from these largely unsupervised diets. Harmful practices included the frequent use of unboiled, unpasteurized goat's milk, unmodified goat's milk in a baby of 4 months and the use of severely restricted diets. Three children (6%) in the diet group had been on diets which were considered by a hospital dietitian to be particularly dangerous. However, there was no evidence of growth retardation, failure to thrive or specific deficiency syndromes in the group we studied. Uncontrolled, unsupervised and often prolonged dietary alterations are commonplace in children with atopic dermatitis. This is a potentially serious health problem.