[Is the DDAVP-test helpful to diagnose children?]. / Trägt ein DDAVP-Test bei Kindern zur Diagnose bei?

UNLABELLED: It is very difficult to determine if patients with a moderate low level of VWF parameters have mild disease or if they are just low normal (so called grey area of VWD). This applies particularly to pediatrics, because it is difficult to evaluate the bleeding history of children. Al our centres every child diagnosed with vWD gets DDAVP to test the response for it. This study was done to evaluate the DDAVP- test as a diagnostic tool. PATIENTS, METHODS: A retrospective analysis of data obtained with routine DDAVP administration for test purposes in 52 patients with borderline von Willebrand disease at the haemophilia centre Graz was done. The increase of VWF:Ag, VWF:RiCof and FVIII:C has been document and compared. RESULTS: All of our patients had a very good response after application of DDAVP. The increase of VWF:Ag, VWF:RiCof and FVIII:C was compared in patients with positive and negative bleeding anamneses. The patients with positive anamneses had significantly lower parameters at the beginning. The increase of VWF parameters did not differ significantly between the groups at the different time-points. These results demonstrate that a positive anamnesis is not significantly associated with a lower increase. On the other side a high increase is not associated with a negative anamnesis. CONCLUSION: It is not possible to use the DDAVP test as a diagnostic tool for patients within the diagnostic grey area of VWD.

An innovative local treatment for staphylococcal scalded skin syndrome.

Staphylococcal scalded skin syndrome (SSSS) is the clinical term used to describe a range of blistering skin disorders induced by the exfoliative toxins of Staphylococcus aureus and prevalently affects neonates, infants and toddlers who lack antibodies to S. aureus toxins. SSSS is a highly contagious disease and is characterised by erythema and fever, followed by the formation of large fragile superficial blisters, which rupture only to leave extensive areas of denuded skin. A diagnosis of SSSS relies on the clinical picture, as well as on histological and microbiological findings. Neonates and young infants are particularly susceptible to a lack of the protective skin barrier, which may cause excessive protein and fluid losses, hypothermia and secondary infection. Due to a complete denudation of skin, the patients also suffer from almost unbearable pain. In our communication, we present an innovative temporary coverage of the denuded skin with Suprathel (PolyMedics Innovations GmbH, Denkendorf, Germany). Suprathel relieves pain, prevents heat loss and secondary infection, accelerates wound healing, does not need to be changed and makes daily care easy for the nurses and is well tolerable for the patient.

Screening for haemorrhagic disorders in paediatric patients by means of a questionnaire.

AIM: In children, screening for haemorrhagic disorders is further complicated by the fact that infants and young children with mild disease in many cases most likely will not have a significant history of easy bruising or bleeding making the efficacy of a questionnaire even more questionable. PATIENTS, METHODS: We compared the questionnaires of a group of 88 children in whom a haemorrhagic disorder was ruled out by rigorous laboratory investigation to a group of 38 children with mild von Willebrand disease (VWD). Questionnaires about child, mother and father were obtained prior to the laboratory diagnosis on the occasion of routine preoperative screening. RESULTS: 23/38 children with mild VWD showed at least one positive question in the questionnaire, while 21/88 without laboratory signs showed at least one positive question. There was a trend to more specific symptoms in older children. Three or more positive questions were found only in VWD patients, but only in a few of the control group. The question about menstrual bleeding in mothers did not differ significantly. Sensitivity of the questionnaire for a hemostatic disorder was 0.60, while specifity was 0.76. The negative predictive value was 0.82, but the positive predictive value was only 0.52. CONCLUSIONS: Our small study shows, that a questionnaire yields good results to exclude a haemostatic disorder, but is not a sensitive tool to identify such a disorder.