Management of chronic immune thrombocytopenia in children and adolescents: lessons from an Austrian national cross-sectional study of 81 patients.

Chronic immune thrombocytopenia (cITP) is often associated with an underlying predisposition towards autoimmunity, recognition of which is relevant to guide treatment. International recommendations on diagnostic steps and therapeutic measures of cITP in childhood exist. However, due to the low prevalence (1-2/100,000) and a variation of availability of immunological and hematological tests and treatments across pediatric units, we postulated that these guidelines are not uniformly adhered to and that immune dysregulation syndromes remained undiscovered. To delineate the current management of children and adolescents with cITP in Austria, we performed a nationwide cross-sectional study. Between 2011 and 2014, 81 children with cITP were seen at seven centers (median age 8.75 years; range 1-17; female:male ratio 47:34) at 641 visits during 180 patient years after diagnosis of cITP (>12 months ITP duration). Additional diagnoses were noted, most frequently immune or autoimmune disorders, hematologic diseases, or infections (in 37.3%, including Evans syndrome, autoimmune lymphoproliferative syndrome, systemic lupus erythematosus, and Fanconi anemia), or other symptoms like bi- or pancytopenia (n=9), lymphoproliferation or granulomatous inflammation (n = 3). Both decision to treat as well as choice of treatment varied: smaller centers tended to observe more frequently, larger centers applied a pattern of treatment modalities that appeared to depend less on bleeding tendency than on center policy. More than 50% of therapeutic interventions occurred in bleedings scores ≤2 (of 5), suggesting a strong psychosocial intention to treat. Platelet increment upon 479 therapeutic interventions of eight types was evaluated, with multiple treatment approaches being pursued sequentially in refractory patients. These data confirm the hypothesis of heterogeneous diagnostic and therapeutic management of cITP in Austrian children and corroborate the need for (1) a precise panel of parameters to exclude underlying disorders and (2) for biomarkers to predict treatment response.

Long-Term Respiratory Support for Children and Adolescents in Austria: A National Survey.

BACKGROUND: Population-based data on pediatric patients on long-term respiratory support (LTRS) in Austria are lacking. This study aimed to record the pediatric departments active in this field, as well as number and characteristics of patients on LTRS. METHODS: A national cross-sectional study was carried out by means of questionnaires sent to all pediatric departments in Austria. RESULTS: All departments answered to the questionnaires. On June 1st, 2013, the reference day for this study, 12 of the 41 pediatric departments in Austria were active in the field. At this time, these centers were caring for 143 patients, 111 (77.6%) of them under 18 years, which corresponds to a prevalence of 7.4 per 100 000. The patients suffered from neuromuscular disorders (44%), other neurological disorders (18.9%), disorders of respiratory drive (9.1%), obstructive sleep apnea (8.4%), thoracal and spinal diseases (8.4%), pulmonary disorders (4.9%) and other diseases (6.3%). Continuous positive airway pressure was used in 6.3%, non-invasive ventilation in 60.1% and invasive ventilation in 33.6% of the patients, respectively. LTRS was performed at home in 92.3%. CONCLUSION: LTRS represents a common management strategy in children and adolescents with a variety of disorders. Census reports such as this one provide the basis for appropriate planning of resource allocation. The age distribution of our patients shows the need for structured transition into adult care.

The burden of pneumococcal meningitis in Austrian children between 2001 and 2008.

UNLABELLED: The present study was conducted to evaluate the burden of pneumococcal meningitis in Austrian children between 2001 and 2008. Clinical outcome was retrospectively analyzed both on discharge and on follow-up investigations. This study was based on a prospective multicentre surveillance study on hospitalized invasive pneumococcal infections in Austrian children with a total annual "study population" of about 399,000 children aged below 5 years per year. Between 2001 and 2008, 74 cases of pneumococcal meningitis were identified in children aged below 5 years. The mean annual incidence rate for pneumococcal meningitis was 2.3 per 100,000 children in this age group. In 57/74 children (mean age on admission 14.5 ± 13.3 months), outcome data on hospital discharge were available: 5 deaths (8.8%), 20 children (35.1%) with sequelae and 32 children (56.1%) without sequelae were observed. Sequelae on discharge included motor impairment in 8 children (14.0%), hearing impairment in 9 children (15.8%) and/or other complications in 14 children (24.6%). In 7/8 children with motor deficits, matching cerebral lesions were identified by neuroimaging: cerebral infarction in five children, cerebral vasculitis and cerebral abscess in one child each. In 40/57 children, long-term outcome (18.9 ± 20.2 months after discharge) could be assessed: 1 child (2.5%) died 9 months after hospital discharge, 11 children (27.5%) had one or two long-term sequelae and 28 children (70.0%) had no sequelae. Long-term sequelae included motor impairment in three children (7.5%), hearing impairment in nine children (22.5%) and other deficits in two children (5.0%). CONCLUSION: Our study confirms that pneumococcal meningitis causes high mortality and severe long-term sequelae. On long-term follow-up, we observed improvements of motor impairment, but not of hearing impairment.

Baby sleeping bag and conventional bedding conditions--comparative investigations by infrared thermography.

BACKGROUND: Thermal stress is a risk factor for sudden infant death syndrome (SIDS). Recently, baby sleeping bags have been recommended as a preventive measure against SIDS. The aim of this study was to describe in which way the use of baby sleeping bags might influence thermoregulation of sleeping infants and maybe the incidence of SIDS. METHODS: Body surface temperature was recorded by use of infrared thermography in 15 infants (median age 49 days). Recordings were done twice: after sleeping for 60 min under a blanket and after sleeping for 60 min in a baby sleeping bag. Temperature was recorded and compared for defined sites of body surface. RESULTS: Infants' mean body surface temperature as well as core temperature after sleeping in a baby sleeping bag did not show significant differences when compared to infants sleeping under a conventional blanket. CONCLUSION: Under controlled conditions, core temperature and mean body surface temperature are comparable, equally if using a baby sleeping bag or conventional bedding. However, under the more uncontrolled conditions of baby care at home, sleeping bags might provide a more constant temperature profile, while other bedding conditions may lead to significant variations of temperature pattern.

Sleep habits and sleep complaints in Austria: current self-reported data on sleep behaviour, sleep disturbances and their treatment.

OBJECTIVES: To acquire current information on sleep habits, disturbances and treatment options in the adult population of Austria and compare results with previously collected data. MATERIALS AND METHODS: A representative sample of the Austrian population (women: n = 522, men: n = 478). RESULTS: Seventy-five percent reported daily sleep-duration between 6 and 8 h. In 76%, sleep latency was <30 min, 15% described difficulties in sleep maintenance. Longer sleep on weekends was prevalent in 54%, 23% took a nap. Concerning sleep environment, 31% reported sleeping alone; the rest had a constant or occasional bed partner. Sleep disturbances such as sleep disruption or prolonged sleep latency were reported by 18%. Predominant symptoms included snoring/apneas (22%), nightmares (22%) and restless legs (21%). Daytime tiredness was reported by 17% and sleepiness by 20%. Twenty-four percent did not take treatment. Only 7% asked for medical help: 96% consulted their physician; 47% tried to change their way of living. Sleep promoting drugs were taken by 7%. Sleep improving measures were: sleep promoters (45%), general measures (20%), consultation of general practitioner (20%), psychotherapy (6%), and technical tools (3%). Comparison with a dataset of 1993 revealed only a slight increase in short sleepers and a slight decrease in long sleepers. CONCLUSIONS: Subjectively reported sleep disorders proved to be relatively stable between 1993 and 2007.

Do wet diapers induce arousals in sleeping infants?

AIM: To find out whether simulated bladder voiding was able to induce arousals in sleeping infants. METHODS: Polygraphic recordings were performed in 34 infants and voiding was simulated by administering water into the diaper. RESULTS: Heart rate, respiratory frequency and electroencephalogram frequency did not change significantly during this procedure. Furthermore, simulated voiding was unable to cause an awakening or to induce body movements in sleeping infants. CONCLUSION: Simulated voiding was unable to induce arousals.

[Uterus duplex with hematometrocolpos and ipsilateral agenesis of the kidney]. / Uterus duplex mit Hämatometrokolpos und ipsilateraler Nierenagenesie.

BACKGROUND: Malformations of the mullerian-duct-system occur with an incidence of about 0.001-0.005% of the population, and they are frequently associated with unilateral agenesis of the kidney. CASE: A 12-year-old girl presented to our emergency department with cyclic abdominal pain which increased with each of her menses. Ultrasound investigation showed a uterus duplex with obstructed hemivagina and hematometrocolpos as well as an ipsilateral absence of the kidney. Additionally, a cystic expansion in the small pelvis was detected which required further investigations (MRT and MR-angiography) to establish the diagnosis of a hematosalpinx. Excision of the vaginal septum resulted in drainage of the hemato-metrocolpos and the hematosalpinx. CONCLUSION: Unilateral hematometrocolpos should be considered as an important differential diagnosis in female adolescents with relapsing lower abdominal pain, even if normal menstruation occurs.

[Pernio as cause of a blue toe syndrome]. / Pernio als Ursache eines Blue-toe-Syndroms.

The evaluation of a blue toe syndrome in children is a diagnostic challenge. The differential diagnosis includes pernio (chilblains) if all diagnostic tests are negative and a corresponding anamnesis is presented. In this report we illustrate the case of a 7 1/2- year-old girl who presented with two blue toes and discuss differential-diagnostic considerations.

Procalcitonin--a marker of invasive fungal infection?

Procalcitonin (PCT) has been described as a marker of bacterial sepsis. However, little is known of its diagnostic value in fungal infections. We calculated the sensitivity of PCT for detection of invasive fungal infections (IFI) by analyzing 55 episodes of proven or probable IFI (three in our series, 52 reported in the recent literature). In the early phase of IFI, PCT was elevated in fewer than half of invasive candidiasis episodes and in only one patient (5.3%) with invasive aspergillosis. Due to low sensitivity and specificity, PCT adds little to the diagnosis of IFI.

[Investigation of sleep disorders in the vicinity of high frequency transmitters]. / Untersuchung von Schlafstörungen um Hochfrequenz-Sendeanlagen.

To investigate the potential impact of RF electromagnetic fields of transmitters on the sleep quality of nearby residents, a new study design is presented. In a double-blind crossover field study the effect of on-site shielding, rather than of additional exposure, is investigated. For improved sleep quality differentiation the polysomnographic parameters are expanded by additional parameters. The feasibility study showed that checking the raw data and correcting the software-generated results by visual reading of the polysomnographic recordings is essential. Long-term RF measurement showed that exposure may vary considerably throughout the night, as well as from one night to the next. This variation may be greater than the GSM contribution itself. Mostly, the contributions of USW radio frequency fields dominated over GSM. Thus, continuous broadband RF recording is required for reliable interpretation of the results, in particular with regard to the potential role of mobile telephony emissions. Results show that simple sleep monitoring systems based on single-channel EEG analysis without acces to original biosignals are not adequate for sleep studies.

Sudden infant death syndrome risk questionnaire: a mirror of parental awareness rather than a prospective diagnostic tool.

AIM: In 1989, a scoring system that aimed to identify infants at risk of sudden infant death syndrome (SIDS) by a structured questionnaire [SIDS risk questionnaire (SRQ)] consisting of 25 items was introduced in Styria (Austria). It was the aim of the study to compare SIDS rates in the population that had access to the SRQ with the population that had no access. Furthermore, for the population receiving the SRQ, the responding (compliant) and non-responding (non-compliant) groups were compared concerning the incidence of SIDS. METHODS: Within the study period, 53 865 births and 57 SIDS cases were recorded (incidence 1.06/1000) and analysed retrospectively. RESULTS: The incidence of SIDS was significantly higher in the non-responding population (2.36/ 1000) than in the responding group (0.81/1000, p < 0.001). However, the incidence of SIDS was not significantly different in the population that had access to the risk questionnaire (1.29/1000) and the group without access (0.86/1000, p = 0.145). CONCLUSION: The value of any questionnaire used for SIDS prevention may be limited by the existence of a non-compliant population which represents a risk group and should be targeted by other preventive measures.