Ariane Biebl

CD14 C-159T and toll-like receptor 4 Asp299Gly polymorphisms in surviving meningococcal disease patients.

Background Carriage of Neisseria meningitidis occurs approximately in 10% of the population, onset of invasive meningococcal disease (IMD) cannot be predicted and differs between ages. It remains unclear, which host factors determine invasion of the bloodstream by the bacteria. Innate immunity has a very important role in the first recognition of invading pathogens. The functional single nucleotide polymorphisms (SNPs) CD14 C-159T and toll-like receptor 4 (TLR4) Asp299Gly have been associated with the risk of gram-negative infections. However, their role in development of IMD still remains unclear. Our aim was to investigate the influence of CD14 C-159T and TLR4 Asp299Gly polymorphisms on the risk of IMD. Methodology/Principal Findings It was a retrospective case control study. Surviving Austrian meningococcal disease patients were enrolled by sending buccal swabs for DNA analysis. 185 cases with a proven meningococcal infection and 770 healthy controls were enrolled. In surviving meningococcal disease patients DNA analysis of CD14 C-159T and TLR 4 Asp299Gly polymorphisms was performed, as they are part of the innate immune response to bacterial determinants. CD14 C-159T and TLR4 Asp299Gly SNPs were not significantly associated with the presence of IMD when compared to healthy controls. The odds ratio for CD14 C-159T SNP was 1.14 (95% confidence interval (CI) 0.91–1.43; p = 0.266). In TLR4 Asp 299 Gly SNP the odds ratio was 0.78 (CI 0.47–1.43; p = 0.359). Conclusion/Significance We could not observe a significant influence of CD14 C-159T and TLR4 Asp299Gly polymorphisms on the risk of developing IMD in surviving meningococcal disease patients. To our knowledge, this is the first study investigating the influence of the CD14 C-159T SNP on the susceptibility to IMD.

Aminoterminal brain-type natriuretic peptide levels correlate with heart failure in patients with bidirectional Glenn anastomosis and with morbidity after the Fontan operation

OBJECTIVE The aims of this study were to generate normal values of aminoterminal pro-brain natriuretic peptide in children with a bidirectional Glenn anastomosis without congestive heart failure and to test the hypothesis that plasma levels of aminoterminal pro-brain natriuretic peptide correlate with the clinical severity of congestive heart failure and morbidity after the Fontan operation. METHODS Aminoterminal pro-brain natriuretic peptide plasma levels of 78 patients after the bidirectional Glenn operation with a median age of 3.2 years and a median follow-up time of 3 years were measured by using an automated enzyme immunoassay. The severity of heart failure was quantified by using the New York University Pediatric Heart Failure Index. RESULTS The 97.5th percentile of aminoterminal pro-brain natriuretic peptide level in patients without congestive heart failure was 339 pg/mL. Aminoterminal pro-brain natriuretic peptide levels strongly correlated with the New York University Pediatric Heart Failure Index score (P < .001). In patients with congestive heart failure (31/78), the aminoterminal pro-brain natriuretic peptide levels were significantly higher (median, 670 pg/mL) than in patients without congestive heart failure (median, 171 pg/mL). In 41 patients who underwent the Fontan operation, the time to removal of chest tubes and the length of hospital stay positively correlated with the preoperative value of aminoterminal pro-brain natriuretic peptide. CONCLUSIONS In children with a bidirectional Glenn anastomosis without signs of heart failure, aminoterminal pro-brain natriuretic peptide levels were within the normal range and correlated with the severity of congestive heart failure. Further studies are needed to determine whether aminoterminal pro-brain natriuretic peptide levels can aide clinicians in the early detection of congestive heart failure in this patient group.

Vaccine strategies of meningococcal disease: results of a 10-year population-based study.

Invasive meningococcal disease (IMD) is an important cause of morbidity and mortality in children and adults. This study was conducted to determine a possible increase in IMD in recent years with special interest focused on serogroup C disease. From January 1st 1993 to December 31st 2002, IMD was studied in one million residents of Austria. We used active, population-based surveillance data from the Office of Public Health. A total of 126 patients with positive blood and/or cerebrospinal fluid culture or positive swabs for Neisseria meningitidis were studied. The median age of all patients was 9.5 years (range 1 month to 63 years). The average incidence of all IMD subgroups was 1.05 cases per 100,000 person years and was highest in children 0–4 years old (7.08 cases per 100,000 person years) followed by young adults aged 15 to 19 years (4.35 cases per 100,000 person years). Serogroup C IMD occurred in 1.30 cases/100,000 person years in patients aged 0 to 4 years and in 1.92 cases/100,000 person years in patients aged 15 to 19 years. Overall mortality was 11.1%. There was a significant increase ( P =0.001) in IMD due to serogroup B disease within the last 10 years. In contrast, serogroup C disease did not increase during the last decade. Conclusion:Currently, we do not recommend mass vaccination against serogroup C disease in Austria, but young adults aged 15 to 19 years display a high incidence of meningococcal C disease. In this age group, vaccination against serogroup C disease should be considered.

Fatal Epstein–Barr virus encephalitis in a 12-year-old child: an underappreciated neurological complication?

Background A 12-year-old boy presented to a community hospital with fever, malaise and fatigue. A polymerase chain reaction test of the cerebrospinal fluid was positive for Epstein–Barr virus. Despite intensive care treatment and antiviral drugs, the patient died 1 month after his initial presentation.Investigations Physical examination, polymerase chain reaction analysis of the cerebrospinal fluid, brain MRI, immunohistochemistry and molecular biological investigations of postmortem brain samples.Diagnosis Epstein–Barr Virus encephalitis.Management Aciclovir, intensive care treatment with intubation, sedation, antiepileptic drugs.

Facial Nerve Paralysis in Children: Is It as Benign as Supposed?

BACKGROUND Facial nerve paralysis is a common disease in children. Most of the patients show complete recovery. This single-center cohort study exclusively included pediatric patients to investigate the outcome of all patients with facial nerve palsy. METHODS Hospital records of all the patients admitted to the Childrens Hospital in Linz between January 2005 and December 2010 with facial nerve paralysis were reviewed. Patients with peripheral facial nerve palsy were invited for clinical reevaluation between July 2011 and October 2011. The House-Brackmann score was used for reassessment. RESULTS Fifty-six patients agreed to return for an additional clinical reevaluation. Study participants were divided in two groups according to their House-Brackmann scores: group 1 (n = 44), with a score <2 were considered good outcomes, and group 2 (n = 12), with a score ≥ 2 showed persistent mild to moderate dysfunction of the facial nerve and were considered moderate outcomes. The most important finding was the difference of the reported time to remission (P = 0.003) between the groups. CONCLUSION The results of this study indicate that facial paralysis in children is not as benign as supposed. It is suggested that patients and their guardians be informed that a slight face asymmetry may persist, but functional recovery in general is excellent.

Confirmation of Host Genetic Determinants in the CFH Region and Susceptibility to Meningococcal Disease in a Central European Study Sample.

Background: Invasive meningococcal disease (IMD) is a leading cause of meningitis and severe sepsis in children and adolescents. Genetic factors are important in determining the susceptibility to and outcome of IMD. Recently, a genome-wide association study from the United Kingdom showed significant associations of single-nucleotide polymorphisms within complement factor H (CFH; rs1065489) and in CFH-related protein 3 (rs426736) with susceptibility of IMD. We report data of a genetic replication study in Central European children. Methods: The study was conducted as a retrospective case-reference study involving 248 patients with confirmed diagnosis of IMD from Austria and Germany and 835 healthy reference individuals from a multicenter German birth cohort. Results: Carriers of the minor alleles of rs1065489 and rs426736 were at lower risk of IMD [allelic odds ratio = 0.60 (0.44–0.82); P = 0.001 and 0.61 (0.45–0.83); P = 0.001]. Also, 2 major haplotypes (GT and TC) derived from the 2 single-nucleotide polymorphisms were significantly associated with IMD (P = 0.001 and P = 0.003, respectively). Conclusions: The consistency of the results between the genome-wide association study and our study population strengthens the association of CFH polymorphisms to the susceptibility of IMD. Our results support the conclusion that CFH is a critical determinant in acquiring meningococcal disease.

Prospective analysis on brain magnetic resonance imaging in children

BACKGROUND Previous studies have addressed the prevalence of incidental findings in adult populations. There are few studies following paediatric patients, most of data were retrieved retrospectively. We conducted a prospective study to determine the prevalence of incidental, pathologic and normal findings in a symptomatic paediatric population. METHODS The subjects of this prospective single centre study are 436 children aged 0-18 years with clinical symptoms and subsequent first brain MRI. Normal, incidental as well as pathologic MRI findings are documented in association with age, gender, neurological examination and previous investigations (CCT, EEG). Secondary outcome parameters are defined as MRI results and their implications. Two board-certified radiologists prospectively analysed MR images without knowing the result from each other. RESULTS The 436 patients with brain MRI were categorized into three groups as follows: 155 (35.5%) patients had normal findings, 163 (37.4%) had incidental findings and 118 (27.1%) had pathological findings in brain MRI. When adding patients with pathologic and incidental findings we report even more (47.9%). We analysed the correlation between neurologic examination and MRI result and it was significant (p-value 0.0008). The p-value for concordance of both radiology reports was <0.001 and therefore highly significant. CONCLUSION To our knowledge this is the first prospective paediatric study reporting the prevalence of normal, pathologic and incidental findings in brain MRI in symptomatic children. Incidental findings are common in paediatric patients but we report the highest prevalence. Our data may help guiding management decision in a consistent and clinically appropriate manner.

Status epilepticus, cardiac resuscitation, and posterior reversible encephalopathy syndrome after ingestion of viscous lidocaine: a plea for more childproof packaging of pharmaceuticals.

Abstract Ingestion of viscous lidocaine in children can lead to potentially lethal neurologic and cardiac effects. We report the case of a 2-year-old boy who developed posterior reversible encephalopathy syndrome 2 days after unobserved ingestion of about 500 mg viscous lidocaine (40 mg/kg of bodyweight). Initially, the child presented with convulsive status epilepticus and subsequent cardiac arrest necessitating cardiopulmonary resuscitation for eight minutes. After 2 days of full recovery, the child presented with progressive disorientation, dizziness, and visual neglect. Lasting for 2 days, these symptoms finally disappeared completely. Combined with the findings on cerebral magnetic resonance imaging, this episode was interpreted as posterior reversible encephalopathy syndrome. Two weeks after the ingestion, no neurologic and visual abnormalities were found. Viscous lidocaine is prescribed routinely for dentition or other painful lesions in the oral cavity in children. Despite the potential hazardousness of the drug, packaging of viscous lidocaine is not childproof. Therefore, physicians have to instruct the parents carefully to minimize the risk of overuse or accidental ingestion. In general, the use of viscous lidocaine should be limited.

Education, school type and screen time were associated with overweight and obesity in 2930 adolescents

This cross‐sectional study analysed the influence of socio‐economic factors on screen time, overweight and obesity.

Fatal cerebral haemorrhage in a hypertensive seven-year-old boy:

A seven-year-old boy died from autopsy-proven brain haemorrhage due to hypertensive vasculopathy. This emphasizes the importance of early diagnosis and therapy of hypertension in children. Brain haemorrhage is a potentially fatal complication of paediatric hypertension.