[Guidelines for the Management of Community Acquired Pneumonia in Children and Adolescents (Pediatric Community Acquired Pneumonia, pCAP) - Issued under the Responsibility of the German Society for Pediatric Infectious Diseases (DGPI) and the German Society for Pediatric Pulmonology (GPP)]. / S2k-Leitlinie Management der ambulant erworbenen Pneumonie bei Kindern und Jugendlichen (pädiatrische ambulant erworbene Pneumonie, pCAP).

The present guideline aims to improve the evidence-based management of children and adolescents with pediatric community-acquired pneumonia (pCAP). Despite a prevalence of approx. 300 cases per 100 000 children per year in Central Europe, mortality is very low. Prevention includes infection control measures and comprehensive immunization. The diagnosis can and should be established clinically by history, physical examination and pulse oximetry, with fever and tachypnea as cardinal features. Additional signs or symptoms such as severely compromised general condition, poor feeding, dehydration, altered consciousness or seizures discriminate subjects with severe pCAP from those with non-severe pCAP. Within an age-dependent spectrum of infectious agents, bacterial etiology cannot be reliably differentiated from viral or mixed infections by currently available biomarkers. Most children and adolescents with non-severe pCAP and oxygen saturation > 92 % can be managed as outpatients without laboratory/microbiology workup or imaging. Anti-infective agents are not generally indicated and can be safely withheld especially in children of young age, with wheeze or other indices suggesting a viral origin. For calculated antibiotic therapy, aminopenicillins are the preferred drug class with comparable efficacy of oral (amoxicillin) and intravenous administration (ampicillin). Follow-up evaluation after 48 - 72 hours is mandatory for the assessment of clinical course, treatment success and potential complications such as parapneumonic pleural effusion or empyema, which may necessitate alternative or add-on therapy.

Pulmonary interstitial glycogenosis - A systematic analysis of new cases.

BACKGROUND: Pulmonary interstitial glycogenosis (PIG) is a rare paediatric interstitial lung disease of unknown cause. The diagnosis can only be made by lung biopsy. Less than 100 cases have been reported. Clinical features, treatment and outcomes have rarely been assessed systematically in decent cohorts of patients. METHODS: In this retrospective multicentre study, the clinical presentation, radiologic findings, pattern of lung biopsy, extrapulmonary comorbidities, treatment and outcome of eleven children with PIG were collected systematically. RESULTS: 10/11 children presented with respiratory distress immediatly after birth and 8/11 needed invasive ventilation. In 8/11 children extrapulmonary comorbidities were present, congenital heart defects being the most common. 7/11 children received systemic glucocorticoids and of these four showed a clear favorable response. During a median follow-up of 3.0 years (range 0.42-12.0) one child died, while 10 patients improved. Chest CT-scans showed ground-glass opacities (7/10), consolidations (6/10), linear opacities (5/10) and mosaic attenuation (4/10) without uniform pattern. Besides interstitial thickening related to undifferentiated glycogen positive mesenchymal cells all tissue samples showed growth abnormalities with reduced alveolarization. CONCLUSIONS: PIG is associated with alveolar growth abnormalities and has to be considered in all newborns with unexplained respiratory distress. Apparent treatment benefit of glucocorticosteroids needs to be evaluated systematically.

Multiple breath washout in pediatric patients after lung transplantation.

Forced expiratory volume in 1 second (FEV1 ) from spirometry is the most commonly used parameter to detect early allograft dysfunction after lung transplantation (LTx). There are concerns regarding its sensitivity. Nitrogen-multiple breath washout (N2 -MBW) is sensitive at detecting early global (lung clearance index [LCI]) and acinar (Sacin ) airway inhomogeneity. We investigated whether N2 -MBW indices indicate small airways pathology after LTx in children with stable spirometry. Thirty-seven children without bronchiolitis obliterans syndrome [BOS] at a median of 1.6 (0.6-3.0) years after LTx underwent N2 -MBW and spirometry, 28 of those on 2 occasions (≤6 months apart) during clinically stable periods. Additional longitudinal data (11 and 8 measurements, respectively) are provided from 2 patients with BOS. In patients without BOS, LCI and Sacin were significantly elevated compared with healthy controls. LCI was abnormal at the 2 test occasions in 81% and 71% of patients, respectively, compared with 30% and 39% of patients with abnormal FEV1 /forced vital capacity (FVC). Correlations of LCI with FEV1 /FVC (r = 0.1, P = .4) and FEV1 (r = -0.1, P = .6) were poor. N2 -MBW represents a sensitive and reproducible tool for the early detection of airways pathology in stable transplant recipients. Moreover, indices were highly elevated in both patients with BOS. Spirometry and LCI showed poor correlation, indicating distinct and complementary physiologic measures.

Nasal Nitric Oxide Measurement and a Modified PICADAR Score for the Screening of Primary Ciliary Dyskinesia in Adults with Bronchiectasis.

Background Determining the underlying diagnosis is essential for the targeted and specific treatment of bronchiectasis. Primary ciliary dyskinesia (PCD) is a rare genetic disease, which is characterized by abnormalities in ciliary structure and/or function and which may result in bronchiectasis. The disease is probably underestimated among adults with bronchiectasis due to the fact that extensive diagnostic testing is required and that the recognition of PCD is low. Objective To evaluate a feasible screening algorithm for PCD among adults with bronchiectasis. Methods Data from all patients who presented to our bronchiectasis outpatient clinic from June 2010 until July 2016 were retrospectively analysed from our database. Nasal NO (nNO) and a modified PICADAR score (PrImary CiliAry DyskinesiA Rule) were measured and compared in the two groups of PCD-bronchiectasis and non-PCD-bronchiectasis. Results 185 of 365 patients (75 males, 110 females) had a sufficient measurement of nNO concentration and complete clinical data and were eligible for analysis. The mean (SD) nNO concentration in nL/ml was significant lower in the PCD group compared to the non-PCD group (25 [31] and 227 [112] nL/min, respectively; p < 0.001). A nNO level of 77 nL/min had the best discriminative value to differentiate between the two groups. Patients with PCD had a significant higher modified PIDACAR score than patients without PCD (5 2 and 1 1, respectively [p < 0.001]). Using ROC curve analysis, the modified PICADAR score of 2 had the best discriminative value with a sensitivity of 1.00 and a specificity of 0.89. Conclusions Low nNO concentration and the modified PICADAR score are suitable and cheap screening tests for PCD in adults with bronchiectasis.

Back to the roots? Dual cannulation strategy for ambulatory ECMO in adolescent lung transplant candidates: An alternative?

Bridging critically ill pediatric patients to lung transplantation still remains a major challenge. Although still controversial, within the last 5 years, ECMO has been increasingly used as a bridge to lung transplantation concept in adult and pediatric patients with acceptable outcomes. The outstanding developments in the field of extracorporeal devices and the introduction of awake ECMO concepts with the avoidance of mechanical ventilation have led to a real paradigm shift in the ICU management of pretransplant candidates with severe respiratory failure. Therefore, ECMO is no longer seen as a contraindication for lung transplantation at least at our center. Nevertheless, how to bridge these patients on ECMO still remains controversial. Thus, we introduced an ambulatory ECMO approach in adolescent lung transplant candidates with acute respiratory failure using a dual cannulation strategy and hereby present first results from this procedure.

Food-induced anaphylaxis and cofactors - data from the anaphylaxis registry.

Food allergens are frequent causes of anaphylaxis. In particular in children and adolescents they are the most frequent elicitors of severe allergic reactions, and in adults food allergens rank third behind insect venom and drugs. Since July 2006 severe allergic reactions from Germany, Austria, and Switzerland are collected in the anaphylaxis registry. Currently 78 hospitals and private practises are connected. From July 2006 until February 2009 1,156 severe allergic reactions were registered. Among children and adolescents (n = 187, age range from 3 months to 17 years) food allergens were the most frequent triggers, comprising 58% of cases. In the adult group (n = 968, 18 - 85 years) food allergens were in the third position (16.3%) behind insect venom and drugs. In children legumes (31%) and in particular peanuts were frequently responsible food allergens, followed by tree nuts (25%) with hazelnut being the most frequent elicitor. In adults fruits (13.4%) most often induced severe food-dependent anaphylaxis, but also animal products (12.2%); among these most frequently crustaceans and molluscs. Cofactors were often suspected in food-dependent anaphylaxis, namely in 39% of the adult group and in 14% of the pediatric group. In adults drugs (22%) and physical activity (10%) were reported to be the most frequent cofactors, in children physical activity was suspected in 8.7% and drugs in 2.6%. Concomitant diseases like atopic dermatitis, allergic asthma, or allergic rhinoconjunctivitis were reported in 78% of children and adolescents and in 67% of the adults. In conclusion, food-induced anaphylaxis, its cofactors and concomitant diseases are age-dependent. The data offers to identify risk factors of anaphylaxis.

Treatment of allergic children - Where is the progress (for the practicing allergist)?

For any kind of therapeutic intervention in allergic diseases such as environmental control, pharmacological, or immunomodulating treatment including educational programs, children are addressed separately from adults. Health authorities like the Food and Drug Administration in the United States of America or the European Medicine Agency in Europe request a specific 'Pediatric investigational plan' with studies addressing dose-response relationship, safety, and efficacy for infants, children, and adolescents. During the last 2 years, promising advances have been reported for the treatment of a variety of allergic and immunologic disorders. This review summarizes the progress in the treatment of pediatric asthma and allergic diseases, based on publications of approximately the last 2.5 years (end of 2013 until May 2016) in and beyond this journal. Meanwhile, it highlights areas with promising novel therapeutic approaches, which are likely to change treatment for allergic children in the near future.

Monitoring of adenovirus (ADV)-specific T cells in a boy with ADV pneumonia and disseminated disease after lung transplantation.

Human adenovirus (ADV) infections are the cause of severe morbidity and mortality in transplant recipients. Cidofovir (CDV) is the current standard antiviral treatment. We report the case of a 3-year-old boy after lung transplantation with severe ADV sepsis, who was monitored for ADV-specific T cells during his disease and recovery. A strong increase in ADV-specific T cells was accompanied by resolution of ADV in blood and bronchoalveolar lavage fluid. Antiviral treatment with CDV was individually adapted according to anti-ADV immune responses, which provides a new method for tailoring antiviral treatment in lung transplant recipients.

Etilefrinhydrochloride tablet ingestion: successful therapy by endoscopic removal of tablet conglomerate.

We report here on a case of etilefrinhydrochloride tablet intoxication in suicidal intention in a female adolescent. Tablet ingestion resulted in the formation of a tablet conglomerate in the stomach, which could neither be effectively treated with activated charcoal nor by gastric lavage. Endoscopic dissemination and removal of the fragments finally led to the elimination of the tables and the symptoms of intoxication resolved completely. The case presented here offers an explanation as to why the use of activated charcoal and/or a gastric lavage may not be successful in some cases of ingestion/intoxication. Endoscopic removal of ingested fragments of the toxic substance, such as etilefrinhydrochloride tablets, may be useful even hours following ingestion and should be considered when treatment with activated charcoal or gastric lavage fail to eliminate toxic substances in cases of tablet ingestion.

Cellulitis with compartment syndrome as a complication of varicella zoster infection.

BACKGROUND: Varicella zoster infection (chickenpox) is a common and usually benign self limiting disease of childhood in non-vaccinated populations. Although varicella usually goes along with mild to moderate illness in immunocompetent patients, serious complications can arise. Varicella is highly infectious, with attack rates in susceptible individuals ranging from 61% to 100%. PATIENT: This case describes a rare but life threatening complication of varicella zoster infection in a child. CONCLUSIONS: High morbidity in non-vaccinated populations that led to rare cases of severe complications became a significant health burden all over the world. This aspect must be considered in the discussion about the importance and benefit of a population wide varicella vaccination programme as it was added to the childhood immunization schedule in Germany in 2004. The case described here might have been avoided by vaccination.

[Rare case of an epidemiologically important tuberculosis in childhood]. / Seltener Fall einer epidemiologisch bedeutsamen Tuberkulose im Kindesalter.

BACKGROUND: Tuberculosis is an infectious disease which is nearly forgotten in Germany because of its low incidence. CASE REPORT: We report on a 14-year-old german girl who was disregarded when active case-finding of her uncle's active pulmonary tuberculosis was carried out three years before. As a result she herself developed a severe infectious pulmonary tuberculosis. The delay between onset of symptoms and diagnosis gives cause for concern and led to active tuberculosis in her brother and her girl friend as well. The lack of information about tuberculosis in population and delay of medical detection in this case led unnecessarily to the continuing chain of infection. CONCLUSIONS: This case report shows that there are severe infectious courses of tuberculosis even in childhood which might get epidemiologically important. For earlier diagnosis and successful interruption of chains of infection tuberculosis in the German population even today has to be taken into account. Case detection through contact investigation of adults is of great importance in childhood and adolescence.

[Gianotti-Crosti syndrome in an infant following immunization]. / Gianotti-Crosti-Syndrom nach Impfungen bei einem Kleinkind -- Fallbericht und Ubersicht der Literatur.

Gianotti-Crosti syndrome (GCS), first described by F. Gianotti in 1955, was originally reported to be associated with hepatitis B virus infection in children. The typical clinical picture allows diagnosis of GCS at first glance. The pathogenesis is still unknown. Besides HBV infection, a large number of infectious agents, mostly viruses, have been described as a trigger of GCS. Here we report the occurrence of GCS in an infant five days after the fourth immunization against poliomyelitis, DTPa, Hib, hepatitis B and Streptococcus pneumoniae. Our data and review of the literature suggest that GCS is rarely associated with immunizations, especially when performed with inactivated vaccines.