[German S1 Guideline Long-/Post-COVID]. / S1-Leitlinie Long-/Post-COVID.

The German Society of Pneumology initiated 2021 the AWMF S1 guideline Long COVID/Post-COVID. In a broad interdisciplinary approach, this S1 guideline was designed based on the current state of knowledge.The clinical recommendations describe current Long COVID/Post-COVID symptoms, diagnostic approaches, and therapies.In addition to the general and consensus introduction, a subject-specific approach was taken to summarize the current state of knowledge.The guideline has an explicit practical claim and will be developed and adapted by the author team based on the current increase in knowledge.

[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.

The validation of a computer-adaptive test (CAT) for assessing health-related quality of life in children and adolescents in a clinical sample: study design, methods and first results of the Kids-CAT study.

PURPOSE: Recently, we developed a computer-adaptive test (CAT) for assessing health-related quality of life (HRQoL) in children and adolescents: the Kids-CAT. It measures five generic HRQoL dimensions. The aims of this article were (1) to present the study design and (2) to investigate its psychometric properties in a clinical setting. METHODS: The Kids-CAT study is a longitudinal prospective study with eight measurements over one year at two University Medical Centers in Germany. For validating the Kids-CAT, 270 consecutive 7- to 17-year-old patients with asthma (n = 52), diabetes (n = 182) or juvenile arthritis (n = 36) answered well-established HRQoL instruments (Pediatric Quality of Life Inventory™ (PedsQL), KIDSCREEN-27) and scales measuring related constructs (e.g., social support, self-efficacy). Measurement precision, test-retest reliability, convergent and discriminant validity were investigated. RESULTS: The mean standard error of measurement ranged between .38 and .49 for the five dimensions, which equals a reliability between .86 and .76, respectively. The Kids-CAT measured most reliably in the lower HRQoL range. Convergent validity was supported by moderate to high correlations of the Kids-CAT dimensions with corresponding PedsQL dimensions ranging between .52 and .72. A lower correlation was found between the social dimensions of both instruments. Discriminant validity was confirmed by lower correlations with non-corresponding subscales of the PedsQL. CONCLUSIONS: The Kids-CAT measures pediatric HRQoL reliably, particularly in lower areas of HRQoL. Its test-retest reliability should be re-investigated in future studies. The validity of the instrument was demonstrated. Overall, results suggest that the Kids-CAT is a promising candidate for detecting psychosocial needs in chronically ill children.

Implementation of the Kids-CAT in clinical settings: a newly developed computer-adaptive test to facilitate the assessment of patient-reported outcomes of children and adolescents in clinical practice in Germany.

PURPOSE: To describe the implementation process of a computer-adaptive test (CAT) for measuring health-related quality of life (HRQoL) of children and adolescents in two pediatric clinics in Germany. The study focuses on the feasibility and user experience with the Kids-CAT, particularly the patients' experience with the tool and the pediatricians' experience with the Kids-CAT Report. METHODS: The Kids-CAT was completed by 312 children and adolescents with asthma, diabetes or rheumatoid arthritis. The test was applied during four clinical visits over a 1-year period. A feedback report with the test results was made available to the pediatricians. To assess both feasibility and acceptability, a multimethod research design was used. To assess the patients' experience with the tool, the children and adolescents completed a questionnaire. To assess the clinicians' experience, two focus groups were conducted with eight pediatricians. RESULTS: The children and adolescents indicated that the Kids-CAT was easy to complete. All pediatricians reported that the Kids-CAT was straightforward and easy to understand and integrate into clinical practice; they also expressed that routine implementation of the tool would be desirable and that the report was a valuable source of information, facilitating the assessment of self-reported HRQoL of their patients. CONCLUSIONS: The Kids-CAT was considered an efficient and valuable tool for assessing HRQoL in children and adolescents. The Kids-CAT Report promises to be a useful adjunct to standard clinical care with the potential to improve patient-physician communication, enabling pediatricians to evaluate and monitor their young patients' self-reported HRQoL.

The 2009 pandemic influenza A(H1N1) coincides with changes in the epidemiology of other viral pathogens causing acute respiratory tract infections in children.

BACKGROUND: In Germany, the outbreak of the novel pandemic 2009 influenza A(H1N1) virus A(H1N1)pdm09 caused a wave of high activity between November 2009 and January 2011. The aim of this study was to investigate the prevalence of 19 respiratory pathogens in children hospitalized for lower respiratory tract infections during the winter influenza seasons of 2009/2010 and 2010/2011 and to observe a possible impact of influenza A(H1N1)pdm09 on the epidemiology of other epidemic viruses. MATERIALS AND METHODS: Specimens were nasopharyngeal aspirates which had been collected from children admitted to the participating hospitals in the area of Mainz, Wiesbaden, and Kiel, Germany, with acute community-acquired lower respiratory tract infections. The specimens were subjected to a previously described multiplex reverse transcription PCR assay to detect the following microorganisms: enterovirus, influenza virus types A and B, respiratory syncytial virus (RSV), parainfluenzavirus types 1-4, adenovirus, Mycoplasma pneumoniae, Chlamydophila pneumoniae, rhinovirus, human metapneumovirus (hMPV), coronavirus OC43 and 229E, influenza A(H1N1)pdm09, Bordetella pertussis, Bordetella parapertussis, and Legionella pneumophila. RESULTS: A total of 3,998 clinical specimens were collected from July 2009 to March 2011, of which 296 were positive for A(H1N1)pdm09. An epidemic of seasonal influenza A or B was not observed in the 2009/2010 season, but a minor epidemic of seasonal influenza B was observed in January/February 2011. Influenza A(H1N1)pdm09 coincided with the absence of the seasonal influenza A of former years. The RSV and hMPV epidemics of 2009/2010 erupted several weeks later than expected based on data collected in the PID-ARI-Network during the past 10 years, whereas in the 2010/2011 influenza season they occurred as expected. CONCLUSIONS: The emergence of the novel influenza A(H1N1)pdm09 virus may have been influenced the epidemiology of other epidemic viruses, such as the RSV and hMPV. No epidemic of seasonal influenza was observed in the 2009/2010 influenza season.

Congenital Central Hypoventilation Syndrome due to PHOX2b gene defects: inheritance from asymptomatic parents.

BACKGROUND: Congenital Central Hypoventilation Syndrome (CCHS, Ondine's curse) is a rare syndrome of dysfunction of the autonomic nervous system characterized by a decreased response to hypercarbia requiring mechanical ventilation in most cases. CCHS is an autosomal-dominant disease associated with tumors of neural crest origin, segmental aganglionosis of the colon, and diffuse autonomic dysregulation symptoms. Most cases of CCHS are caused by de novo heterozygous in-frame expansions within in the PHOX2b gene. PATIENTS AND MAIN RESULTS: Here we report two families in which a PHOX2b defect was inherited from an asymptomatic parent. In family 1 an asymptomatic mother carried a mild mutation (15 bp expansion within the polyalanine repeat) also found in her daughter who was symptomatic immediately after birth but did not require mechanical ventilation. In family 2, two newborn infants with respiratory failure due to insufficient respiratory drive requiring mechanical ventilation were born to asymptomatic parents. A 39 pb expansion within the PHOX2b polyalanine repeat was found in one patient in whom DNA was available, but not in blood leukocytes from any parent. Microsatellite analyses confirmed the identity of the parents, such that a germline mosaicism has to be deduced. CONCLUSIONS: Carriers of mild PHOX2b mutations causing disease in their offspring may be asymptomatic; Modifier genes determining the clinical course may exist. Germline mosaicism may lead to CCHS in children from unaffected parents. Genetic counseling should include these variations.

Holoprosencephaly and low molecular weight proteinuria: the human homologue of murine megalin deficiency.

We encountered a child with holoprosencephaly, pulmonary insufficiency, absent circulating vitamin D metabolites, mild albuminuria, and urinary excretion of vitamin D-binding protein. The child displayed a phenotype highly reminiscent of that observed in mice genetically deficient for megalin, a member of the low-density lipoprotein receptor superfamily. Only the Guthrie card was available from the child; the DNA sufficed for a limited haplotype analysis. We were not able to implicate the megalin gene locus directly; however, the possibility of a functional megalin defect in this child remains. To the best of our knowledge, this patient represents the first report that pathologic abnormalities consistent with megalin deficiency are present in humans.

[High-resolution computed tomography of the lungs in pediatric patients]. / Hochauflösende Computertomographie der Lunge im Kindesalter.

Since the introduction of the high-resolution technique more than ten years ago, HRCT has become an established modality for diagnosing diseases of the respiratory system. This is especially true for the diagnosis of lung diseases in adults. Experience in HRCT of the lungs is limited in pediatric patients. This review gives an overview of frequent and less frequent HRCT findings in pulmonary diseases in childhood. A purely reticular pattern is rarely observed in infants. Pulmonary diseases associated with overinflation are relatively frequent. Paired inspiratory-expiratory scans combine morphological analysis with functional information and have improved the diagnosis of air trapping, e. g. in post-infectious bronchiolitis obliterans or bronchopulmonary dysplasia. Especially in children the high radiation exposure is a problem. Even when applying a low-dose protocol the radiation dose of HRCT will still exceed the dose of a chest X-ray by 100 times. The indication for pediatric pulmonary HRCT is limited to selected cases and it should be decided in agreement with the pediatric radiologists and the pediatric pulmonologists.

[Mediastinal tumour in children misdiagnosed as bronchial asthma]. / Fehldiagnose "Asthma bronchiale" bei Mediastinaltumor im Kindesalter.

HISTORY: Three school children (2 girls and 1 boy, aged 9-15 years) complained about exercise-induced shortness of breath and stridor. The children were treated with inhalation steroids, one of them with systemic steroids, without a significant effect. INVESTIGATIONS: Because of lack of treatment response a chest X-ray was done. In all cases a mediastinal mass was visible. No radiological signs of an obstructive pulmonary disease was demonstrated. In none of the three cases did a pulmonary function tests demonstrate an obstructive pulmonary disease. DIAGNOSIS, TREATMENT, AND COURSE: Bone marrow aspiration in one girl showed an acute lymphoblastic leukemia of T-cell immunophenotype with a large mediastinal lymphoma. Lymph node biopsy in the boy demonstrated a nodular sclerosing subtype of Hodgkin's disease. In the other girl a ganglioneuroma was found. CONCLUSION: In children and adolescents a chest radiograph and pulmonary function tests should be performed if history and clinical signs are suggestive of bronchial asthma, before a specific inhalative or systemic treatment is started.

[Transoral treatment of severe laryngomalacia. Review and presentation of a modified surgical technique]. / Transorale Behandlung der schweren Laryngomalazie. Ubersicht und Beschreibung einer modifizierten Operationstechnik.

BACKGROUND: Laryngomalacia is the most common cause of stridor in children. So far various authors have described surgical techniques in a sense of a supraglottoplasty or an epiglottoplasty to handle the severe form of this disease. We present a modified technique of the so called epiglottopexy. METHODS: Under the different types of laryngomalacia this study focuses on the treatment of three patients (5 to 10 months) with a posterior displacement of the epiglottis during inspiration. The first step of our technique was to denude a small portion of the base of the tongue from mucosa. A corresponding lesion was created on the lingual surface of the epiglottis. The vaporisation of the mucosa was performed with the CO2 laser at a power setting of 1 Watt and a 0.25 mm spot size. The epiglottis was then fixed transorally to the base of the tongue with 2 single stitch sutures and 1 inverse mattress suture, using a resorbable material. RESULTS: No intra- or postoperative complications were observed. All three patients demonstrated a significant airway improvement without stridor. CONCLUSION: The presented technique of transoral laser surgical epiglottopexy seems to be a method suitable for the treatment of laryngomalacia caused by posterior displacement of the epiglottis.

Repyloromyotomy for recurrent infantile hypertrophic pyloric stenosis after successful first pyloromyotomy.

The authors report on a male infant aged 4(1/2) weeks who underwent a pyloromyotomy for hypertrophic pyloric stenosis. After an uncomplicated postoperative course with normal feeding and weight gain, projectile vomiting reoccurred. The boy underwent a repyloromyotomy for recurrent hypertrophic pyloric stenosis. The underlying cause of a recurrent hypertrophic pyloric stenosis after a successful pyloromyotomy may be explained by the natural history of the disease.

Elimination and haemodynamic effects of nitrendipine in patients with chronic renal failure.

In sixteen patients with arterial hypertension and differing degrees of renal function the pharmacokinetics and haemodynamic effects of nitrendipine have been studied after treatment for 7 days. The AUC (0-24) and the elimination half-life of nitrendipine were significantly increased; the AUC (0-24) in patients with renal failure (median creatinine clearance 27.1 ml x min-1) was 196 ng x ml-1 x h compared to 97.8 ng x ml-1 x h in control subjects (median creatinine clearance 94.4 ml x min-1). The corresponding elimination half-lives were 13.5 h in renal failure and 4.4 h in the controls. The haemodynamic effects of nitrendipine were not enhanced in the patients.

Stereoselective pharmacokinetics of oral nitrendipine in elderly hypertensive patients with normal and impaired renal function.

The pharmacokinetics of the enantiomers of nitrendipine has been studied in seven elderly patients with chronic renal failure (CRF) and in six control subjects (mean creatinine clearance 30 and 97 ml.min-1 respectively). Racemic nitrendipine 20 mg was given once daily for seven days and the pharmacokinetics of the enantiomers over the last dosage interval were determined using a stereospecific assay. In both groups nitrendipine exhibited stereoselective pharmacokinetics (AUC, Cmax), but the half-lives of the enantiomers did not differ in individual subjects. As an index of stereoselectivity, the mean S/R ratio of AUCs in control subjects (2.07) was not significantly different from the ratio in patients with CRF (2.68). The mean AUCs of (S)- and (R)-nitrendipine during the last dosage interval were increased in CRF by 132% and 85%, respectively. The observed doubling of the half-lives and the increases in Cmax did not reach significance because of the large variability in each group. Thus, the pharmacokinetics of oral nitrendipine is altered in CRF, but there was no change in the stereoselectivity of its pharmacokinetics.

Tubulointerstitial nephritis and uveitis in association with Epstein-Barr virus infection.

The case of a 13.5-year-old girl with acute tubulointerstitial nephritis and uveitis (TINU syndrome) is presented. The etiology of this rare syndrome, which in most cases involves female adolescents and usually regresses spontaneously, is still unknown. An infection-triggered pathological immune reaction has been considered to play a role in the pathogenesis of this disorder. Here we report for the first time the association of TINU syndrome and Epstein-Barr virus infection.

Epiglottopexy for the treatment of severe laryngomalacia.

Laryngomalacia is the most common cause of stridor in newborns and infants. Up until now, different surgical techniques for the treatment of this disease have been described. We report on a modified technique, the so called epiglottopexy, which is a laser-surgical treatment strategy in severe laryngomalacia. A total number of six children (ages: 6 weeks-10.4 years) were treated for life-threatening stridor, which was due to an isolated posterior displacement of the epiglottis during inspiration. In two patients, shortened aryepiglottic folds contributed to the stridor. In all of the children, a modified technique of epiglottopexy was performed transorally; in two cases, this was followed by laser surgical transection of the aryepiglottic folds. Epiglottopexy on the base of the tongue was performed using single suture stitches following laser-surgical vaporization of the corresponding mucosal areas of the epiglottis and the base of the tongue. No intra- or postoperative complications were observed. All six children demonstrated significant airway improvement without any further stridor. Deglutation was not impaired. The presented laser-surgical technique seems to be an appropriate therapy for treatment of severe forms of laryngomalacia characterized by an isolated posterior displacement of the epiglottis during inspiration.