Klinische Padiatrie最新文献

{"title":"Editorial.","authors":"Anna Wiemers, Stefanie Dillenhöfer, Anne Schlegtendal","doi":"10.1055/a-2241-9672","DOIUrl":"10.1055/a-2241-9672","url":null,"abstract":"","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":"236 2","pages":"55"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139931748","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pulmonary Alveolar Proteinosis and new therapeutic concepts.","authors":"Claudio Rodriguez Gonzalez, Hannah Schevel, Gesine Hansen, Nicolaus Schwerk, Nico Lachmann","doi":"10.1055/a-2233-1243","DOIUrl":"10.1055/a-2233-1243","url":null,"abstract":"<p><p>Pulmonary alveolar proteinosis (PAP) is an umbrella term used to refer to a pulmonary syndrome which is characterized by excessive accumulation of surfactant in the lungs of affected individuals. In general, PAP is a rare lung disease affecting children and adults, although its prevalence and incidence is variable among different countries. Even though PAP is a rare disease, it is a prime example on how modern medicine can lead to new therapeutic concepts, changing ways and techniques of (genetic) diagnosis which ultimately led into personalized treatments, all dedicated to improve the function of the impaired lung and thus life expectancy and quality of life in PAP patients. In fact, new technologies, such as new sequencing technologies, gene therapy approaches, new kind and sources of stem cells and completely new insights into the ontogeny of immune cells such as macrophages have increased our understanding in the onset and progression of PAP, which have paved the way for novel therapeutic concepts for PAP and beyond. As of today, classical monocyte-derived macrophages are known as important immune mediator and immune sentinels within the innate immunity. Furthermore, macrophages (known as tissue resident macrophages (TRMs)) can also be found in various tissues, introducing e. g. alveolar macrophages in the broncho-alveolar space as crucial cellular determinants in the onset of PAP and other lung disorders. Given recent insights into the onset of alveolar macrophages and knowledge about factors which impede their function, has led to the development of new therapies, which are applied in the context of PAP, with promising implications also for other diseases in which macrophages play an important role. Thus, we here summarize the latest insights into the various forms of PAP and introduce new pre-clinical work which is currently conducted in the framework of PAP, introducing new therapies for children and adults who still suffer from this severe, potentially life-threatening disease.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"73-79"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10883756/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139575564","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Kids Lung Registry and Child-EU Project - Progress in Rare and Interstitial Lung Diseases in Childhood Through Collaboration].","authors":"Matthias Griese, Angelika Gold, Florian Gothe, Hannah Kaiser, Birgit Kammer, Matthias Kappler, Ingrid Krueger-Stollfuss, Julia Ley-Zaporozhan, Katarzyna Michel, Christina K Rapp, Simone Reu-Hofer, Hans Rock, Andrea Schams, Nguyen-Binh Tran, Nicolaus Schwerk","doi":"10.1055/a-2214-7090","DOIUrl":"10.1055/a-2214-7090","url":null,"abstract":"<p><strong>Background: </strong>Progress in rare and interstitial lung disease in childhood can most usefully be achieved through systematic, registry-based collection.</p><p><strong>Question and methods: </strong>What are the practicalities and benefits of participating in the pediatric lung registry/chILD-EU project? We report our clinical experiences.</p><p><strong>Results: </strong>Pediatricians and pediatric pulmonologists identify children with rare lung diseases. These are reported to the Kid's Lung Register after parental consent. Clinical data, imaging, and blood are sent to the registry. Genetic analysis can be arranged if desired. With completeness of the data, a peer-review process by pediatric radiology, possibly lung pathology, clinical and possibly genetic experts takes place in an interdisciplinary conference. A working diagnosis is established and communicated to the responsible physician via the registry and, if necessary, further discussed in case-related discussions. Assistance in entering the data is provided by the registry. Follow-ups are performed annually, and all registered physicians are invited to regular, web-based case discussions. Significant questions are answered in scientific projects and jointly published (>110 publications to date).</p><p><strong>Conclusions: </strong>Due to voluntary additional work of all participants beyond clinical routine, more than 1000 children with rare lung diseases have been included in the registry with biobank to date. A deeper understanding of the clinical courses of large cohorts of rare diseases and the initial description of new entities contributes to better care for these children.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"80-96"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138806555","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Subjective Smell Disturbances in Children with Sars-Cov-2 or Other Viral Infections do not Correspond with Olfactory Test Results.","authors":"Hanna Grote, Anna Hoffmann, Sebastian Kerzel, Hannah Lukasik, Christoph Maier, Claire Mallon, Anne Schlegtendal, Michaela Schwarzbach, Konstantin van Ackeren, Stefan Volkenstein, Folke Brinkmann","doi":"10.1055/a-2208-6245","DOIUrl":"10.1055/a-2208-6245","url":null,"abstract":"<p><strong>Background: </strong>Olfactory dysfunction associated with SARS-CoV-2 infection in children has not been verified by a validated olfactory test. We aimed to determine whether these complaints are objectifiable (test-based hyposmia), how often they occur during acute SARS-CoV-2 infection compared to other upper respiratory tract infections (URTI), as well as in children recovered from COVID-19 compared to children with long COVID.</p><p><strong>Methods: </strong>Olfactory testing (U-sniff test; hyposmia<8 points) and survey-based symptom assessments were performed in 434 children (5-17 years; 04/2021-06/2022). 186 symptom-free children served as controls. Of the children with symptoms of acute respiratory tract infection, SARS-CoV-2 PCR test results were positive in 45 and negative in 107 children (URTI group). Additionally, 96 children were recruited at least 4 weeks (17.6±15.2 weeks) after COVID-19, of whom 66 had recovered and 30 had developed long COVID.</p><p><strong>Results: </strong>Compared to controls (2.7%), hyposmia frequency was increased in all other groups (11-17%, p<0.05), but no between-group differences were observed. Only 3/41 children with hyposmia reported complaints, whereas 13/16 children with complaints were normosmic, with the largest proportion being in the long-COVID group (23%, p<0.05).</p><p><strong>Conclusion: </strong>Questionnaires are unsuitable for assessing hyposmia frequency in children. Olfactory complaints and hyposmia are not specific for SARS-CoV-2 infection. The number of complaints in the long-COVID group could result from aversive olfactory perception, which is undetectable with the U-sniff test.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"129-138"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139541091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Impact of Reanalysis of Nitrogen Multiple-Breath Washout on its Relationship with Chest Magnetic Resonance Imaging Findings in Clinically Stable and Pulmonary Exacerbated Children with Cystic Fibrosis.","authors":"Maria Meißner, Eva Steinke, Mark Oliver Wielpütz, Cornelia Joachim, Olaf Sommerburg, Marcus Alexander Mall, Mirjam Stahl","doi":"10.1055/a-2214-7217","DOIUrl":"10.1055/a-2214-7217","url":null,"abstract":"<p><strong>Rationale: </strong>Multiple-breath washout (MBW)-derived lung clearance index (LCI) detects lung disease in children with cystic fibrosis (CF). Correction of a cross-talk error in the software of the MBW device Exhalyzer D in a new software version has generated significant interest regarding its impact on previous MBW findings. Since LCI and chest magnetic resonance imaging (MRI) correlated before in CF children, this study aims to reassess previous MBW data after correction.</p><p><strong>Patients/methods: </strong>Reanalysis of the main findings from a previously published study comparing MBW and MRI in a pediatric CF cohort by reassessment of nitrogen (N₂) MBW of 61 stable children with CF, 75 age-matched healthy controls (HC), and 15 CF children with pulmonary exacerbation (PEx) in the corrected software version.</p><p><strong>Results: </strong>The corrected LCI (N₂LCI_cor) decreased in the entire cohort (-17.0 (11.2)%), HC (-8.5 (8.2)%), stable CF children (-22.2 (11.1)%), and within the PEx group at baseline, at PEx and after antibiotic therapy (-21.5 (7.3)%; -22.5 (6.1)%; -21.4 (6.6)%; all P<0.01). N₂LCI_cor and N₂LCI_pre correlated with chest MRI scores in stable CF (r=0.70 to 0.84; all P<0.01) without a significant difference between N₂LCI_cor and N₂LCI_pre. Change in LCI from baseline to PEx and from PEx to after therapy decreased from N₂LCI_pre to N₂LCI_cor, but these changes remained significant (all P=0.001).</p><p><strong>Discussion/conclusions: </strong>Our results indicate that N₂LCI_cor is significantly lower than N₂LCI_pre, but key results published in the original study demonstrating N₂MBW and MRI as complementary methods for clinical surveillance in children with CF remain unaffected.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"106-115"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138806557","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Are Questionnaires Helpful To Predict Exercise-Induced Bronchoconstriction (EIB) And Exercise-Induced Laryngeal Obstruction (EILO)?","authors":"Melanie Dreßler, Hannah Lassmann, Celine Eichhorn, Jordis Trischler, Martin Hutter, Stefan Zielen, Johannes Schulze","doi":"10.1055/a-2151-2269","DOIUrl":"10.1055/a-2151-2269","url":null,"abstract":"<p><strong>Objective: </strong>Exercise induced laryngeal obstruction (EILO) is an important differential diagnosis to exercise induced bronchoconstriction (EIB) and diagnosed via continuous laryngoscopy while exercising (CLE). However, availability of CLE is limited to specialized centres. And without CLE EILO is often misdiagnosed as EIB. Therefore it is essential to carefully preselect potential EILO candidates. Aim of this study was to investigate whether two short questionnaires -Asthma Control Test (ACT) and Dyspnea Index (DI) evaluating upper airway-related dyspnea- can differentiate between EIB and EILO.</p><p><strong>Methods: </strong>Patients with dyspnea while exercising were analysed with an exercise challenge in the cold chamber (ECC) to diagnose EIB in visit 1 (V1), as appropriate a CLE in visit 2 (V2, 4-6 weeks after V1) and ACT and DI in V1 and V2. EIB patients were treated with asthma medication after V1.</p><p><strong>Results: </strong>Complete dataset of 36 subjects were gathered. The ACT showed lower values in V2 in EILO compared to EIB patients. A lack of improvement in ACT in V2 after asthma medication of EIB patients is suspicious for additional EILO diagnosis. The DI showed higher values in V1 in EILO compared to EIB patients. A score≥30 can predict a positive CLE reaction.</p><p><strong>Conclusion: </strong>ACT and DI are valuable tools in preselecting CLE candidates to assure timely diagnostic despite limited diagnostic capabilities.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"139-144"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139575559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Necessity of Tobramycin trough Levels in Once Daily Iv-Treatment in Patients with Cystic Fibrosis.","authors":"Anne Schlegtendal, Sophia Rettberg, Christoph Maier, Folke Brinkmann, Cordula Koerner-Rettberg","doi":"10.1055/a-2244-6903","DOIUrl":"10.1055/a-2244-6903","url":null,"abstract":"<p><strong>Background: </strong>Once daily intravenous (iv) treatment with tobramycin for Pseudomonas aeruginosa infection in patients with cystic fibrosis (pwCF) is frequently monitored by measuring tobramycin trough levels (TLs). Although the necessity of these TLs is recently questioned in pwCF without renal impairment, no study has evaluated this so far. The aim of this observational study was to evaluate the frequency of increased tobramycin TLs in pwCF treated with a once daily tobramycin dosing protocol.</p><p><strong>Methods: </strong>Patient records of all consecutive once daily iv tobramycin courses in 35 pwCF between 07/2009 and 07/2019 were analyzed for tobramycin level, renal function, co-medication and comorbidity.</p><p><strong>Results: </strong>Eight elevated TLs (2.9% of 278 courses) were recorded in four patients, two with normal renal function. One of these resolved without adjustment of tobramycin dosages suggesting a test timing or laboratory error. In the other patient the elevated tobramycin level decreased after tobramycin dosage adjustment. Six of the elevated levels occurred in two patients with chronic renal failure. In 15 other patients with reduced glomerular filtration rate (GFR) (36 courses) but normal range creatinine no case of elevated tobramycin trough levels was detected. Neither cumulative tobramycin dosages nor concomitant diabetes or nutritional status were risk factors for elevated TLs.</p><p><strong>Conclusion: </strong>Our data show that elevated tobramycin TLs are rare but cannot be excluded, so determination of tobramycin TLs is still recommended for safety.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"116-122"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139575561","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"[Emergency Care Plans for the Management of Emergencies in Children on Home Mechanical Ventilation].","authors":"Florian Stehling, Anke Wendt, Michael Berger, Sebastian Kerzel, Dejan Vlajnic, Hans Fuchs, Lennart Gunst","doi":"10.1055/a-2235-7805","DOIUrl":"10.1055/a-2235-7805","url":null,"abstract":"<p><p>In pediatrics chronic respiratory insufficiency is increasingly treated on an outpatient basis with home mechanical ventilation. Nursing and medical teams with different structures take care of the often complex ill children in the outpatient setting. Structured treatment processes, especially emergency plans for the management of respiratory emergencies of home mechanical ventilated children are lacking. This article is a proposal for emergency management of respiratory infections, emergencies of non-invasively ventilated and invasively ventilated, tracheotomized children. In addition to resuscitation measures according to ERC/AHA, the focus is primarily on secretion management, as well as on the handling of ventilators and devices.</p>","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":"57-63"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10883754/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139575531","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editorial.","authors":"Philippe Stock","doi":"10.1055/a-2241-9696","DOIUrl":"10.1055/a-2241-9696","url":null,"abstract":"","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":"236 2","pages":"56"},"PeriodicalIF":1.0,"publicationDate":"2024-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139931749","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"False Laboratory Thyroid Function Tests after Biotin Treatment in a Neonate with Shock and Suspect of Metabolic Disorder.","authors":"Martin Mavany, Oliver Blankenstein, Christoph Bührer, Christof Dame","doi":"10.1055/a-2209-3722","DOIUrl":"https://doi.org/10.1055/a-2209-3722","url":null,"abstract":"","PeriodicalId":17846,"journal":{"name":"Klinische Padiatrie","volume":" ","pages":""},"PeriodicalIF":1.0,"publicationDate":"2024-01-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139642464","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}