Pott's disease: a major issue for an unaccompanied refugee minor

Chest clinic The incidence of TB in children in Germany has been a rise since 2008, especially among foreign-born individuals. With rapidly increasing numbers of refugees from the numerous areas of conflict, this increase in incidence is not expected to halt, neither in Germany in Europe in general. We report a case of insufficient tracking in a 16-year-old unaccompanied refugee minor from Somalia who had a positive interferon. release assay on arrival in Germany. No actions were undertaken, until 6 months later, an X-ray showed prominent hilar enlargement. Nine months later, the patient presented to our hospital with abdominal pain, vomiting and B symptoms. Workup revealed a paravertebral abscess due to Pott's disease, a skeletal manifestation of Mycobacterium tuberculosis disease. The patient made a full recovery after a combination therapy for a total of 9 months

Connected to the wrong pipe: esophageal bronchi mimicking bilateral bronchial atresia

Infants frequently present with respiratory symptoms, but diagnosing the underlying pulmonary condition is sometimes challenging. Here, we describe an infant presenting with respiratory distress due to a rare pulmonary condition. Different investigations eventually led to the correct diagnosis and the patient successfully treated. Rare respiratory conditions need to be considered to ensure early and appropriate care. A 2-week-old neonate was referred with a postnatal history of tachypnea, poor feeding and elevated plasma inflammation markers. The patient presented with mild episodes of coughing after feeding and diminished breath sounds of the right upper lung. A chest radiograph revealed bilateral consolidations. Whereas bronchoscopy was suggestive of bilateral bronchial atresia, computed tomography supported bronchial atresia of the right upper and middle lobe and a left-sided broncho-esophageal communication. Surprisingly, an upper gastrointestinal series revealed bilateral esophageal insertion of bronchi and the diagnosis of a communicating bronchopulmonary foregut malformation (CBPFM) was made. Two-stage lobectomy of the affected lobes and segments was performed at days 31 and 41 after birth. Histopathological examination exhibited hamartomatous lung tissue with purulent bronchopneumonia. At a follow-up examination after 4 years, the patient was asymptomatic and thriving well with oral feeds. CBPFM are rare malformations. This case highlights the clinical challenge of diagnosing this rare condition. There is a need to raise awareness for such uncommon conditions and improve diagnostic accuracy. For optimal management a multidisciplinary approach is essential

One-year outcomes in a multicentre cohort study of incident rare diffuse parenchymal lung disease in children (ChILD)

We performed a prospective, observational, cohort study of children newly diagnosed with children's interstitial lung disease (ChILD), with structured follow-up at 4, 8, 12 weeks and 6 and 12 months. 127 children, median age 0.9 (IQR 0.3-7.9) years had dyspnoea (68%, 69/102), tachypnoea (75%, 77/103) and low oxygen saturation (SpO(2)) median 92% (IQR 88-96). Death (n=20, 16%) was the most common in those <6 months of age with SpO(2)<94% and developmental/surfactant disorders. We report for the first time that ChILD survivors improved multiple clinical parameters within 8-12 weeks of diagnosis. These data can inform family discussions and support clinical trial measurements

FusionVAC22_01: a phase I clinical trial evaluating a DNAJB1-PRKACA fusion transcript-based peptide vaccine combined with immune checkpoint inhibition for fibrolamellar hepatocellular carcinoma and other tumor entities carrying the oncogenic driver fusion

The DNAJB1-PRKACA fusion transcript was identified as the oncogenic driver of tumor pathogenesis in fibrolamellar hepatocellular carcinoma (FL-HCC), also known as fibrolamellar carcinoma (FLC), as well as in other tumor entities, thus representing a broad target for novel treatment in multiple cancer entities. FL-HCC is a rare primary liver tumor with a 5-year survival rate of only 45%, which typically affects young patients with no underlying primary liver disease. Surgical resection is the only curative treatment option if no metastases are present at diagnosis. There is no standard of care for systemic therapy. Peptide-based vaccines represent a low side-effect approach relying on specific immune recognition of tumor-associated human leucocyte antigen (HLA) presented peptides. The induction (priming) of tumor-specific T-cell responses against neoepitopes derived from gene fusion transcripts by peptide-vaccination combined with expansion of the immune response and optimization of immune function within the tumor microenvironment achieved by immune-checkpoint-inhibition (ICI) has the potential to improve response rates and durability of responses in malignant diseases. The phase I clinical trial FusionVAC22_01 will enroll patients with FL-HCC or other cancer entities carrying the DNAJB1-PRKACA fusion transcript that are locally advanced or metastatic. Two doses of the DNAJB1-PRKACA fusion-based neoepitope vaccine Fusion-VAC-XS15 will be applied subcutaneously (s.c.) with a 4-week interval in combination with the anti-programmed cell death-ligand 1 (PD-L1) antibody atezolizumab starting at day 15 after the first vaccination. Anti-PD-L1 will be applied every 4 weeks until end of the 54-week treatment phase or until disease progression or other reason for study termination. Thereafter, patients will enter a 6 months follow-up period. The clinical trial reported here was approved by the Ethics Committee II of the University of Heidelberg (Medical faculty of Mannheim) and the Paul-Ehrlich-Institute (P-00540). Clinical trial results will be published in peer-reviewed journals.Trial registration numbersEU CT Number: 2022-502869-17-01 and ClinicalTrials.gov Registry (NCT05937295)

International management platform for children's interstitial lung disease (chILD-EU)

BACKGROUND: Children's interstitial lung diseases (chILD) cover many rare entities, frequently not diagnosed or studied in detail. There is a great need for specialised advice and for internationally agreed subclassification of entities collected in a register.Our objective was to implement an international management platform with independent multidisciplinary review of cases at presentation for long-term follow-up and to test if this would allow for more accurate diagnosis. Also, quality and reproducibility of a diagnostic subclassification system were assessed using a collection of 25 complex chILD cases. METHODS: A web-based chILD management platform with a registry and biobank was successfully designed and implemented. RESULTS: Over a 3-year period, 575 patients were included for observation spanning a wide spectrum of chILD. In 346 patients, multidisciplinary reviews were completed by teams at five international sites (Munich 51%, London 12%, Hannover 31%, Ankara 1% and Paris 5%). In 13%, the diagnosis reached by the referring team was not confirmed by peer review. Among these, the diagnosis initially given was wrong (27%), imprecise (50%) or significant information was added (23%).The ability of nine expert clinicians to subcategorise the final diagnosis into the chILD-EU register classification had an overall exact inter-rater agreement of 59% on first assessment and after training, 64%. Only 10% of the 'wrong' answers resulted in allocation to an incorrect category. Subcategorisation proved useful but training is needed for optimal implementation. CONCLUSIONS: We have shown that chILD-EU has generated a platform to help the clinical assessment of chILD. TRIAL REGISTRATION NUMBER: Results, NCT02852928

Organ doses in preterm and full-term neonates and infants - a retrospective study on 1,064 chest radiographs

Background Chest radiography is the most frequent X-ray examination performed in the neonatal period. However, commonly used dosimetric entities do not describe the radiation risk sufficiently. Objective The aim of this study was to investigate selected organ doses and total body dose of chest radiographs in preterm and full-term neonates and infants. Materials and methods In this retrospective study, we evaluated 1,064 chest radiographs of 136 preterm and 305 full-term babies with respect to field size and centering. We calculated the entrance dose from the dose-area product. Upper and lower field borders referred to the corresponding vertebrae. We calculated individual organ doses of the thyroid, the breast, the liver and active bone marrow for each chest radiograph using the neonatal PCXMC program, a Monte Carlo program for calculating patient doses in medical X-ray examinations. Results The median field size of chest radiographs ranged from 90 cm(2) in preterm neonates at birth to 290 cm(2) in full-term infants at the age of 6 months. Median values of entrance dose varied, depending on age, from 15 mu Gy to 25 mu Gy. The median organ doses ranged 1-20 mu Sv for the thyroid, 3-30 mu Sv for the breast, 2-20 mu Sv for the liver and 0.5-3.5 mu Sv for the bone marrow in preterm and full-term neonates and infants, respectively. Conclusion The analysis of chest radiographs in preterm and full-term neonates and infants revealed high variability in field size. By contrast, the entrance dose varied to a minor extent. Organ dose calculations using the PCXMC program might be a valuable tool to calculate the individual radiation risk in neonates and infants

Digitalisierte historische Bauzeitschriften auf dem Digitalen Repositorium (OPUS)

Die Universitätsbibliothek der BTU Cottbus-Senftenberg digitalisiert seit 2005 historische Bauzeitschriften, um häufig nachgefragte Titel digital verfügbar zu machen. Diese Maßnahme dient nebenbei auch dem Bestandsschutz. Um Überschneidungen zu vermeiden, wird zunächst überprüft, ob bereits durch andere Einrichtungen wie z.B. der Zentral- und Landesbibliothek Berlin, beziehungsweise Projekte (z.B. ANNO, ZDB) digitalisiert wird. Der Workflow des Digitalisierungsprojekts verläuft folgendermaßen: Nach dem Scannen werden die Dokumente mittels OCR (Optical Character Recognition) in maschinenlesbare PDF-Dateien umgewandelt. Anschließend erfolgt der Upload in Opus und die Vergabe der Metadaten für die inhaltliche und formale Beschreibung. Diese dienen der Such– und Auffindbarkeit der Bauzeitschriften auch außerhalb von OPUS. Die Digitalisate werden auf dem Digitalen Repositorium der BTU Cottbus-Senftenberg archiviert und sind frei zugänglich. Die digitalisierten Zeitschriften werden in der Zeitschriftendatenbank und der Elektronischen Zeitschriftenbibliothek nachgewiesen. Bei der Digitalisierung wurde darauf geachtet, dass die Bestände möglichst vollständig abgebildet sind und es wurde ein Zeitschnitt bei 1920 gemacht. Dieser Service wird von den Mitarbeitern der BTU, anderen Einrichtungen und externen Nutzern sehr gut angenommen

Organ doses in preterm and full-term neonates and infants — a retrospective study on 1,064 chest radiographs

Abstract Background Chest radiography is the most frequent X-ray examination performed in the neonatal period. However, commonly used dosimetric entities do not describe the radiation risk sufficiently. Objective The aim of this study was to investigate selected organ doses and total body dose of chest radiographs in preterm and full-term neonates and infants. Materials and methods In this retrospective study, we evaluated 1,064 chest radiographs of 136 preterm and 305 full-term babies with respect to field size and centering. We calculated the entrance dose from the dose–area product. Upper and lower field borders referred to the corresponding vertebrae. We calculated individual organ doses of the thyroid, the breast, the liver and active bone marrow for each chest radiograph using the neonatal PCXMC program, a Monte Carlo program for calculating patient doses in medical X-ray examinations. Results The median field size of chest radiographs ranged from 90 cm2 in preterm neonates at birth to 290 cm2 in full-term infants at the age of 6 months. Median values of entrance dose varied, depending on age, from 15 μGy to 25 μGy. The median organ doses ranged 1–20 μSv for the thyroid, 3–30 μSv for the breast, 2–20 μSv for the liver and 0.5–3.5 μSv for the bone marrow in preterm and full-term neonates and infants, respectively. Conclusion The analysis of chest radiographs in preterm and full-term neonates and infants revealed high variability in field size. By contrast, the entrance dose varied to a minor extent. Organ dose calculations using the PCXMC program might be a valuable tool to calculate the individual radiation risk in neonates and infants. </jats:sec