Key4OI Recommendations for Lung Function Guidance in Osteogenesis Imperfecta: Based on an Internationally Performed Comprehensive International Consortium for Health Outcomes Measurement Procedure.

INTRODUCTION: Pulmonary involvement in Osteogenesis Imperfecta (OI) can be severe but may be overlooked in milder cases. The Care4BrittleBones Foundation initiated this project to develop a set of global outcome measures focusing on respiratory-related issues in patients with OI. The objective was to reach an international consensus for a standardized set of outcomes and associated measuring instruments for the pulmonary care of individuals with OI. Based on the initial tests and questionnaires, we suggest parameters for when pulmonologists should seek guidance from the growing literature on OI pulmonary care and/or recognized experts in the field. STUDY DESIGN AND METHODS: The project team consisted of a multidisciplinary mix of 12 people from six countries, including an OI patient representative, and facilitated by the Care4BrittleBones Foundation director. The International Consortium for Health Outcomes Measurement (ICHOM) process was followed, which includes the Delphi method, used to collect the opinions of the expert team. Patient input was present in each meeting due to the inclusion of a patient representative. In addition, online focus groups were held. They consisted of adults with OI from different countries, and they determined which questions matter the most to the OI community worldwide. RESULTS: After three Delphi rounds, the expert team reached a consensus on the final set of measuring instruments, which included pulmonary function testing and patient self-reporting of symptoms related to breathing and sleep. Two questionnaires were decided upon: St. George's Respiratory Questionnaire (shortened version) and four questions regarding sleep. Patients should be screened for a history of pneumonia. Advanced testing for select patients by a pulmonologist would include further pulmonary function tests and a chest radiograph. CONCLUSIONS: A standardized set of outcome measures related to pulmonary care of individuals with OI was determined based on what is important to both experts and patients. This included patient-reported outcome measures and basic pulmonary function testing. Using these outcome measures, it can be determined which patients are at high risk for pulmonary complications.

A 79-Year-Old Woman With Advanced Lung Carcinoma and Bilateral Infiltrates.

CASE PRESENTATION: A 79-year-old woman was admitted to the hospital for progressive dyspnea and severe hypoxemia, requiring oxygen supplementation. The dyspnea started approximately 3 to 4 weeks before presentation and was slowly progressive throughout the following weeks. Her medical history mentioned an adenocarcinoma with an epidermal growth factor receptor (EGFR) exon 19 deletion of the lung with metastases to the bones and brain for which treatment with osimertinib was started 14 months earlier. Furthermore, she was treated with rivaroxaban for a first episode of a pulmonary embolism. In the months leading up to her current presentation there were no changes in medication and no use of antibiotics. She had no known exposure to toxic fumes or substances, she was a nonsmoker, and her family history was unremarkable for autoimmune disorders or interstitial lung disease (ILD).

Benefit of Concurrent Versus Sequential Chemoradiotherapy in Elderly Patients With Stage III Non-Small Cell Lung Cancer.

INTRODUCTION: Lung cancer is the largest cause of cancer-related deaths worldwide. Eighty-five percent of patients is diagnosed with non-small cell lung cancer (NSCLC). Almost a third of patients is aged over 75, but this group is poorly represented in clinical trials. This study compares the effects of therapy in non-operable stage III NSCLC in elderly patients compared to their younger counterparts. PATIENTS AND METHODS: This is a retrospective cohort study. Patients are divided into three groups; patients younger than 65, patients aged between 65 and 75 and patients of 75 years or older. Concurrent chemoradiotherapy is compared to sequential chemoradiotherapy using Cox regression analysis. The primary outcome is survival. A sub analysis is performed for the presence of toxicity using logistic regression. RESULTS: Seven hundred and fifty patients were diagnosed with stage III NSCLC and treated with concurrent (442) or sequential (308) chemoradiotherapy. Concurrent chemoradiotherapy provides a decreased HR of death of 0.72 (0.560-0.85) compared to sequential chemoradiotherapy, even when corrected for age. Elderly patients receiving concurrent chemoradiotherapy do not have a significantly larger risk of toxicity. CONCLUSIONS: Patients of all ages with stage III NSCLC benefit from concurrent chemoradiotherapy compared to sequential chemoradiotherapy. Age is not a deciding factor in this prospect, nor do the patients experience more severe toxicity than their younger counterparts.

Digital versus analogue chest drainage system in patients with primary spontaneous pneumothorax: a randomized controlled trial.

BACKGROUND: Patients with a primary spontaneous pneumothorax (PSP) who are treated with chest tube drainage are traditionally connected to an analogue chest drainage system, containing a water seal and using a visual method of monitoring air leakage. Electronic systems with continuous digital monitoring of air leakage provide better insight into actual air leakage and changes in leakage over time, which may lead to a shorter length of hospital stay. METHODS: We performed a randomized controlled trial comparing the digital with analogue system, with the aim of demonstrating that use of a digital drainage system in PSP leads to a shorter hospital stay. RESULTS: In 102 patients enrolled with PSP we found no differences in total duration of chest tube drainage and hospital stay between the groups. However, in a post-hoc analysis, excluding 19 patients needing surgery due to prolonged air leakage, hospital stay was significantly shorter in the digital group (median 1 days, IQR 1-5 days) compared to the analogue group (median 3 days, IQR 2-5 days) (p 0.014). Treatment failure occurred in 3 patients in both groups; the rate of recurrence within 12 weeks was not significantly different between groups (16% in the digital group versus 8% in the analogue group, p 0.339). CONCLUSION: Length of hospital stay was not shorter in patients with PSP when applying a digital drainage system compared to an analogue drainage system. However, in the large subgroup of uncomplicated PSP, a significant reduction in duration of drainage and hospital stay was demonstrated with digital drainage. These findings suggest that digital drainage may be a practical alternative to manual aspiration in the management of PSP. TRIAL REGISTRATION: Registered 22 September 2013 - Retrospectively registered, Trial NL4022 (NTR4195).

[A man with a yellow coloured abnormality of his finger]. / Een man met een geelkleurige afwijking van de vinger.

A 83-year-old man presented to the emergency department with signs of diabetes mellitus de novo and a yellow coloured abnormality of his left index finger. A plain radiograph of the left hand showed destruction of the proximal interphalangeal joint of digit II. With use of polarised light microscopy, the diagnosis 'gouty tophus' was established.

[Increased INR from concomitant use of acenocoumarol and capecitabine]. / Doorgeschoten INR door gelijktijdig gebruik van acenocoumarol en capecitabine.

BACKGROUND: A drug interaction between capecitabine and coumarin may result in an increased INR and bleeding complications. CASE DESCRIPTION: We describe an 80-year-old woman who presented with rectal bleeding and an increased INR due to the concomitant use of acenocoumarol and capecitabine for atrial fibrillation and metastatic cecal cancer, respectively. CONCLUSION: In patients with a compelling indication for treatment with capecitabine and anticoagulant therapy, conversion to low-molecular weight heparin should be considered.

[A woman with skin lesions over her whole body]. / Een vrouw met huidafwijkingen over het hele lichaam.

A 37-year-old woman was admitted to the hospital because of generalized skin and mucosa lesions after she had started with carbamazepine. It appeared the lesions were part of Stevens-Johnson syndrome.

[A woman with painful, swollen legs]. / Een vrouw met pijnlijke, gezwollen benen.

A 72-year-old woman from the inlands of Surinam was admitted to the hospital because of chronic painful swelling of both legs, caused by filariasis.

[A progressive vomiting pre-schooler]. / Een progressief brakende kleuter.

A 4-year-old boy with persistent vomiting had a peptic stenosis of the oesophagus.