- Bilateral breast calciphylaxis in a patient who survived earlier extensive tissue necrosis 5 years previously: A case report. [Journal Article]
- IJInt J Surg Case Rep 2018 May 07; 48:22-25
- CONCLUSIONS: Calciphylaxis is a rare diagnosis that should be considered in patients with renal insufficiency developing painful patches of skin necrosis.A multidisciplinary treatment approach including hyperparathyroidectomy, modified hemodialysis and wound treatment is recommended. There is limited evidence for surgical intervention.
- Heritage of Stanislaw Ciechanowski (1869-1945)-Discoverer of pathogenesis of prostatic hyperplasia-passionis insignia signis fulgent mirificis . [Journal Article]
- PProstate 2018 May 18
- CONCLUSIONS: Ciechanowski gained such a high authority, that his opinion was found crucial in prewar Poland in the field of medical publications, but his world-famous achievement was scientific explanation of prostate overgrowth as inflammation induced hyperplasia.
- Risk factors for lung disease progression in children with cystic fibrosis. [Journal Article]
- EREur Respir J 2018 May 17
- To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch...
To identify potential risk factors for lung disease progression in children with cystic fibrosis (CF), we studied the longitudinal data of all children with CF (aged ≥5 years) registered in the Dutch CF Registry (2009-2014). Lung disease progression was expressed as a decline in lung function (FEV1%pred) and the pulmonary exacerbation rate (PEx). Potential risk factors at baseline included: sex, age, best FEV1%pred, best forced vital capacity (FVC)%pred, genotype, BMI z-score, pancreatic insufficiency, medication use (proton pump inhibitors [PPI], prophylactic antibiotics, and inhaled corticosteroids), CF-related diabetes, allergic bronchopulmonary aspergillosis, and colonisation with Pseudomonas aeruginosaThe data of 545 children were analysed. PPI use was associated with both annual decline of FEV1%pred (p=0.017) and future PEx (p=0.006). Moreover, lower FEV1%pred at baseline (p=0.007), prophylactic inhaled antibiotics use (p=0.006), and PEx in the baseline year (p=0.002) were related to PEx in subsequent years.In a cohort of Dutch children with CF followed for five years, we were able to identify several risk factors for future exacerbations. Especially the association between PPI use and lung disease progression definitely requires further investigation.
- Health-related quality of life measurement in patients with chronic respiratory failure. [Review]
- RIRespir Investig 2018; 56(3):214-221
- The improvement of health-related quality of life (HRQL) is an important goal in managing patients with chronic respiratory failure (CRF) receiving long-term oxygen therapy (LTOT) and/or domiciliary ...
The improvement of health-related quality of life (HRQL) is an important goal in managing patients with chronic respiratory failure (CRF) receiving long-term oxygen therapy (LTOT) and/or domiciliary noninvasive ventilation (NIV). Two condition-specific HRQL questionnaires have been developed to specifically assess these patients: the Maugeri Respiratory Failure Questionnaire (MRF) and the Severe Respiratory Insufficiency Questionnaire (SRI). The MRF is more advantageous in its ease of completion; conversely, the SRI measures diversified health impairments more multi-dimensionally and discriminatively with greater balance, especially in patients receiving NIV. The SRI is available in many different languages as a result of back-translation and validation processes, and is widely validated for various disorders such as chronic obstructive pulmonary disease, restrictive thoracic disorders, neuromuscular disorders, and obesity hypoventilation syndrome, among others. Dyspnea and psychological status were the main determinants for both questionnaires, while the MRF tended to place more emphasis on activity limitations than SRI. In comparison to existing generic questionnaires such as the Medical Outcomes Study 36-item short form (SF-36) and disease-specific questionnaires such as the St. George's Respiratory Questionnaire (SGRQ) and the Chronic Respiratory Disease Questionnaire (CRQ), both the MRF and the SRI have been shown to be valid and reliable, and have better discriminatory, evaluative, and predictive features than other questionnaires. Thus, in assessing the HRQL of patients with CRF using LTOT and/or NIV, we might consider avoiding the use of the SF-36 or even the SGRQ or CRQ alone and consider using the CRF-specific SRI and MRF in addition to existing generic and/or disease-specific questionnaires.
- Ribcage deformity and the altered breathing pattern in children with osteogenesis imperfecta. [Journal Article]
- PPPediatr Pulmonol 2018 May 15
- CONCLUSIONS: An altered breathing pattern in severe OI is present since childhood and it worsens with age. This is caused by the combination of pectus carinatum, brittle ribs and spinal deformity that put the ribcage muscles in mechanical disadvantage. These results suggest that in severe OI the assessment of the respiratory function should start in early childhood in order to try to reduce the incidence of premature death.
- Spondylocostal Dysostosis: A Literature Review and Case Report with Long-Term Follow-Up of a Conservatively Managed Patient. [Journal Article]
- CRCase Rep Orthop 2018; 2018:1795083
- Patients with spondylocostal dysostosis (SCD) have congenital spine and rib deformities associated with frequently severe thoracic insufficiency and respiratory compromise. The literature is largely ...
Patients with spondylocostal dysostosis (SCD) have congenital spine and rib deformities associated with frequently severe thoracic insufficiency and respiratory compromise. The literature is largely composed of case reports and small cohorts, and there is little information regarding adults with this condition. In this report, we describe the natural history of a conservatively treated patient and include quality-of-life issues such as childbearing, athletic participation, and occupational selection.
- [CF Lung Disease - a German S3 Guideline: Module 2: Diagnostics and Treatment in Chronic Infection with Pseudomonas aeruginosa]. [Journal Article]
- PPneumologie 2018; 72(5):347-392
- Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Con...
Cystic Fibrosis (CF) is the most common autosomal-recessive genetic disease affecting approximately 8000 people in Germany. The disease is caused by mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene leading to dysfunction of CFTR, a transmembrane chloride channel. This defect causes insufficient hydration of the epithelial lining fluid which leads to chronic inflammation of the airways. Recurrent infections of the airways as well as pulmonary exacerbations aggravate chronic inflammation, lead to pulmonary fibrosis and tissue destruction up to global respiratory insufficiency, which is responsible for the mortality in over 90 % of patients. The main aim of pulmonary treatment in CF is to reduce pulmonary inflammation and chronic infection. Pseudomonas aeruginosa (Pa) is the most relevant pathogen in the course of CF lung disease. Colonization and chronic infection are leading to additional loss of pulmonary function. There are many possibilities to treat Pa-infection. This is a S3-clinical guideline which implements a definition for chronic Pa-infection and demonstrates evidence-based diagnostic methods and medical treatment for Pa-infection in order to give guidance for individual treatment options.
- [Doppler ultrasonography of the renal artery: Guidelines and predictive factors for the presence of a tight stenosis. Retrospective analysis of 450 consecutive examinations]. [Journal Article]
- JMJ Med Vasc 2018; 43(3):163-173
- CONCLUSIONS: This study confirms the relevance of published guidelines. The diagnostic-effectiveness of Duplex ultrasonography examinations to search for renal artery stenosis depends upon compliance with these guidelines.
- Cardiac examination in children with Laron syndrome undergoing mecasermin therapy. [Journal Article]
- JPJ Pediatr Endocrinol Metab 2018 May 11
- CONCLUSIONS: In this study, we showed that cardiac findings were consistent with previous studies. To the best of our knowledge, the observed pulmonary hypertension in children with LS, who received treatment with or without mecasermin, is reported for first time in the literature.
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- Characteristics of pulmonary mucormycosis and predictive risk factors for the outcome. [Journal Article]
- IInfection 2018 May 10
- CONCLUSIONS: Pulmonary mucormycosis is a rare infection with a high mortality. Invasive approach for histopathology and culture are crucial for a definite diagnosis. Acute onset patients had a poorer prognosis, and early treatment with antifungal therapy is imperative. Surgical approach is recommended in appropriate patients for a better outcome.