Lungen- und Atemwegsmedizin

Unilateral absence of pulmonary artery (UAPA) is a rare congenital condition occurring isolated or in conjunction with other congenital heart defects (CHD). While none-isolated UAPA is mostly detected early in life, detection of isolated UAPA is often delayed due to diverse and unspecific symptoms. While there is consensus on the therapeutic strategy in young symptomatic patients, the incidental finding of UAPA in young or adult asymptomatic patients is a matter of on-going debate and limited evidence. Here, we will present two previously unpublished, illustrative cases with different extent of symptom severity and discuss the difficulties in determining an appropriate therapeutic strategy. Along with the cases, we will review the literature, present typical symptoms and possible pitfalls, and provide an overview of therapeutic options with a focus on the adult patient.

Regulatory T-cells (Tregs) express the high-affinity chain of the interleukin 2 (IL-2) receptor, CD25, encoded by the IL2RA gene. IL2RA is one of the most extensively characterized genes regarding its regulatory regions and their functional links to cell surface receptors and their associated signal transduction cascade in the context of the antigen-dependent activation of mature CD4+ T lymphocyte. However, converging evidences strongly suggested that they were some missing pieces in this already complex puzzle made of 6 well-characterized regulatory regions. Here, by combining principally in silico genomic footprinting approach and meta-analysis of several ChIP-seq studies, we identified and characterized 2 new putative CD28-responsive elements. We show that a recently-characterized intronic enhancer at +11 kb harbors a functional CREB site. Further, we evidence a repressor element consisting of two GAAA repeats located 5’-most of a previously identified enhancer 4 kb upstream of the IL2RA gene. Massspectrometry analyses revealed Poly ADP-ribose polymerase 1 (PARP-1) as part of the complexes binding this element. Altogether, our observations extend our understanding of the molecular basis of the multiple options offered by such a complex organization in term of T cell responses.

Introduction: Chronic obstructive pulmonary disease (COPD) is the major cause of morbidity and mortality which results in an economic and social burden. Osteoporosis is considered as one of the major systemic manifestations of COPD.
Aim and Objective: It is to determine the prevalence of osteoporosis in COPD patients and its disease severity and also to evaluate various risk factors involved in reduced bone mineral density in patients with COPD.
Methods: A total of 30 COPD patients and 15 healthy controls were enrolled and all individuals were subjected to pulmonary function test and DEXA scan. Various risk factors for COPD associated with osteoporosis and association of osteoporosis with severity of COPD were studied and analyzed. Statistical analysis was done for parametric data using t-test. p value <0.05 was considered as significant.
Results: The incidence of osteoporosis was found to be significantly higher in COPD patients (60%) compared to healthy adults (20%) (p=0.017). Osteoporosis within the COPD patients of age group of 51-60 was found to be significantly higher compared to other age group in COPD. Obesity, overweight and smoking habit as well as exacerbations may be the risk factors in COPD patients. The average number of exacerbation in COPD patients was 2.13. COPD Patients in Gold Stage 2 had less osteoporosis while majority of patients in stage 3 and stage 4 had osteoporosis. T score and BMD differed significantly between the cases and control (p=0.007). BMD was higher in healthy controls compared to COPD patients (p=0.012). BMD has higher in age group of 41-50 compared to age group 51-60. (p=0.021).
Conclusion: The risk of osteoporosis and osteopenia increases with the increase of COPD severity. There are multiple risk factors which are found to be associated with osteoporosis and osteopenia in COPD. Thus the present study warrants further COPD study.

Nivolumab is a recently approved medication that shows significant clinical activity for various types of malignancies such melanoma, renal cell carcinoma and non-small cell carcinoma. Pneumonitis is a rare, but, possibly fatal manifestation that still remains ambiguous. This article presents a case of nivolumab induced pneumonitis in a patient with melanoma and discusses management plans.

Spinal cord injury (SCI) can result in serious respiratory compromise, impaired cough ability and respiratory failure. Complications include atelectasis and pneumonia. Respiratory failure is the primary cause of morbidity and mortality in high cervical cord injuries. Various methods have been used to assist coughing in SCI, including manual and mechanical techniques. Physical therapists can apply certain exercises and maneuvers to augment tidal breathing and expiratory effort, such as respiratory muscle training. For patients with vital capacities <10 to 15 mL/ kg, noninvasive methods such as abdominal binding, the pneumobelt, and face mask-applied ventilators are used to maintain adequate respiration. Phrenic nerve and diaphragmatic pacing provide increased patient mobility, comfort and lower health care costs; breathing pacemakers have increased survival and improved quality of life in individuals with upper cervical cord and brain stem lesions. Tracheostomy should be used only for those patients that have severe bulbar impairment and cannot successfully use airway clearance methods. Even patients with tracheostomyassisted ventilation can be eventually weaned off respirators, provided they meet criteria for spontaneous breathing. Peak expiratory flows should exceed 160 L/m to assure expulsion of airway secretions and the negative inspiratory pressure should exceed -20 cm H2O (variables measured with the tube cuff inflated) before the patient is decannulated. Appropriate vaccinations should be provided for any individual with compromised respiratory function, particularly with regularly scheduled influenza and pneumococcal pneumonia vaccines. Management of the physically impaired patient can be a major challenge for family, leading to adverse physical and psychological consequences. Long-term management requires a multidisciplinary approach that includes respiratory, physical and occupational therapists, nutritionists, social workers, psychologists, and home health agencies, all of whom contribute to key aspects of maintaining optimum respiratory function. Life satisfaction is a major consideration in this group of individuals, but it may have a more positive outlook than one would think in someone with significant physical and psychological challenges.

Introduction: A Tension Hydrothorax is defined as a massive pleural effusion presenting with hemodynamic abnormalities secondary to mediastinal compression. In these patients the pleural volume increases the intrathoracic pressure to the point of compromising diastolic filling and cardiac output simulating a cardiac tamponade physiology. This is an uncommon yet potentially fatal medical emergency that if left untreated may lead to cardiac arrest. Early detection and rapid intervention of these patients prevents cardiorespiratory collapse.
Case presentation: We present a two-case report of patients presenting with a malignant tension hydrothorax managed in the emergency room with ultrasound-guided decompression thoracentesis and the other patient with tube thoracostomy.
Conclusion: Although these life-saving decompressive interventions appear simple, not having proper training is an independent factor for chest tube and thoracentesis complications. Courses and simulation training can benefit physicians to minimize potential complications when managing emergency conditions and pathologies such as a Tension hydrothorax which continues to be a medical challenge. Whether thoracentesis or thoracostomy is superior in managing this condition remains in debate and a clinical dilemma.

Background and aim: There are currently many devices on the market, which have been used for training of the respiratory muscles. The knowledge of these devices may help professionals to carefully select the best one to be used. However, due to the numerous available devices available, this selection represents a challenge. Although previous studies have attempted to describe all respiratory muscle training devices, important ones with proven efficacy were not included. Therefore, the purpose of this review was to describe the mechanisms and characteristics of all available respiratory muscle training devices, and discuss their merits and limitations.
Methods: Searches were conducted in databases, books, website selling products related to rehabilitation, and reference lists of the retrieved papers.
Results: The present review included 14 devices currently available on the market and reported by published studies. However, three could not be described in details, due to lack of information. Amongst the 11 evaluated devices, all of them showed positive aspects and limitations that should be considered.
Conclusion: Although some devices appear to be more advantageous than others, it is not possible to choose the best one, based only upon their technical information and clinical utility. To select the most appropriate one, it is also necessary to consider the specific health condition, the nature of the impairments, the purpose of the training, and whether it is for use within research or clinical contexts.