Nasal epithelial cells from very preterm infants have a functional defect in their ability to repair beyond the first year of life, and failed repair may be associated with antenatal steroid exposure https//bit.ly/39OFJs7.
Reduction of COL4A3, one of the six isoforms of collagen 4, in asthmatic airways results in increased inflammation and angiogenesis, implicating it as a central part of asthma pathogenesis. However, to date, the path underlying these diminished COL4A3 levels has been elusive. This study investigated a possible mechanism underlying the reduction of COL4A3 expression.

Bronchial biopsies of 76 patients with asthma and 83 controls were subjected to RNA-sequencing and DNA methylation bead arrays to identify expression and methylation changes. The binding of ZNF263 was analysed by chromatin-immunoprecipitation sequencing coupled with quantitative (q)PCR. Effects of
silencing, using small interfering RNA, on the
expression were studied using qPCR.

expression was significantly reduced in bronchial biopsies compared to healthy controls, whereas DNA methylation levels at cg11797365 were increased.
expression levels were significantly low in asthmatics without inhaled corticosteroid (ICS) use, whereas the expression was not statistically different between asthmatics using ICS and controls. https://www.selleckchem.com/products/olprinone.html Methylation levels at cg11797365
were increased upon consecutive rhinovirus infections.

Our data indicate an epigenetic modification as a contributing factor for the loss of
expression in asthmatic airway epithelium.
Our data indicate an epigenetic modification as a contributing factor for the loss of COL4A3 expression in asthmatic airway epithelium.
This cross-sectional study aimed to investigate the prevalence of self-reported and clinically screened swallowing dysfunction (dysphagia) in COPD patients with severe exacerbations and to identify any associated factors. Findings were then compared to a control group.

Participants included 30 patients hospitalised due to a COPD exacerbation. The control group consisted of 30 adults hospitalised with acute cardiac symptoms. Data were derived from spirometry, the 150 mL timed water swallow test, a cookie swallow test and a dyspnoea questionnaire (modified Medical Research Council (mMRC)). Scores from the 10-item Eating Assessment Tool (EAT-10) were calculated to assess patient perception of swallowing dysfunction.

Self-reported swallowing dysfunction and clinical signs thereof were more common in COPD patients than in the control group (67%
23% and 80%
37%, respectively; p≤0.001). Clinical signs of swallowing dysfunction in the group with acute exacerbation of COPD were associated with self-reportef COPD. Both groups also experienced xerostomia.A combination of airway clearance techniques are applied for people with bronchiectasis, together with recommendations for exercise and suggestions for management of common comorbidities https//bit.ly/2U3c99H.
Childhood interstitial and diffuse lung diseases (chILD) encompass a broad spectrum of rare pulmonary disorders. In most developing Middle Eastern countries, chILD is still underdiagnosed. Our objective was to describe and investigate patients diagnosed with chILD in a tertiary university hospital in Egypt.

We analysed data of consecutive subjects (aged <18 years) referred for further evaluation at the Children's Hospital, Ain Shams University (Cairo, Egypt). Diagnosis of chILD was made in accordance with the ChILD-EU criteria. The following information was obtained demographic data, clinical characteristics, chest computed tomography findings, laboratory studies, spirometry, bronchoalveolar lavage and histopathology findings.

22 subjects were enrolled over 24 months. Median age at diagnosis was 7 years (range 3.5-14 years). The most common manifestations were dyspnoea (100%), cough (90.9%), clubbing (95.5%) and tachypnoea (90.9%). Systematic evaluation led to the following diagnoses hypersensitivitydy highlights the need for an Egyptian chILD network with genetic testing, as well as the value of collaborating with international groups in improving healthcare for children with chILD.
Real-world trial data comparing single- with multiple-inhaler triple therapy (MITT) in COPD patients are currently lacking. The effectiveness of once-daily single-inhaler fluticasone furoate (FF)/umeclidinium (UMEC)/vilanterol (VI) and MITT were compared in usual clinical care.

INTREPID was a multicentre, randomised, open-label, phase IV effectiveness study comparing FF/UMEC/VI 100/62.5/25 µg
the ELLIPTA inhaler with a clinician's choice of any approved non-ELLIPTA MITT in usual COPD clinical practice in five European countries. Primary end-point was proportion of COPD Assessment Test (CAT) responders (≥2-unit decrease in CAT score from baseline) at week 24. Secondary end-points in a subpopulation included change from baseline in forced expiratory volume in 1 s (FEV
) and percentage of patients making at least one critical error in inhalation technique at week 24. Safety was also assessed.

3092 patients were included (FF/UMEC/VI n=1545; MITT n=1547). The proportion of CAT responders at week 24 was significantly greater with FF/UMEC/VI
non-ELLIPTA MITT (OR 1.31, 95% CI 1.13-1.51; p<0.001) and mean change from baseline in FEV
was significantly greater with FF/UMEC/VI (77 mL
28 mL; treatment difference 50 mL, 95% CI 26-73 mL; p<0.001). The percentage of patients with at least one critical error in inhalation technique was low in both groups (FF/UMEC/VI 6%; non-ELLIPTA MITT 3%). Safety profiles, including incidence of pneumonia serious adverse events, were similar between treatments.

In a usual clinical care setting, treatment with once-daily single-inhaler FF/UMEC/VI resulted in significantly more patients gaining health status improvement and greater lung function improvement
non-ELLIPTA MITT.
In a usual clinical care setting, treatment with once-daily single-inhaler FF/UMEC/VI resulted in significantly more patients gaining health status improvement and greater lung function improvement versus non-ELLIPTA MITT.

In 2016, World Health Organization guidelines conditionally recommended standardised shorter 9-12-month regimens for multidrug-resistant (MDR) tuberculosis (TB) treatment. We conducted a prospective study of a shorter standardised MDR-TB regimen in Karakalpakstan, Uzbekistan.

Consecutive adults and children with confirmed rifampicin-resistant pulmonary TB were enrolled between September 1, 2013 and March 31, 2015; exclusions included prior treatment with second-line anti-TB drugs, and documented resistance to ofloxacin or to two second-line injectable agents. The primary outcome was recurrence-free cure at 1 year following treatment completion.

Of 146 enrolled patients, 128 were included 67 female (52.3%), median age 30.1 (interquartile range 23.8-44.4) years. At the end of treatment, 71.9% (92 out of 128) of patients achieved treatment success, with 68% (87 out of 128) achieving recurrence-free cure at 1 year following completion. Unsuccessful outcomes during treatment included 22 (17.2%) treatment faiDR-TB regimen was moderate with considerable treatment failure and amplification of fluoroquinolone resistance. When introducing standardised shorter regimens, baseline drug susceptibility testing and minimising missed doses are critical. High rates globally of pyrazinamide, ethambutol and ethionamide resistance raise questions of continued inclusion of these drugs in shorter regimens in the absence of drug susceptibility testing-confirmed susceptibility.This article provides an overview of outstanding sessions that were (co)organised by the Allied Respiratory Professionals Assembly during the European Respiratory Society International Congress 2020, which this year assumed a virtual format. The content of the sessions was mainly targeted at allied respiratory professionals, including respiratory function technologists and scientists, physiotherapists, and nurses. Short take-home messages related to spirometry and exercise testing are provided, highlighting the importance of quality control. The need for quality improvement in sleep interventions is underlined as it may enhance patient outcomes and the working capacity of healthcare services. The promising role of digital health in chronic disease management is discussed, with emphasis on the value of end-user participation in the development of these technologies. Evidence on the effectiveness of airway clearance techniques in chronic respiratory conditions is provided along with the rationale for its use and challenges to be addressed in future research. The importance of assessing, preventing and reversing frailty in respiratory patients is discussed, with a clear focus on exercise-based interventions. Research on the impact of disease-specific fear and anxiety on patient outcomes draws attention to the need for early assessment and intervention. Finally, advances in nursing care related to treatment adherence, self-management and patients' perspectives in asthma and chronic obstructive pulmonary disease are provided, highlighting the need for patient engagement and shared decision making. This highlights article provides readers with valuable insight into the latest scientific data and emerging areas affecting clinical practice of allied respiratory professionals.The American Thoracic Society and European Respiratory Society commissioned a task force to update the technical standards for spirometry testing with the aim of increasing the accuracy, precision and quality of spirometry measurements and improving the patient experience. To inform the task force with patient experiences, the European Lung Foundation, in collaboration with the task force, conducted an online survey in 10 languages between August and September 2018. There were 1760 respondents from 52 countries. The majority were adults (97.1%); the most common reasons for spirometry referral were diagnosis (35.0%) and management of an ongoing condition (60.1%). 53.2% reported regularly using inhalers. Respondents were very experienced with spirometry 89.9% completed more than one test; 48% completed 10 or more tests. However, most reported not knowing what forced expiratory volume in 1 s (FEV1) means (59.4%) and only 39.6% knew their most recent FEV1; the exception was respondents with cystic fibrosis who reported much greater knowledge. Respondents rated as moderately or seriously problematic being told to keep blowing when they felt nothing is coming out (31.4%), coughing (30.4%), tiredness (26.3%) and concern about shortness of breath (20.1%). Overall, respondents found spirometry to be acceptable; however, an important minority (17%) found it difficult. Patients want clear information before, during and after the test, including information on stopping medications. Operators have an important role in increasing the ease of patients and changes to the testing environment can increase patient comfort. Patients want access to their results and want to understand how they relate to their individual health.
Interrupted aortic arch (IAA) is a rare congenital heart disease defined by an interruption of the lumen and anatomical continuity between the ascending and descending major arteries. It is usually found within a few hours or days of birth. Without surgery, the chances of survival are low. If IAA patients have an effective collateral circulation established, they can survive into adulthood. However, IAA in adults is extremely rare, with few reported cases.

A 27-year-old woman presented with a 6-year history of progressively worsening shortness of breath and chest tightness on exertion. She had cyanotic lips and clubbing of the fingers. A transthoracic echocardiogram revealed an enlarged heart and dilation of the main pulmonary artery. There was an abnormal 9 mm passage between the descending aorta and pulmonary artery. The ventricular septal outflow tract had a 14 mm defect. Doppler ultrasound suggested a patent ductus arteriosus and computed tomographic angiography showed the absence of the aortic arch. The diagnoses were ventricular septal defect, patent ductus arteriosus, and definite interruption of the aortic arch. https://www.selleckchem.com/products/heparan-sulfate.html Although surgical correction was recommended, the patient declined due to the surgical risks and was treated with medications to reduce pulmonary artery pressure and treat heart failure. Her condition has been stable for 12 mo of follow-up.

Although rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.
Although rare, IAA should be considered in adults with refractory hypertension or unexplained congestive heart failure.

Night practice is attested from 1838; night-school from 1520s; night-life "recurring nocturnal carousing" is attested from 1852. Commercial clubs immediately capitalized on the scenario inflicting a increase in "Superclubs" in the UK, similar to Ministry of Sound (London), Renaissance, and Cream (Liverpool). San Diego’s low-lit lounges are the place you’ll find beautiful crowds, attractive beats and funky cocktails. Less frenetic than conventional clubs, these venues provide the opportunity for conversation while nonetheless satisfying the need to see and be seen. For more on San Diego's nightlife scene, watch Guide to the Good Stuff videos from DJ Ayla Simone and DJ Schoeny as they take you on a tour of what is scorching and taking place in San Diego after darkish. Dance all weekend with world ’s most famous resident DJs like The Chainsmokers, David Guetta, Kygo and Marshmello.
Presale tickets holders will achieve entry via the ticket area on the membership entrance. Use promo code NOCOVER for an immediate discount on all EBC At Night tickets. Yohan's dialogue indicates he finds Connie engaging, but Connie tells the participant that she thinks he is a creep. The membership will turn into emptier, beginning with the VIP Lounge and then the VIP bar space, progressing to the basic public bar and eventually the dance flooring. The participant can drink at any of the three bars or devour their very own Pißwasser from their stock anywhere. There are also drinks sitting on tables throughout the club that the gamers can freely drink.
It was an inventive incubator on the coronary heart of New York’s early 80s cultural renaissance. It turned an area for musicians, artists, and activists to seek inspiration. It was the membership the place Madonna performed on stage for the primary time and Keith Haring painted art straight onto the walls, And within the midst of the AIDs crisis, it became the place New Yorkers escaped to find neighborhood, joy and the cha... Learn extra about San Diego’s greatest nearby nightclub by visiting our nightlife in San Diego venue web page. Additionally, our rooftop makes for a superb rental opportunity, try our non-public events rental choices – San Diego Event Space Venue Rentals. EBC At Night on the Encore Hotel in Las Vegas proves that pool events aren’t only for the daytime.
A short broad stairway on the left leads directly up to the office house and this offers the quickest entry to the office without having to stroll via multiple ranges of nightclub floor. When entering by way of the storage on foot, choices to enter the nightclub (the default parking space area) or entry any of the storage or warehouse ranges are offered. Situated on the higher degree of Avalon, Bardot is greatest known for School Night, a long-running Monday evening live music showcase. Keep tabs via Instagram for other upcoming occasions at Bardot. These discothèques were additionally patronized by anti-Vichy youth known as zazous. In Nazi Germany, there were underground discothèques patronized by anti-Nazi youth referred to as the "Swing Kids".
However, the seismic shift in nightlife was the emergence of rave tradition in the UK. A combination of free and commercial outdoor events have been held in fields, warehouses, and abandoned buildings, by various teams corresponding to Biology, Sunrise, Confusion, Hedonism, Rage & Energy, and plenty of others. This laid the bottom for what was unfold within the Nineties, initially in the United Kingdom, Germany, and the United States after which worldwide from the 2000s onwards. From about 1900 to 1920, working class Americans would collect at honky tonks or juke joints to bop to music performed on a piano or a jukebox.
The primary bar is straight ahead and is staffed by an unnamed female bartender. If the employees improve option is bought, a second female bartender shall be behind the bar. This 13,000 square-foot Hollywood Boulevard venue kicks off the weekends on Thursday nights with La Cultura, featuring a combine of reggaeton, Latin and hip-hop. The hip-hop beats proceed to drop throughout the weekend with TBA Fridays and The Scene on Saturdays. Our historic 1930’s building has been reimagined to perform as a nightclub and event space! Venue rental obtainable for company award exhibits, film shoots, charity galas and extra, for as a lot as 1,500 guests within the heart of DTLA.
Probably this degeneracy of coaches was as a result of practice of travellers clubbing together to rent a post-chaise for the journey. Shagarach and Dr. Silsby stationed themselves every at one aspect, the former empty-handed, the latter clubbing his stout cane. Instead of a brute clubbing a girl virtually to dying, we see the pleading lover, cautiously and earnestly wooing his bride.
Another bouncer might be monitoring the door to the office area on the far end of the VIP lounge space. If the participant is not a member of the groups that have been allowed entry, he'll stop the player from accessing both the office or the stairway all the way down to the DJ booth. A smaller garage space able to storing one private car. https://oplovesite.com/ delivered in management missions shall be parked in here but will turn into static props.
Opened in spring 2022, the $10-million MainRó is the first LA venture for hospitality magnate Romain Zago, proprietor of Miami’s famend Mynt Lounge and Myn-tu. Delivering sensory overload from the second you cross safety, this 7,500 square-foot “clubstaurant” features touch-sensitive tables and shifting 4K digital screens together with velvet booths and Vegas-style showgirls. Picture Moulin Rouge staged by Cirque du Soleil on New Year’s Eve and you’re getting close. Located on Vine Street close to Hollywood Boulevard, Avalon has a protracted and storied history in the neighborhood.

resulted in stable or improved vision. Careful monitoring is required because of possible development of CNVM.
CSCR in elderly patients can mimic occult CNVM, especially on FA. In this group, many patients were monitored without treatment, which typically resulted in stable or improved vision. Careful monitoring is required because of possible development of CNVM.The cells in glomus body regulate blood flow to the skin and play a role in temperature regulation. Glomuvenous malformations (GVMs), previously reported in literature as glomus tumors or glomangiomas, are usually present in the nail bed. In 1972, Carroll and Berman described the triad of paroxysmal stabbing pain, point tenderness, and cold hypersensitivity associated with these malformations. We present an unusual case of GVM in the neck of a 17-year-old female presenting with a painful right-sided neck mass. Physical examination revealed an approximately 2.5 × 2 cm right-sided tender neck mass; pain was exacerbated by probing with a pinhead. Computed tomography identified multiple rounded masses in the neck, with the delayed contrast filling. The patient underwent excisional biopsy of the lesion and samples were sent for histopathological examination. The histopathology report, along with the radiological findings and the clinical presentation, confirmed the diagnosis of GVM. The patient remained symptom-free on long-term follow-up. Despite absence of previous reports, GVM can occur in neck. Surgical excision can lead to complete resolution of symptoms.Objectives  Memory impairment has been only rarely reported in association with acute aortic dissection type A. We report a patient with pure anterograde amnesia and memory impairment of contents occurring after the event, accompanying acute aortic dissection type A. Case Report  A previously healthy 53-year-old Caucasian male was admitted because of sudden chest pain after having lifted a heavy object. https://www.selleckchem.com/products/dwiz-2.html Clinical examination and electrocardiogram showed no abnormalities. Since blood tests showed leukocytosis, anemia, and elevated D-dimer level, either pulmonary embolism or aortic dissection was suspected; therefore, computed tomography was suggested. The patient seemed disoriented to time, and neurologic investigation confirmed that the patient was disoriented to time; short time memory was severely impaired and concentration was reduced. An amnestic episode with anterograde amnesia was diagnosed. Computed tomography showed type A aortic dissection. A supracoronary replacement of the ascending aorta was performed. The patient was discharged on the 7th postoperative day. Three months postoperatively, the patient is clinically stable; however, amnesia for the interval between pain onset and cardiac surgery persists. Conclusions  Transient amnesia, usually considered a benign syndrome, may be more common than generally recognized in aortic dissection. The suspicion for aortic dissection or other cardiovascular emergencies is substantiated when amnesia is associated with sudden onset of chest pain, leukocytosis, and elevated D-dimer levels. Computed tomography of the aorta with contrast medium is the imaging method of choice to confirm or exclude the diagnosis.A 43-year-old male presented with shortness of breath and palpitations. Physical examination was significant for skin lichenification, an erythematous maculopapular rash with annular plaques, a fissured tongue, and digital clubbing. Electrocardiogram captured a supraventricular tachycardia and right bundle branch block. Left heart catheterization found several proximal left coronary artery branch aneurysms, a right coronary-pulmonary artery fistula, venous-luminal vessels between the right heart chambers and cardiac veins, and thebesian venous lakes. Functional cardiac capacity was reduced, with left ventricular ejection fraction of 35 to 40%, on ventriculogram. A diagnosis of discoid lupus erythematosus was established after skin biopsies revealed systemic lupus erythematosus-like features but negative anti-nuclear, anti-Smith, anti-ds-deoxyribonucleic acid, anti-Ro, and anti-La antibodies. The case reported is unique, in that while our patient had pathologic electrophysiologic changes typically seen with autoimmune disease, the patient had many anatomical cardiac anomalies without atherosclerotic coronary disease. It is uncertain whether these findings are purely incidental or are related to an underlying genetic disorder.This case report aims to highlight a successful example of using novel oral anticoagulants (NOACs), such as apixaban, to prevent recurrent venous thromboembolism (VTE) in patients with solid-organ malignancy, as an alternative to low-molecular weight heparin (LMWH). Discussed is the case of a 67-year-old woman diagnosed with recurrent thrombosis in the upper-right limb and malignancy of right breast with metastasis to the axillary lymph nodes. Over a follow-up period of more than 1 year, there was no recurrence of VTE and D-dimer decreased, illuminating the possibility of apixaban as an alternative form of treatment for recurrent VTE in patients with malignancy. This appears to be one of the first case reports in Singapore.Ischemia and no obstructive coronary arteries (INOCA) is a common clinical presentation, with a variety of causes that are often not fully investigated in routine clinical practice. The goal of our study was to characterize a real-world population of patients with INOCA, with a deeper focus on symptoms and stress test findings. The study population consisted of 435 patients who underwent diagnostic coronary angiography for anginal symptoms and/or evidence of myocardial ischemia at non-invasive imaging. In all patients angiography demonstrated nonobstructive coronary artery disease (CAD, less than 30% luminal diameter stenosis or fractional flow reserve > 0.8 and/or instantaneous wave-free ratio  > 0.89). Fifty-four percent of the patients were women. Atypical clinical presentation was more common in women (59.5 vs. 49.5%, p  = 0.037). Women were more likely to have normal coronary arteries than men (41.8 vs. 16.2%, p   less then  0.001), and less likely than men to have hemodynamically non-significant CAD (32.

He denied fever, weight loss, rash, dysphagia, proximal muscle weakness, wheeze, sinus congestion, and peripheral numbness/tingling. Medical history included paroxysmal atrial fibrillation, hypertension, Hashimoto's thyroiditis, and OSA. Long-term medications included aspirin, flecainide, atorvastatin, amlodipine, levothyroxine, and candesartan. He was a lifelong nonsmoker. There was no history of recent travel.
A 43-year-old man experienced sudden onset of chest pain and shortness of breath onboard a domestic flight. After consultation with the airline's operations center, a decision was made to land the plane in its destination airport. After landing, an ambulance was ready, and the patient was transferred to the ED in our facility. Patient evaluation was immediately started, and oxygen supply and venous access were secured.
A 43-year-old man experienced sudden onset of chest pain and shortness of breath onboard a domestic flight. After consultation with the airline's operations center, a decision was made to land the plane in its destination airport. After landing, an ambulance was ready, and the patient was transferred to the ED in our facility. Patient evaluation was immediately started, and oxygen supply and venous access were secured.
A 26-year-old man presented with a 2-week history of productive cough and a 1-year history of effort-related dyspnea. His medical history was significant for hay fever and exertion-triggered asthma. He was not taking medicines regularly but was using inhaled salbutamol as needed. He was an ex-smoker, with a previous history of 2-pack years.
A 26-year-old man presented with a 2-week history of productive cough and a 1-year history of effort-related dyspnea. His medical history was significant for hay fever and exertion-triggered asthma. He was not taking medicines regularly but was using inhaled salbutamol as needed. He was an ex-smoker, with a previous history of 2-pack years.
A 67-year-old woman presented to her primary physician with a year of periumbilical abdominal discomfort with diarrhea, bloating, and unintentional weight loss. While undergoing workup for the abdominal pain, she presented to the ED with dyspnea that worsened over a few weeks. She had no associated chest pain, lower extremity edema, fevers, or chills, but endorsed orthopnea. The patient's medical history included hypothyroidism and diabetes mellitus. Her family history was significant for coronary disease in her father and hypertension in her mother. She had no recent travel; she is a nonsmoker and rare alcohol drinker. She worked in administration.
A 67-year-old woman presented to her primary physician with a year of periumbilical abdominal discomfort with diarrhea, bloating, and unintentional weight loss. While undergoing workup for the abdominal pain, she presented to the ED with dyspnea that worsened over a few weeks. She had no associated chest pain, lower extremity edema, fevers, or chills, but endorsed orthopnea. The patient's medical history included hypothyroidism and diabetes mellitus. Her family history was significant for coronary disease in her father and hypertension in her mother. She had no recent travel; she is a nonsmoker and rare alcohol drinker. She worked in administration.
A 74-year-old man was referred to a pulmonologist for evaluation of a 1-year history of nonproductive cough and progressive exertional dyspnea. He was initially evaluated by his primary care physician, where he had spirometry that was negative for any obstructive or restrictive lung disease. An echocardiogram showed a normal left ventricular ejection fraction, with no wall motion abnormality or valvular heart disease. He had an outpatient chest radiograph performed (Fig 1), and he was subsequently treated empirically for a COPD exacerbation with 5days of oral prednisone and azithromycin. He was eventually referred to a pulmonologist because of a lack of clinical improvement. On seeing his pulmonary physician, he described the same exertional dyspnea and a nonproductive cough. A review of systems was negative for fever, chills, wheezing, angina, arthralgia, myalgia, rash, or leg swelling. He denied any medical illness and was not taking any medications. He was currently retired and had worked as a cashier hike" inspiratory crackles on lung examination. There was no digital clubbing, nor was there peripheral edema in his lower extremities. https://www.selleckchem.com/products/h3b-120.html He had no muscle tenderness and demonstrated normal muscle strength against resistance.High spinal cord injured patients (SCI) are susceptible to respiratory muscle impairments. Transcranial direct current stimulation (tDCS) and peripheral electrical stimulation (PES) may influence the diaphragm's central control, but until now they are not described as a therapeutic resource for difficult weaning. We present two case reports of SCI patients (P1 and P2) with long-term tracheostomy (>40 days) and hospital stay (>50 days). In association with respiratory exercise, P1 received a combined application of anodal tDCS over the supplementary motor area plus sensory PES in the thoracic-abdominal muscles, and P2 received isolated excitatory PES in the abdominal muscles, applied daily except on weekends. Maximum inspiratory/expiratory pressure, peak cough flow, diaphragm excursion, and thickening fraction were measured in the first and last days of the protocol. Both patients had improvements, with clinical impact such as cough effectiveness, decannulated after 15 applications of stimulation. Augmentation of neural respiratory drive and corticospinal excitability is suggested.Niemann-Pick Disease type B (NPDB) is a rare autosomal recessive disease belonging to the family of lysosomal storage disorders. NPDB is caused by mutations of sphingomyelin phosphodiesterase 1 gene (SMPD1) and is characterized by hepatosplenomegaly, interstitial lung disease, recurrent pulmonary infections, and neurologic disorders. Bronchiectasis are atypical. Until now, only three cases of lung transplantation for severe respiratory impairment have been reported. We describe a case of NPDB that was diagnosed after lung transplantation for cystic bronchiectasis. In 2016, a 31-year-old woman who was experiencing hypoxemic respiratory failure and recurrent pulmonary infections due to cystic bronchiectasis received a double-lung-transplantation. Histopathologic study on removed lungs revealed clusters of CD68 foamy lipid-laden macrophages with concentric and palisade arrangement, compatible with the diagnosis of NPDB, which was confirmed after SMPD1 genetic sequencing. Twenty-three months after transplantation, allograft function is stable (FEV1 was 100% of best-FEV1).