Academic literature on the topic 'Respiratory insufficiency Etiology'

Pycnodysostosis (PDO) is a rare genetic disorder characterized by cathepsin K deficiency which plays an important role in bone metabolism. Among clinical features of this disease which are mainly caused by altered bone remodeling are craniofacial abnormalities such as hypoplastic maxilla and obtuse gonial angle which consequently lead to respiratory insufficiency in forms of pharyngeal narrowing and severe snoring. In this paper, another case of this rare disorder is presented along with a review on etiology and management issues of respiratory insufficiency in these patients.

AbstractSpinal muscular atrophy with respiratory distress type 1 (SMARD1) is a rare autosomal recessive neuromuscular disorder that involves the anterior horn motor neurons. It is a disease with a poor prognosis presenting with progressive distal motor weakness and respiratory insufficiency from diaphragmatic paralysis followed by distal muscle weakness before 6 months of age. With the intent to spread the awareness of this rare and life-threatening disease, we report a 2.5-month-old female infant with a subsequent diagnosis of SMARD1, who was admitted in our pediatric intensive care unit with chief complaint of progressive respiratory distress and poor feeding.

The problem of etiologic diagnosis of bilateral pleural effusions is important because of the relatively large number and variety of diseases accompanied by this syndrome, the complexity of diagnosis verification, and the frequent diagnostic errors. The aim of this review is to describe the spectrum of diseases causing bilateral pleural effusion and to consider a set of diagnostic measures to clarify the etiology of the process. Analysis of 60 literature sources showed that the most common causes of bilateral transudative pleural effusions are cardiac, hepatic, and renal insufficiency. Exudative bilateral pleural effusions are found in inflammatory processes in the pleura, including tuberculosis, and develop when inflammation is transmitted by contact or lymphohematogenous routes from the lungs or other organs. Bilateral localization of pleural effusion in tumor processes is observed in 5.7% of cases. Bilateral pleural effusion is seen in pulmonary embolism, diffuse connective tissue diseases, acute idiopathic pericarditis, postinfarction Dressler syndrome, after pericardotomy, and after pacemaker placement. It may be observed in such rare diseases as sarcoidosis, yellow nail syndrome, and Meigs syndrome, and may be induced by some drugs. Conclusion. The choice of therapeutic measures for bilateral pleural effusion is determined by an accurate etiological diagnosis of the underlying disease. The diagnosis should be based on the patient's clinical data and cytologic, microbiologic, and biochemical analysis of pleural fluid obtained by pleural puncture. In some cases, additional examination methods such as pleural biopsy, bronchoscopy, ultrasound, computed, magnetic resonance imaging of the chest and abdomen, and positron emission tomography are required.

The authors of this paper wish to present a case of fatal cor pulmonale with right ventricular hypertrophy complicated by a congenital myopathy. It is our intention to demonstrate the importance of vigilant clinical assessment of children with a congenital myopathy, regardless of the exact etiology of their disease, or family history of disease severity. This case highlights the risk for fatal complications if hypoventilation and respiratory insufficiency go unrecognized in myopathic children. Consequently, we recommend respiratory and cardiac monitoring surveillance as well as appropriate referral to specialists in the management of such children.

Canine chronic bronchitis (CCB) is considered to be one of the most common chronic respiratory diseases of dogs. The hallmark of CCB is chronic and progressive cough that leads to poor life quality of the dog. Severe cases may progress to respiratory insufficiency and death. The cause is an inflammatory process of unknown origin. The disease is most common in adult and older dogs of small animal breeds. In severe cases, this chronic inflammatory process progresses to irreversible structural changes in the airways, resulting in respiratory failure. Despite this, information about the disease is extremely limited in the literature, and the recommended treatment is based on poor scientific evidence. Thus, the aim of this study is to review the subject and describe the etiology, pathophysiology, clinical features, complementary diagnosis and treatment of CCB.

Among the differential diagnoses that should be considered in acute respiratory failure (ARF) are infectious processes, autoimmune diseases, interstitial pulmonary fibrosis, and pulmonary neoplasia. Timely diagnosis of lung neoplasia is complicated in the early stages. An opportune diagnosis, as well as the specific treatment, decrease mortality. ARF occurs 1 in 500 pregnancies and is most common during the postpartum period. Among the specific etiologies that cause ARF during pregnancy that must be considered are: (1) preeclampsia; (2) embolism of amniotic fluid; (3) peripartum cardiomyopathy; and (4) trophoblastic embolism. The case of a 36-year-old patient with a 33-week pregnancy and ARF is presented. The patient presented dyspnea while exerting moderate effort that progressed to orthopnea and type 1 respiratory insufficiency. Imaging studies showed bilateral alveolar infiltrates and predominantly right areas of consolidation. Blood cultures, a galactomannan assay and IgG antibodies against mycoplasma pneumoniae, were reported as negative. Autoimmune etiology was ruled out through an immunoassay. A percutaneous pulmonary biopsy was performed and an invasive pulmonary adenocarcinoma with lepidic growth pattern (i.e. lepidic pulmonary adenocarcinoma, LPA) result was reported. This etiology is rare and very difficult to recognize in acute respiratory failure cases. After infectious, autoimmune and interstitial lung fibrosis have been excluded the clinician must suspect of lung cancer in a patient with acute respiratory failure and chest imaging compatible with the presence of ground-glass nodular opacities, a solitary nodule or mass with bronchogram, and lung consolidation. In the presence of acute respiratory failure, the suspicion of pulmonary neoplasia in an adult of reproductive age must be timely. Failure to recognize this etiology can lead to fatal results.

A leptospirose é uma zoonose de alta morbidade em humanos e um importante problema de saúde pública. Causada por bactérias do gênero Leptospira, a doença apresenta diversas formas clínicas e é especialmente importante em países em desenvolvimento. Síndrome pulmonar hemorrágica é a maior causa de óbito em pacientes com formas severas da doença. Os mecanismos patogênicos relacionados à síndrome pulmonar hemorrágica na leptospirose humana são desconhecidos. Com o objetivo de avaliar estes mecanismos patogênicos, 30 necrópsias (tecido pulmonar) de pacientes com síndrome pulmonar hemorrágica na leptospirose e 7 controles foram avaliados. Para determinar a participação os mecanismos patogênicos envolvidos, experimentos de histologia e imunohistoquímica (IgM, IgG, IgA, and C3) foram realizados em amostras de tecidos pulmonares, bem como dosagem sérica de auto-anticorpos específicos (anticardiolipina e anti-membrana basal) de amostras pareadas de soros de pacientes com leptospirose com e sem síndrome hemorrágica pulmonar e de indivíduos doadores de banco de sangue. Nos achados patológicos, os pacientes com síndrome hemorrágica pulmonar na leptospirose diferem dos controles com hemorragia pulmonar em alguns aspectos: moderada ou intensa presença de macrófagos na luz alveolar (97% versus 29%, respectivamente; p < 0.01); presença de membrana hialina na superficie alveolar (100% versus 0% respectivamente; p < 0.01); intensa necrose e regeneração de pneumócitos II (100% versus 0%, respectivamente; p < 0.01); e presença de plasmócitos no septo aveolar (80% versus 29%; p < 0.02). Nenhuma diferença estatisticamente significativa foi observada em relação ao número de outras células no septo alveolar. Leptospiras intactas foram raramente observadas. A detecção de antígeno de leptospira não foi correlacionada com a intensidade de hemorragia pulmonar. Em nenhum dos tecidos pulmonares estudados foi evidenciado alterações microscópicas sugestivas de coagulação intravascular disseminada. Deposição de imunoglobulina foi detectada na superfície alveolar de 18 de 30 pacientes com síndrome pulmonar hemorrágica na leptospirose. Três padrões de marcação de imunoglobulina e complemento foram observados em tecido pulmonar de pacientes com hemorragia pulmonar e leptospirose: (A) marcação linear delicada, como uma membrana, recobrindo a superfície luminal alveolar de pneumócitos I e II; (MF) marcação multifocal, aleatória ao longo do septo; e (I) marcação fraca granular, focal, intra-alveolar. Não houve diferenças significativas na concentração de auto-anticorpos contra membrana basal nos diferentes grupos estudados. Observamos diferenças significativas nos títulos de anticorpos IgM anticardiolipina entre a primeira e segunda amostra, nos pacientes com e sem hemorragia pulmonar (p<0.01 e p=0.04, respectivamente). Aumento significativo nos títulos de anticorpos anti-cardiolipina da classe IgG, bem como na relação IgG/IgM, foi observado apenas nos pacientes com hemorragia pulmonar (p=0.01 e p=0.01). Nós concluímos que o comprometimento pulmonar na leptospirose humana grave ocorre principalmente sob a forma de uma pneumopatia hemorrágica com características peculiares, cujo quadro morfológico difere de outras hemorragias pulmonares. Caracteriza-se pela deposição linear de imunoglobulina (IgM, IgG e IgA) e complemento(C3) na superfície luminal alveolar de pneumócitos I e II e multifocal nos septos alveolares. Associa-se à intensa necrose de pneumócitos I e II, regeneração de pneumócitos II, além de inflamação septal e alveolar
Leptospirosis is a zoonotic disease that is a cause of high morbidity and mortality in humans and is an important public health problem. Caused by bacteria of Leptospira genus, this disease presents diverse clinical manifestations and is especially important in developing countries. Leptospirosis pulmonary hemorrhage syndrome is the major cause of death in patients with the severe form of leptospirosis. The pathogenic mechanisms of this syndrome are unknown. With the purpose of identifying these pathogenic mechanisms, 30 necropsies (pulmonary samples) from patients with leptospirosis pulmonary hemorrhage syndrome and seven controls were evaluated. . To determine whether the immune system is involved, histology and immunohistochemistry (IgM, IgG, IgA, and C3) experiments were performed on lung tissue samples, as well sera measurements of autoantibodies (against the basal membrane and anti-cardiolipin) were performed in leptospirosis patients with and without pulmonary hemorrhage syndrome (in paired samples) and in healthy donors from a blood bank. We found that patients with leptospirosis pulmonary hemorrhage syndrome differed from control pulmonary hemorrhage patients in several features: the presence of moderate to high levels of macrophages in the alveolar space (77% versus 29%, respectively; p = 0.02), the presence of the focal hyaline membrane on alveolar surface (100% versus 0%; p < 0.01), extensive necrosis and regeneration of pneumocyte II cells (100% versus 0%; p < 0.01) and the presence of plasma cells in the alveolar septum (77% versus 29%, respectively; p =0.02). No statistically significant differences were observed in the number of others cells in the alveolar septae. Intact leptospires were rarely detected. Leptospiral antigen was not correlated with the intensity of the lesions. None of the patients showed microscopic evidence for disseminated intravascular coagulation. Immunoglobulin deposits were detected on the alveolar surface of 18/30 leptospirosis patients with pulmonary hemorrhage. Three staining patterns were observed for the immunoglobulins and C3 in the lung tissues of leptospirosis patients with pulmonary hemorrhage syndrom: (A) delicate linear staining adjacent to the alveolar surface, like a membrane covering the luminal surface of type I and II pneumocyte cells; (MF) random, multifocal staining along the alveolar septum; and (I) weak, focal intra-alveolar granular staining.. We were not able to show any significant difference in autoantibodies concentration in the different groups. We found significant difference between the titles of anticardiolipin IgM antibodies in the first and second sera sample from leptospirosis patients with and without pulmonary hemorrhage (p<0.01 e p=0.04, respectively). The increased in the titles of anti-cardiolipin IgG antibodies, as well IgG/IgM ratio was observed only in patients with pulmonary hemorrhage(p=0.01 and p=0.01). We concluded that the pulmonary involvement on severe human leptospirosis have particular characteristics, which the morphologic aspect differ from the others causes of lung hemorrhage. It was distinguished by linear deposition of immunoglobulin and complement (C3C) on the luminal alveolar surface of pneumocyte I and II cells. This event was associated with pneumocyte I and II cells necrosis, pneumocyte II regeneration and septal and alveolar inflammation

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disorder of unknown etiology that affects upper and lower motor neurons resulting in progressive atrophy of skeletal muscles. There are two forms of ALS: spinal motor neuron injury and bulbar paresis. Dysphagia is a highly prevalent severe and invalidating symptom in ALS: almost 80% of ALS patients with bulbar paresis will develop dysphagia. Also, dysphagia is one of the most common and serious complications, with respiratory insufficiency, in patients with ALS as it exposes them to malnutrition, dehydration and aspiration pneumonia. These conditions are reported to be associated with a minor survival in patients with ALS. Screening for dysphagia must be performed in all ALS patients at diagnosis and during the follow-up to approach dysphagia as soon as possible. This chapter includes the latest developments in the assessment and approach of dysphagia in ALS patients.