[AUTOIMMUNE PATHOGENESIS IN THE BACKGROUND OF PULMONARY ALVEOLAR PROTEINOSIS]
LOSONCZY György
JANUARY 21, 2006
Lege Artis Medicinae - 2006;16(01)
LOSONCZY György
JANUARY 21, 2006
Lege Artis Medicinae - 2006;16(01)
[Pulmonary alveolar proteinosis (PAP) is a rare form of interstitial lung disease. The etiology and pathogenesis of PAP has remained uncertain. It is characterized by the accumulation of surfactant in alveolar space, which leads to impaired oxygen diffusion and lung restriction. Patients develop chronic respiratory failure. Traditional treatment of the disease has been whole-lung lavage. In recent years the role of alveolar macrophages in the removal of surfactant has been discovered with granulocyte-macrophage colony-stimulating factor (GM-CSF) triggering this function of macrophages. In the most frequent “idiopathic” subtype of this disease autoantibodies against GM-CSF have been shown to be present in blood. These autoantibodies block GM-CSF-induced surfactant removal by the alveolar macrophages. Clinical studies indicated that longterm, high dose therapy with GM-CSF can be effective in the cleaning the alveolar space and treating respiratory failure. This new treatment modality makes high-risk whole-lung lavage avoidable.]
Lege Artis Medicinae
Lege Artis Medicinae
Lege Artis Medicinae
Lege Artis Medicinae
Clinical Neuroscience
CANOMAD (chronic ataxic neuropathy, ophthalmoplegia, M-protein agglutination, disialosyl antibodies) syndrome is a rare polyneuropathy. IgM paraproteins react with ganglioside-containing disialylated epitopes resulting in dorsal root ganglionopathy and B-lymphocyte infiltration of cranial and peripheral nerves. Clinical features include ataxia, slight muscle weakness, areflexia, sensory- and cranial nerve symptoms. Case studies have reported the efficacy of rituximab and intravenous immunoglobulin (IVIg) treatments. We present the case of a 57-year-old man, who had difficulty walking, with numbness and clumsiness in all limbs. He had areflexia, vibratory sensation loss and ataxia. Laboratory tests showed IgM monoclonal components and disialosyl antibodies in the serum. Nerve conduction studies indicated severe sensorimotor demyelinating polyneuroradiculopathy. Despite IVIg and rituximab treatments, the patient’s disease course gradually worsened and he died of respiratory failure. Neuropathological examination revealed dorsal column- and dorsal root atrophy with mixed mononuclear cell infiltration. This article aims to draw attention to this syndrome, and the use of early potent immunosuppressive treatment to improve patients’ quality of life.
Lege Artis Medicinae
[Over the past decade, the use of noninvasive ventilation in the setting of acute exacerbations of chronic obstructive pulmonary disease (COPD) has gained popularity, and is recommended by evidence-based guidelines. The evidence that it should be effective in chronic COPD is much weaker, and large, prospective, randomised, controlled studies that would also provide a guide for the selection of the best candidates, are still lacking. It has bee established, however, that home mechanical ventilation is certainly beneficial for a well-defined subgroup of patients. This includes the so called “blue bloater” patients (with hypercapnia and polyglobulia), other cases with increased hypercapnia, and patients with "overlapping" syndrome (COPD accompanied by sleep apnoe). Also, for patients with acute respiratory failure who refuse intratracheal intubation noninvasive mechanical ventilation remains the only choice. The past years have unequivocally proved the superiority of the positive pressure ventilation mode for either short term or long term use.]
Journal of Nursing Theory and Practice
[Acute respiratory distress syndrome (ARDS) is a medical condition characterized by a high mortality rate. ARDS may be triggered by various pathologies such as sepsis and can have a significant impact on the overall outcome of primary disease. Prone positioning as a supportive strategy in the treatment of ARDS that has been investigated since 1974. Lying face down has become more and more popular because it might have helped to improve oxygenisation in 70 percent of patients with ARDS. Occurence of ARDS did not change in the last 10 years in Europe and the syndrome is still associated with a quite high death rate between 40-50% despite of technologic and therapeutic improvement of last decades. It has already been investigated whether prone positioning may increase survival in patients with ARDS. However, approaches to the exact use of position are often occasional. Guideline development would be crucial to emphasize beneficial effect of prone positioning in patients suffering from ARDS and describe the process by which the manoeuvre may be performed. Primarily, it is substantial to improve oxygenation through the use of the prone position whilst promoting patient safety. Complications can be minimized by using a predefined strategy of placing patients in a prone position and a related checklist. ]
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