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Therapeutic Apheresis in Children
Published in James L. MacPherson, Duke O. Kasprisin, Therapeutic Hemapheresis, 2019
Goodpasture’s syndrome is an autoimmune disease in which the development of antibasement membrane antibodies leads to severe renal disease and pulmonary hemorrhage. Since the introduction of PE and immunosuppressive drugs in the treatment of Goodpasture’s, the mortality has decreased dramatically. Since this disease is much more common in adults, there have been few reports in the pediatric literature. An 876 and 15 year old77 with this disease have been treated with PE. In both cases the patients responded with rapid improvement of their pulmonary problems, but treatment could not prevent renal failure. However, in the eight-year-old patient the renal disease progressed after the family refused further PE. A 10 year old with Goodpasture’s syndrome, who had renal compromise and severe respiratory disease, was treated with immunosuppressive therapy and PE. The patient’s respiratory and renal function returned to normal following the therapy.78
Pulmonary Immunology
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Hemant H. Kesarwala, Thomas J. Fischer
Cytotoxic (type II) reactions result from the binding of either IgG or IgM antibody to cell-bound antigens. This antigen-antibody binding activates the complement cascade with subsequent destruction of the cell (cytolysis) to which the antigen is bound. Clinical examples include immune hemolytic anemia and Rh hemolytic disease of the newborn. In the lung, Goodpasture’s syndrome is a clinical example of a type II reaction where antibodies reactive against alveolar and glomerular basement membrane are present in these patients. These antibodies bind to the alveolar basement membrane giving rise to pulmonary hemorrhage and an alveolitis.32 The inciting agent for such antibody formation is not known. Clinical improvement follows the use of therapy which suppresses this immune response.
Noninfectious Pulmonary Manifestations of Renal Disease In Children
Published in Lourdes R. Laraya-Cuasay, Walter T. Hughes, Interstitial Lung Diseases in Children, 2019
Stephen T. Lawless, H. Jorge Baluarte
Approximately 60 to 80% of patients will have both pulmonary hemorrhage and glomerulonephritis. The latent period between the first episode of hemoptysis and the discovery of the renal disease (usually in the form of abnormal urinalysis) may vary from a few weeks to several years, but averages about 3 months. Unfortunately the renal lesion progresses rapidly over time requiring some form of dialysis usually after 3 1/2 months (range <1 to 14 months). The glomerular injury is mediated by the interaction of the circulating autoantibody with intrinsic GBM glycoproteins, resulting in activation of the complement cascade and thus leading to the infiltration by inflammatory cells. Coagulation mechanisms and monocytes are seen to participate actively in the generation of crescents. Lung injury may be induced in a similar fashion; however, the lack of correlation between levels of anti-GBM antibody and pulmonary manifestations is difficult to account for. It is possible that nonantibody factors, perhaps released by the acutely damaged kidney, participate in the pulmonary injury. The triggering factor for Goodpasture’s syndrome is not known, but it is perhaps a combination of genetic factors and an exposure to an environmental agent like influenza, hydrocarbon, or penicillamine that damage the alveolar walls which then release an autoantigen.3
The impact of race on hospitalization outcomes for goodpasture’s syndrome in the United States: nationwide inpatient sample 2003–2014
Published in Hospital Practice, 2021
Wisit Cheungpasitporn, Charat Thongprayoon, Michael A. Mao, Boonphiphop Boonpheng, Tarun Bathini, Saraschandra Vallabhajosyula, Juan Medaura, Api Chewcharat, Swetha R. Kanduri, Karthik Kovvuru, Sohail Abdul Salim, Wisit Kaewput
Goodpasture’s syndrome, also known as anti-glomerular basement membrane (anti-GBM) disease, is an extremely rare but serious small vessel vasculitis [1,2]. It is an autoimmune disorder mediated by circulating anti-GBM autoantibodies against the noncollagenous domains (NC1) of type IV collagen alpha 3 chains (α3(IV)NC1). This causes direct injury to renal and pulmonary capillaries [3–6]. Thus, patients with Goodpasture’s syndrome typically present with ‘pulmonary-renal syndrome’ in the form of rapidly progressive glomerulonephritis (RPGN) with diffuse alveolar hemorrhage [1]. Kidney biopsy classically reveals crescentic and necrotizing glomerulonephritis on light microscopy with bright linear polyclonal IgG deposits on glomerular basement membrane (GBM) by immunofluorescence staining [3,4,7–9].
Hypnosis and end-stage renal disease: Review and treatment
Published in American Journal of Clinical Hypnosis, 2020
Although not extensive, there are some examples of successful individualized psychotherapy using hypnosis for dialysis patients. Dy and Fabbri (1972) treated a very anxious, multisymptomatic woman. She was admitted to the hospital in great distress and diagnosed with Goodpasture’s syndrome, a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and kidney failure. She was started on dialysis and became so dyspneic that she had to be tracheotomized and given 100% oxygen. She anxiously demanded oxygen whenever she was awake. The staff observed that she could be off oxygen while asleep without suffering from dyspnea. This unusual dependency on artificial respiration suggested a role for multiple psychological factors. Using an alternating tension and relaxation induction, she was given calming suggestions to imagine a peaceful, relaxing tranquil scene. She chose a warm beach. Suggestions were then made (a) to allow herself to breathe slowly and deeply, to feel the invigorating, refreshing air that was flowing into her lungs, (b) to feel better and more relaxed (post hypnotically), and (c) to enter a trance quickly in the future sessions. Out of trance, she was instructed to continue practicing the relaxation exercises several times each day. Eventually, she learned to perform the exercises on her own and was weaned off the oxygen which, originally, she said she needed desperately. Three months after admission, she was discharged.
Coronavirus-associated bronchiolitis in an immunocompetent adult with anti-glomerular basement membrane disease
Published in Canadian Journal of Respiratory, Critical Care, and Sleep Medicine, 2019
Anti-GBM disease is caused by circulating autoantibodies directed against alpha-3(IV)NC1, a major component of glomerular, alveolar, and other specialized basement membranes.11 This mechanism leads to glomerulonephritis associated with rapidly progressive renal failure and in 40-60% of the cases, alveolar hemorrhage.12 The presence of the latter is termed Goodpasture’s syndrome. Diffuse alveolar filling pattern from hemorrhage can be nonspecific. Radiological features of Goodpasture’s syndrome can range from diffuse nodular opacities with no zonal predominance but sparing of costophrenic angles and apices, to ill-defined areas of ground glass opacification.16,17 However, diffuse bronchiolitis is not a typical presentation in this disease, therefore making it difficult to reconcile our patient’s radiological findings with alveolar hemorrhage on bronchoscopy.