Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019079 (hemoptysis)
 6,129 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Goodpasture's syndrome, or anti-glomerular basement membrane (anti-GBM) disease, is a rare pulmonary-renal syndrome that results from autoantibody-mediated destruction of alveolar and glomerular basement membranes. It is defined by the triad of pulmonary hemorrhage (hemoptysis), glomerulonephritis (hematuria), and circulating anti-GBM antibodies. Although kidney disease can occur with or without lung involvement, isolated alveolar hemorrhage as an incipient manifestation of the syndrome is rare. We report the case of a patient with anti-GBM disease who initially presented with seronegative hemoptysis and normal urine. It was not until relapse of his condition that we found acute glomerulonephritis and circulating antibodies. In this report, we briefly review the pathogenesis and clinical manifestations of anti-GBM disease and discuss the relevance of isolated alveolar hemorrhage.
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PMID:Goodpasture's syndrome: a case of delayed appearance of autoantibodies and renal disease. 1156 50

Anti-basement membrane antibody disease is a rare disorder characterized by the presence of autoantibodies binding to the alveolar and glomerular basement membranes, and mediating both alveolar hemorrhage and acute glomerulonephritis. We retrospectively analyzed 28 cases of anti-basement membrane antibody disease with alveolar hemorrhage proven by bronchoalveolar lavage. The median age of patients at diagnosis was 23 years; 68% were male, 89% were active smokers, and 36% were exposed to some other inhaled agent. At diagnosis, 46% had predominant pulmonary involvement with normal initial serum creatinine. Lung function tests disclosed a restrictive ventilatory defect in 28% (n = 11) and hypoxemia (moderate in 29% and severe in 29%, n = 21). Carbon monoxide transfer factor was elevated in only 25% (n = 12). Bronchoalveolar lavage was more sensitive than any other criterion for detecting alveolar hemorrhage. After onset of treatment, new hemoptysis or transient worsening of hypoxemia occurred in 29% but did not affect pulmonary outcome. In contrast, worsening of renal function occurred in 33% and adversely affected renal outcome. At last follow-up (median, 2.6 yr; n = 24), all patients were alive and a complete cure was achieved in 50%. Long-term dialysis or renal transplantation was required in 42%, and 8% had mild chronic renal insufficiency. Last chest X-ray was normal in all cases, and no patient had respiratory insufficiency. All patients with predominant pulmonary involvement at presentation maintained independent renal function. In summary, this cohort was characterized by frequent exposure to tobacco smoking and other inhaled agents, and a constantly favorable pulmonary outcome contrasting with frequent chronic renal failure. Renal outcome was excellent in the subgroup of patients with predominant pulmonary involvement.
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PMID:Alveolar hemorrhage in anti-basement membrane antibody disease: a series of 28 cases. 1750 57

A 10-year-old girl was admitted with fever, cough, maculopapular rash, hemoptysis, dark-colored urine, edema, multiple lymphadenopathies, and hepatosplenomegaly. She developed acute glomerulonephritis during the course of these complex clinical features. Laboratory data showed hematuria, proteinuria, and hypocomplementemia. Serological tests showed positive human parvovirus B19 (HPVB19)-specific immunoglobin M (IgM) and HPVB19 DNA was detected in the patient's serum using polymerase chain reaction (PCR). Renal biopsy revealed acute endocapillary proliferative glomerulonephritis (AEPGN) with coarse granular C3 depositions in a "starry sky pattern," which is more peculiar to poststreptococcal glomerulonephritis. Electron microscopy showed subendothelial and small hump-shaped subepithelial electron-dense deposits in glomerular capillary walls. There was no evidence of either any mycobacterial, staphylococcal, or streptococcal infection, and the diagnosis of Goodpasture syndrome and connective tissue disorders was excluded during clinical and laboratory investigations. A diagnosis of HPVB19-induced pleuropneumonitis and glomerulonephritis was made. Through a literature search there was no documented pediatric case of AEPGN induced by HPVB19, and this case represents, to our knowledge, the first time that a direct relationship between parvovirus infection and AEPGN has been demonstrated in a child.
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PMID:Parvovirus B19-induced multisystemic vasculitis and acute endocapillary proliferative glomerulonephritis in a child. 2044 92

Pulmonary-renal syndromes (PRS) are characterized by the simultaneous presence of diffuse alveolar hemorrhage and acute glomerulonephritis. The most common causes of PRS are ANCA-associated vasculitides, Goodpasture's syndrome and systemic lupus erythematosus. The clinical picture of PRS includes hemoptysis (not always present), acute-onset anemia and renal abnormalities ranging from isolated urinary abnormalities to rapidly progressive glomerulonephritis. The severity of the pulmonary involvement determines the mortality risk as well as the need for mechanical ventilation in intensive care. The diagnosis of PRS is based upon clinical, serological, radiological and histological findings. Immunosuppressive therapy, along with an adequate support therapy (especially aimed at avoiding microbial infection), needs to be started promptly and effectively to reduce both the mortality risk and long-term complications such as end-stage renal disease.
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PMID:[Pulmonary-renal syndromes]. 2134 Dec 46