Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0348321 (Haemophilus)
 15,372 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diffuse panbronchiolitis (DPB) is an idiopathic inflammatory disease, largely restricted to Japan, that is characterized by progressive suppurative and obstructive airway disease, which, if left untreated, progresses to bronchiectasis, respiratory failure, and death. The lesion was first described in the early 1960s. In 1969 the name diffuse panbronchiolitis (DPB) was proposed to distinguish it from chronic bronchitis. Diffuse refers to the distribution of the lesions throughout both lungs, and pan refers to the involvement of inflammation in all layers of the respiratory bronchioles. Its distinctive imaging and histologic features, the coexisting sinusitis, and the isolation of Haemophilus influenzae and Pseudomonas aeruginosa in the sputum should enhance disease recognition. Neutrophils and T-lymphocytes, particularly CD8- cells, together with cytokines IL-8 and macrophage inflammatory protein-1 are believed to play key roles in the development of this disease. Significant improvement in the prognosis of this potentially fatal disease has been reported after the use of long-term therapy with macrolide antibiotics, the effect of which is attributed to an anti-inflammatory and immunoregulatory action.
Sarcoidosis Vasc Diffuse Lung Dis 2004 Jun
PMID:Diffuse panbronchiolitis. 1528 30

TNFRSF13B/TACI defects have been associated with CVID pathogenesis and/or phenotype, especially the development of benign lymphoproliferation and autoimmunity. Our purpose was to investigate the role of TNFRSF13B/TACI defects in the pathogenesis of two common lymphoproliferative disorders, namely, sarcoidosis and tonsillar hypertrophy (TH). 105 patients (71 with sarcoidosis and 34 with TH, including 19 without infectious causative and 15 due to Haemophilus influenzae) were analyzed for TNFRSF13B/TACI defects. Two out of 19 TH patients without infectious cause (10.5%) and 2 patients with sarcoidosis (2.8%) displayed rare TNFRSF13B/TACI defects (I87N, L69TfsX12, E36L, and R202H, resp.). Both mutations identified in TH patients have been assessed as deleterious for protein function, while the patient with the R202H mutation and sarcoidosis exhibited also sIgG4D. Our study further supports the notion that TNFRSF13B/TACI defects alone do not result in CVID but may be also found frequently in distinct clinical phenotypes, including benign lymphoproliferation and IgG subclass deficiencies.
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PMID:Heterozygous alterations of TNFRSF13B/TACI in tonsillar hypertrophy and sarcoidosis. 2395 60

Among 547 Haemophilus influenzae isolates recovered in our center, 45 displayed a phenotype of loss of PBP 3 affinity (8.2%). Two isolates with 6 substitutions in PBP 3 showed decreased susceptibility to third-generation cephalosporins. Clinical data revealed clinical failure after ceftriaxone treatment in a context of bronchitis in a patient with pulmonary sarcoidosis.
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PMID:Prevalence of Haemophilus influenzae with alteration of PBP 3 sequence over a 1-year period in a French hospital: focus on a clinical failure after ceftriaxone treatment. 3024 13