Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0034069 (pulmonary fibrosis)
 7,050 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The primary hypogammaglobulinemias, with onset of acute and recurrent bacterial infections in infancy and early childhood, consist of a heterogeneous group of largely genetically determined antibody deficiency states including that congenital sex-linked form, Bruton's agammaglobulinemia. Patients with panhypogammaglobulinemia require continuous gamma globulin therapy; in spite of this, they continue to develop infections of the upper and lower respiratory tract in the form of otitis media, mastoiditis, sinusitis, rhinitis, pharyngitis, tracheobranchitis, or pneumonia of a chronic and recurrent nature. The frequency and severity of these infections vary from patient to patient. These episodes all respond to antibiotic administration, often with a prolonged course. Many patients develop permanent pulmonary sequelae in the form of atelectasis, bronchiectasis, and pulmonary fibrosis. Most of these changes involve focal areas of the lower right middle and left lingular lobes. Occasionally, the patient may develop generalized bronchiectasis but without hilar lymphadenopathy. Management emphasizes early detection, early institution of gamma globulin treatment, and administration of appropriate antibiotic therapy at the earliest onset of infection. Good pulmonary toilet, nutritional care, emotional care, and a loving home environment are of utmost importance. These patients should be followed in a medical center with the joint effort of specialists in various disciplines.
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PMID:Respiratory complications of primary hypogammaglobulinemia. 7 Jul 74

Orbito-ocular involvement in Wegener's granulomatosis is the result of both focal ischaemic vasculitis and granulomatous soft tissue inflammation. Necrotising keratoscleritis and orbital inflammation are two most characteristic ophthalmic presentations. We describe a 56-year-old man with systemic limited Wegener's granulomatosis, presenting with pulmonary fibrosis, pansinusitis and left mastoiditis. This was complicated by the development of a left severe necrotising anterior scleritis, peripheral ulcerative keratitis and orbital apex syndrome. Both c-ANCA and anti-PR3 were positive. Despite mainstay systemic immunosuppressive therapy with cyclophosphamide and prednisolone, the visual prognosis remained very poor. This was largely due to the presence of an irreversible ischaemic optic neuropathy, extensive corneoscleral melt and corneal neovascularisation. This case highlights the possible extent of orbital and ocular surface involvement in Wegener's granulomatosis, and hence the importance of vigilance by the physician.
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PMID:Systemic Wegener's granulomatosis with severe orbito-ocular involvement. 1894 92