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At least 19 recordsLinked to original sources
PubMed2025

Exploiting pleiotropy to enhance variant discovery with functional false discovery rates.

The cost of recruiting participants for genome-wide association studies (GWASs) can limit sample sizes and hinder the discovery of genetic variants. Here we introduce the surrogate functional false discovery rate (sfFDR) framework that integrates summary statistics of related traits to increase power. The sfFDR framework provides estimates of FDR quantities such as the functional local FDR and q value, and uses these estimates to derive a functional P value for type I error rate control and a functional local Bayes' factor for post-GWAS analyses. Compared with a standard analysis, sfFDR substantially increased power (equivalent to a 52% increase in sample size) in a study of obesity-related traits from the UK Biobank and discovered eight additional lead SNPs near genes linked to immune-related responses in a rare disease GWAS of eosinophilic granulomatosis with polyangiitis. Collectively, these results highlight the utility of exploiting related traits in both small and large studies.

Humans↗
PubMed1979

Lymphomatoid granulomatosis of the larynx in a renal transplant recipient.

This is a case of lymphomatoid granulomatosis in a renal transplant patient, which presented as an ulcerating pharyngeal lesion and caused death by exsanguination from carotid artery erosion. The diagnosis was established at limited necropsy on the basis of a characteristic vaso-infiltrative and atypical lymphoreticular infiltration as defined by Liebow, et al, in 1972. As far as can be ascertained, this is the second reported case of lymphomatoid granulomatosis in an immunosuppressed patient and also is a presumed localized extrapulmonary form of this disorder which has recently been reported. The striking histologic similarities to midline malignant reticulosis are discussed.

Adult↗
PubMed1978

Radiation therapy in lymphomatoid granulomatosis.

Two female patients with lymphomatoid granulomatosis have received radiation therapy for local, progressive, symptomatic lesions. One patient was a 56-year-old woman with concomitant brain and pulmonary lesions which progressed on cyclophosphamide and prednisone therapy. The second patient was a 62-year-old woman with a supraorbital mass which appeared and progressed on prednisone therapy. Both patients exhibited rapid response to radiation therapy, in terms of relief of symptoms and objective evidence of subsidence of local disease. A discussion of the nature of lymphomatoid granulomatosis and its treatment is presented with a review of the literature. One hundred sixty-five cases have now been described including the two in this study, and in only one previous case was radiation therapy utilized to treat local disease, prior to the two cases described herein.

Cyclophosphamide↗
PubMedDate not supplied

Wegener's granulomatosis and midline (nonhealing) "granuloma".

There has been considerable controversy over the years regarding the distinctions between various disorders characterized by a necrotizing and granulomatous inflammation of the tissues of the upper respiratory tract and oral cavity. It now seems clear that if infections and other known agents can be excluded, three clinicopathologic entities remain: Wegener's granulomatosis (a systemic disease), idiopathic midline (nonhealing) granuloma, and premalignant or malignant lymphoreticular lesions. The antigenic stimulus for all three may be related but remains unidentified.

Diagnosis, Differential↗
PubMed1978

Cerebral angiitis.

Explore the source record for details and available documents.

Arteriosclerosis↗
PubMed1979

A rapid unfavorable outcome of Wegener's granulomatosis in early childhood.

Wegener's Granulomatosis was suspected in a 27-month-old female with a nodular, necrotizing lesion of the nose, diffuse subcutaneous nodules, and erythematous desquamation of the entire body. From 20 months of age on she had a purulent nasal discharge, recurrent infections of the upper and lower respiratory tract, a Coombs positive anemia, and enlargement of the spleen and liver. Treatment with azathioprine and corticosteroids produced transient improvement but three months later a dramatic relapse occurred. Cyclophosphamide was substituted for azathioprine but 10 days later the patient died and the autopsy confirmed the diagnosis of Wegener's Granulomatosis. The early age of onset of the disease may explain the unfavorable outcome, despite treatment with cytotoxic agents.

Adrenal Cortex Hormones↗
PubMed1977

The ocular manifestations of Wegener's granulomatosis. Fifteen years experience and review of the literature.

Ocular manifestations of Wegener's granulomatosis may occur secondary to contiguous granulomatous sinusitis or as a result of focal vasculitis. Contiguous granulomatous sinus disease causes nasolacrimal duct obstruction, proptosis and ocular muscle or optic nerve involvement. Focal vasculitis unrelated to contiguous upper respiratory tract disease is manifested by conjunctivitis, episcleritis, scleritis, corneoscleral ulceration, uveitis, and granulomatous vasculitis of the retina and optic nerve. A review of 29 cases of Wegener's granulomatosis and three cases of lymphomatoid granulomatosis studied over the past 15 years at the National Institute of Allergy and Infectious Diseases (NIAID) disclosed single or multiple ocular manifestations of disease in 15 patients (47 per cent). The pattern of ocular disease, its relationship to systemic involvement, diagnostic methods and the response to therapy are discussed.

Adolescent↗
PubMed1979

Diffuse pulmonary hemorrhage and rapidly progressive renal failure. An uncommon presentation of Wegener's granulomatosis.

A 57 year old woman presented with rapidly progressive renal failure and diffuse pulmonary hemorrhage and life-threatening respiratory failure promptly developed; these conditions resolved after bilateral nephrectomy. Renal pathology revealed rapidly progressive glomerulonephritis and vasculitis with granular deposition of immunoglobulin on immunofluorescent staining. One year later, multiple nodular cavitating pulmonary infilrates developed, and lung biopsy was diagnostic of Wegener's granulomatosis. Therapy with cyclophosphamide resulted in resolution of the pulmonary lesions. Diffuse pulmonary hemorrhage and rapidly developing renal failure mimicking Goodpasture's syndrome was the initial manifestation of Wegener's granulomatosis in this patient.

Acute Kidney Injury↗
PubMed1979

Multiple cranial nerve palsies in late metastasis of midline malignant reticulosis.

A 12-year-old man had a sudden onset of multiple cranial nerve palsies after treatment for a necrotizing lesion of the soft palate two years previously. It was thought that neurologic signs were secondary to extension of the local disease and radiation therapy to the base of the brain was begun. The patient died shortly thereafter. A diffuse atypical histiocytic lymphoma involving multiple cranial nerves, lumbosacral nerves, orbital muscles, and other organs was found on autopsy. Initial nasopharyngeal biopsy and autopsy findings were compatible with midline malignant reticulosis, a malignant lesion of the upper airway sometimes associated with metastasis. Our case is the first reported autopsy-documented case, to the best of our knowledge, of metastatic involvement of the cranial nerves in midline malignant reticulosis.

Adult↗
PubMed1979

Wegener granulomatosis. Unusual cause of necrotizing urethritis.

We report a case of Wegener granulomatosis presenting as a destructive urethral mass. The initial clinical impression was carcinoma, and a urinary diverting procedure was considered before the correct diagnosis was established. The importance of recognizing this unusual disease and its dramatic response to appropriate therapy are emphasized.

Cyclophosphamide↗
PubMed1979

Wegener's granulomatosis in childhood. A clinical report based on 3 cases.

Wegener's granulomatosis is a very rare disease in children; only 10 cases have been reported in the literature. The present report describes 3 cases where Wegener's granulomatosis developed at an early stage, beginning with upper respiratory tract symptoms. All the 3 children have been successfully treated with a combination of azathioprine and corticosteroids.

Adolescent↗
PubMed1978

Peripheral corneal degeneration and occlusive vasculitis in Wegener's granulomatosis.

A 61-year-old white woman, who had progressive arthritis over a two-year period, developed bilateral peripheral corneal degeneration. The superior cornea thinned to a thickness of approximately two thirds of normal. She died suddenly in the hospital and a primary occlusive vasculitis of the anterior ciliary arteries within the superior rectus muscles was found. There was necrotizing arteritis in the lungs, kidneys, adrenal caps, spleen, fallopian tubes, and skeletal muscle. The observation of an occlusive vasculitis of the anterior ciliary arteries in this patient supports the belief that ischemia may play a major role in peripheral marginal degeneration of the cornea.

Arterial Occlusive Diseases↗