1/14. Coexistent yellow nail syndrome and selective antibody deficiency.BACKGROUND: yellow nail syndrome (YNS) is a rare, often underdiagnosed condition of unknown origin. The clinical features of the syndrome include yellow nails, chronic sinusitis, bronchiectasis, pleural effusion, and lymphoedema. Despite the frequent occurrence of upper and lower respiratory tract infections in patients with YNS, comprehensive analysis of their humoral immunity has not been previously reported. OBJECTIVE: To present the case of a patient with YNS whose recurrent upper and lower respiratory tract infections may have been caused by an underlying selective antibody deficiency that manifests as impaired IgG antibody response to polysaccharide antigens. methods: The patient underwent cultures of purulent sputum for streptococcus pneumoniae and haemophilus influenzae, bronchial washings for H. influenzae, and nail scrapings for fungi. Her serum levels of IgG, IgA, IgM, IgG subclasses, and serum titers of IgG antitetanus toxoid, anti-H. influenzae, and anti-S. pneumoniae antibodies were measured. RESULTS: Cultures of purulent sputum were positive on multiple occasions for S. pneumoniae and H. influenzae and bronchial washings were positive for H. influenzae. Nail scrapings were consistently negative for fungi. She had no reductions in serum levels of IgG, IgA, IgM, or IgG subclasses and had normal serum titers of IgG antitetanus toxoid antibodies. However, she demonstrated impaired IgG antibody responses following immunization with Pneumovax and an H. influenza B vaccine. CONCLUSIONS: This case report describes the first comprehensive analysis of humoral immune function in a patient with YNS. The finding of a selective antibody deficiency in our patient provides a potential explanation for the occurrence of respiratory infections in YNS. Accordingly, we recommend that functional antibody determinations and quantitative serum immunoglobulins be evaluated in patients diagnosed as having this unusual, enigmatic syndrome.- - - - - - - - - - ranking = 1keywords = respiratory tract infection, respiratory tract, tract infection, tract, upper (Clic here for more details about this article) |
2/14. Clinical evaluation and treatment of the adult patient with suspected primary immunodeficiency disease: a case analysis.PURPOSE: To describe immunoglobulin G2 (IgG2) subclass deficiency in the context of primary immunodeficiency disorders, their pathophysiology, epidemiology, clinical evaluation, and management. DATA SOURCES: Actual case study and extensive review of the scientific and medical literature. CONCLUSIONS: Consideration should be given to primary immunodeficiency diseases as one possible cause of recurrent upper and lower respiratory tract infections in patients at any age. early diagnosis and intervention can significantly reduce the burden of these diseases. IMPLICATIONS FOR PRACTICE: Advanced practice nurses need to keep pace with the ever-expanding field of immunity. knowledge of basic principles of the immune system facilitates a logical approach to the evaluation and management of primary immunodeficiency diseases.- - - - - - - - - - ranking = 0.5keywords = respiratory tract infection, respiratory tract, tract infection, tract, upper (Clic here for more details about this article) |
3/14. Recurrent infections and joint pain.A seven-year-old white male presented with recurrent bouts of paranasal sinusitis, streptococcal pharyngotonsillitis, lower respiratory tract infections, continuous low-grade fever, and conjunctivitis, which required frequent use of antibiotics over a period of two years. A careful review of systems also revealed a six-month history of arthralgia affecting his knees, elbows, and hands, which limited his daily activities. Prominent in the history were recurrent bouts of a generalized salmon-red, nonpruritic rash, which was most pronounced on the face and trunk and which was exacerbated by fever. His past medical history revealed severe bouts of gastroesophageal reflux disease, chronic intermittent bloody mucous diarrhea, and atopic dermatitis. A detailed review of the patient's family pedigree over five generations revealed a strong genetic predisposition for autoimmune diseases of several types. His physical examination revealed a thin, pale, chronically ill-appearing male, bilateral conjunctivitis, and pale nasal mucosae with no lymphadenopathy, organomegaly, arthritis, or rash. All laboratory results were unremarkable except for a positive rheumatoid factor and a suboptimal antibody response to immunization with pneumococcal vaccine. A diagnosis of juvenile rheumatoid arthritis of the systemic onset type was established, and, based upon his humoral immune deficiency, treatment with intravenous immunoglobulin was initiated with remarkable improvement in his symptomatology.- - - - - - - - - - ranking = 0.4999963067983keywords = respiratory tract infection, respiratory tract, tract infection, tract (Clic here for more details about this article) |
4/14. New and controversial uses of intravenous gamma-globulin.During the last few years the use of intravenous immunoglobulin (IVIG) has attracted increasing interest for the treatment of patients who do not have a classical humoral antibody deficiency syndrome. In certain situations this approach has revolutionized medical management, e.g. in immune thrombocytopenia. In other areas, such as in Kawasaki's syndrome, IVIG therapy have been shown to be highly beneficial in preventing long term disease sequelae by some investigators, but the field remains controversial. Conditions under which IVIG therapy has been shown to be of potential benefit are: (1) intractable childhood epilepsy; (2) autoimmune diseases, e.g. myasthenia gravis, systemic lupus erythematosus, idiopathic thrombocytopenic purpura, idiopathic neutropenia and aplastic anemia; (3) atopic allergy with IgG subclass deficiency including bronchial asthma; (4) in severe infections in combination therapy with antibiotics and as an antipyretic; (5) in Kawasaki's disease; (6) in multiple myeloma and chronic lymphocytic leukemia. Oral and intraventricular administration of IVIG have also been tried, the former for severe diarrhea and the latter to try to rescue the central nervous system from damage by a pathogen. Carefully controlled clinical trials are needed to establish the efficacy of gamma-globulin therapy in these and other conditions.- - - - - - - - - - ranking = 0.00016378382657764keywords = tract (Clic here for more details about this article) |
5/14. Juvenile onset pernicious anemia, partial intestinal villous atrophy, ulcerative colitis, and squamous metaplasia of the stomach.We report a case of a 13-yr-old white boy with juvenile onset pernicious anemia in association with igg deficiency. He had marked gastric atrophy, intestinal metaplasia of the stomach, and an intractable antral ulcer that required surgery. In addition, his gastric mucosa showed evidence of a progressive squamous metaplasia. Diffuse squamous metaplasia of the stomach, a very rare gastric lesion, has not previously been described either in association with pernicious anemia, atrophic gastritis, or hypogammaglobulinemia. This patient also has ulcerative colitis involving the entire colon and partial villous atrophy noted on small intestinal biopsy.- - - - - - - - - - ranking = 8.1891913288822E-5keywords = tract (Clic here for more details about this article) |
6/14. Serial immunologic and histopathologic studies in the treatment of necrotizing fasciitis with combined immunodeficiency by a bovine thymic extract (thymostimulin).A 25-day-old Chinese female baby developed necrotizing fasciitis (NF) with indurated erythematous patches with superficial necrosis over two thirds of the back. skin cultures of the lesions yielded streptococcus pyogenes, enterococcus, pseudomonas aeruginosa, and candida species. The patient had lymphopenia with low T cells and T cell subsets. The lymphoproliferative response to mitogen and macrophage migration (MIF) production were also poor. Her lymphocytes were unable to produce IgG in vitro. Also, IgG bearing cells were not demonstrable. The initial lymph node biopsy demonstrated an absence of follicular formation and depletion of lymphocytes in both thymic-dependent and thymic-independent areas. Five weeks after bovine thymic extract (Thymostimulin) treatment, a second lymph node biopsy demonstrated germinal centers containing IgG bearing cells. Three weeks later, a third lymph node biopsy showed germinal centers with cuffs of lymphocytes. Differentiation of the cortex and medulla of the node was demonstrated. IgG was also detectable in an in vitro Ig synthesis study. The total T cells, T cell subsets, lymphoproliferative response MIF production also increased gradually after Thymostimulin treatment. This investigation demonstrated the therapeutic effectiveness of Thymostimulin in NF with underlying combined immuno-deficiency, both serious and frequently fetal diseases, by the histologic and immunologic reconstitution of T and B cell function.- - - - - - - - - - ranking = 0.00040945956644411keywords = tract (Clic here for more details about this article) |
7/14. Thymic hormone dependent immunodeficiency in an infant with partial trisomy of chromosome 22.An infant with partial trisomy 22 syndrome and recurrent infections showed severe cellular immunodeficiency and serum IgG reduction. A marked increase of E-RFCs was observed after in vitro incubation of peripheral blood lymphocytes with thymostimulin (TS), a calf thymic extract. The treatment with TS resulted in reduction of infections, correction of cellular immunodeficiency and increase of IgG levels. Such a primary thymic hormone dependent immunodeficiency may be due to an altered development of branchial arches, commonly present in trisomy 22 syndrome. The same pathogenetic mechanism may account for thymus dysplasia observed in other chromosomal syndromes.- - - - - - - - - - ranking = 8.1891913288822E-5keywords = tract (Clic here for more details about this article) |
8/14. A rare case of variable immune deficiency with type II dysgammaglobulinaemia, light chain defect, gut associated iga deficiency and progressive neutropenia.This report describes in detail an unusual variant of a common variable immunodeficiency disease in a seven-year-old boy. The unique features were progressive neutropenia due to defective myelopoiesis, serum IgG and IgA deficiencies, defective immunoglobulin light-chain synthesis, absence of secretory IgA and IgM gammopathy. He had been born healthy, but following a thermal injury at the age of 1 1/2 years, he suffered recurrent attacks of sinopulmonary and urinary tract infections, enteritis due to enteropathogenic E. coli, giardia lamblia and E. histolytica, developed pulmonary tuberculosis and died of deep mycotic infection of the oral cavity and obstruction of the bronchial tree. The cause of the defective myelopoiesis could not be determined, but it might have been due to prolonged sulphomamide therapy administered for controlling his persistent urinary tract infection due to paraphymosis.- - - - - - - - - - ranking = 0.032528454608072keywords = tract infection, tract (Clic here for more details about this article) |
9/14. Selective immunoglobulin G4 deficiency and recurrent infections of the respiratory tract.Using a newly developed radioimmunoassay, 4 of 422 subjects tested were found to have an isolated absence of the immunoglobulin subclass IgG4. All had severe recurrent sinopulmonary infection. The association of these disorders with an isolated absence of IgG4 suggested that this immunoglobulin subclass may have a unique role in host respiratory tract defense mechanisms.- - - - - - - - - - ranking = 0.17548153470484keywords = respiratory tract, tract (Clic here for more details about this article) |
10/14. Acquired total immunoglobulin g deficiency in a patient with primary biliary cirrhosis.A 38-year-old Japanese woman was hospitalized for susceptibility to respiratory tract infections. Clinical examinations revealed asymptomatic primary cholestasis, abnormally elevated immunoglobulin m (IgM) and antimitochondrial antibody, being consistent with asymptomatic primary biliary cirrhosis. Three years later her serum immunoglobulin g (IgG) decreased remarkably, whereas other immunoglobulins were unchanged. Immunological examinations on the peripheral blood lymphocytes demonstrated spontaneous over-synthesis of serum IgM and decreased synthesis of IgG due to abnormal function of both T and B cells. Our case suggests a new possible association between primary biliary cirrhosis and igg deficiency.- - - - - - - - - - ranking = 0.4999963067983keywords = respiratory tract infection, respiratory tract, tract infection, tract (Clic here for more details about this article) |
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