1/62. Selective antibody deficiency to bacterial polysaccharide antigens in patients with netherton syndrome.Three patients with netherton syndrome, recurrent sinopulmonary infections, and humoral immune deficiency are described. Although quantitative serum immunoglobulin levels were generally normal, two patients had selective antibody deficiency to bacterial polysaccharide antigens, one associated with IgA-IgG-2 deficiency. A third patient had an antibody deficiency to protein antigens. This is the first report, to our knowledge, that describes antibody deficiency in patients with netherton syndrome. This finding demonstrates the importance of evaluating functional antibody responses to both protein and bacterial polysaccharide antigens and not relying on IgG subclass determination.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
2/62. fucosidosis: immunological studies and chronological neuroradiological changes.A 3.5-y-old boy of Arabic origin had the clinical features of both type 1 and type 2 fucosidosis, consistent with an intermediate form of the disease. The activity of his leucocyte alpha L-fucosidase was absent. He presented with recurrent sinopulmonary infection and otitis media in addition to paronychia and a periapical dental abscess. Investigation of his systemic immune function did not reveal a significant underlying defect, but subtle abnormalities, particularly of antibody production and secretory IgA, cannot be excluded. The cranial magnetic resonance images showed periventricular and subcortical white matter abnormalities and mild cortical atrophy in addition to globus pallidus changes.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
3/62. Reversible total iga deficiency associated with phenytoin treatment.A 55-y-old male developed long-standing upper respiratory symptoms during phenytoin treatment of epileptic fits. Complete lack of serum IgA was noted repeatedly. Following replacement of phenytoin, normal IgA levels were found and a slow improvement in respiratory symptoms occurred. Immunoglobulin deficiency, in particular low levels of IgA, are not uncommon among phenytoin treated subjects. The occurrence of frequent respiratory infections in such patients justifies the investigation of immunoglobulin levels.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
4/62. Evolution of iga deficiency to IgG subclass deficiency and common variable immunodeficiency.FIRST REPORT: male child with repeated pulmonary infections from the age of 4 months. He was diagnosed as iga deficiency (undetectable IgA levels) at the age of 3 years, when he presented repeated bouts of pneumonia and tonsillitis. Several immunologic evaluations were made between the ages of 4 months and 8 years. At 8 years and 9 months, the diagnosis of iga deficiency was confirmed, and associated IgG2 and IgG4 deficiency (29.0 mg/dl y 0.01 mg/dl) with normal total IgG serum level was found. With the administration of intravenous gammaglobulin, the lung infections remitted and the subsequent clinical course has been uneventful up to now. SECOND REPORT: a boy with repeated infections since the age of 2 months. iga deficiency was diagnosed at 1 year 7 months (undetectable serum IgA levels). At age 51/2 years, his clinical course worsened and more serious infections appeared. A new immunologic study revealed iga deficiency associated with CD4 cell deficiency (432 cells/mm3) and normal CD3, CD19, and CD8 levels. Despite intensive antibiotic treatment and care, the child died. The findings suggest an association of iga deficiency and common variable immunodeficiency.- - - - - - - - - - ranking = 4keywords = infection (Clic here for more details about this article) |
5/62. Rapidly progressive tabes dorsalis associated with selective iga deficiency.tabes dorsalis is uncommon and progresses slowly from infection to clinical manifestation. We report a rare case of rapidly progressive tabes dorsalis associated with selective iga deficiency (sIgAD). A 28-year-old man was hospitalized with lightning back pain, nausea, and bladder bowel dysfunction. serum and cerebrospinal fluid (CSF) revealed high titers of treponema pallidum antibody, and the serum IgA level was less than 5 mg/dl. Thl-dominant cytokine expression was observed, as is usually seen in neurosyphilis. He was treated with ceftriaxone and CSF pleocytosis disappeared. We postulate sIgAD influenced the atypical rapid clinical course of tabes dorsalis in this patient.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
6/62. Sonographic detection of multiple brain abscesses in a newborn with iga deficiency.We report the case of a neonate with selective iga deficiency and multiple brain abscesses diagnosed with sonography. brain sonography revealed multiple abscesses in the left hemisphere; the abscesses ranged from 10 to 20 mm. Cultures obtained from the cerebrospinal fluid and blood were positive for proteus mirabilis. The neonate responded promptly to broad-spectrum antibiotic therapy and had no neurologic sequelae. Because iga deficiency is associated with infections, we believe it was a predisposing factor for the brain abscesses.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
7/62. plasma cell gingivitis.plasma cell gingivitis (PCG), an infrequent benign inflammatory condition of unknown etiology, is a type of plasma cell orificial mucositis, which includes a wide spectrum of conditions. We present the case of a 13-year-old girl who had PCG with an erythematous congestive plaque on the anterior maxillary gingiva for 4 years. Occasionally, the lesion became increasingly swollen and painful and bled. Results of a histopathologic examination showed dense plasmacytic infiltrate in the dermis, affecting the dermoepidermal border, with immunohistochemical positivity in the K and A light chains and vascular proliferation. "Lozenge" keratinocytes, "watery" spongiosis, and exocytosis were seen in the epidermis. Laboratory analysis showed notably low levels of both serum IgA and secretory IgA. We consider whether secretory IgA at low levels has an important etiopathogenic role favoring the development of localized subclinical repetitive infections that could lead to chronic PCG.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
8/62. The progressive appearance of multiple urinary Bence-Jones proteins and serum paraproteins in a child with immune deficiency.Multiple urinary Bence-Jones proteins and serum paraproteins were found in a child with type I dysgammaglobulinaemia (Seligmann et al., 1968). These showed a continually evolving pattern over a period of 4 months in relation to systemic infections and with no evidence of underlying malignancy.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
9/62. Community-acquired pseudomonas aeruginosa pneumonia complicated with loculated empyema in an infant with selective iga deficiency.pseudomonas aeruginosa is widely prevalent in the hospital environment, especially in intensive care units. Selective iga deficiency is characterized by a serum IgA level less than 5 mg/dl with no deficiency of other immunoglobulins. The occurrence of community-acquired P. aeruginosa pneumonia with empyema is rare in pediatric patients. We present a 10-month-old male infant who was referred due to persistent fever and progressive respiratory distress for 1 week. A chest radiograph revealed a right lobar pneumonia with pleural effusion. P. aeruginosa that was subsequently isolated from both blood and pleural effusion cultures. The patient received treatment with ceftazidime and intrapleural instillation of urokinase to promote drainage of empyema. Subsequent immunological screening revealed a very low serum IgA level (<5 mg/dl). We present our experience in successfully treating a loculated empyema with intrapleural instillation of urokinase in an infant. It is also important for pediatricians to be aware that they should be alert for the patient who present with respiratory infections due to unusual organisms. An advanced immunological study to investigate the underlying disorders in these patients is mandatory.- - - - - - - - - - ranking = 1keywords = infection (Clic here for more details about this article) |
10/62. iga deficiency: a report of three cases from thailand.Selective iga deficiency has been reported to be the most common primary immunodeficiency disease in Western countries. A markedly lower frequency of this condition has been reported in the Japanese population. While most of the IgA deficient cases are healthy, some patients develop significant recurrent sinopulmonary infections, allergic disorders and autoimmune diseases. Herein, we report three cases of iga deficiency among Thai patients, all of whom suffered from chronic sinopulmonary infections. Two of the three patients had absolute iga deficiency while the third had a partial iga deficiency. The associated conditions found in these three patients were deficiencies of an IgG subclass, allergic rhinitis and lupus nephritis. The youngest child (5 years old boy with lupus nephritis) expired from pneumocystis carrinii pneumonia complicated with adult respiratory distress syndrome.- - - - - - - - - - ranking = 2keywords = infection (Clic here for more details about this article) |
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