1/55. Rapid respiratory deterioration and sudden death due to disseminated cryptococcosis in a patient with the acquired immunodeficiency syndrome.We report the case of a patient with the acquired immunodeficiency syndrome (AIDS) whose death occurred within 30 hours of hospitalization due to disseminated cryptococcosis, manifested by dizziness, cough, and shortness of breath. The clinical picture was consistent with pneumocystis pneumonia, and antibiotic therapy with corticosteroids was initiated. Despite initial improvement, the patient's condition quickly worsened, resulting in cardiorespiratory arrest and death. autopsy revealed cryptococci in several organs. Sudden, rapid deterioration and death are rare consequences of disseminated cryptococcosis, and steroids may worsen the course of the disease. On the basis of this case and review of similar cases in the literature, we recommend early consideration of disseminated cryptococcosis in AIDS patients with pneumonia. early diagnosis and appropriate therapy are essential to reduce morbidity and mortality.- - - - - - - - - - ranking = 1keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
2/55. toxoplasma gondii pneumonia in a pancreas transplant patient.A 41-year-old woman had fever of 3 days' duration. She had had pancreas transplantation 2 years previously and had recently completed a course of antirejection medication. temperature spikes occurred during treatment with broad spectrum antibiotics. No obvious cause for the fever was found. The patient's condition worsened, with development of shortness of breath, bilateral pulmonary infiltrates on chest radiographs, sepsis, and shock. Fiberoptic bronchoscopy with bronchoalveolar lavage showed the presence of toxoplasma gondii. pyrimethamine and clindamycin were started, and the patient improved. toxoplasma gondii occurs in contaminated food containing oocysts or cysts. organ transplantation and blood transfusions are other routes of transmission. Most recent cases have occurred in human immunodeficiency virus (hiv) patients with reactivation of previous infection. serology and tissue biopsies are used for diagnosis. Treatment includes a combination of pyrimethamine and sulfadiazine or trisulfapyrimidines.- - - - - - - - - - ranking = 0.078088375673965keywords = immunodeficiency (Clic here for more details about this article) |
3/55. Organizing pneumonia related to common variable immunodeficiency. case report and literature review.A 68-year-old woman suffering from common variable immunodeficiency (CVI) developed a typical picture of organizing pneumonia. Causative factors other than CVI were eliminated. Several antibiotic regimens failed to improve the patient's condition, while the clinical manifestations rapidly disappeared under steroid therapy, with complete radiological recovery, but relapsed after steroid withdrawal. Finally, organizing pneumonia was definitely demonstrated by pathological findings obtained by open lung biopsy. Interestingly, pathological examination exhibited two other well-known CVI-associated lesions, i.e. benign lymphoid hyperplasia and noncaseating granuloma. In view of reports in the literature, we speculate that these different histological patterns could have resulted in a spectrum of symptomatic CVI-associated pulmonary disorders that improved under steroid therapy.- - - - - - - - - - ranking = 0.39044187836983keywords = immunodeficiency (Clic here for more details about this article) |
4/55. Recurrent pneumonia with mild hypogammaglobulinemia diagnosed as X-linked agammaglobulinemia in adults.BACKGROUND: X-linked agammaglobulinemia (XLA) is a humoral immunodeficiency caused by disruption of the Bruton's tyrosine kinase (BTK) gene. Typical XLA patients suffer recurrent and severe bacterial infections in childhood. methods: Flow cytometric analysis of the peripheral monocytes using the anti-BTK antibody was used to characterize a 27 year old male patient with mild hypogammaglobulinemia (IgG, 635 mg/dl; IgM, 11 mg/dl; IgA, <5 mg/dl). He had suffered from frequent pneumonia since age 25 but had no history of frequent infections in his childhood or in adolescence. Sequencing of the BTK cDNA obtained from an Epstein-Barr virus-transformed B lymphoblastoid cell line derived from the bone marrow of the patient was performed to confirm a genetic defect. RESULTS: Flow cytometric analysis of cytoplasmic BTK protein in peripheral monocytes indicated that the patient presents a rare case of adult-onset XLA and that his mother is an XLA carrier. Sequencing of the BTK gene revealed a deletion of AG in the codon for Glu605 (AGT), resulting in an aberrant stop codon that truncates the BTK protein in its kinase domain. CONCLUSIONS: This case suggests that some XLA cases may remain undiagnosed because they only show mild hypogammaglobulinemia and they lack repeated infections in childhood. Flow cytometric analysis is a powerful method to screen these patients.- - - - - - - - - - ranking = 0.078088375673965keywords = immunodeficiency (Clic here for more details about this article) |
5/55. Bilateral ophthalmic artery occlusion in a patient with acquired immunodeficiency syndrome and central nervous system lymphoma.PURPOSE: Clinical course and autopsy findings in a patient with human immunodeficiency virus-1 immunodeficiency, central nervous system lymphoma, and bilateral, simultaneous ophthalmic artery occlusions. DESIGN: Observational case report. methods: Clinical examination, fundus photography, gross and microscopic pathologic study. RESULTS: Fundus photographs disclosed stasis in retinal arterioles, the absence of a cherry-red spot; internal carotid arteriography disclosed bilateral ophthalmic artery occlusions; postmortem histopathologic examination disclosed bilateral ophthalmic artery atherosclerosis, retinal ischemic necrosis, ischemic optic neuropathy, diffuse large-cell lymphoma of multiple areas of the central nervous system, cytomegalovirus encephalitis, atherosclerosis, and bronchopneumonia. CONCLUSIONS: A 47-year-old male with acquired immunodeficiency syndrome, profound immunodeficiency, systemic hypertension, and central nervous system lymphoma, developed deep vein thrombosis, bilateral ophthalmic artery occlusions, and died of pneumonia 7 weeks after the onset of blindness. Postmortem study revealed bilateral ophthalmic artery hemorrhagic atherosclerosis, ischemic optic neuropathy, ischemic retinal necrosis, diffuse large-cell central nervous system lymphoma, cytomegalovirus encephalitis, pneumonitis, and systemic atherosclerosis.- - - - - - - - - - ranking = 1.2342651270219keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
6/55. Successful transplantation of haploidentical CD34 selected bone marrow cells for an infantile case of severe combined immunodeficiency with aspergillus pneumonia.A 5-month-old boy with X-linked severe combined immunodeficiency (SCID) and aspergillus pneumonia was successfully transplanted. Before and during transplantation, the patient received O2 administration, systemic amphotericin b, and itraconazole. The transplant was performed with a conditioning regimen of busulfan/cyclophosphamide and 2.9 x 10(6)/kg of CD34 selected bone marrow cells from his HLA haploidentical mother. Acute grade II graft-versus-host disease (GvHD) was well controlled. Neutrophil counts reached >0.5 x 10(9)/L by day 15 and platelet counts reached > 50 x 10(9)/L by day 48. The T-cell subset (counts) in peripheral blood increased to 42.2% (0.31 x 10(9)/L) by day 46. The pneumonia improved by day 54. The patient has been doing well with limited chronic GvHD of the gut with a follow-up of longer than 40 months after BMT. Conquest of aspergillus pneumonia in SCID infants could be achieved by CD34 bone marrow cell transplantation together with appropriate anti-fungal treatment.- - - - - - - - - - ranking = 0.39044187836983keywords = immunodeficiency (Clic here for more details about this article) |
7/55. toxoplasma gondii pneumonitis in patients infected with the human immunodeficiency virus.Pulmonary toxoplasmosis is a rarely recognized opportunistic infection in immunocompromised patients. A few case reports have described pulmonary toxoplasmosis in human immunodeficiency virus-infected patients in association with toxoplasma gondii central nervous system disease. We encountered six cases of pulmonary toxoplasmosis in human immunodeficiency virus-infected patients who presented with a protracted febrile illness, respiratory symptoms, and an abnormal chest roentgenogram in the absence of neurologic findings. No clinical or roentgenographic features distinguished T gondii pneumonitis from more common opportunistic pulmonary infections. As the acquired immunodeficiency syndrome epidemic progresses, the presenting illnesses have evolved. toxoplasma gondii must be considered a potential cause of pulmonary disease during the evaluation of human immunodeficiency virus-infected patients with respiratory symptoms.- - - - - - - - - - ranking = 0.74661862971776keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
8/55. A familial case of hyper-IgM immunodeficiency.A 22-year-old male was diagnosed as having immunodeficiency with hyper-IgM based upon recurrent pneumonia, marked elevation of serum IgM and markedly decreased level of IgG. IgG-or IgA-bearing B cells were not detected in peripheral blood while a number and a proportion of peripheral blood T lymphocytes were normal. Peripheral blood lymphocytes from this patient proliferated normally in response to T-independent and T-dependent B cell mitogens, and to T cell mitogens. Furthermore, the same type of dysgammaglobulinemia with increased IgM was found in the patient's father and brother. From these observations, it is suggested that it is a rare case of autosomal dominant or polygenal inheritance of hyper-IgM immunodeficiency.- - - - - - - - - - ranking = 0.46853025404379keywords = immunodeficiency (Clic here for more details about this article) |
9/55. rhodococcus equi cavitary pneumonia in hiv-infected patients: an unsuspected opportunistic pathogen.Two patients seropositive for human immunodeficiency virus (hiv) and with no previous acquired immunodeficiency syndrome-defining conditions developed cavitary pneumonia and pleural disease caused by rhodococcus equi. R. equi was isolated from these patients' sputum and lung biopsy specimens, respectively, but the microorganism was initially considered to be a contaminant (patient 1) or misidentified as a nontuberculous mycobacterium (patient 2). The R. equi infection was fatal in one patient, who died after 4 months without specific antimicrobial therapy; the second patient was unresponsive to combination therapy with various antimicrobial agents. R. equi may cause life-threatening infections in hiv-infected patients. microbiology laboratories should be cognizant of the need to exclude R. equi as a cause of infection in highly immunosuppressed patients.- - - - - - - - - - ranking = 0.27808837567397keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
10/55. common variable immunodeficiency in an adult with recurrent pneumonia.Primary immunodeficiency syndromes are rarely diagnosed among adults. common variable immunodeficiency (CVID) is a congenital immunological disorder characterized by defective antibody production. In this report, we describe a 35-year-old male suffering from a common variable immunodeficiency, referred to us because of a lobar pneumonia. He had a history of recurrent pulmonary infections, which was present months before presentation, suggesting hypogammaglobulinemia. We found a severe hypogammaglobulinemia, which confirmed the diagnosis of CVID. His immunoglobulin profiles upon admission before infusion of immunoglobulin (normal ranges) were: IgG < 1.41 (8-17) g/l, IgA 0.25 (0.85-4.9) g/l, IgM 0.182 (0.5-3.7) g/l, and IgE < 2 (< 120) IU/ml. His HLA profiles were HLA A2 A26, B18 B38, Cw7, DR11 and DQ7 DQ9. He was treated with intravenous immunoglobulin. After this regimen, his IgG was maintained at > 6.0 g/L. On follow up, he has been free of opportunistic infections. In conclusion, CVID should be considered in the differential diagnosis of recurrent pneumonia in adults.- - - - - - - - - - ranking = 0.66853025404379keywords = immunodeficiency syndrome, immunodeficiency (Clic here for more details about this article) |
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