Cases reported "Disease Susceptibility"

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1/99. Two separate episodes of hemophagocytic syndrome at a two-year interval in an apparently immunocompetent male.

    We describe two separate episodes of hemophagocytic syndrome (HPS) at an interval of two years in a seemingly immunocompetent male. This case suggests the possible existence of an inherent predisposition to HPS, in which otherwise negligible self-limited viral infection may trigger HPS. Laboratory data for a 16-year-old boy admitted with persistent high grade fever and severe thrombocytopenia disclosed coagulation abnormality, liver damage, and hypercytokinemia. A bone marrow aspiration revealed a proliferation of histiocytes with fresh hemophagocytosis. We diagnosed that he was suffering from HPS. Responding to steroid pulse therapy, he recovered completely and was discharged. After two years of healthy life, he became febrile again and was readmitted. The fever was refractory to antibiotics and was associated with a sudden drop in platelet count. Laboratory data and the bone marrow picture were consistent with those of HPS. He was again successfully treated with steroid. After the second episode, he has been healthy for more than two years.
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2/99. pseudomonas aeruginosa septicaemia from an oral source.

    Oral colonisation with aerobic Gram-negative bacilli (AGNB) is abnormal and usually indicates a medically compromised state in the host. It has been postulated that oral colonisation with AGNB may predispose a patient to serious systemic infection, but proof of this assertion is lacking. This report describes an elderly patient who had oral colonisation of pseudomonas aeruginosa and developed septicaemia from an identical strain of this bacterium.
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3/99. Leukocyte functions in 2 cases of Papillon-Lefevre syndrome.

    AIM: To investigate the role of leukocytes in the pathogenesis of Papillon-Lefevre syndrome (PLS). methods: Peripheral blood polymorphonuclear neutrophils (PMNs), monocytes (MNs) and gingival crevicular fluid (GCF) were obtained from 2 cases of PLS with typical features. The chemotaxis of PMNs and MNs were evaluated using a modified Boyden chamber. The adherence of PMNs was determined by adherence of PMNs to petri dishes. interleukin-8 (IL-8) in GCF was detected by sandwich ELISA. Elastase activity in GCF was measured with a low molecular weight substrate (S-2484) specific for granulocyte elastase. RESULTS: PMNs from both patients showed depressed chemotactic response to FMLP and IL-8. Total amounts of IL-8 in GCF from the 2 patients were much higher than those of the normal controls. Elastase activity was not significantly different from that of the controls. The adherence of PMN and the chemotaxis of MN in the 2 patients were normal. CONCLUSION: The depressed chemotactic response of PMN leads to decreased recruitment of PMN and/or release of lysozyme from PMN in the diseased gingival tissue, increasing the susceptibility of PLS patients to periodontal infection.
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4/99. 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.
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ranking = 4
keywords = infection
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5/99. Primary pseudomonas meningitis in an adult, splenectomized, multitransfused thalassaemia major patient.

    A 19-year-old splenectomized, multitransfused female patient with beta-thalassaemia major developed primary meningitis due to P. putida. Her blood cultures were negative. P. putida is an unusual nosocomial organism to cause primary meningitis. infection due to this organism carries high mortality. However, owing to early diagnosis and energetic treatment this patient survived without any sequelae. A review of serious infections over the last 7 years in patients in our thalassaemia care centre revealed 11 serious infections among our splenectomized patients (n = 46) and none in the non-splenectomized group (n = 106). Surprisingly, all overwhelming infections (23.8% in the splenectomized group) were caused by Gram-negative bacilli like klebsiella, Pseudomonas, aeromonas and campylobacter species. As all our splenectomized patients had prior pneumococcal vaccination and oral penicillin prophylaxis, overwhelming septicaemia due to S. Pneumoniae was successfully prevented, but an increasing incidence of overwhelming sepsis due to Gram-negative bacilli, against which no vaccination or suitable prophylactic antibiotics are available, is now posing a new threat to this vulnerable group of patients.
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ranking = 3
keywords = infection
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6/99. Acute renal failure complicating nonfulminant hepatitis a in HLA-B27 positive patient.

    Here we report the case of a previously healthy 32-year-old HLA B27 positive male who developed completely reversible acute oliguric renal failure in the course of nonfulminant hepatitis A infection. This is a rare complication of uncertain etiology. B27 positive individuals are prone to a number of immune system derangements including overreacting in interactions with some microbiologic agents. We made our consideration on the basis of some experimental models and the presumed pathogenesis of reactive arthritis in these individuals. As a result here we postulate a hepatitis a virus-triggered, immune mediated mechanism of renal injury restricted to genetically susceptible (i.e. B27 positive) individuals. In regard to this hypothesis we warrant additional HLA-profiles and future similar cases for further confirms and conclusions.
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7/99. Familial persistent polyclonal B-cell lymphocytosis.

    We report the occurrence of the syndrome of persistent polyclonal B-cell lymphocytosis in a brother and a sister. Both showed the morphological and immunophenotypic features of this rare disorder. In addition both had mild splenomegaly, increase of serum IgM and serological evidence of previous EBV infection. Of interest, two additional brothers had no evidence of PPBL but were indistinguishable in terms of HLA haplotype (HLA-DR7), smoking habits or evidence of EBV infection. These observations provide additional support for a genetic basis of the syndrome but suggest that pathogenic factors other than those known so far may be required for its full expression.
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keywords = infection
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8/99. Prepubertal periodontitis associated with chronic granulomatous disease.

    BACKGROUND: Generalised prepubertal periodontitis is a rare entity that is usually a consequence of severe systemic diseases. Chronic granulomatous disease is one of the extremely rare inherited immunodeficiency diseases, which predisposes the patient to recurrent severe bacterial and fungal infections. AIM: The purpose of this report is to describe a 5-year old male patient suffering from prepubertal periodontitis associated with chronic granulomatous disease, who was referred to the Department of Periodontology for treatment of severe gingival inflammation. methods: A detailed past history was obtained and thorough clinical and laboratory examinations were performed. RESULTS: Medical tests revealed the only immunodeficiency sign as the extremely low burst test result. The patient was diagnosed as having an autosomal recessive (AR) form of chronic granulomatous disease. He was put on prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) and also a periodontal maintenance regimen with regular 1-month intervals. CONCLUSION: This case report emphasises the importance of the differential diagnosis of severe immunodeficiency in the background of prepubertal periodontitis.
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keywords = infection
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9/99. Isolated renoureteric aspergilloma due to aspergillus flavus: case report and review of the literature.

    In this paper we describe a case in which acute renal colic was associated with elimination of multiple hyphal masses of aspergillus flavus. Also, we reviewed the literature on similar cases and we found a similar pattern characterized by a marked male predominance, association with at least one underlying medical condition that predisposes to fungal infection, the presence of local symptoms resembling acute ureteral colic, and the absence of systemic manifestations. Moreover, our data suggest that Aspergillus balls must be suspected when a diabetic and intravenous drug user presents with acute renal colic and that non-obstructive renal aspergillosis may be initially treated with itraconazole.
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keywords = infection
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10/99. Immunologic and virologic analyses of an acutely hiv type 1-infected patient with extremely rapid disease progression.

    The immunologic and virologic factors that impact on the rate of disease progression after acute infection with human immunodeficiency virus (hiv) type 1 are poorly understood. A patient with an extraordinarily rapid disease course leading to AIDS-associated death within 6 months of infection was studied intensively for the presence of anti-hiv immune reactivities as well as changes in the genetic and biologic properties of virus isolates. Although altered humoral responses were evident, the most distinctive immunologic feature was a nearly complete absence of detectable hiv-specific CTL responses. In addition to a rapid decline in CD3 CD4 cells, elevated percentages of CD8 CD45RA and CD8 CD57 cells and diminished CD8 CD45R0 and CD8 CD28 cells were evident. Primary viral isolates recovered throughout the course of infection exhibited limited sequence diversity. Cloned viral envelopes were found to have unusually broad patterns of coreceptor usage for cell-cell fusion, although infectivity studies yielded no evidence of infection via these alternative receptors. The infectivity studies demonstrated that these isolates and their envelopes maintained an R5 phenotype throughout the course of disease. The absence of demonstrable anti-hiv CTL reactivities, coupled with a protracted course of seroconversion, highlights the importance of robust hiv-specific immune responses in the control of disease progression.
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ranking = 4
keywords = infection
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