Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An 8-year-old male underwent excision of an encapsulated thymoma. Four months later he presented with disseminated, fatal varicella. Evaluation of his immune status during the terminal illness revealed hypogammaglobulinemia and lymphopenia consistent with a diagnosis of Good's syndrome (immunodeficiency with thymoma). This is the first case of Good's syndrome reported in a child and the first case of fatal varicella associated with Good's syndrome. The combination of this rare pediatric tumor and immunodeficiency is discussed. Despite specific antiviral therapy, varicella remains a deadly disease in the immunocompromised host.
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PMID:Fatal varicella infection in a child associated with thymoma and immunodeficiency (Good's syndrome). 232 70

A 63 yo female was hospitalized with a 2 week history of vomiting, epigastric pain, anorexia and weight loss. She had an incidental finding of left anterior upper mediastinal mass on Chest Xray and TSP of 2.2 gm% and globulins of 1.2 gm% along with endobronchial larvae of Ascaris lumbricoides and malignant cells and Strongyloides stercoralis in the gastric mucosa. She died after a progressively deteriorating course and at autopsy a thymoma predominantly composed of spindle shaped cells was found. This rare variant of thymoma has been associated with red cell agenesis and with immunodeficiency (Good's syndrome).
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PMID:[Thymoma of fusiform cells. Report of a case]. 276 92

We treated a case of thymoma with immunodeficiency (Good's syndrome) associated with a rare combination of other parathymic syndromes including myasthenia gravis, benign IgG lambda M component, pernicious anemia, and diabetes. The characterization of the patient's immunologic capacity disclosed practically normal T-cell number and mitogenic responses but impaired lymphokine production as well as B-cell function.
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PMID:Thymoma with immunodeficiency (Good's syndrome) associated with myasthenia gravis and benign IgG gammopathy. 389 87

A 63-year-old man was admitted for Campylobacter fetus sepsis and immunodeficiency syndrome with thymoma (Good's syndrome). Serological examination demonstrated hypoimmunoglobulinemia. Analysis of lymphocyte subsets in the peripheral blood and bone marrow showed marked decreases in the proportion of cells bearing B cell markers. However, there were no abnormalities of cellular immunity. This is a rare case of Good's syndrome in Japan in which the pathogenic mechanism involved a block in the early stage of B cell differentiation. Moreover, this is the first case ever reported of Campylobacter fetus sepsis associated with Good's syndrome.
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PMID:Good's syndrome with a block in the early stage of B cell differentiation and complicated by Campylobacter fetus sepsis. 780 19

We have described the first case, to our knowledge, in which recurrent respiratory tract infections were the primary manifestation of thymoma with immunodeficiency (Good's syndrome) associated with cobalamin malabsorption and immunoglobulin M-kappa (IgM-kappa) M component. The intrinsic factor receptor activity was dramatically decreased in a mucosal homogenate prepared from ileal biopsies. This decreased activity could be the principal cause of the malabsorption of labelled cobalamin which was observed in the presence of intrinsic factor. However, it could be the consequence of the cobalamin deficiency, as it is known that a cobalamin deficiency can affect the assimilation of cobalamin, even in presence of exogenous intrinsic factor.
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PMID:Thymoma with immunodeficiency (Good's syndrome) associated with selective cobalamin malabsorption and benign IgM-kappa gammopathy. 830 82

We report a bronchopulmonary infection with Mycobacterium malmoense in a patient with severe immunosuppression due to insulin-dependent diabetes mellitus, humoral immunodeficiency after thymoma (Good's syndrome) and prolonged immunosuppressive treatment after myasthenic crisis. It presented as non-resolving pneumonia of the left lower lobe. Bronchoscopically, a bronchoesophageal fistula was detected. Numerous acid-fast organisms were found in the sputum specimen and in the bronchial biopsy around the fistula. M. malmoense was isolated from sputum, bronchoalveolar lavage and bronchial biopsy. Whereas conventional in vitro susceptibility testing revealed susceptibility only to ethambutol, multi-drug susceptibility testing confirmed susceptibility to rifampicin, ethambutol, clarithromycin and prothionamide. The clinical outcome after 12 months of therapy resulted in a stable remission and considerable suppression of the mycobacterial load, but not in complete eradication.
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PMID:Bronchopulmonary infection with Mycobacterium malmoense presenting as a bronchoesophageal fistula. 875 16

A 58-year-old man was admitted to our hospital because of recurrent pulmonary infections that began three years previously. Laboratory data showed hypogammaglobulinemia and a chest computed tomogram showed diffuse bilateral micronodular shadows and an anterior mediastinal tumor. Immunodeficiency with thymoma (Good's syndrome) was diagnosed. After undergoing a thymectomy, he received intravenous gamma-globulin injections once a month for prophylaxis. Good's syndrome occurs rarely in Japan. A solid tumor-like shadow is not necessarily observed in routine chest X-ray studies, and hypogammaglobulinemia is one sign of this syndrome. The hypogammaglobulinemia of Good's syndrome should be carefully differentiated from that of other immunodeficiency diseases such as common variable immunodeficiency, the acquired immunodeficiency syndrome, chronic lymphocytic leukemia, non-Hodgkin's lymphoma, and multiple myeloma (non-secretory type).
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PMID:[Immunodeficiency with thymoma (Good's syndrome) similar to sino-bronchial syndrome]. 881 Jul 67

Here we present the case of a patient affected with a unique association of Sweet's syndrome, idiopathic myelofibrosis, spindle-cell thymoma, myasthenia gravis and Good's syndrome (a rare form of thymoma-related, combined immunodeficiency presenting with recurrent respiratory infections). Conventional therapies (corticosteroids, colchicine, DDS, clofazimine) were ineffective or were contraindicated. Treatment with etretinate (50 mg/day) proved effective on skin lesions. Moreover, the patient's general condition unexpectedly improved, with long-lasting (11 months) suppression of respiratory infections and a slight but consistent improvement of hematological parameters such as Hct, MCV, Hb level, RBC, WBC and lymphocyte absolute counts.
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PMID:Sweet's syndrome in a patient with idiopathic myelofibrosis and thymoma-myasthenia gravis-immunodeficiency complex: efficacy of treatment with etretinate. 890 Aug 54

Immunodeficiency with a thymoma (Good's syndrome) is a rare condition occurring in 7% to 13% of patients with adult-onset hypogammaglobulinemia. In 80% of cases, hypogammaglobulinemia is detected within 5 years of the identification of the thymoma. A 70-year-old man was found to have hypogammaglobulinemia 18 years after a thymoma had been resected. Cellular immunophenotyping revealed there were no detectable B cells, decreased CD4+ cells, and increased CD8+ cells. Both CD4+ and CD8+ subsets expressed increased populations of CD38+ DR+ cells and CD45RO+ cells. The CD8+ CD28+ population was markedly reduced. Inducible cytokine production by the patient's peripheral blood mononuclear cells revealed decreased IL-2, IL-10, and interferon-gamma production. These data suggest that patients with Good's syndrome have activated memory T cells that have dysregulated cytokine production.
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PMID:Hypogammaglobulinemia and anemia 18 years after thymoma resection. 943 96

Immunodeficiency with thymoma (Good syndrome, GS) is a rare, adult-onset condition that is characterized by thymoma, hypogammaglobulinemia, and low numbers of peripheral B cells. CD4+ T lymphopenia and an inverted CD4:CD8+ T-cell ratio may be present. Here we report 5 patients with GS and infectious complications who were seen at 3 institutions between 1983 and 1999. Three patients had recurrent sinopulmonary infections, 3 had severe cytomegalovirus (CMV) disease, and 1 had Pneumocystis carinii pneumonia. Review of the literature identified 46 other reports of infections in GS patients. The infections reported in all 51 patients included recurrent sinopulmonary infection (19 cases with documented respiratory pathogens), generally with encapsulated bacteria, most often Haemophilus influenzae (11 cases); CMV disease (5 cases); bacteremia (7 cases); oral or esophageal candidiasis (6 cases); persistent mucocutaneous candidiasis (5 cases); chronic diarrhea (5 cases with documented stool pathogens); urinary tract infections (4 cases); P. carinii pneumonia (3 cases); tuberculosis (2 cases); Kaposi sarcoma (1 case); disseminated varicella (1 case); candidemia (1 case); wound infection with Clostridium perfringens (1 case); Mycoplasma arthritis (1 case); and other infections. Patients with GS present with a spectrum of sinopulmonary infections and pathogens similar to common variable immunodeficiency (CVID). Compared with patients with CVID, opportunistic infections, including severe CMV disease, P. carinii pneumonia, and mucocutaneous candidiasis, appear to be more common in patients with GS, and patients with GS may have a worse prognosis. GS should be ruled out in patients with thymoma or CVID who develop severe, especially opportunistic, infections. Treatment with intravenous immune globulin is recommended for all patients with GS.
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PMID:Infections in patients with immunodeficiency with thymoma (Good syndrome). Report of 5 cases and review of the literature. 1130 88


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