Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 61-year-old woman who had undergone an operation for thymoma 17 years previously suddenly became dyspneic and showed bilateral pulmonary infiltrates on a chest radiograph. In the bronchoalveolar lavage fluid cells contained characteristic cytomegalic inclusion bodies, as well as cytomegalovirus DNA demonstrated by a polymerase chain reaction. Immunological findings included hypogammaglobulinemia, deficient numbers of circulating B cells, and impaired blast transformation of peripheral blood T cells in response to mitogens in vitro. Considering all of the findings, the patient was diagnosed with Good's syndrome presenting with cytomegalovirus pneumonia.
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PMID:Good's syndrome presenting with cytomegalovirus pneumonia. 1119 98

Common variable immunodeficiency (CVID) is a heterogeneous immunodeficiency syndrome characterized by hypogammaglobulinemia, recurrent bacterial infections, and various immunologic abnormalities. The clinical presentation is generally that of recurrent pyogenic sinopulmonary infections. Our objectives were to study the prevalence of lung involvement and the response to intravenous immunoglobulin replacement therapy in 19 patients with CVID. Nineteen patients (12 men) with a mean age (SD) of 33.1 (17.1) years had a previous diagnosis of CVID and were treated with intravenous immunoglobulin replacement. All patients underwent complete pulmonary function tests and high-resolution computed tomography (HRCT) examination. Bronchiectasis was diagnosed in 11 (58%) patients and eight (42%) were multi-lobar bronchiectasis. Chronic airflow limitation (CAL) was present in 10 (53%) patients and a restrictive pattern was seen in one case. Eleven patients (58%) presented a decrease in single-breath carbon monoxide diffusing capacity of the lung (DL(CO)). Before intravenous immunoglobulin replacement therapy (INIRT), 84% of patients had suffered from at least one episode of pneumonia. Episodes of lower respiratory tract infection decreased significantly from 0.28 per patient and year before replacement therapy to 0.16 per patient and year after treatment. The mean duration of replacement therapy was 7.5 years. In conclusion lung involvement was frequent in patients with CVID. Long-term administration of intravenous gammaglobulin resulted in a substantial reduction of pneumonic episodes.
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PMID:Respiratory disorders in common variable immunodeficiency. 1126 36

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

A 3-month-old, full-term female infant was hospitalized with pneumonia and bronchiolitis. Laboratory studies revealed a profoundly low level of IgG (41 mg/dL) and low level of IgA (< 6.67 mg/dL). Other causes of immunodeficiency were ruled out, and there was no evidence of protein loss to account for the low immunoglobulin levels. The immunoglobulin levels normalized over time. Our patient had a transient hypogammaglobulinemia of infancy, with severely low IgG and low IgA levels. We found no other reports of cases with such low values of IgG that proved to be transient.
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PMID:Spontaneous resolution of profound hypogammaglobulinemia. 1181 63

Recurrent infections are a common cause of morbidity in childhood. Several reports have associated this condition to low levels of IgA and IgG subclasses and/or lack of specific antipolysaccharide antibody response, although the relevance of these defects in terms of prognosis and therapeutic approach is still unclear. The aim of our study was to determine the frequency and the clinical relevance of humoral immunodeficiency (HID) other than hypogammaglobulinemia in children affected by recurrent infections. We recruited 67 pediatric patients affected by recurrent infections. Serum IgG, IgA, IgM, IgG2, IgG3, and specific anti-Haemophilus influenzae (anti-Hib) antibodies were determined. Thirty-seven out of 67 patients showed antibody defects (55%). IgA deficiency was observed in 21 out of 67 patients (31%), followed by IgG2 (18%), IgG3 (15%) and IgM (6%) defects. Anti-Hib deficiency was present in three out of 44 patients (7%). A tendency for a higher occurrence of pneumonia and otitis, although not statistically significant (p > 0.05), was observed in HID patients compared to children with normal humoral function. No statistical difference as to the frequency of mild infections (URI) was found between HID and non-HID patients. We therefore suggest that the therapeutic program is based on the clinical status of the patients. Long-term follow-up with repeated determinations of antibody levels is crucial, however, to detect those defects that might evolve into more complex immunodeficiencies.
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PMID:Evaluation of the relevance of humoral immunodeficiencies in a pediatric population affected by recurrent infections. 1248 21

The majority of bronchial and interstitial diseases of the adult are accompanied by bronchiolar inflammation, but over time the use of the term "bronchiolitis" has been limited to a few specific affections. Bronchiolitis with predominantly alveolar involvement Some "bronchiolites" emphasize the problem of an interstitial pneumopathy, since the disease predominantly involves the alveolar spaces. Only a few bronchiolites are severely damaging: bronchiolitis obliterans with organizing pneumonia and interstitial pneumopathy with respiratory bronchiolitis. These predominantly alveolar affections reveal the clinical (crepitant rales), radiographic (multiple or even diffuse opacities), and functional aspects (restrictive ventilation problems). Brochiolitis with obstructive airway problems In this case the disease predominantly involves the bronchioles and spares the alveolar tissue. The term "bronchiolitis" is in this case perfectly justified. The clinical picture is evocative with obstructed airway and a clear pulmonary parenchyma on the thoracic x-ray. These affections belong to the obstructive broncho-pneumopathy group. The prototype is brochiolitis obliterans, the anatomic correlation of which is generally constrictive bronchiolitis obliterans. Occasionally primitive, it frequently complicates the progression of many morbid states (transplants, collagenosis, inhaled or ingested toxic substances.). Diffuse panbronchiolitis Other "bronchiolites" deviate from this framework and are accompanied by marked lesions of other respiratory tracts (membrane bronchioles, cartilage bronchi, mucosa, ear nose and throat). The prototype is panbronchiolitis, described in the Far East. It is exceptional in Europe, where similar but nosologically different clinical aspects are observed during various diseases: cystic fibrosis, Young's syndrome, hypogammaglobulinemia, bone marrow transplant, context of HIV or haemorrhagic recto-colitis.
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PMID:[Measures to be taken in adults with bronchiolitis]. 1261 Apr 49

Agammaglobulinemia is characterized by failure of B-cell differentiation (hypogammaglobulinemia) and increased susceptibility to bacterial infections. The present study was set up in order to evaluate the effectiveness of intravenous immunoglobulin (IVIG) treatment on the incidence of pneumonia in patients with agammaglobulinemia. We carried out chart reviews of 23 patients with agammaglobulinemia (mean age 11.5+/-5.4 years), who had been observed in a 22-year period (July 1981-January 2003) in Iran's referral center for primary immunodeficiency disorders. Nineteen of these 23 (82.5%) had been infected with pneumonia at least once before receiving the immunoglobulin treatment and 11 of them had experienced multiple episodes. During treatment with gamma-globulin - over a mean period of 6.8+/-4.1 years (range: 0.8-15.3 years) - the incidence of pneumonia requiring treatment or hospitalization decreased from 0.82 to 0.12 per patient per year (P=0.006). During IVIG replacement, hospitalization due to pneumonia decreased from 0.58 to 0.05 per patient per year (P=0.08) and the immunoglobulin G level (mean+/-S.D.) changed from 66.2+/-63.9 (range: 0-210 mg dl(-1)) to 552.4+/-199.1 (range: 136-942 mg dl(-1)) (P<0.001). Treatment of agammaglobulinemia with IVIG significantly reduced the incidence of pneumonia and hospital admission. Intensive management and regular monitoring is required in order to fully prevent severe respiratory complications.
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PMID:Efficacy of intravenous immunoglobulin on the prevention of pneumonia in patients with agammaglobulinemia. 1498 29

In renal allograft recipients, infection disease complications remain an important cause of morbidity and mortality during the post-transplant period. This complication occurs more frequently from 1 to 6 months after transplant. The epidemiology of infection during the postoperative period is less well characterized, because recipients routinely reside at home. We describe a case of late onset Candida albicans and HSV-1 esophagitis, and Pneumocystis carinii pneumonia, that occurs 9 years after renal transplantation in a patient with severe CD4+ T-lymphocytopenia and hypogammaglobulinaemia. We underline the importance of monitoring immunosuppressive therapy in these patients and the usefulness of prophylaxis against P. carinii pneumonia
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PMID:Late onset opportunistic infections in a renal allograft recipient: a case report. 1531 1

The immunodeficiency, centromeric instability, and facial anomalies (ICF) syndrome is a rare autosomal recessive disorder characterized by variable immunodeficiency, instability of the pericentromeric heterochromatin, and facial dysmorphism. Here we report a new case of ICF syndrome who died of rubella pneumonitis. A six year-old-girl who was the first child of consanguineous parents was admitted to the hospital because of bronchopneumonia. Laboratory investigations revealed pan-hypogammaglobulinemia, lymphoperria, normal proportions of peripheral blood lymphocytes with an inverted CD4/CD8 ratio, and interstitial pneumonia with a positive serology of acute rubella infection. The ICF syndrome was diagnosed by centromeric instability in the standard cytogenetic analysis. An inclusion body was demonstrated in the lung biopsy after the death of the patient. Chromosomal investigation could be helpful along with other tests for diagnosis of variable immunodeficiency accompanied by facial dysmorphism.
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PMID:A case with ICF syndrome lost to rubella pneumonitis. 1588 37

Common variable immunodeficiency (CVID) is the most common clinically manifested primary immunodeficiency disease. A 29-year-old female patient presented with pneumonia and enlarged thoracal and abdominal lymph nodes. Frequently recurring infections, especially in the respiratory tract were observed in the patient's history. A hypogammaglobulinaemia could be detected. By exclusion of other disorders and a complete analysis of the immune status a CVID Ib/B was diagnosed. Regular ambulatory treatment with immune globulin substitution reduced the incidence and severity of infections.
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PMID:[29-year-old female patient with pneumonia and enlarged abdominal lymph nodes]. 1592 64


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