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)

Chronic bronchitis is defined for epidemiologic and clinical purposes as the presence of productive cough for three months in each of two successive years. Based on symptoms, the term 'chronic bronchitis', therefore, does not describe one distinct disease. It is rather a collective name for the clinical manifestation of numerous different congenital or acquired chronic diseases of the trachea, the bronchi and the bronchioli. Cigarette smoking is the most consistently important (and preventable) determinant of chronic bronchitis. There are, however, other rare etiologic factors, including malformations, tumors, recurrent aspirations and bronchiectasis. The latter often occur in association with systemic disorders such as cystic fibrosis, immotile cilia syndrome, immunodeficiency, alpha 1-antitrypsin deficiency and others.
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PMID:[Chronic bronchitis: rare etiology]. 144 Apr 38

Intrahepatic biliary stones in seven non-Oriental patients were studied in all by sonography, in four patients by computed tomography and in four patients by percutaneous transhepatic cholangiography. For patients had extrahepatic biliary atresia treated with portoenterostomies, one patient had undergone partial liver transplantation and of the remaining two, one had cystic fibrosis and the other immunodeficiency syndrome. All sonograms were abnormal and showed echogenic foci within the liver, with or without associated signs of biliary tract dilatation. CT confirmed the biliary tract dilatation yet calculi were identified in one patient only. PTC was particularly helpful in the patient with immunodeficiency in whom features typical of sclerosing cholangitis were found. This report emphasizes the variable radiological appearance of bile stones which to our knowledge have rarely been described in children with entities other than Oriental cholangitis.
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PMID:Intrahepatic biliary stones in children. 152 54

A distinctive feature of Alzheimer's disease (AD) is the presence of dystrophic neurites that immunoreact with antibodies to amyloid precursor protein (APP) and ubiquitin (Ub). The authors examined dystrophic axons (DA) present in other chronic conditions such as familial infantile neuroaxonal dystrophy (INAD), aging, cystic fibrosis, and biliary obstruction as well as in conditions of shorter duration such as human immunodeficiency virus (HIV) leucoencephalopathy, infarction and radiation therapy to determine whether APP and Ub immunoreactivity was unique to the DA of AD. A large number of DA immunoreacted with antibodies to the A4, C- and N-terminal regions of APP as well as to Ub. Ub and APP immunoreactivities often, but not always, colocalized. "Acute" DA generally reacted more intensely and in larger number with antibodies to APP than to Ub, whereas the reverse was true for "chronic" DA. Structureless DA immunostained diffusely. In DA with cores or granules, the Ub immunoreaction was occasionally limited to these structures, whereas reaction with antibodies to APP was more diffuse. In view of the contention that impairment of proteolysis is the common pathogenetic step in the formation of DA, Ub immunoreactivity in all DA may indicate a vicarious attempt to degrade accumulated components through an activation of the Ub system. The role of APP in the formation of DA remains to be determined.
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PMID:Amyloid precursor protein and ubiquitin immunoreactivity in dystrophic axons is not unique to Alzheimer's disease. 171 43

Cultures from 105 children with chronic sinusitis who had failed aggressive medical management were retrospectively studied. Patients with immunodeficiency and cystic fibrosis were excluded from the study. Because the most common sites of disease were the infundibula and anterior ethmoid sinuses, samples of mucosa removed from the anterior ethmoid bullae during endoscopic ethmoidectomy were routinely cultured for aerobic and anaerobic organisms. Fungal cultures were performed for 55 bullae. The principal organisms isolated were alpha-hemolytic Streptococcus, Staphylococcus aureus, Moraxella catarrhalis, Streptococcus pneumoniae, and Haemophilus influenzae non-type B. Only 12 anaerobic organisms and four fungi were isolated. Of the 204 bullae cultured, multiple organisms were found in 61 bullae and 40 showed no growth. Isolates of other less common organisms were also found. These data are analyzed on the basis of age and duration of symptoms, and antibiotic treatment is described.
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PMID:Bacteriology of the ethmoid bullae in children with chronic sinusitis. 199 Oct 59

The preparation, pharmacokinetics, clinical uses, dosage and administration, and adverse effects of intravenous immune globulin (IVIG) are reviewed. IVIG, which consists primarily of immunoglobulin G (IgG), is initially prepared from pooled human plasma by using the Cohn-Oncley fractionation procedure. Secondary treatments render the preparation suitable for i.v. use. The specific antibody content of IVIG depends on the geographic location of the plasma donors, the product, and the product lot. The metabolism of IgG appears to follow a multicompartmental, first-order process. The half-life of IgG is dependent on the half-lives of the IgG subclasses; three of the four subclasses have half-lives in the range of 23-25 days. IVIG is indicated in the treatment of idiopathic thrombocytopenic purpura (ITP) and as replacement therapy in primary humoral immunodeficiencies (PHI). IVIG has also been used for antimicrobial prophylaxis in bone marrow transplant and burn patients and in patients with malignancies. Patients with HIV infection, cystic fibrosis, neonatal sepsis, and respiratory syncytial virus infection may also benefit from prophylaxis or treatment with IVIG. The recommended dosage of IVIG in ITP is 400 mg/kg/day for two to five days. For the treatment of PHI, the usual dosage is 100-400 mg/kg every three or four weeks. Adverse reactions are often mild and are usually related to the infusion rate. Intravenous immune globulin is a valuable therapeutic tool in several immunodeficiency and autoimmune states, but IVIG products are expensive, and conclusive data on their efficacy in the treatment of many disorders remain to be obtained.
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PMID:Clinical uses of intravenous immune globulin. 229 73

Patients with asthma who have incomplete control of their symptoms or require regular systemic steroidal therapy are said to have recalcitrant asthma. A systematic approach may significantly improve quality of life. Factors that should be evaluated include living with an antigen, occupational exposure, use of beta-adrenoreceptor blockers, use of nonsteroidal anti-inflammatory agents, sensitivity to dietary chemicals, endocrinopathies, gastroesophageal reflux, sinusitis, bronchopulmonary aspergillosis, and noncompliance. Other diseases may mimic asthma or exacerbate nonspecific bronchial hyperreactivity. These include congestive heart failure, chronic infectious bronchitis resulting from cystic fibrosis, ciliary dysfunction syndrome, and immunodeficiency syndromes, upper airway obstruction, pertussis syndrome, psychogenic coughs, bronchiolitis obliterans, chronic eosinophilic pneumonia, and vasculitides. A systematic approach to the evaluation of coexisting factors and potential exacerbating diseases is presented.
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PMID:Recalcitrant asthma: an allergist's approach. 229 75

A 3 1/2 year old girl presented with failure to thrive and a five month history of diarrhoea and recurrent cough. The results of sweat sodium tests suggested a diagnosis of cystic fibrosis; but atypical organisms were found (Haemophilus influenzae, Candida albicans, but no Staphylococcus aureus), she failed to respond to treatment, and her sweat sodium concentrations fell in response to fludrocortisone. She also had hyperglobulinaemia, neutropenia, and reduced numbers of T4 lymphocytes, which prompted the performance of a test for antibody to human immunodeficiency virus (HIV). This proved positive, and she was treated with co-trimoxazole, zidovudine, and human immunoglobulin. Both parents and two siblings were also positive for HIV, though all had normal sweat sodium concentrations. Children with symptoms suggestive of cystic fibrosis but who also show atypical features, as in this case, should have their HIV state checked.
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PMID:Abnormal sweat electrolytes in symptomatic human immunodeficiency virus infection in a child. 312 Oct 56

Physicians who think epidemiologically are rare. A method is suggested for detecting their aptitude early in their career when help may be offered to make the most of their special talent. Clusters geographically or in families may provide clues to cancer etiology. Clusters have been systematically thought by mapping cancer mortality in the US and independently in China. Case-control studies have revealed environmental exposure responsible for some of the clusters. Clusters noted by alert clinicians or other astute observers have revealed most of the known environmental causes of human cancers. Genetic influence in carcinogenesis has been identified by studies of peculiar cancer occurrence, such as familial aggregation, multiple primary cancer or the occurrence of cancer with other diseases as, for example, congenital malformations and immunodeficiency disorders. Ethnic differences in cancer occurrence may be revealing. Thus, in Japan there is low frequency of B-cell lymphoma but high frequency of certain autoimmune diseases, as if inherent protection against one predisposes the other. As a rule of thumb, the occurrence of three rare observations is not likely to be due to chance. Examples include ideal carcinoma in three persons with cystic fibrosis of the pancreas who survived to about 30 years of age, and the occurrence in Klinefelter's syndrome of germ cell tumor of the pineal--a neoplasm that has an unusually high frequency in Japan. Finally, the history of discoveries concerning cancer etiology, an aspect of what Comroe has called "research on research", can point the way to new discoveries in the future.
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PMID:Nakahara memorial lecture. Rare events and cancer epidemiology. 333 98

Our studies have revealed that patients with Cystic Fibrosis CF who are infected with P. aeruginosa have grossly elevated serum levels of IgG antibodies to the opsonic immunodeterminant, type-specific LPS. Second, this elevation is distributed among all four IgG subclasses, with a significant shift towards IgG3. Third, sera from colonized CF patients shows diminished opsonic capacity, although complement dependent human neutrophil phagocytosis is not notably impaired. Fourth, functional polyclonal or monoclonal antibody opsonins exhibit prozone inhibition of phagocytosis at high concentrations. Fifth, sera from uninfected CF patients have lower levels and proportions of IgG2 antibodies to P. aeruginosa LPS, and higher levels and proportions of IgG4 antibodies, than normal controls. Finally, levels of IgG4 antibodies, but not IgG1, 2, or 3, correlate inversely with opsonic capacity. We therefore make several speculations. High levels of IgG4 antibodies to opsonic immunodeterminants may inhibit normal pulmonary clearance of P. aeruginosa by alveolar macrophages in vivo. Second, high levels of opsonic antibodies may also contribute to the problem in vivo by the phenomenon of prozone inhibition. Third, reduced levels of IgG2 antibodies in uninfected CF patients raises the intriguing possibility of an wider polysaccharide antigen-related isotype-restricted immunodeficiency, with an attempted compensatory shift to IgG4 doomed to failure.
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PMID:The role of IgG subclass antibodies in chronic infection: the case of cystic fibrosis. 336 9

An 18-year follow-up of a young woman who presented at the age of 10 years with a malabsorption syndrome and recurrent chest infections is reported. The serum immunoglobulins, and in particular the IgA, were increased, cell-mediated immunity was impaired but free alpha heavy chains were not detected. A jejunal biopsy showed plasma cell infiltration of the bowel with villous atrophy. Bronchiectasis, recurrent skin infections, and sinusitis were treated with frequent courses of antibiotics and corticosteroids. An initial presumptive diagnosis of cystic fibrosis was excluded by a normal sweat test. Although difficult to classify her exact type of immune deficiency state, the data would appear to conform to the syndrome of cellular immunodeficiency with normal or near normal immunoglobulins and lymphopenia (Nezelof's syndrome).
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PMID:Plasma cell infiltration of the small intestine, recurrent pulmonary infections, and cellular immunodeficiency (Nezelof's syndrome). 405 Jul 63


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