Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019158 (hepatitis)
30,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We present our experience with 5 pediatric patients, 3 males and 2 females, with alpha 1 antitrypsin deficiency. These patients were between the ages of 15 months and 8 years and 4 were of the PI ZZ phenotype and 1 of the PI SZ phenotype. All cases presented with liver disease (neonatal cholestasis, cirrhosis, hepatitis). We comment on the different clinical forms of this entity during childhood, most of which are liver diseases, whereas in the adult it is generally manifest as lung disease.
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PMID:[Alpha-1 antitrypsin deficiency in infancy and childhood]. 158 Apr 32

This study examines the hospitalization experience of a sample of chronic hemodialysis patients, using primary data sources. There were multiple causes of hospitalization over the six-month tracking period, with stays extending from 1 to 87 days. Patients were more likely to be hospitalized if they had a negative hepatitis antigen, lower functional status scores, lower phosphate and protein levels, repeated access procedures, other cardiovascular conditions, arthritis, psychiatric disorders, ischemic peripheral vascular disease, lung disease, or larger households. Hospitalization for access-related problems was associated with arthritis, previous access procedures, and blood pressure levels. Sociodemographic and treatment characteristics did not have a significant influence on the risk of hospitalization. Improved management in these clinical areas may improve the quality of life of chronic hemodialysis patients and reduce the high level of expenditures associated with delivering inpatient services to this segment of the Medicare population.
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PMID:Factors associated with hospitalization in a sample of chronic hemodialysis patients. 174 74

Aside from disease induced by the direct pharmacological effect of heroin or cocaine, the occurrence of several medical complications not directly related to the drug itself is becoming an increasingly serious problem. In addition to the well-known occurrence of infective diseases, including AIDS, related to the i.v. use of heroin, heroin addicts also seem significantly more at risk for chronic nephropathy. Amyloidotic nephropathy is especially frequent in addicts who use heroin by subcutaneous route (skin popping); it seems to be mediated by an immunologic mechanism. Amyloidotic nephropathy is the main cause of renal failure among drug addicted subjects. Cardiovascular and cerebrovascular diseases are the most frequent medical complications observed in cocaine users. However, the occurrence of infective disease, such as endocarditis or hepatitis related to the parenteral use of the drug, is becoming frequent in these patients. Pulmonary disease is also common due to the route of administration of crack. These medical complications of drug addiction belong to the specific field of internal medicine and should be promptly recognized and treated by the physician.
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PMID:[Medical complications connected with the use of drugs]. 177 47

Alpha 1-protease inhibitor can exist as over 70 different biochemical variants (the Pi system) which are inherited as autosomal-codominant alleles. The majority of these variants are of no clinical significance. Epidemiologically, the most abundant are Pi types M, S, and Z. Homozygotes of type Z have only 10%-20% of the normal serum concentration of the inhibitor and have an increased risk of developing pulmonary emphysema. Cigarette smoking is the most important risk factor. A minority of Pi Z homozygotes (10%-20%) develop a form of neonatal hepatitis and a proportion of these suffer from liver cirrhosis in adult life. Heterozygotes of Pi type SZ have about one third of the normal serum alpha 1-protease inhibitor concentration but this phenotype does not in itself appear to be a significant emphysema risk factor. Heterozygotes of Pi type MZ are thought to have a moderately increased risk of developing emphysema but only if they smoke; there is also evidence for an increased risk of cirrhosis among subjects of type MZ. No excessive risk appears to be attached to the MS phenotype. Cumulative survival curves have suggested that type Z homozygotes have a poor prognosis but such estimates are based on clinic or hospital patients who already have respiratory symptoms. Calculations based on population frequencies however, suggest that about 90% of the total number of type Z subjects are not accounted for in such surveys. Their whereabouts remains unclear at present; some will undoubtedly have died of liver or lung disease but it is possible that the majority escape and live undetected among the general population.
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PMID:The epidemiology of alpha 1-antitrypsin deficiency. 211 62

Alpha 1-protease inhibitor can exist as over seventy different biochemical variants (the Pi system) which are inherited as autosomal codominant alleles. The majority of these variants are of no clinical significance. Epidemiologically, the most abundant are Pi types M, S and Z. Homozygotes of type Z have only 10 to 20% of the normal serum concentration of the inhibitor and have an increased risk of developing pulmonary emphysema. Cigarette smoking is the most important risk factor. A minority of Pi Z homozygotes (10 to 20%) develop a form of neonatal hepatitis and a proportion of these suffer from cirrhosis in adult life. Heterozygotes of Pi type SZ have about one third of the normal serum alpha 1-protease inhibitor concentration but this phenotype does not in itself appear to be a significant emphysema risk factor. Heterozygotes of Pi type MZ are thought to have a moderately increased risk of developing emphysema but only if they smoke; there is also evidence for an increased risk of cirrhosis among subjects of type MZ. No excessive risk appears to be attached to the MS phenotype. Cumulative survival curves have suggested that type Z homozygotes have a poor prognosis but such estimates are based on clinic or hospital patients who already have respiratory symptoms. Calculations based on population frequencies however, suggest that about 90% of the total number of type Z subjects are not accounted for in such surveys. Their whereabouts remains unclear at present; some will undoubtedly have died of liver or lung disease but it is possible that the majority escape and live undetected among the general population.
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PMID:Epidemiology of alpha 1-protease inhibitor deficiency. 234 48

Serum neopterin was measured in patients with acquired 'common variable' hypogammaglobulinaemia (CVH), X-linked agammaglobulinaemia (XLA) and healthy subjects. There was a highly significant increase in neopterin levels in the CVH patients as compared with that of the other groups, particularly in those CVH patients with non-A non-B or granulomatous hepatitis. There was no association between raised neopterin levels and chronic infective lung disease. Since chronic viral infections are associated with raised serum neopterin, this data add some support to a viral aetiology for CVH.
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PMID:Serum neopterin patients with X-linked and acquired 'common variable' hypogammaglobulinaemia. 272 61

Common variable immunodeficiency (CVI) or hypogammaglobulinemia is a heterogeneous primary immunodeficiency disease in which B cells produce little or no antibody. Since the disease is relatively rare and the spectrum of associated illnesses is broad, patients are given care by a variety of specialists. Thus it has been difficult to determine the incidence of specific complications. In these studies we analyzed 103 consecutively referred CVI patients of age range 3-71 years (average, 29 years) who were followed for a period of 1-13 years (total of 750 patient years). The average serum IgG was 174.4 mg/dl for untreated patients and 301 mg/dl for patients treated with intramuscular immunoglobulin at the time of the first visit. The average IgA was 14.5, and the average IgM was 80.7, with no difference between or after immunoglobulin treatment. About one-half of the patients had T-cell dysfunction, but lymphocyte stimulation responses were inversely related to age, which implies worsened T-cell immunity with age. Serum IgG and IgA levels were found to be statistically associated (P = 0.008), and serum IgG was related to lymphocyte stimulation with concanavalin A (P = 0.01). By 1986, 79 patients were alive, 23 had died, and 1 could not be located. Recurrent bacterial illnesses were common to all patients, and 22% had developed chronic lung disease, 22% autoimmune disease, 15% cancer, 13% hepatitis, and 9% malabsorption. Autoimmune disease was more common in females, and cancer was more likely to develop in the fifth and sixth decades. In 11% of the group, other family members were found to be immunodeficient (hypogammaglobulinemic or IgA deficient). Nine patients died of respiratory insufficiency (with or without other complications), and seven patients died of cancer. These data provide valuable information about the immunologic abnormalities and the spectrum and frequency of illnesses associated with hypogammaglobulinemia.
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PMID:Clinical and immunologic analyses of 103 patients with common variable immunodeficiency. 278 95

An extremely premature infant was born to a mother who had antibodies to cytomegalovirus (CMV). Despite having acquired transplacental antibodies, the baby developed an episode of presumed CMV hepatitis at the age of 10 weeks after blood transfusions from seropositive donors. Although the hepatitis settled, the baby developed chronic lung disease from which he died at the age of 5 months. Post mortem, widespread infection with CMV was demonstrated. The fact that serious CMV infection can develop in an infant of a seropositive mother further justifies the routine use of blood from CMV-negative donors for all transfusions given to infants of low birth weight.
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PMID:Fatal acquired cytomegalovirus infection in a neonate with maternal antibody. 285 Oct 21

Pasteurella ureae is uncommonly isolated from patients and rarely causes disease. The case of a patient with spontaneous bacterial peritonitis caused by P. ureae is described and contrasted with other reported cases of P. ureae colonization and infection. Respiratory colonization has been described in older patients, predominantly men, with underlying lung disease consisting principally of bronchitis, bronchiectasis, and tumors. Only 11 patients (including the present patient) have been reported to have illness with P. ureae. Seven had meningitis, two had pneumonia, and one had hepatitis. Skull fracture, alcoholism, and liver disease are common antecedents of P. ureae infection.
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PMID:Spontaneous bacterial peritonitis caused by Pasteurella ureae. 381 35

Cytomegalovirus (CMV) infection is the most frequent systemic viral opportunistic infection in AIDS, occurring in almost 40% of patients, at a stage of profound immunodeficiency, with a CD4 cell count lower than 50/microL. The most frequent localizations are retinal and gastrointestinal. Diagnosis of retinis, which can be totally asymptomatic, is based on fundus examination, which should be performed regularly in patients with AIDS and/or low CD4 count. Diagnosis of colitis, as of other rare manifestation (oesophagitis, hepatitis, encephalitis, myeloradiculitis, pneumopathy), relies on the association of suggestive clinical symptoms and CMV inclusions in biopsy specimens and/or CMV positive culture. The 2 drugs available for treatment of CMV disease, ganciclovir and foscarnet, are administered by intravenous route, with 2 infusions per day for induction therapy (usually 2 to 3 weeks), then once a day as lifelong maintenance therapy, to lessen or delay recurrences. Active drugs which could be given orally, combination of 2 drugs, new potent drugs and the development of prophylaxis in at-risk patients should help to improve the prognosis of CMV infection in AIDS.
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PMID:[Cytomegalovirus infections in AIDS]. 775 12


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