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)

The human herpes virus 6 (HHV 6) may induce not only the wellknown condition of exanthem subitum, but also a number of more common (cf. Part 1) or rare, even previously unknown, clinical manifestations. Part 2 of this paper deals with the more rarely observed manifestations. These include complications of ARD (sinusitis, otitis media, bronchial pneumonia) hepatitis, encephalitis or Pfeiffer's disease (mononucleosis-like syndrome). In individuals with a relevant disposition (genetic HLA/DR type?) initiation or (re-)activation of rheumatoid arthritis (JCA = juvenile chronic arthritis) or chronic iridocyclitis may occur. Although, on account of the high prevalence of vaccination in our population (approximately 95%), prenatal infections are extremely rare, they may manifest in a severe "septic" form (fatalities have occurred) or may lead to neurological deficits (comparable with cytomegalovirus infection). To date, no specific therapy (e.g. gammaglobulin, virostatics) or reliable preventive measures (e.g. vaccination) are available.
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PMID:[Infections with herpesvirus 6--really only "exanthema subitum"? Part 2: Rare or unknown disease pictures]. 133 53

Physicians treating adolescents should take a complete sexual history, including sexual orientation and practices, to determine whether their patients are homosexually active. Lesbians are at very low risk for sexually transmitted diseases, but they do have other health concerns. Four general groups of conditions may be encountered in homosexually active men: classical sexually transmitted diseases (gonorrhea, infections with Chlamydia trachomatis, syphilis, herpes simplex infections, genital warts, pubic lice, scabies); enteric diseases (infections with Shigella species, Campylobacter jejuni, Entamoeba histolytica, Giardia lamblia, hepatitis A, hepatitis B, hepatitis non-A, non-B, and cytomegalovirus); trauma (fecal incontinence, hemorrhoids, anal fissure, foreign bodies, rectosigmoid tears, allergic proctitis, penile edema, chemical sinusitis, inhaled nitrite burns, and sexual assault of the male patient); and the acquired immunodeficiency syndrome (AIDS). Clinicians can assist homosexual teenagers by understanding their special health needs, by counseling them about safe sexual practices, and by accepting their relationships nonjudgmentally.
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PMID:Medical problems of the homosexual adolescent. 383 19

We retrospectively studied 94 children with urticaria longer than six weeks in duration. The disease was equally distributed among the sexes and the following age subgroups (0-3.9 years, 4.0-7.9 years, 8.0-11.9 years and 12.0-15.9 years). A cause of the urticaria was identified or suspected in 15 of the patients. These included eight patients with cold urticaria, two with infection (hepatitis, sinusitis), two with food allergy, one patient with juvenile rheumatoid arthritis, one with arthralgia associated with a positive ANA and one with a low level of total hemolytic complement (CH50). Follow-up of a year of more on 52 patients revealed a median duration of urticarial symptoms of 16.0 months, with 58% of children becoming symptom free for six months or more, whereas the remaining 42% continued to have recurrent symptoms but without the development of an underlying serious illness. Results of the present study indicate that the etiology of chronic urticaria in childhood remains mostly undetermined but that the prognosis is generally favorable. However, one must consider an underlying infection or autoimmune disease as a potential etiology.
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PMID:Chronic urticaria in childhood: natural course and etiology. 688 5

Fungal infections are associated with a high mortality rate after liver transplantation. To describe risk factors for fungal infections, 405 consecutive liver transplant recipients were analyzed. Forty-five patients (11%) developed invasive fungal infection. Median posttransplantation time to the first episode was 60 days. Pathogens were Candida species (spp) (n=24, 53%), Cryptococcus neoformans (n=10, 22%), Aspergillus spp (n=6, 13%), Rhizopus spp (n=l), and others (n=4). Presentations of infection included disseminated (n=9), intra-abdominal (n=9), esophageal (n=9), lung (n=8), blood (n=6), and central nervous system infections (n=3), and sinusitis with esophagitis (n=1). Eighteen patients (40%) with invasive fungal infection died, and 13 (72%) of these deaths were attributable to fungi. Mortality in the nonfungal infection group was 12%. Univariate analysis identified separate risk factors for Candida (intra-abdominal bleeding), Aspergillus (fulminant hepatitis), and cryptococcal (symptomatic cytomegalovirus infection) infections. In both univariate and multivariate analyses, a high intratransplant transfusion requirement and posttransplant bacterial infection were identified as significant risk factors for all types of fungal infection. The risk factor analysis reported here suggests that different pathogenic processes lead to Candida and non-Candida infection in liver transplant recipients. Their identification should prompt specific prophylactic measures to reduce morbidity and mortality in this population.
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PMID:Risk factors of invasive Candida and non-Candida fungal infections after liver transplantation. 887 86

Microsporidia are ubiquitous in nature. Several clinical syndromes have been associated with microsporidiosis, especially in HIV-infected individuals, and include enteropathy, keratoconjunctivitis, sinusitis, tracheobronchitis, encephalitis, interstitial nephritis, hepatitis, cholecystitis, osteomyelitis, and myositis. Diarrhea and malabsorption are the most common clinical problems. Enterocytozoon bieneusi is the most common microsporidial cause of intestinal disease. A second species, Encephalitozoon intestinalis (originally named Septata intestinalis) is associated with disseminated as well as intestinal disease. Microsporidiosis has been seen worldwide, and is recognized as a frequent enteric infection in patients with AIDS. The pathogenesis of intestinal disease is related to excess death of enterocytes as a result of cellular infection. Clinically, microsporidiosis most often presents with diarrhea and weight loss as a result of small intestinal injury and malabsorption. However, microsporidia have been detected in virtually all organs, and may provoke symptoms related to their specific localization. The diagnosis of microsporidiosis is made histologically, either from tissue biopsies or secretions. While transmission electron microscopy was required for diagnosis in the past, special stains and light microscopy, as well as immunohistochemical and molecular techniques are capable of providing a firm diagnosis. Therapeutic options are limited. Enc. intestinalis responds well to albendazole, while no antiparasitic therapy has documented efficacy in Ent. bieneusi infections.
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PMID:Clinical syndromes associated with microsporidiosis. 955 78

Human microsporidiosis is a parasitic infection due to species of four different genera: Encephalitozoon; Enterocytozoon; Nosema; and Pleistophora. Although well known as a cause of disease in animals, microsporidiosis was only occasionally reported in humans. Recently, in human immunodeficiency virus (HIV)-infected patients, microsporidia belonging to Encephalitozoon and Enterocytozoon species have proved to be important opportunistic pathogens. Enterocytozoon bieneusi is associated with chronic intermittent diarrhea, cholangiopathy and sinusitis whereas Encephalitozoon intestinalis, Encephalitozoon hellem and Encephalitozoon cuniculi, the three Encephalitozoon species found in humans, are associated with diarrhea, rhinosinusitis, keratoconjunctivitis, nephritis and hepatitis. Diagnosis of microsporidial infections in humans was until recently an invasive, laborious procedure including electron microscopy of small intestine biopsies. However, new simple staining methods using Uvitex 2B or modified trichrome stain for feces and other body fluids have facilitated clinical diagnosis as well as drug evaluation and epidemiological studies. The application of monoclonal antibodies and molecular techniques such as the polymerase chain reaction have further improved microsporidial diagnosis. Treatment of Entero. bieneusi has, until now, been unsuccessful whereas albendazole has proved to be an effective treatment for Encephalitozoon species infection. Identification of effective treatment for Entero. bieneusi infections and further study of the pathogenicity of these microsporidial infections in immunocompetent hosts are important future challenges.
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PMID:Human microsporidiosis: Clinical, diagnostic and therapeutic aspects of an increasing infection. 1186 34

Fatal disseminated toxoplasmosis was diagnosed in a Risso's dolphin (Grampus griseus) dam and its fetus on the basis of pathologic findings, immunohistochemistry, and structure of the parasite. The dolphin was stranded alive on the Spanish Mediterranean coast and died a few hours later. At necropsy the dam was in good condition. From the standpoint of pathology, however, it had generalized lymphadenomegaly and splenomegaly, enlargement of and multifocal hemorrhage in the adrenal glands, diffuse mucosal hemorrhage of the glandular and pyloric stomach, ulcerative glossitis and stomatitis, focal erosions and reddening of the laryngeal appendix, and severe paraotic sinusitis with intralesional nematodes Crassicauda grampicola. The dolphin was pregnant, most probably in the first gestational trimester. The most prominent microscopic lesions were multifocal granulomatous encephalomyelitis, diffuse subacute interstitial pneumonia, mild multifocal necrotizing hepatitis and nonsuppurative cholangiohepatitis, gastritis and adrenalitis, mild lymphoid depletion, medullary sinus and follicular histyocitosis, and systemic hemosiderosis. The fetus had foci of coagulative and lytic necrosis in the kidneys, the lung, and the heart. Most lesions were associated with tachyzoites and tissue cysts of Toxoplasma gondii. The diagnosis was confirmed immunohistochemically. This is the first report on toxoplasmosis in a Risso's dolphin (G. griseus) and on transplacental transmission to an early-stage fetus in any cetaceans.
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PMID:Disseminated toxoplasmosis in a Mediterranean pregnant Risso's dolphin (Grampus griseus) with transplacental fetal infection. 1243 53

The X-linked hyper-IgM (XHIGM) syndrome is an uncommon primary immunodeficiency disease caused by mutations in the gene for CD40 ligand and characterized by normal or elevated serum IgM, reduced levels of IgG and IgA, and defective T-cell function. Because of its rarity, it has been difficult for any single investigator or institution to develop a comprehensive clinical picture of this disorder. Accordingly, a national registry was developed in the United States to provide demographic, genetic, immunologic, and clinical information on a relatively large number of patients with the XHIGM syndrome.A total of 79 patients from 60 unrelated families were registered between January 1997 and July 2002. The estimated minimal incidence was approximately 1/1,030,000 live births. All of the patients had significant IgG deficiency and most had IgA deficiency, but only one-half had elevated IgM levels. Most patients presented initially with a history of an increased susceptibility to infection including Pneumocystis carinii pneumonia. The average age of diagnosis was significantly earlier in patients born into a family with a previously affected individual. However, only one-third of the patients born into a family with a previously affected individual were diagnosed exclusively because of the presence of the positive family history before any clinical symptoms developed. Over half the patients developed symptoms of immunodeficiency and were diagnosed by 1 year of age, and over 90% by 4 years of age. The most prominent clinical infections were pneumonia (81% of patients), upper respiratory infections (49%) including sinusitis (43%) and recurrent otitis (43%), recurrent/protracted diarrhea (34%), central nervous system infections (14%), sepsis (13%), cellulitis (13%), hepatitis (9%), and osteomyelitis (1%). In addition to infections caused by encapsulated bacteria, opportunistic infections were relatively common and were caused by P. carinii, members of the herpes virus family (including cytomegalovirus), Cryptosporidium, Cryptococcus, Candida, Histoplasma, and Bartonella. Sclerosing cholangitis occurred in 5 patients and in 4 of these was associated with Cryptosporidium infection. Eight patients had died at the time of their entry into the Registry; 2 of pneumonia (1 P. carinii and 1 cytomegalovirus), 2 of encephalitis (1 ECHO virus and 1 cytomegalovirus), 2 of malignancy (both hepatocellular carcinoma), 1 of sclerosing cholangitis caused by Cryptosporidium, and 1 of hemolytic uremic syndrome.
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PMID:The X-linked hyper-IgM syndrome: clinical and immunologic features of 79 patients. 1466 87

X-linked agammaglobulinemia (XLA) is a primary immunodeficiency caused by mutations in the gene for Bruton tyrosine kinase (BTK) that result in the deficient development of B lymphocytes and hypogammaglobulinemia. Because the disorder is uncommon, no single institution has had sufficient numbers of patients to develop a comprehensive clinical picture of the disorder. Accordingly, a national registry of United States residents with XLA was established in 1999 to provide an updated clinical view of the disorder in a large cohort of patients. A total of 201 patients were registered by 66 physicians. The estimated birth rate for the 10-year period of 1988-1997 was 1/379,000. Infection was the most common initial clinical presentation (85%), followed by a positive family history (41%) and neutropenia (11%). Although the average age of diagnosis was younger in patients with a positive family history (mean, 2.59 yr) than in patients with a negative family history (mean, 5.37 yr) (p < 0.001), only 34.5% of patients with a positive family history at the time of their birth were diagnosed before clinical symptoms developed-that is, based on family history alone. Seventy percent of patients had at least 1 episode of otitis, 62% at least 1 episode of pneumonia, 60% at least 1 episode of sinusitis, 23% at least 1 episode of chronic/recurrent diarrhea, 21% at least 1 episode of conjunctivitis, 18% at least 1 episode of pyoderma and/or cellulitis, 11% at least 1 episode of meningitis/encephalitis, 10% at least 1 episode of sepsis, 8% at least 1 episode of septic arthritis, 6% at least 1 episode of hepatitis, and 3% at least 1 episode of osteomyelitis. Fourteen of 201 (6.9%) patients were dead at the time they were entered in the Registry. However, in a prospective 4 /4-year follow-up of living patients, only 3/80 (3.75%) patients died. Causes of death included disseminated enterovirus infection (n = 6), pulmonary insufficiency (n = 5), adenovirus infection (n = 1), sepsis (n = 1), acquired immunodeficiency disease syndrome (AIDS) (n = 1), myocarditis (n = 1), hepatitis (n = 2), and stem cell transplantation (n = 1).
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PMID:X-linked agammaglobulinemia: report on a United States registry of 201 patients. 1686 44

An adult female, albino South African Clawed frog (Xenopus laevis) from a research colony at the Biological Resources Facility of the College of Agriculture and Life Sciences at North Carolina State University (NCSU) was presented with depression, lethargy, loss of diving reflex, and a distended abdomen. Cytologic examination of coelomic effusion fluid at the NCSU veterinary teaching hospital revealed a mixed population of inflammatory cells, including heterophils and a predominance of large mononuclear cells (macrophages) that often contained intracytoplasmic, negatively-stained, rod-shaped to filamentous organisms consistent with Mycobacterium sp. Ziehl-Neelsen stain revealed bright pink to red, acid-fast organisms with a beaded appearance. Histopathologic findings in tissues obtained at necropsy included marked, multifocal to coalescing, heterophilic, granulomatous and fibrinous coelomitis as well as severe multifocal heterophilic and granulomatous hepatitis, interstitial pneumonia and sinusitis/rhinitis. Slender gram-positive, acid-fast bacterial rods were identified in sections of coelomic pleura, kidneys, nasal cavities, spleen, liver, and pulmonary interstitium, indicative of systemic mycobacteriosis. Based on mycobacterial culture, the organism was identified as M marinum complex. Mycobacteria are variably gram-positive, often acid-fast, small rods that are ubiquitous in aquatic environments. The clinical and pathologic spectrum of disease in amphibians depends on host and pathogen status. Xenopus sp and several other frogs are good models for studying the pathogenesis of M tuberculosis infection. In addition to culture, polymerase chain reaction assays may be used for definitive identification of the organisms; accurate speciation may require further genetic investigation.
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PMID:Reluctant to dive: coelomic effusion in a frog. 1696 23


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