Diagnosing Wilson disease.
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Biomedical subjects
Publications and source records attributed to L S Friedman.
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BACKGROUND: It is unknown whether patients want primary care physicians to inquire about physical abuse (PA) or sexual abuse (SA) or how frequently physicians make such inquiries. METHODS: To determine patient preferences and physician practices, we surveyed 164 patients and 27 physicians at private and public primary care sites. Data were collected using confidential, anonymous, written, multiple-choice questionnaires and were evaluated using univariate analysis. RESULTS: Among patients, routine PA inquiry was favored by 78% and routine SA inquiry was favored by 68%. Only 7% were ever asked about PA and 6% about SA. A history of PA was reported by 16% and a history of SA by 17%. Ninety percent believed physicians could help with problems from PA and 89% felt physicians could help with problems from SA. Among physicians, one third believed that PA and SA questions should be asked routinely. However, SA inquiries were never made by 89% at initial visits or by 85% at annual visits. Physical abuse inquiries were never made by 67% at initial visits, or by 60% at annual visits. Eighty-one percent believed they could help with problems associated with PA and 74% with SA. CONCLUSIONS: Most patients favor inquiries about physical and sexual abuse and believe physicians can help with these problems. Physicians believe they can help with these problems though they frequently do not inquire.
Alpha-interferon is the first agent with proven therapeutic efficacy in humans with chronic HBV infection. Obviously, further research is needed to clarify and expand the role of interferon in this setting. In addition, an overall response rate of less than 50% illustrates the need for continuing innovation in the treatment of HBV infection especially in patients with predictors of poor outcome. Despite the advent of effective vaccines against HBV, this pathogen is likely to remain a source of serious human morbidity and mortality for the foreseeable future. Continuing efforts must be directed towards finding more effective therapies against HBV. A recent preliminary report suggests that thymosin. like interferon on immune modulator, may also be efficacious in the treatment of chronic HBV and deserves further study.
OBJECTIVE: To review current concepts about the pathogenesis, clinical manifestations, and treatment of Wilson's disease, with an emphasis on recent developments. DATA IDENTIFICATION: Published information was identified using MEDLINE and through extensive manual searching of bibliographies in identified sources. RESULTS: The basic biochemical alteration responsible for deranged hepatobiliary copper homeostasis in Wilson's disease has yet to be identified. The gene for Wilson's disease has been mapped to chromosome 13, but the function of its gene product has not yet been determined. The clinical manifestations of Wilson's disease are varied and often nonspecific and include a range of hepatic, neurologic, and psychiatric findings. Penicillamine remains the drug of choice for the treatment of Wilson's disease, but recent experience suggests that trientine and zinc may be safe, effective alternatives. All three drugs are probably safe for use in pregnant patients with Wilson's disease. Liver transplantation is the only effective treatment for Wilsonian fulminant hepatic failure and corrects the underlying metabolic defect. CONCLUSIONS: Wilson's disease is a disorder of hepatobiliary copper excretion manifested predominantly by hepatic and neurologic copper toxicosis and inherited in an autosomal recessive pattern. Although the specific underlying biochemical defect remains to be defined, specific therapy is available and usually successful. Maintaining a high index of suspicion is critical in diagnosing this readily treatable inherited disease.
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Social reinforcers such as spouse behaviors have been hypothesized to be important in maintaining chronic pain behavior. This study used direct observation to test whether solicitous and aggressive spouse behaviors systematically precede and follow patient pain behaviors. Fifty chronic pain patients and spouses and 33 control couples were videotaped performing specified tasks. Spouse solicitous behaviors were significantly more likely to precede and follow nonverbal pain behaviors, and nonverbal pain behaviors were significantly less likely to follow spouse aggressive behaviors in pain than in control couples. Within couples, spouse solicitous behaviors preceded and followed verbal and nonverbal pain behaviors beyond chance levels more often in pain than in control couples. Results support an operant conceptualization of factors maintaining chronic pain behaviors.
Listeria monocytogenes is a Gram-positive bacillus that is pathogenic in both the normal and compromised host. We describe Listeria peritonitis and cerebritis in a patient with cirrhosis due to non-A, non-B hepatitis, and review the 11 other cases of Listeria peritonitis reported in the English-language literature. Listeria is a rare cause of peritonitis in debilitated, older patients, with two-thirds of the cases occurring in patients with chronic liver disease. Listeria peritonitis may also occur in patients undergoing peritoneal dialysis, or in those with malignancy. Peritonitis due to Listeria is clinically similar to spontaneous bacterial peritonitis, and is associated with fever, variable abdominal pain, and neutrocytic ascites; bacteremia commonly accompanies Listeria peritonitis. This syndrome can be successfully treated with antimicrobial drugs, although the third-generation cephalosporins commonly used in the therapy of spontaneous bacterial peritonitis are not recommended. Ampicillin may be the drug of choice, with combination therapy with an aminoglycoside reserved for cases that do not respond to ampicillin alone.
Liver transplantation is now considered definitive therapy for end-stage liver disease and has been playing an increasingly important role in the management of fulminant hepatic failure. With the advent of effective immunosuppression and improved surgical techniques, high survival rates can be expected for most transplanted patients. It has become apparent, however, that transplantation for patients with viral hepatitis is associated with some unique problems because of the propensity for viral reinfection of the grafted liver. Patients with actively replicating hepatitis B viral infection pretransplantation appear to be most likely to experience clinically significant recurrent hepatitis. Recurrent hepatitis D (delta) and hepatitis C appear to be relatively less serious in the transplanted liver. Interventions to prevent or treat graft reinfection have thus far met with limited success. Further studies are needed to define more precisely which patients with viral hepatitis are likely to do poorly after liver transplantation, and to develop strategies for treating recurrent hepatitis in transplant recipients.
Although adolescents currently compose fewer than 1% of AIDS cases nationally, they represent an expanding and pivotal population. This article is written for those who currently provide health care for adolescents and for those who will do so in the future. Much of what is known about AIDS in adolescents will be reviewed. Epidemiology, specific risk groups and behavior as well as legal, ethical, educational, and preventive issues unique to adolescents are discussed. In addition, some recommendations and techniques for interviewing teenagers are suggested.
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Collagenous colitis, a cause of watery diarrhea characterized by a distinctive band of collagen under the surface epithelium of the colon, has been recognized with increasing frequency in recent years. The pathophysiology of collagenous colitis remains obscure. The thickening of the subepithelial collagen layer may be a response to chronic inflammation or a local abnormality of collagen synthesis. The precise mechanism of the diarrhea in collagenous colitis is also unclear, and it has not been possible to link the diarrhea directly to the excess collagen deposition. The relationship between collagenous colitis and lymphocytic colitis, another type of microscopic colitis, remains to be defined; elucidating the relationship between the two disorders may provide clues to the pathophysiology of both.
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