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Risk of Uterine Rupture during Labor among Women with a Prior Cesarean Delivery
Many women attempt a trial of labor after prior cesarean section, yet there is concern that this may increase the risk of uterine rupture. This large study, involving 20,095 primiparous women with one previous cesarean section who delivered a second singleton child, found an increased risk of uterine rupture associated with spontaneous or induced labor, particularly with labor induced with a prostaglandin.
This study provides compelling evidence that uterine rupture is more likely in women who have a trial of labor following prior cesarean section, and that the risk is particularly increased when labor is induced with use of prostaglandins. Since misoprostol was generally available only during the last year of the study, the findings argue for caution in the use of other prostaglandins, as well as misoprostol, for induction of labor.
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Recombinant Human  -Galactosidase A Replacement Therapy in Patients with Fabry's Disease
Fabry's disease, an X-linked inborn error of metabolism caused by a lysosomal  -galactosidase A deficiency, is associated with early death from microvascular disease owing to the progressive deposition of glycosphingolipids in the kidneys, heart, and brain. In this randomized, multicenter, double-blind study, 58 patients with Fabry's disease received recombinant human -galactosidase A or placebo. The results indicate that replacement therapy can clear microvascular deposits of globotriaosylceramide and lower plasma concentrations.
Recombinant human -galactosidase A replacement therapy reverses a number of changes in Fabry's disease and thus may prevent disability and early death.
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Familial Hemiplegic Migraine Associated with Mutations in a Neuronal Calcium Channel
Familial hemiplegic migraine is an autosomal dominant disorder characterized by attacks of hemiparesis followed by a migraine headache. In some cases, there are other features, such as ataxia, nystagmus, or other permanent cerebellar signs. In about half the families affected, a mutation in CACNA1A, a gene that encodes a neuronal calcium channel, is present. In this study of 117 subjects with mutations in CACNA1A, particular clinical features were found to be partially accounted for by the nature of the mutation.
This study is of interest because it confirms that mutations in a gene encoding a neuronal calcium channel may be responsible for some cases of familial hemiplegic migraine. The findings also indicate that various clinical features may be explained by mutations located in different regions of the gene.
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Galactose Infusions for the Cardiac Variant of Fabry's Disease
Cardiomyopathy is the main feature of the cardiac variant of Fabry's disease, in which mutant -galactosidase A has some enzymatic activity but is malformed and rapidly degraded. Galactose attaches to the enzyme's binding site, thereby correcting the malformation and allowing the enzyme to degrade deposits of glycosphingolipids. A patient with Fabry's disease who was disabled by severe cardiomyopathy had a response to intravenous galactose and was able to return to work.
The spectacular therapeutic effect of galactose infusions in this patient suggests that other inherited diseases resulting from malformed proteins with residual enzymatic activity may respond to such treatment, described by the authors as "chaperone-mediated therapy."
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Clinical Practice: Hormone-Replacement Therapy
A 52-year-old woman who is not at increased risk for breast cancer has hot flashes and disturbed sleep. A 58-year-old woman whose mother had breast cancer has osteopenia. A 65-year-old woman with a history of coronary revascularization has osteoporosis. Each woman wants to know whether she should use hormone-replacement therapy. This article reviews the benefits and risks of hormone-replacement therapy, with particular attention to the conundrum of coronary heart disease. A strategy for decision making is presented.
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Medical Progress: Hepatitis C Virus Infection
Approximately 170 million people worldwide are infected with the hepatitis C virus, which usually causes progressive liver disease, is a common reason for liver transplantation, and can result in hepatocellular carcinoma. The combination of interferon alfa and ribavirin is a promising new approach to therapy.
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