Journal of nephrology
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Journal of nephrology · May 2008
ReviewMetabolic syndrome and chronic kidney disease: a Japanese perspective on a worldwide problem.
Metabolic syndrome and obesity have causative roles in the development of chronic kidney disease (CKD). CKD leads to end-stage renal disease (ESRD), cardiovascular disease and death. The prevalence of metabolic syndrome is increasing worldwide in both developing and developed countries. ⋯ A fundamental scientific question is the ethnic factor for calculating the glomerular filtration rate (GFR) using the Modification of Diet in Renal Disease Study equation. The incidence and prevalence of CKD are closely related with lifestyle factors such as diet, exercise, tobacco use, as well as other cultural differences. Research on the relationship between CKD and metabolic syndrome may provide clues to better understand the role of lifestyle-related factors and the age-related decline in GFR.
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The goal of palliative care is to achieve good quality of life for patients with chronic life-limiting illnesses, and their families, through assessment and management of physical, psychosocial and spiritual problems and needs. Patients with kidney diseases present a particular target of such care from the time of diagnosis to the time of family bereavement family. ⋯ Pain and other physical, psychosocial and spiritual symptom management appear to be an important issue in nephrology. Withdrawal from dialysis is another problem that may require special attention in the form of legal and ethical guidance.
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Journal of nephrology · Jan 2008
ReviewUpdate on Fabry disease: kidney involvement, renal progression and enzyme replacement therapy.
Fabry disease is an X-linked lysosomal storage disorder which is caused by a deficiency of the lysosomal enzyme alpha-galactosidase A. The lack of enzyme causes a progressive intracellular accumulation of glycosphingolipids, mainly globotriaosylceramide (GL3). Affected organs are, among others, the vascular endothelium, heart, brain and kidneys, as well as the central and peripheral nervous system. ⋯ Long-term treatment outcomes in patients with severe organ manifestations, in particular proteinuria and renal function impairment, are still critical and warrant further investigation. Besides ERT being an optimized adjunctive therapy, timely initiation of ERT is important to assure optimal medical care. Subsequent follow-up assessments should be carried out in all patients on a regular basis to evaluate treatment outcomes.
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The anion gap (AG) is an important tool in the evaluation of metabolic acidosis. It is affected by many variables including serum albumin and globulin concentrations. HIV patients may have lower serum albumin and higher serum globulin concentrations. We hypothesized that the AG in HIV patients may differ from that of normal controls. ⋯ Our results indicate that the AG is lower in HIV patients and that this decrement may be due to the increase in serum globulin concentrations. Since a high serum AG metabolic acidosis may be masked by a deceitfully normal AG in patients with elevated serum globulin concentrations, calculation of corrected AG should be undertaken to avoid a costly delay in diagnosis and treatment.
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Journal of nephrology · Nov 2007
Review Case ReportsCatastrophic antiphospholipid syndrome: report of 4 cases.
Catastrophic antiphospholipid syndrome (CAPS), described by Asherson in 1992, is a rare form of antiphospholipid syndrome resulting in multiorgan failure with a mortality rate of about 50%. The syndrome occurs in patients with either systemic lupus erythematosus and other rheumatic diseases (systemic sclerosis, rheumatoid arthritis, primary Sjogren syndrome) or alone. Whereas in "classic" antiphospholipid syndrome (APS), medium-large vessels are involved, a diffuse small vessel ischemia and thrombosis (microangiopathic disease) leading to a severe multiorgan dysfunction is predominant in CAPS. "Trigger" factors have been demonstrated in 45% of patients, but in the majority, they remain unknown. ⋯ Disseminated intravascular coagulation occurs in approximately 13% of patients. The present study reports the clinical and serological features of 4 patients affected by this rare form of antiphospholipid syndrome. Nephrologists should be aware of the possibility of this syndrome as a cause of multiorgan failure since prompt recognition is essential for effective treatment.