Collapsing glomerulopathy in sickle cell disease: a case report

<p>Abstract</p> <p>Introduction</p> <p>Sickle cell disease has been associated with many renal structural and functional abnormalities. Collapsing glomerulopathy or the collapsing variant of focal segmental glomerulosclerosis is a rare clinicopathologic entity in patients with sickle cell disease that requires timely diagnosis and aggressive management.</p> <p>Case presentation</p> <p>In this case report we describe a 21-year-old African-American woman with a medical history of significant sickle cell disease and asthma. She was admitted for pain, decreased urine output, bilateral leg swelling and reported weight gain. During her period of hospitalisation she developed acute renal failure requiring dialysis. Further investigation revealed the collapsing variant of focal segmental glomerulosclerosis.</p> <p>Conclusions</p> <p>Although focal segmental glomerulosclerosis is a common feature of sickle cell nephropathy, the collapsing variant of focal segmental glomerulosclerosis or collapsing glomerulopathy has been rarely documented. Even when other risk factors are controlled, collapsing glomerulopathy has a very poor prognosis. This is a rare case of a patient with massive proteinuria presenting as acute renal failure with a very poor response to corticosteroids and a much faster rate of progression to end-stage renal disease.</p

Essential fatty acids for premenstrual syndrome and their effect on prolactin and total cholesterol levels: a randomized, double blind, placebo-controlled study

<p>Abstract</p> <p>Objective</p> <p>To evaluate the effectiveness and safety of polyunsaturated fatty acids for the treatment of the premenstrual syndrome (PMS) using a graded symptom scale and to assess the effect of this treatment on basal plasma levels of prolactin and total cholesterol.</p> <p>Methods</p> <p>A randomized, double-blind, placebo-controlled study was conducted with 120 women with PMS divided into three groups and treated with 1 or 2 grams of the medication or placebo. Symptoms were recorded over a 6-month period using the Prospective Record of the Impact and Severity of Menstruation (PRISM) calendar. Total cholesterol and prolactin levels were measured. Analysis of variance (ANOVA), Pearson's chi-square test, Wilcoxon's nonparametric signed-rank test for paired samples and the Mann-Whitney nonparametric test for independent samples were used in the statistical analysis.</p> <p>Results</p> <p>There were no differences in age, marital status, schooling or ethnicity between the groups. In the group treated with 1 gram of the medication, a significant reduction was found when the median PRISM score recorded in the luteal phase at baseline (99) was compared with the median score recorded in the 3^rdmonth (58) and in the 6^thmonth of evaluation (35). In the 2-gram group, these differences were even more significant (baseline score: 98; 3^rdmonth: 48; 6^thmonth: 28). In the placebo group, there was a significant reduction at the 3^rdbut not at the 6^thmonth (baseline: 96.5; 3^rdmonth: 63.5; 6^thmonth: 62). The difference between the phases of the menstrual cycle was greater in the 2-gram group compared to the group treated with 1 gram of the medication. There were no statistically significant differences in prolactin or total cholesterol levels between baseline values and those recorded after six months of treatment.</p> <p>Conclusion</p> <p>The difference between the groups using the medication and the placebo group with respect to the improvement in symptomatology appears to indicate the effectiveness of the drug. Improvement in symptoms was higher when the 2-gram dose was used. This medication was not associated with any changes in prolactin or total cholesterol levels in these women.</p

The glomerulopathy of sickle cell disease

Sickle cell disease (SCD) produces many structural and functional abnormalities in the kidney, including glomerular abnormalities. Albuminuria is the most common manifestation of glomerular damage, with a prevalence between 26 and 68% in adult patients. The pathophysiology of albuminuria in SCD is likely multifactorial, with contributions from hyperfiltration, glomerular hypertension, ischemia-reperfusion injury, oxidative stress, decreased nitric oxide (NO) bioavailability, and endothelial dysfunction. Although its natural history in SCD remains inadequately defined, albuminuria is associated with increased echocardiography-derived tricuspid regurgitant jet velocity, systemic blood pressure, and hypertension, as well as history of stroke, suggesting a shared vasculopathic pathophysiology. While most patients with albuminuria are treated with angiotensin converting enzyme inhibitors/angiotensin receptor blockers, there are no published long-term data on the efficacy of these agents. With the improved patient survival following kidney transplantation, SCD patients with end-stage renal disease should be considered for this treatment modality. Given the high prevalence of albuminuria and its association with multiple SCD-related clinical complications, additional studies are needed to answer several clinically important questions in a bid to adequately elucidate its pathophysiology, natural history, and treatment

Hypertension and microalbuminuria in children with congenital solitary kidneys.

Contains fulltext : 69925.pdf (publisher's version ) (Closed access)AIM: According to the hyperfiltration hypothesis, a low nephron endowment will lead to hyperfiltration in the remaining glomeruli and is associated with systemic hypertension, proteinuria and glomerulosclerosis. Being born with one functioning kidney instead of two, for instance because of unilateral renal agenesis or multicystic dysplastic kidney, is a cause of congenital renal mass reduction. METHODS: In order to study the effect of congenital renal mass reduction on renal function and blood pressure, a retrospective chart review of 66 patients at the Pediatric Renal Center of the VU University Medical Center was performed. As intrauterine growth restriction is associated with a low nephron endowment, the additional effect of birthweight was also studied. RESULTS: A total of 50% of patients with congenital renal mass reduction is found to be hypertensive, using anti-hypertensive drugs, and/or having microalbuminuria (>20 mug/min). Patients born small for gestational age have significantly smaller kidneys and lower estimated glomerular filtration rate than patients with a normal birthweight. CONCLUSIONS: We conclude that microalbuminuria and/or hypertension is present in 50% of patients with congenital solitary kidneys, which warrants a systematic follow-up of blood pressure, proteinuria and renal function in all patients with congenital solitary functioning kidneys, especially in patients with a low birthweight