Paricalcitol as an Antiproteinuric Agent Can Result in the Deterioration of Renal and Heart Function in a Patient with Fabry Disease.

BACKGROUND Fabry disease is a rare and progressive X-linked inherited disorder of glycosphingolipid metabolism that is due to deficient or absent lysosomal a-galactosidase A activity. Among its other associated signs and symptoms, patients present with renal failure and proteinuria, which are markers of disease progression. Renin-angiotensin-aldosterone system (RAAS) blockers can slow the progression of chronic renal failure and proteinuria. In fact, some studies have shown the beneficial effects of paricalcitol on proteinuria. CASE REPORT We present a case of a female patient with the classic variant of Fabry disease. She was treated with a high dose of paricalcitol as an antiproteinuric agent due to unsatisfactory double-RAAS blockage, which resulted in transient worsening of cardiac and renal function. CONCLUSIONS Despite the positive effects of paricalcitol as an antiproteinuric agent, as previously shown by some authors, our case highlights the possible serious adverse effects associated with the use of high doses of this drug.

Urinary immunoglobulin G to albumin ratio and N-Acetyl-Beta-D-Glucosaminidase as early predictors of therapeutic response in ANCA-associated glomerulonephritis.

BACKGROUND: The aim of our study was to evaluate the prognostic value of glomerular and tubular proteinuria and tubular enzymuria as early indicators of therapeutic response to induction therapy with i.v. pulse cyclophosphamide (CyC) and methylprednisolone (MP) in patients with antineutrophil cytoplasmic antibody (ANCA) associated glomerulonephritis. METHODS AND FINDINGS: An observational single-center study was conducted in 30 patients with ANCA-associated glomerulonephritis. Patients were divided into subgroups with good or poor response to CyC therapy according to clinical and laboratory parameters. The diagnosis of ANCA-associated glomerulonephritis was based on the Chapel-Hill disease definitions. Good response to induction therapy was significantly associated with higher absolute values of urine N-acetyl-beta-D-glucosaminidase (NAG) to creatinine ratio (above 14.83 microcat/mol) and urine immunoglobulin G (IgG) to albumin ratio (above 0.09) at the time of diagnosis, while albuminuria or proteinuria did not have any early predictive value. The remission of renal disease was anticipated as early as 3 months after introduction of induction therapy in patients with reduction of urine NAG to creatinine ratio below the baseline value and in patients with at least 24% rise in eGFR. CONCLUSIONS: Urine IgG to albumin and urine NAG to creatinine ratio are better early predictors of treatment response in patients with ANCA-associated glomerulonephritis than proteinuria or albuminuria.

Electrocardiographic findings in children with erythema migrans.

OBJECTIVE: To assess electrocardiographic findings in children with erythema migrans and to compare them with findings obtained in a healthy control group of comparable age and with a similar proportion of boys and girls. METHODS: Electrocardiograms were carried out in 147 children under the age of 15 years before treatment with antibiotics for erythema migrans (solitary 68 patients, multiple 79 patients) and in the control group of 148 healthy children. RESULTS: Abnormal electrocardiographic findings were detected more often in healthy children than in patients (14% versus 5%; P = 0.0303) and among patients more often in boys than in girls (10% versus 0%; P = 0.0107). Electrocardiographic abnormalities characteristic for Lyme borreliosis, such as atrioventricular blocks, were rare: in patients with erythema migrans only one child had first-degree atrioventricular block; in the control group one child had first-degree and another had second-degree atrioventricular block. Patients with erythema migrans had shorter PR and RR intervals and lower R and S wave voltages in V1 than the healthy children. Comparison among patients with solitary and multiple erythema migrans did not reveal significant electrocardiographic differences. The frequency of electrocardiographic abnormalities in patients with erythema migrans was not associated with the presence of systemic symptoms, or with the presence of meningitis or the isolation of Borrelia burgdorferi sensu lato from the blood. CONCLUSIONS: Electrocardiographic abnormalities in children with erythema migrans are mild, nonspecific and rare. The presence of clinical signs and symptoms indicative or suggestive of disseminated Lyme borreliosis is not associated with higher frequency of such abnormalities. Comparison of findings in patients with erythema migrans and healthy children revealed several distinctions, some of which might have been interpreted as a result of altered activity of the autonomic nervous system.