Relationship between serum cystatin-c and coronary lesion severity in coronary artery disease patients with a normal glomerular filtration rate

Objective Cardiovascular disease is a major cause of death. This study evaluated the relationship between serum cystatin-c and coronary lesion severity in coronary artery disease (CAD) patients with a normal glomerular filtration rate. Methods Nine hundred and fifty-nine patients were retrospectively included and divided into non-CAD and CAD groups according to coronary angiography results. CAD patients were classified into three groups by Gensini score tertiles. Multivariable logistic regression was used to study the relationship between serum cystatin-c and coronary lesion severity. Results Serum cystatin-c levels were significantly higher in CAD patients than in non-CAD patients. Correlation analysis revealed significant correlations between serum cystatin-c levels with the Gensini score and the number of diseased vessels. The area under the receiver operating characteristic curve of serum cystatin-c was 0.544 and 0.555 for predicting a high Gensini score and three-vessel disease, respectively. Multivariate stepwise regression analysis demonstrated that the serum cystatin-c level was an independent predictor of a high Gensini score [odds ratio (OR) = 2.177, 95% confidence interval (CI) 1.140–3.930] and three-vessel disease (OR = 1.845, 95% CI 0.994–3.424) after adjusting for the conventional CAD risk factors. Conclusions Serum cystatin-c was elevated in CAD patients and may be an independent predictor of CAD severity.

The uraemic hypertensive patient: a therapeutic challenge—right you are (if you think so)

High blood pressure (BP) is a leading cause of chronic kidney disease (CKD) and at the same time represents its most frequent complication. High BP is an independent risk factor for advanced CKD; on the other hand, at least 40% of patients with normal glomerular filtration rate (GFR) and virtually all patients with GFR <30 mL/min are hypertensive. CKD and microalbuminuria are powerful risk factors for cardiovascular morbidity and mortality. Consequently, in uraemic hypertension, it is of utmost importance to carefully manage both high BP and microalbuminuria, in order to slow down the progression of kidney damage and to reduce the incidence of cardiovascular events. The first purpose of the medical treatment in hypertensive patients is to normalize BP, regardless of the drug used. Nevertheless, some drugs have an ‘additional’ nephroprotective effect at the same BP target achieved. In this regard, first-line drugs are definitely renin–angiotensin–aldosterone inhibitors, mainly for their proved efficacy in reducing hypertension-related kidney damage and proteinuria. Anyway, a combined approach (two or more drugs) is usually needed to achieve the optimal BP target and reduce the worsening of CKD.

Activity of lysosomal urine enzymes in children after acute kidney injury

The importance of the problem of acute kidney injury in children is due to the high risk of developing chronic kidney disease as a consequence. Lysosomal enzymes of β-galactosidase (GAL) and N-acetyl-β-D-hexozoaminidase (NAG) in urine are considered to be informative markers of renal parenchyma damage. The objective of this study - to determine the activity of lysosomal enzymes in urine as markers of progression of interstitial nephritis in children after acute kidney injury. Methods. 41 children were examined after acute kidney injury, achievement of self-diuresis and improvement. Group I included 22 patients with a disease period of up to 2 years after acute kidney injury, group II - 19 patients with a disease period of 2 years or more. The control (reference) group consisted of 28 children who were conditionally healthy, without kidney disease, as well as without acute diseases and severe metabolic disorders and anatomical defects. Results. NAG and GAL activity were found to exceed 8 and 3 times parameters in the reference group of healthy children, respectively, in patients who had acute kidney injury during the year (p <0.001). In patients with a history of 2 years or more, enzyme levels decreased, but remained higher than normal up to 4 times (p <0.001). The highest level of NAG activity was observed in patients of group 1 with the combination of hemolytic-uremic syndrome with hemolytic anemia, and the lowest - in children with toxic kidney. Determination of the activity of lysosomal enzymes NAG and GAL is a more informative marker of torpid course of interstitial nephritis with a progressive decrease in renal function than indicators of glomerular filtration rate. In twelve months after acute kidney damage in 75% of children, the level of NAG and GAL activity remained significantly elevated at normal glomerular filtration rate. A correlation between glomerular filtration rate and NAG activity (r = -0.473) and GAL (r = -0.333) and a direct correlation between NAG and GAL activity (r = 0.845) were observed. Conclusions. The levels of lysosomal enzymes of NAG and GAL were found to be 8 and 3 times higher than normal, respectively, in patients who suffered acute kidney injury during the year, and in patients with a history of 2 years or more, enzyme activity levels decreased, but remained higher than normal 4-fold (p <0.001). In a year after acute kidney damage in 75% of children, the level of NAG and GAL activity remained significantly elevated despite of normal glomerular filtration rate. Therefore, the detection of NAG and β-galactosidase in the urine of children after acute kidney injury are informative markers of renal parenchyma damage, which may serve as objective criteria for progressive decline in renal functional status, and the lack of normalization of their levels is not a sign of progression of interstitial nephritis in corresponding group of children.

Proximal renal tubular acidosis with primary Fanconi syndrome

Renal tubular acidosis (RTA) is associated with normal or near normal glomerular filtration rate. Proximal RTA is associated with impaired bicarbonate reabsorption. This is manifested as bicarbonate wastage in the urine, and this reflects the defect in proximal tubular transport. Osteopenia or full-blown rickets may develop. Type 2 RTA is rare and occurs in association with conditions such as Fanconi syndrome. This is manifested as glycosuria, aminoaciduria, phosphate wasting and mild proteinuria. The basis of therapy is the continuous administration of appropriate amounts of alkali in the form of either bicarbonate or citrate, as well as the treatment of the cause.

Congenital Anomalies of the Kidney and Urinary Tract in Children Born Small for Gestational Age

Introduction: Congenital anomalies of the kidney and urinary tract (CAKUT) represent several types of malformations with occurrence of 1 in about 500 live births. Objective: Small for gestation age (SGA) may influence in prevalence of CAKUT and progression of chronic kidney disease (CKD) in children. The aim of this study was to elaborate our experiences with detected CAKUT in a cohort of SGA born children in Macedonia. Methods: Our cohort consisted of 100 SGA born children investigated for associated congenital anomalies of urinary tract. We analyzed anthropometric and clinical birth data in children with diagnosed CAKUT and estimated the stage and time of onset of CKD by biochemical and imaging technics. Results: We revealed 7 (7.0%) SGA born children with congenital anomalies of the urinary tract. Their mean birth weight was very low 1855 gr (-3.93 SDS) and the birth length 45.57cm (-2.17 SDS), as well. A significant growth failure with reduced weight and BMI were noticed at the time of diagnosis. A diagnosis of CAKUT in 4/7 was established in the first few months of life, but in others 3 later in early childhood. Three children revealed with unilateral kidney agenesis, 2 had hypo-dysplastic kidneys and in 2 children was found vesicoureteral reflux. Normal glomerular filtration rate was estimated in 2 children with CAKUT. Stage 2 CKD with GFR 60-90 ml/minx1.73m2 had 3 children, 1 patient was graded in stage 3 and one child needed kidney transplantation, stage 5 CKD. Conclusions: We presented 7 SGA born children with CAKUT. An early recognition, assessment and treatment of these anomalies might improve their quality of life.

Three cases of Gordon syndrome with dominant KLHL3 mutations

Background:Gordon syndrome (GS) is a rare form of monogenic hypertension characterized by low renin hypertension, hyperkalemia, hyperchloremic metabolic acidosis, and normal glomerular filtration rate. To date, four genes causing GS have been identified as:Case presentation:We report three cases of GS in two families. All patients presented with typical clinical features of GS and had a known dominantConclusions:GS should be considered in patients with low renin hypertension and hyperkalemia. Although it is a rare disease, the correct diagnosis of GS is clinically important, as it can easily be treated with a low sodium diet or thiazides. In addition, family studies can identify individuals with undiagnosed GS as all mutations causing this disease, except for some recessive

Renal tubular acidosis--underrated problem?

Renal tubular acidosis (RTA) is a hyperchloremic metabolic acidosis characterized by a normal anion gap and normal (or near normal) glomerular filtration rate in the absence of diarrhoea. Inherited isolated forms of renal tubular acidosis are not common. However, they can also be a part of a more generalized tubule defect, like in Fanconi syndrome. In recent years more and more gene mutations have been found which are associated with RTA (mutations in the gene SLC4A4, encoding a Na(+)-HCO(3)(-) cotransporter (NBC-1); in the gene SLC4A1, encoding Cl(-)/HCO3(-) exchanger (AE1); in the gene ATP6B1, encoding B1 subunit of H(+)-ATPase; in the gene CA2 encoding carbonic anhydrase II; and others) and allow better understanding of underlying processes of bicarbonate and H(+) transport. Isolated renal tubular acidosis can be frequently acquired due to use of certain drug groups, autoimmune disease or kidney transplantation. As the prevalence of acquired forms of RTA is common, new therapeutic options for the currently used supplementation of oral alkali, are awaited.