Essential fatty acid deficiency profile in patients with nephrotic-range proteinuria (original) (raw)
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Frontiers in Pediatrics
Steroid-sensitive nephrotic syndrome is an immunological disorder mediated by still poorly defined circulating factor(s) that target the podocyte and damage the filtration barrier. Fatty acids (FA) have several biological roles and, in particular, are strictly involved in cell to cell communication, inflammatory processes and regulation of lymphocyte pools. Studies of FAs during INS have been mainly focused on biochemical changes during the phase of proteinuria; while no information is available about FA profile in patients with idiopathic nephrotic syndrome (INS) on stable remission. Aim of this study is to assess differences in blood FA profile between pediatric patients with INS during the phase of stable remission. Blood fatty acid profile of 47 pediatric patients on stable remission and 47 matched healthy controls were evaluated with gas chromatography. Patients with INS on stable remission had significantly higher levels of PUFA and omega-6 than controls (40.17 vs. 37.91% and ...
Perinatally Imposed Essential Fatty Acid Deficiency Changes Renal Function of the Adult Rat
Food and Nutrition Sciences, 2014
This study was designed to investigate whether essential fatty acid deficiency early during development could change the content of phospholipids and cholesterol in whole membranes of the kidney and renal function at adult life. For this, female Wistar rats were maintained on a standard diet or on an essential fatty acid deficient diet (EFAD) from the age of 30 days, throughout the pregnancy, at age of 90 days and until the weaning, for evaluation of their offspring. Weanling rats were maintained on a standard diet until the age of 13 weeks. Systolic blood pressure (SBP), glomerular filtration rate (GFR), urinary sodium excretion (UNa + V), positive cells for angiotensin II (Ang II) and cholesterol and phospholipids in whole membranes of the kidney were evaluated. Cholesterol, total phospholipids and the relative content of classes of phospholipids were unaltered in the cortex and medullary kidney. SBP, GFR and UNa + V were also unaltered in the EFAD group. However, the number of positive cells for Ang II in the tubulointerstitial area of the renal cortex was higher in the EFAD group. Therefore, these findings indicated that although cholesterol and phospholipids were unaltered and urinary sodium excretion was unchanged, Ang II expression in the kidney was erroneously programmed and later hindering of renal function was not ruled out.
Urinary Organic Acids in Infant Malnutrition
Pediatric Research, 1998
The metabolic derangements in severe protein-energy malnutrition (PEM) are only partially known, due to the limitations of blood collection in these patients. Urinary excretion of organic acids was studied by gas chromatography-mass spectrometry in 39 infants with four types of PEM:1) upon hospital admission, as soon as eventual infections had been cleared, and salt and water deficits corrected, but before oral feeding was started; 2) after start of protein alimentation;3) on the day of discharge. All of the patients showed an increased excretion of various organic acids at some point of their hospital stay, regardless of the clinical type of PEM. In nearly half of the malnourished children, results were suggestive of blocks in the pathways of propionate (15.4% with increased methylmalonate and 25.6% with 2-methylcitrate), of fatty acid β-oxidation (30.8% with raised dicarboxylic acids with low or low normal 3-hydroxybutyrate), or of both pathways (12.8%). These abnormalities may have been caused by cofactor deficiencies (biotin, vitamin B 12 , riboflavin, carnitine, niacin). Dicarboxylic acids were excreted in high amounts since the initial sample, probably due to increased mobilization of fatty acids. Increased 2-methylcitrate and methylmalonate excretion was observed more frequently once patients started to be orally fed. The accumulation of potentially toxic acyl-CoA precursors of these compounds could contribute to the known clinical worsening of some malnourished infants after suddenly increased protein intake. Other less specific metabolites, such as 3-hydroxybutyrate, lactate, 4-hydroxyphenyllactate, fumarate, succinate, and 4-hydroxyphenylacetate, were also abnormally excreted in some patients. The analysis of urinary organic acids provides a new approach for the metabolic study of PEM and may have diagnostic and therapeutic implications.
Nutrition in Pediatric Nephrotic Syndrome
Journal of pediatric nephrology, 2017
Nephrotic Syndrome is a collection of symptoms due to glomerular damage, characterized by proteinuria ≥3.5g/day or a protein-creatinine ratio ≥2. From an etiological point of view, there are three forms of nephrotic syndrome, including congenital, primary, and secondary. The first sign of the disease is periorbital edema, especially in the morning. Diagnostic confirmation is done through evaluation of urine protein, serum electrolytes, BUN, Cr, Albumin, and cholesterol. The main treatment goals of nephrotic syndrome are decreasing proteinuria, preventing complications, and protecting the renal function via appropriate drugs and diet. The main objective of this study was to review diets required in nephrotic syndrome in children: Protein at a diose of 0.8 mg/kg/day is the most effective diet in nephrotic patients. Lowfat diets (calorie intake <30% and cholesterol ≤200mg/day) can improve hyperlipidemia. Salt and water intake should be restricted in the range of less than 2 gr/day and 1-1.5 liters/day, respectively. Nephrotic syndrome patients have iron, copper, zinc, and calcium deficiency due to increased urine protein excretion or concomitant metabolic disorders. Nephrotic syndrome, Diet, Pediatrics
Low Molecular Weight Proteinuria in Children with Distal Renal Tubular Acidosis
PRILOZI, 2018
Distal renal tubular acidosis (dRTA) (MIM #267300, #602722 and #179800) is a rare inherited tubulopathy characterized by the inability of the distal tubule to acidify the urine with consecutive systemic acidosis. The clinical features include polyuria, polydipsia, poor appetite, failure to thrive, short stature and rickets. Prominent biochemical features are hypokalemia, hypercalciuria and hypocitraturia. There are reports on patients who presented with unusual biochemical features such as low molecular proteinuria, hypophosphatemia, hypouricemia, generalized hyperaminioaciduria, hyperoxaluria and other making diagnostic confusion to the clinicians. In this work, we report on a series of 8 children with clinically, biochemically and genetically proven dRTA who present with low molecular proteinuria at the disease onset. With metabolic compensation of the disease, there was complete resolution of the low molecular weight protenuria and other proximal tubular abnormalities in all chil...
The Spectrum of Kidney Diseases in Children Associated with Low Molecular Weight Proteinuria
Open Access Macedonian Journal of Medical Sciences, 2018
BACKGROUND: Proteinuria, in addition to haematuria, is the most important laboratory parameter in patients with nephro-urological diseases. Low molecular weight proteinuria (LMWP) is of particular importance because some diseases genetic and tubulointerstitial are diagnosed based on its presence.AIM: The purpose of this study is to describe the clinical features, the course and outcome of pediatric patients with a renal disease associated with LMWP.MATERIAL AND METHODS: This retrospective observational study included 250 pediatric patients with various kidney diseases in which the type of proteinuria was defined by 4-20% gradient gel sodium dodecyl sulphate polyacrylamide gel (SDS-PAG) electrophoresis.RESULTS: Isolated LMWP was detected in 12% of patients, while mixed glomerulotubular proteinuria was detected in 18% of patients. It was detected in all patients with the Dent-1/2 disease, Lowe's syndrome and secondary Fanconi syndrome. Transient LMWP was also detected in a series ...
Pediatric Nephrotic Syndrome; Clinical Characteristics and Nutritional Status
Al-Mukhtar Journal of Sciences, 2020
Nephrotic syndrome (NS), is the most common chronic renal disorder in children, with multifactorial risk factors and complex etiology. Therefore, the aim of the present study was to determine the age and gender distribution and also determine the clinical characteristics and nutritional status of pediatric nephrotic syndrome. A total of 75 patients with nephrotic syndrome series were selected through semi-constructed questionnaires. The age of our subjects ranged between 1-18 years. Body weight and height were extracted from patient files or self-reported to calculate BMI percentile. Laboratory tests such as blood glucose, lipid profile vitamin D, and HbA1C were included. All samples were analyzed through either mean ±SEM or Chi-square for determining significant differences. The present study showed that 75 patients were diagnosed as nephrotic syndrome. The average age of patients was 9 years old and the age which showed significance was 6-10 years (p=0.04). In comparison to female...
Nutritional status and muscle amino acids in children with end-stage renal failure
Kidney International, 1992
Nutritional status and muscle amino acids in children with end-stage renal failure. Nutritional status, assessed by anthropometric and biochemical methods, and muscle water, protein and amino acid composition, were evaluated in a control group of 10 children with normal renal function who were undergoing elective surgery, and in 15 children with end-stage chronic renal failure. Samples of the rectus abdominis muscle were taken when surgery was performed in the control children and when a peritoneal catheter was implanted in the uremic children. Height and body weight were reduced in the uremic children compared to the controls but skinfold thickness, arm muscle circumference and serum proteins (total protein, albumin, transferrin, pseudocholinesterase) were essentially normal. The muscle contents of total, extracellular and intracellular water, and of alkali-soluble protein (ASP), DNA and the ASP-DNA ratio were not significantly different in uremic children from those in the controls. Plasma leucine, isoleucine, tyrosine, valine, and serine levels were significantly decreased, whereas plasma citrulline, 1-methylhistidine and 3-methylhistidine levels were increased. Muscle isoleucine and valine levels and the valine/glycine ratio were low in the uremic children. Our results demonstrate that children with chronic renal failure and growth retardation may maintain a satisfactory nutritional status but exhibit amino acid abnormalities typical of ure-There are numerous reports concerning abnormal plasma amino acid (AA) concentrations in patients with chronic renal failure [1-3]. The plasma concentrations do not necessarily reflect the intracellular concentrations, and the determination of free AA in muscle, which is the largest pool of free AA in the body, is of particular interest in the study of AA metabolism in uremia. In untreated adult patients with chronic renal failure (CRF) a typical muscle intracellular AA pattern has been described [4, 5]. Intracellular AA abnormalities similar to those in adult patients have also been reported in children with CRF [6, 7]. The role of nutritional factors and of uremia itself in these abnormalities is still not clear. The aim of this study was to investigate plasma and muscle AA concentrations in relation to anthropometric and biochemical nutritional parameters in children with end-stage renal failure.
Pediatric nephrology (Berlin, Germany), 2017
Differential diagnosis of prenatally detected hyperechogenic and enlarged kidneys can be challenging as there is a broad phenotypic overlap between several rare genetic and non-genetic disorders. Metabolic diseases are among the rarest underlying disorders, but they demand particular attention as their prognosis and postnatal management differ from those of other diseases. We report two cases of cystic, hyperechogenic and enlarged kidneys detected on prenatal ultrasound images, resulting in the suspected diagnosis of autosomal recessive polycystic kidney disease (ARPKD). Postnatal clinical course and work-up, however, revealed early, neonatal forms of disorders of fatty acid oxidation (DFAO) in both cases, namely, glutaric acidemia type II, based on identification of the novel, homozygous splice-site mutation c.1117-2A > G in the ETFDH gene, in one case and carnitine palmitoyltransferase II deficiency in the other case. Review of pre- and postnatal sonographic findings resulted i...