Adherence to the Mediterranean Diet Improves Fatty Acids Profile in Pediatric Patients with Idiopathic Nephrotic Syndrome.

The fatty acid profiles of patients with idiopathic nephrotic syndrome (INS) are different from that of healthy controls, even during remission, revealing an increase of the pro-inflammatory omega 6 series. It is still unknown whether the concomitance of nephrotic syndrome affects the potential positive effects of the Mediterranean diet on the levels of omega 3 and 6 fatty acids. We performed a cross-sectional study to evaluate the association between the adherence to the Mediterranean diet and fatty acid profile in 54 children with INS. The dietary habits were assessed through the validated Kidmed questionnaire. Patients with higher adherence had lower levels of linoleic acid and total omega-6. Moreover, a negative correlation between proteinuria and the anti-inflammatory omega-3 series was found. In conclusion, patients with INS with proteinuria and low adherence to the Mediterranean diet have an imbalance in the omega-6/omega-3 ratio that may benefit from following the Mediterranean diet.

Round Table Discussion.

The 1st International Carnitine Working Group concluded with a round table discussion addressing several areas of relevance. These included the design of future studies that could increase the amount of evidence-based data about the role of carnitine in the treatment of fatty acid oxidation defects, for which substantial controversy still exists. There was general consensus that future trials on the effect of carnitine in disorders of fatty acid oxidation should be randomized, double-blinded, multicentered and minimally include the following diagnoses: medium-chain acyl coenzyme A (CoA) dehydrogenase deficiency, very long-chain acyl-CoA dehydrogenase deficiency, long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency and mitochondrial trifunctional protein deficiency. Another area that generated interest was trials of carnitine in cardiomyopathy and, especially, the use of biomarkers to identify patients at greater risk of cardiotoxicity following treatment with anthracyclines. The possibility that carnitine treatment may lead to improvements in autistic behaviors was also discussed, although the evidence is still not sufficient to make any firm conclusions in this regard. Preliminary data on carnitine levels in children and adolescents with primary hypertension, low birth weight and nephrotic syndrome was also presented. Lastly, the panelists stressed that there remains an objective need to harmonize the terminology used to describe carnitine deficiencies (e.g., primary, secondary and systemic deficiency).

Looking at appearance of urine before performing a renal biopsy in nephrotic syndrome.

Chyluria results from an abnormal connection between lymphatic bed and urinary tract, causing lymph leakage into the urine. The clinical picture often begins with the appearance of cloudy, milky urines accompanied by monolateral flank pain, malnutrition, weight loss and weakness. We report a case of chyluria that occurred in a young woman who was referred to our unit for nephrotic-range proteinuria. Before performing a renal biopsy, we found that urine analysis demonstrated a massive lipiduria. Therefore, we collected urine samples from each kidney with a selective ureteral catheterization, demonstrating a monolateral source of lipids and proteins. We suspended the renal biopsy and performed a lymphography that showed an inherited lymphangioma on the left lumbar lymphatic bed. Sclerosing solution instillation, renal pedicle lymphatic disconnection or laser therapy are invasive therapeutical options that may cause severe adverse effects. Instead of these procedures, a conservative therapy based on a low-fat diet supplemented with medium-chain triglycerides was chosen. This dietetic schedule was followed by complete resolution of proteinuria and lipiduria. The patient progressively gained body weight and improved quality of life. No relapses were observed after 3 years of follow-up. This case emphasizes the possible role of a noninvasive therapeutical option for patients with chyluria.

Renal effects of captopril, indomethacin and nifedipine in nephrotic patients after an oral protein load.

BACKGROUND: In this study we investigated whether the increase in proteinuria induced by an oral protein load may be prevented by the angiotensin-converting enzyme inhibitor (ACEI) captopril in patients with nephrotic syndrome, and whether the effects of captopril on renal haemodynamics and/or glomerular selectivity are comparable to those obtained with the nonsteroidal anti-inflammatory drug (NSAID) indomethacin and the calcium-channel blocker (CaCB) nifedipine. METHODS: Twelve subjects underwent the following treatments: (1) low-protein meal (0.2 g protein/kg body wt), (2) high-protein meal (1.3 g protein/kg body wt), (3) high-protein meal plus oral captopril (50 mg), (4) high-protein meal plus oral nifedipine (10 mg), (5) high-protein meal plus oral indomethacin (50 mg). Urine and blood samples were obtained after meals and tested for total protein, immunoglobulin G and albumin. GFR and renal plasma flow (RPF) were calculated from iothalamate and p-aminohippuric acid clearances respectively. RESULTS: Mean arterial pressure decreased significantly after both captopril (-4%, P = 0.001) and nifedipine (-5%, P = 0.0019). Compared with the low-protein meal, mean values of GFR and RPF increased significantly after the high-protein meal alone (+21%, P = 0.0002; +10%, P = 0.0491 respectively), and after captopril (+18%, P = 0.0025; +24%, P = 0.0034 respectively) or nifedipine administration (+30%, P = 0.0001; +21%, P = 0.0036 respectively), whereas they remained unchanged after the high-protein meal plus indomethacin administration. FF did not change significantly under the five experimental conditions. The increase in urinary protein excretion induced by the meat load (total protein +18%, P = 0.0102; albumin +26%, P = 0.0316; IgG +28%, P = 0.0203) was entirely blocked by both captopril and indomethacin, whereas it was further increased by nifedipine administration. CONCLUSIONS: Both captopril and indomethacin, but not nifedipine, are able to prevent the increase in urinary protein excretion rate following a meat meal. The antiproteinuric effect of captopril is comparable to that of indomethacin, but the renal haemodynamic changes induced by these drugs differ considerably, because the filtration capacity and the renal functional reserve were preserved by captopril and decreased by indomethacin. The reduction in systemic blood pressure following administration of both captopril and nifedipine does not account for changes in proteinuria, since, with a similar degree of blood pressure lowering, urinary protein excretion is reduced by captopril and increased by nifedipine.

Nutritional therapy for progressive renal failure.

Results of the Diabetes Control and Complications Trial indicate that intensive insulin treatment of patients with type I diabetes would greatly reduce the incidence of diabetic nephropathy. Another multicenter trial indicates that modest protein restriction is of no value in children with chronic renal failure. The relationship between urea nitrogen excretion and total nitrogen excretion in children differs from that in adults. A repeated crossover study found that ketoacids slow progression of renal failure, in comparison with amino acid supplements to the same diet. Long-term protein restriction does not lead to protein deficiency at the onset of dialysis. When combined with essential amino acid supplements, a low-protein diet may gradually correct hypoalbuminemia in nephrotic subjects.

Treatment of proteinuric patients with a vegetarian soy diet and fish oil.

Our aim was to determine whether a longer period of treatment with a vegetarian soy diet with addition of fish oil supplements would accentuate the beneficial effects on hyperlipidemia and proteinuria of nephrotic patients we found in a previous study. After an 8-week baseline period on free diet, patients were randomly allocated either on soy diet alone (SD) or to SD plus 5 g/day of fish oil (SD + FO) orally for two months. Then they crossed over to the other treatment for two additional months. They finally resumed eating the free diet for 3 months. We selected 20 outpatients with chronic glomerulonephritis, proteinuria in the nephrotic range, fasting serum cholesterol > 250 mg/dl, mean serum creatinine concentrations 1.75 +/- 0.23 mg/dl. Serum lipid profile, urinary protein loss and nutritional parameters were monitored. With the soy diet, we obtained a significant decrease both of hyperlipidemia and of proteinuria. The effect of the soy diet on proteinuria increased over the 4 months. The addition of a moderate amount (5 g/day) of fish oil in a randomized cross-over design had no further beneficial effect. Stability of serum albumin, transferrin and the body mass index documented good nutritional status. In conclusion, the dietary manipulation with our vegetarian soy diet confirmed the beneficial effects on hyperlipidemia and proteinuria of nephrotic patients. Such effects persisted and even ameliorated after 4 months of diet. The addition of moderate oral supplements of fish oil did not potentiate the beneficial effect.

Vegetarian diets for treating nephrotic syndrome.

Vegetarian diets based on soy-protein appeared to be effective in treating the hyperlipidemia of nephrotic syndrome. Whether there is a unique effect of soy or whether all very low-fat, low-saturated-fat, low-cholesterol, and low-protein diets have similar effects remains unknown.

Omega-3 fatty acid supplementation in primary nephrotic syndrome: effects on plasma lipids and coagulopathy.

The effect of fish oil dietary supplementation on the dyslipidemia and coagulopathy of seven patients with nephrotic syndrome and hypoalbuminemia due to primary kidney disease was studied. Plasma lipids, platelet aggregation studies, simplate bleeding time, and fibrinogen levels were determined before and after 6 wk of treatment with fish oil (15 g/day of MaxEPA; 2.7 g of eicosapentenoic acid (EPA) and 1.8 g of docosahexenoic acid. Urea kinetics were determined from urine-urea concentration, urinary proteina, and urine volume. A 3-day dietary intake record was obtained from each patient before and after 6 wk of fish oil supplementation. There was no significant dietary change in protein, fat, or carbohydrate intake over the time period of the study. At study end, total triglycerides decreased from 2.98 +/- 1.31 to 2.18 +/- 1.14 mmol/L (P = 0.002), and very low-density lipoprotein-triglycerides decreased from 2.35 +/- 1.34 to 1.28 +/- 1.07 mmol/L (P = 0.01). Low-density lipoprotein (LDL) cholesterol increased from 5.18 +/- 1.74 to 7.35 +/- 2.83 mmol/L (P = 0.005). No significant changes occurred in bleeding time, platelet count, hematocrit, red blood cell flexibility, or whole blood viscosity. Platelet aggregation responses to collagen and arachidonic acid were consistently reduced after treatment, but there was no change in platelet response to ADP. The platelet membrane phospolipids showed a significantly increased incorporation of EPA after the fish oil diet (P = 0.03).(ABSTRACT TRUNCATED AT 250 WORDS)

Low protein supplemented diet corrects altered serum thyroid hormone and TSH concentrations in patients with chronic nephrotic syndrome.

Creatinine clearance, daily urinary protein excretion, serum concentrations of thyroid hormones (TT4, TT3, fT4, fT3), TSH and parathyroid hormone (PTH), have been measured before and after 2 or 6 months of a low protein diet supplemented with aminoacids and ketoanalogues in 18 patients affected by chronic nephrotic syndrome without significant impairment of renal function. Mean creatinine clearance and mean serum protein concentration (79.5 +/- 13.8 ml/min and 5.4 +/- 0.6 g/dl, mean +/- S.D., respectively) did not significantly change (79.1 +/- 17.3 ml/min and 5.5 +/- 0.6 g/dl) after the diet. Mean daily urinary protein excretion (7.1 +/- 2.2 g/day basally) significantly decreased (5.5 +/- 1.9 g/day) after the diet (p less than 0.05). Mean serum TT4 concentration (5.6 +/- 1.8 micrograms/dl basally) significantly increased (6.7 +/- 2 micrograms/dl, p less than 0.05) after the diet. Mean serum TT3 concentration (106.7 +/- 28.5 ng/dl, basally) significantly increased (126.7 +/- 22.6 ng/dl) after the diet (p less than 0.01). Mean serum fT4 and fT3 concentrations (8.0 +/- 2.9 pg/ml and 4.5 +/- 1.6 pg/ml, respectively) did not significantly change (9.4 +/- 2.7 pg/ml, and 4.9 +/- 1.9 pg/ml, respectively) after the diet. In some patients low basal serum concentration values of TT4, TT3, fT4, fT3 became normal after the diet. Mean serum TSH concentration (3.1 +/- 2.3 microU/ml basally), significantly decreased (1.5 +/- 1.3 microU/ml) after the diet (p less than 0.05). Mean serum PTH concentration (0.7 +/- 0.3 ng/ml basally) significantly decreased (0.4 +/- 0.2 ng/ml) after the diet (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

[Idiopathic nephrotic syndrome and food hypersensitivity. Value of an exclusion diet]. / Syndrome néphrotique idiopathique et hypersensibilité alimentaire. Valeur des régimes d'éviction.

A case of idiopathic nephrotic syndrome (INS) evolving since more than 7 years with 4 successive corticosensitive relapses is reported. At the time of a 5th relapse corticosteroid treatment was refused by the parents and evolution went on without any spontaneous tendency to remission. Three months later, allergy tests detected various sensitizations, especially for food allergens. An antiallergic treatment including dietary exclusions induced a complete and durable remission. Five similar cases are reported. These facts, compared to data from the literature, indicate the possible role of food hypersensitivity in INS. Its mechanism has been discussed; the possible part of a lymphocyte activation has been emphasized.

[Phosphorus-calcium metabolism and dietetic correction of its disorders in chronic kidney diseases in children]. / Sostoianie fosforno-kal'tsievogo obmena i dieticheskaia korrektsiia ego narushenii pri khronicheskikh bolezniakh pochek u detei.

Ninety children with varying renal diseases were under observation. The investigations conducted have shown that disorders in phosphoric-calcium metabolism depend on the type, activity of chronic glomerulonephritis (CGN) and etiology of chronic renal insufficiency (CRI). Significant disorders in phosphoric-calcium metabolism were detected in patients with nephrotic and mixed types of CGN. Most manifest clinical and x-ray changes of the osseous system were observed in patients with CRI that developed as a result of the tubulointerstitial pathologic process. Low-phosphate diets with preset amounts of Ca and P were developed, composed of products with relatively low content of P, and of new dietetic products rich in Ca. The diets were used for correction of hyperphosphatemia in children with CGN and in those with CRI, simultaneously with drug therapy, to prevent or diminish disorders in phosphoric-calcium metabolism, and to reduce the risk of invalidism among children with chronic renal diseases.

Dietary fat in experimental nephrotic syndrome: beneficial effects of fish oil on serum lipids and, indirectly, on the kidney.

Three isocaloric diets with different fat composition were fed to rats for seven weeks after the production of nephrotic syndrome by adriamycin. The effects of feeding 3% and 14% fish oil were compared with those of feeding beef fat. At the fourth week of feeding the levels of triglycerides and cholesterol were lower in both fish oil fed groups. At the seventh week these levels, and the LDL cholesterol, were lower only in the 14% fish oil group. In rats fed beef fat, but not in those fed fish oil, there was a striking positive correlation of the four-week serum triglycerides and cholesterol with the seven-week serum creatinine level and with the degree of glomerular hyalinosis and endothelial swelling. The favorable effects of fish oil feeding on serum lipids may have a protective effect on the development of glomerular damage.