The Kidney Disease Outcome and Quality Initiative (KDOQI) Group recommended guidelines for the monitoring and treatment of chronic kidney disease (CKD) in 2002. These recommendations were based on the prevalence of known complications as seen in adults. In children, the exact prevalence of these complications is unknown. We therefore conducted a cross-sectional study of 366 patients with CKD in a single center to analyze the prevalence of these complications across all stages of kidney disease. Patients were categorized to their KDOQI stage of CKD according to their estimated renal function as determined from serum cystatin C. Fifty seven percent of patients had CKD stage 1, 29.0% stage 2, 10.4% stage 3 and 4.1% stages 4+5. Uropathies (31%) were the most prevalent causes of CKD. Glomerular disease accounted for 27%. The overall prevalence of complications was as follows: hypertension 70.2%, anemia 36.6%, proteinuria 11.5%, and metabolic bone disease 16.9%. Metabolic bone disease and anemia occurred frequently, even with a glomerular filtration rate >60 ml/min/1.73 m2. Growth failure (11.5%) was also common and is not a component of the KDOQI guidelines for CKD in children. The prevalence of all complications increased with worsening stage of kidney disease (all P-values significant). In summary, this study supports the KDOQI guidelines in defining and staging CKD in children. This study also highlights the differences in the causes and complications that occur in CKD between adults and pediatrics. We recommend modification of the KDOQI guidelines for children to reflect the differences described in this paper.

BACKGROUND:Reports on the worldwide increase in focal segmental glomerulosclerosis (FSGS) in childhood may have been hampered by referral bias. A true increase in FSGS possibly could alter the current practice of withholding renal biopsy in childhood nephrotic syndrome (NS) unless the patient fails to respond to a 28-day course of corticosteroid therapy.METHODS:With these questions in mind, we analyzed a 17-year database covering a 275,000-child population with mandatory referral. The incidence of NS per 100,000 childhood population per year was calculated, charts of 159 patients with NS seen between 1985 and 2002 were reviewed, and a receiver operating characteristic (ROC) plot analysis was performed to analyze the diagnostic performance of remission time.RESULTS:Results show that 115 of 159 patients had minimal change NS, diagnosed either on the basis of corticosteroid response (n = 89), verified by renal biopsy (n = 14), or with minimal change plus mesangial immunoglobulin M on histological examination (n = 12). The remaining 44 patients underwent a renal biopsy showing FSGS (n = 29; 18.2%), diffuse mesangial hypercellularity (n = 8; 5%), membranoproliferative glomerulonephritis (n = 1; 0.6%), membranous nephropathy (n = 3; 1.9%), or other diagnoses (n = 3). The incidence of FSGS increased significantly (P = 0.0253) from 0.37 to 0.94/100,000-child population/y in the two 8(1/2)-year intervals of our study. ROC plot analysis confirmed diagnostic sensitivity and specificity greater than 80% for remission time between 21 and 28 days of therapy.CONCLUSION:We confirm the increasing incidence of FSGS in children with idiopathic NS in a well-defined catchment area and, at the same time, find no reason to change the initial therapy and current indications to perform renal biopsy in childhood NS.

Rats made uremic by 2-stage 5/6 nephrectomy and sham-operated control animals were fed either a normal laboratory chow, a high-sucrose (60%) or a high-fat (10% cholesterol; 20% olive oil) diet, all containing 21% protein and identical amounts of electrolytes, vitamins and trace elements. Serum creatinine levels remained unchanged in the control animals but rose in the 5/6 nephrectomised uremic animals by a factor of 2.7 from a mean of 0.44 +/- 0.05 mg/dl to 1.20 +/- 0.11 mg/dl at 8 weeks, without differences between the dietary groups. During 8 weeks of dietary regimen the high-sucrose and high-fat diets induced significant hypertriglyceridemia, generally similar in control and uremic rats. The uremic animals on a high-sucrose and high-fat diet had the most pronounced rise in serum triglycerides, 331.5 +/- 89.0 and 298.0 +/- 45.0 mg/dl, respectively (control: 159.9 +/- 14.0 mg/dl). After 4 and 8 weeks, only the animals on the high-fat diet had significant hypercholesterolemia, most pronounced in the uremic animals (356 +/- 56.3 mg/dl; control: 71.6 +/- 12.9 mg/dl). The animals in the latter group also had significant proteinuria and renal histologic abnormalities consisting of xanthoma-like glomerular lesions, infiltrates and fibrosis not seen in the other groups of animals. These data indicate that dietary-induced hyperlipidemia of short duration causes or aggravates renal damage in the rat with mild-moderate uremia, induced by ablation.

The effect of 8 weeks of daily oral fish oil supplementation in a dose of 3 to 8 g/d on serum lipid levels was studied in 16 patients, 7 to 8 years of age, who had end-stage renal disease and were receiving renal replacement therapy. Fasting serum cholesterol (CHOL), triglyceride (TG) levels, and lipoprotein profiles were measured before therapy, 8 weeks after fish oil supplementation, and 4 weeks after its cessation. During 8 weeks of treatment the mean serum CHOL level did not change. The mean serum TG level, however, decreased significantly (P less than .01) from 236 +/- 31 mg/dL to 171 +/- 21 mg/dL (27.5%). Four weeks after treatment was stopped, the mean serum TG level returned to a value not significantly different from the pretreatment level (208 +/- 30 mg/dL). In a subgroup of 11 excessively hyperlipidemic patients, with serum CHOL and TG levels greater than or equal to 50% of the 90th percentile for age and sex, the mean serum TG level decreased even more (30.8%), from 286 +/- 35 mg/dL to 198 +/- 24 mg/dL (P less than .01), and the mean CHOL/high-density lipoprotein CHOL ratio decreased from 8.4 +/- 1.2 to 7.4 +/- 1.3 (P less than .05). Blood pressure and platelet counts remained stable during the entire study period. Side effects of the treatment were minimal. These results show that dietary fish oil supplementation reduces serum TG levels in young patients receiving renal replacement therapy and improves their "atherogenic" serum lipoprotein profile.

We studied plasma vitamin E levels in children/adolescents 3-19 years of age (10.9 +/- 4.5; mean +/- 1 SD) with chronic renal failure treated conservatively, on hemodialysis, on continuous ambulatory peritoneal dialysis and after renal transplantation. In all 4 groups of patients vitamin E levels (0.66-0.90 mg/dl) were within the normal range (0.76 +/- 0.19 mg/dl). The lowest levels of vitamin E (0.59 +/- 0.1 mg/dl) were found in nonuremic patients with a good functioning renal transplant. These data do not support the notion that uremia is accompanied by vitamin E malabsorption.

In the past we have shown that patients with chronic renal failure (CRF) on hemodialysis show evidence of intestinal malabsorption of fat. The present study was designed to verify this finding in an animal model. Male rats weighing +/- 200 g were studied. Uremia (U) was induced by 2-stage subtotal (5/6) nephrectomy. Control (C) animals were sham-operated. Fat absorption was studied after 6 weeks of uremia with the oral fat loading test. Twenty percent intralipid (0.25 g/100 gBW) was given by gastric tube feeding to fasting animals and the appearance of chylomicrons (CHYL) and the rise of triglycerides (TG) in the serum was followed for 5 hrs. In order to isolate the effect of fat absorption, an additional group of U and C animals was pretreated with orotic acid and triton, thus blocking hepatic TG synthesis and neutralizing peripheral lipoprotein lipase activity. The absorption of CHYL was significantly (p less than 0.01) impaired in all U animals and averaged 43 and 70 percent of that of the C animals, 1 and 2 hrs after the load respectively. The rise in serum TG did not differ from C in mildly U animals (Scr 1.0 +/- 0.04). In the more severely uremic animals (Scr 2.6 +/- 0.2), however, pretreated with orotic acid and triton, the rise in serum TG was far less (p less than 0.01) than in C animals (111 +/- 26-903 +/- 111 delta % V.780 +/- 170-5032 +/- 746 delta %) 1 and 5 hrs after the load.(ABSTRACT TRUNCATED AT 250 WORDS)

The outcome of acute poststreptococcal glomerulonephritis (APSGN) is still controversial. Whereas many studies show a good prognosis, particularly in children, late morphologic and functional renal abnormalities have been described. To verify this latter finding, we evaluated the sodium handling of the kidney several years after APSGN. Eleven subjects (9 children and adolescents and 2 adults) were studied 2 to 3 years after APSGN. At the time of the study all patients were well, without pathologic urinary findings and with normal blood pressures. Five healthy subjects (3 adults and 2 children) served as controls. Following two control periods of 30 min, an i.v. saline load (2.5% NaCl, 1 l/1.73 m2 body surface area) was administered over 45 min. Sodium excretion was measured during the control and salt-loading periods, and during four additional 30-min collection periods. No exaggerated natriuresis was found. We therefore conclude that epidemic APSGN in Israel carries not only an excellent clinical but also a good renal functional prognosis.

We report the case histories of 2 patients with a rapidly fatal form of acute lymphatic leukemia, who showed severe hypercalcemia during the acute phase of the disease. The pathogenetic mechanism of the hypercalcemia in these two cases was different. A short review of present day theories regarding the pathogenesis of tumor hypercalcemia is presented.

An 8-year-old girl sustained 65–70 per cent body surface area burns when her clothes caught fire. Two weeks later, she developed acute renal failure (ARF) and needed dialysis therapy. The extensive, infected burn area prevented the establishment of a safe vascular access for haemodialysis. Continuous peritoneal dialysis proved to be a valid alternative form of treatment and even had advantages over haemodialysis. Large amounts of fluids, calories and electrolytes could be administered via the peritoneal route. This facilitated the treatment of the catabolic state of the uraemic burn patient and served to correct the electrolyte losses via the skin. Peritoneal dialysis is a valuable adjunct to the treatment of acute renal failure in burn patients who need renal replacement therapy.

We performed computerized tomography (CT) of the brain in 22 patients, 2 to 18 years of age, with stable chronic renal failure (n = 6), on dialysis (n = 14) and after renal transplantation (n = 2). None suffered from a systemic disease known to affect the central nervous system (CNS) and none had overt CNS dysfunction at the time of the CT examination. The most striking pathological finding was brain atrophy, which was present in 13 patients (59%). In two patients cortical infarcts were present and one patient was found to have a hypodense area in the basal ganglia. The brain atrophy could not be related to the type of basic renal disease, the age of the patient, corticosteroid dosage, the duration of the renal failure or the presence of hypertension However, the mode of treatment, i.c. hemodialysis, seemed to be a risk factor. We assume that metabolic derangements and/or the accumulation of toxic substances due to the uremic state may be responsible for the brain atrophy in young patients with CRF. Recurrent osmotic changes of the brain during hemodialysis may aggravate the process of brain atrophy.

A 22-month-old child with familial infantile nephrotic syndrome due to diffuse mesangial sclerosis is presented. The case history of this patient is exceptional because of evidence of an immunologic pathogenesis of the renal disease, documented by characteristic immunofluorescent and electron-microscopy findings.

We report our experience with continuous, transcutaneous monitoring of capillary oxygen and carbon dioxide tension (PtcO2, PTlcCO2 ) during the entire length of hemodialysis treatment in 8 pediatric patients. The relative changes in PtcO2 and PtcCO2 observed with this method were in accordance with those reported in the literature, obtained with frequent arterial blood sampling. There was a temporary fall of PtcO2, averaging 26.2% after 2h of dialysis, with a concomitant rise of PtcCO2 of 9.9%. The noninvasive, transcutaneous measurement of PtcO2 and PtcCO2 is a reliable and valuable adjunct for the monitoring of the dialysis patient who is prone to develop hypoxemia. It is particularly useful in the very young.

An eight-year old girl with extensive burns and aminoglycoside-induced acute renal failure was treated with peritoneal dialysis. She developed chyloperitoneum after medical insertion of a Tenckhoff catheter. Conservative dietary treatment with medium-chain triglycerides, supplemented by parenteral hyperalimentation and temporary cessation of the peritoneal dialysis allowed the chylous fistula to heal, without the need for surgical intervention. Leakage of milky chyle into the peritoneal cavity (chyloperitoneum or chylous ascites) is a rare clinical entity. Generally it is due to trauma or obstruction of the lymphatic system. It has been observed in adults with intra-abdominal malignancies and/or inflammations and in children with congenital malformations of the abdominal lymphatics (I). Chyloperitoneum also has been seen after abdominal trauma, including surgery (1–5); chyloperitoneum has been mentioned before as a complication of peritoneal dialysis but no details have been provided (1,6). This paper will report the occurrence and treatment of chyloperitoneum in an eight-year-old child undergoing peritoneal dialysis for acute renal failure.

A sixteen day old infant developed candiduria after surgery on a single functioning, hydronephrotic kidney with ureteropelvic junction stenosis. Masses of candida albicans caused obstruction of the ureter with acute anuria. Endoscopic relief of the obstruction together with aggressive antifungal therapy led to irradication of the fungal infection. This case history emphasizes the fact that candida infection in early childhood should be evaluated carefully. Unfortunately no guidelines are yet available for the indications and the preferred mode of treatment, and length of therapy of infantile renal candidiasis.

Un garcon de 2 ans 1/2 est pique, a 5 km de Jerusalem, par un «scorpion noir» Androctonus crassicauda crassicauda. Survenue de symptomes d'envenimation generalisee. Traitement avec succes par un serum anti-scorpion jaune Leimus quinquestriatus. Confirmation chez la souris de l'efficacite du serum non homologue