Initial presentation of childhood-onset systemic lupus erythematosus: a French multicenter study.

OBJECTIVE: To describe the clinical and laboratory manifestations of childhood-onset systemic lupus erythematosus (SLE) at presentation. STUDY DESIGN: This retrospective French multicenter study involved 155 patients in whom SLE developed before the age of 16 years. Mean patient age at onset was 11.5 +/- 2.5 years (range, 1.5-16 years). The female to male ratio was 4.5. RESULTS: The most common initial manifestations were hematologic (72%), cutaneous (70%), musculoskeletal (64%), renal (50%), and fever (58%). Thirty-two percent of children had atypical symptoms, mainly including abdominal involvement in 26 patients, which lead to negative laparotomy results for presumed appendicitis. Severe renal, neurologic, hematologic, abdominal, cardiac, pulmonary, thrombotic, and/or cutaneous manifestations occurred within the first month after the diagnosis in 40% of patients. The mean erythrocyte sedimentation rate was 72 +/- 29 mm/h, and the mean C-reactive protein value 22 +/- 21 mg/L. Antinuclear antibodies an, anti-double stranded DNA antibodies, and low C3 or C4 level were retrieved in 97%, 93%, and 78 % of patients, respectively. CONCLUSION: Initial manifestations of childhood-onset SLE are diverse and often severe. The diagnosis of SLE should be promptly considered in any febrile adolescent with unexplained organ involvement, especially when associated with an increased erythrocyte sedimentation rate.

[Usefulness of procalcitonin for the diagnosis of acute pyelonephritis in children]. / Apport du dosage de la procalcitonine pour le diagnostic de pyélonéphrite aiguë de l'enfant.

BACKGROUND: Acute pyelonephritis can induce parenchymal scarring. The aim of this study was to evaluate the usefulness of procalcitonin (PCT) to predict renal involvement in febrile children with urinary tract infection (UTI). METHODS: In a prospective study serum PCT was measured and compared with others commonly used inflammatory markers in children admitted to the emergency unit with acute pyelonephritis. Renal parenchymal involvement was assessed by a (99 m)Tc-labeled dimercaptosuccinic acid (DMSA) renal scar performed in the first 3 days after the admission. RESULTS: Among 42 enrolled patients, 19 (45%) had acute renal involvement (Group A) ; 23 (55%) (Group B) had normal DMSA scan (n = 16), or old scarring (n = 4) or various anomalies related to uropathy (n = 3). In group A, the mean PCT level was significantly higher than in the group B (5.4 ng/ml, vs 0.4 ng /ml, p < 10(-5)). In these 2 groups, mean C reactive protein (CRP) levels were 99.1 mg/l and 44.6 mg/l respectively (p < 0.001). For a level of serum PCT > or = 0.5 ng/ml, the sensitivity and specificity to predict the renal involvement were 100% and 87% respectively; for a level> or= 20 mg/l CRP had a sensitivity of 94% but a specificity of 30%. CONCLUSION: Serum PCT levels were significantly increased in febrile children with UTI when acute renal parenchymal involvement was present. PCT seems a better marker than CRP for the prediction of patients at risk of renal lesions.

Renal function outcome in pediatric liver transplant recipients.

The orthotopic liver transplantation (OLT) allows survival of children followed for severe hepatic injury, provided that the immunosuppressive treatment is prolonged. The nephrotoxicity of cyclosporine predicts the long-term outcome of the adult patients receiving a liver transplant. The aim of this study was to determine the long-term outcome of renal function in children receiving OLT. This study included 12 children, with a median for age of 7.1 yr (2-15 yr) at the time of OLT. The duration of follow-up was at least 4 yr, being 7 yr in 10 patients and more than 10 yr in seven. Renal function was evaluated with the serum level of creatinine, calculated glomerular filtration rate (cGFR), and measurement of glomerular filtration rate using chrome 51 ethylenediaminetetraacetate ((51)Cr EDTA) clearance performed at least once during follow-up. The doses and the serum concentrations (C(0)) of cyclosporine were reported at each study time. The cGFR decreased significantly 2 yr after the OLT [median (range): 106 mL/min/1.73 m(2) (71-150) at the time of OLT vs. 85 mL/min/1.73 m(2) (57-128) 2 yr after the OLT, p = 0.03], and decreased again between 7 and 10 yr after OLT [median (range): 99 mL/min/1.73 m(2) (76-125) 7 yr after OLT vs. 81 mL/min/1.73 m(2) (66-140) 10 yr after OLT, p = 0.04]. Six patients developed chronic renal failure (cGFR from 57 to 80 mL/min/1.73 m(2)) 2 yr after OLT associated with high doses of cyclosporine [median (range): 8.8 mg/kg/day (3.5-13)]. The cGFR overestimated renal function by 16% compared with the isotopic measurement of GFR (p = 0.03). Using the (51)Cr EDTA measurement, six of seven patients followed up more than 10 yr after OLT presented mild (n = 3) or moderate (n = 3) chronic renal failure. In our study, the majority of OLT recipients developed a chronic renal failure 10 yr after transplantation. Cyclosporine seems to be the most important factor responsible for the impairment of renal function. The use of the mycophenolate mofetil, a new immunosuppressive agent, allowing a reduction in the dose of cyclosporine, could minimize renal dysfunction. While awaiting the results of a prospective long-term study, close drug monitoring is advised.

[Verocytotoxin positive haemolytic uremic syndrome and HIV-associated nephropathy]. / Syndrome hémolytique et urémique typique et néphropathie à VIH.

UNLABELLED: Haemolytic uremic syndrome (HUS) and HIV-associated nephropathy (HIVAN) are common renal diseases in the course of HIV-infected patients. CASE REPORT: We report the case of a 13-month-old Caucasian boy hospitalised for a verocytotoxin positive HUS associated with HIV infection. After the acute phase of HUS the creatinine level returned to normal values. Because of progressive renal failure with severe overload hypertension and glomerular proteinuria despite antiretroviral therapy and angiotensine converting enzyme inhibitor, the child required peritoneal dialysis 12 months later. Clinical and biological course together with pathological findings were consistent with both typical HUS and HIVAN. CONCLUSION: This is the first paediatric case of typical HUS revealing a HIVAN. The association of HUS and HIVAN may explain the progression to end-stage renal failure despite antiretroviral therapy associated with angiotensine converting enzyme inhibitor and a good control of HIV replication. HIVAN is rare in children and may occur in the early phase of HIV infection even not only in black patients.

[Power Doppler sonography and acute pyelonephritis in children: comparison with Tc-DMSA scintigraphy]. / Echographie doppler énergie et pyélonéphrite aiguë de l'enfant: comparaison avec la scintigraphie au DMSA-Tc.

UNLABELLED: Acute pyelonephritis is a common infection in children. The clinical and biological diagnosis is still sometimes difficult. For most authors, Technecium 99m dimercaptosuccinic acid scintigraphy is considered as the gold standard tool for diagnosis but it is invasive and expensive. The aim of our study was to compare the sensitivity and the specificity of B-mode sonography and power doppler to DMSA-Tc scintigraphy in acute pyelonephritis. PATIENTS AND METHODS: Forty-nine children were enrolled in this study with suspicion of pyelonephritis. All infants underwent doppler sonography and scintigraphy within 48 hours after their hospitalization. Doppler sonography criteria were increased kidney size, thickness of sinus wall, vascular defect, and various echogenicity of the kidneys (focal or diffuse hyperechogenicity or focal hypoechogenicity). RESULTS: Among 28 children with a positive scintigraphy, 15 had a positive doppler sonography (sensitivity 54%) and 13 had a negative doppler sonography. Among 21 children with a negative scintigraphy, 20 had a negative doppler sonography (specificity 95%) and one had a positive doppler sonography. CONCLUSION: In clinically suspected acute pyelonephritis, doppler sonography has a high specificity. A positive doppler sonography should avoid the use of scintigraphy.

Percutaneous angioplasty of arteriovenous (Brescia-Cimino) fistulae in children.

The development of a stenosis in a Brescia-Cimino fistula is a major clinical problem that threatens vascular access for dialysis. We reviewed the case notes of 46 children undergoing hemodialysis via Brescia-Cimino fistulae. Ten children (mean age 12.5 years) developed 14 stenoses located in the venous (10), anastomotic (3), or arterial (1) part of the fistula. Three (1 arterial and 2 anastomotic stenoses) of the 14 stenoses were treated surgically; the remaining 11 (10 venous and 1 anastomotic stenoses) were treated by angioplasty. Seventeen angioplasty procedures were performed by the percutaneous venous route under local anesthesia. Mean follow-up was 24 months. Restenosis within 6 months occurred in 5 patients, predominantly those who had angioplasty with low balloon inflation pressures; 1 was treated surgically; 4 underwent repeat angioplasty using higher balloon inflation pressures (3 patients) or a bigger balloon (1 patient). None subsequently developed restenosis. Angioplasty can be safely used to treat stenosis of arteriovenous fistulae, with a high initial (60% freedom from restenosis at 6 months) success rate. In summary, balloon angioplasty, repeated if necessary, is a safe and effective treatment for the majority of stenoses occurring in Brescia-Cimino fistulae. Restenosis can be safely treated by further angioplasty, which is associated with a high rate of ultimate clinical success.

1H-NMR analysis of trimethylamine in urine for the diagnosis of fish-odour syndrome.

This paper reports the use of proton NMR spectroscopy for the analysis of trimethylamine in the urine of a patient with trimethylaminuria. Analysis of this compound was also performed for other members of his family. Qualitative and quantitative determination of trimethylamine and trimethylamine-N-oxide was simultaneously performed on untreated urine within a few minutes. The application of the method is discussed.

Phosphate end dialysis value: a misleading parameter of hemodialysis efficiency. French Society for Pediatric Nephrology.

Despite low end dialysis serum phosphate levels (Pe) the control of phosphate retention remains often unsatisfactory in dialyzed patients. In order to assess the value of Pe in dialyzed children as an indicator of dialytic phosphate removal, we studied serum phosphate kinetics over the period of dialysis and post dialysis and compared these with urea kinetics. A multicenter study was conducted in the 21 French pediatric hemodialysis units and included 144 children under 15 years of age. Blood urea and phosphate concentrations were measured at the beginning, at 45 min later, at the end of dialysis, and 30 min post dialysis. At 60 min and at 360 min post dialysis measurements were made only for a subgroup of 12 children. From the serum levels, reduction ratios for urea (URR) and phosphate (PRR) and post dialysis rebound for urea (PDUR) and phosphate (PDPR) were calculated. URR (over the dialysis session, 72% +/- 9%) was higher than PRR (47% +/- 12%). Moreover, urea removal continued throughout the dialysis period, while most of the reduction in phosphate occurred in the initial dialysis period. Post dialysis urea rebound was limited to the 60th min post dialysis, whereas post dialysis phosphate rebound occurred until the 360th min post dialysis; by this time the serum phosphate levels had almost reached the predialysis levels. In summary, serum phosphate kinetics over dialysis and post dialysis periods in children appear to be misleading for the quantification of phosphate removal, i.e., phosphate clearance is a poor indicator of dialytic phosphate removal.

Risk factors for chronic rejection in pediatric renal allograft recipients.

To determine the risk factors predictive of graft loss from chronic rejection in pediatric renal allograft recipients, we reviewed the collaborative study database of the Société de Néphrologie Pédiatrique which registered 314 grafts from January 1987 to December 1991. Of the 289 grafts analyzed, 71 failed during follow-up, chronic rejection being the most common cause of graft loss (35%). The clinical features of the chronic rejection group (n = 25) were compared with those of the group without failure (n = 218). The variables tested by monovariate analysis were cyclosporine dose at 1 year, donor type, donor and recipient age, and acute rejection episodes. The incidence of graft loss due to chronic rejection was 4% (4/109) in patients who had no acute rejection and 16% (21/134) in those with at least one acute rejection episode (P = 0.002). Donor age (< or = 5 years) was a risk factor for chronic rejection (P = 0.024). Recipient age and donor type were not significantly different between the chronic rejection group and the control group. Using time-dependent covariates, the risk factors were an acute rejection episode (P = 0.003) and low cyclosporine doses at 1 year (P = 0.02). We conclude that acute rejection and low cyclosporine doses in these pediatric patients were risk factors for graft loss due to chronic rejection.

Collagen type III glomerulopathy: a new type of hereditary nephropathy.

A new type of hereditary glomerulopathy was observed in ten children presenting with early and progressive glomerular symptoms, often associated with hypertension. Light microscopy showed a diffuse increase in the mesangial matrix and generalized widening of the capillary walls. Electron-microscopic examination of renal tissue, after phosphotungstic acid treatment, revealed the presence of fibrillar collagen within the mesangial matrix and the subendothelial aspect of the glomerular basement membrane, adjacent to normal lamina densa. Immunohistochemical studies identified the fibrillar collagen not usually present within the glomerular extracellular matrix as type III collagen. Clinical and family studies ruled out the diagnosis of nail-patella syndrome, an autosomal dominant disorder with typical extrarenal symptoms, which is also characterized by the presence of fibrillar collagen within the glomerular basement membranes. The poor renal outcome, the possible extrarenal haematological and pulmonary involvement and the transmission as an autosomal recessive trait strongly suggest that collagen type III glomerulopathy is a new type of hereditary disease. From the high incidence of superimposed haemolytic uraemic syndrome in patients or their siblings, it may be hypothesized that collagen type III glomerulopathy is the underlying defect in some of the familial cases of haemolytic uraemic syndromes.

[Urological complications following kidney transplantation in childhood]. / Urologichni uslozhneniia sled bubrechna transplantatsiia v detskata vuzrast.

Five urologic complications in the posttransplantation period were performed in a case material of 68 renal transplantations in children, performed at the Transplantation Center in Lille, Northern France over the period 1981-July 1990. Three patients had ureteral stenosis (4.4 per cent), 1 urinary fistula (1.5 per cent) and 1 calculosis (1.5 per cent). All these cases are described and illustrated with figures. Comparing the results with those reported from other leading transplantation centers, the authors point to the low incidence of urologic complications in their case material (7.3 per cent). This encouraging result is attributed to the adequate selection of recipients, preliminary removal of accompanying urologic anomalies, early transplantation (82 per cent before the end of the second year after initiation of hemodialysis), as well as to precise explantation and transplantation.

[Blood flow measurement in arteriovenous fistula. Comparison of 2 ultrasonic methods, direct and indirect by compression of the fistula]. / Mesure du débit dans les fistules artério-veineuses. Comparaison de deux méthodes ultrasonores, directe et indirecte par compression de la fistule.

Measuring blood flow in arteriovenous fistulae in patients under chronic dialysis is of interest to evaluate the repercussions of the fistula on the heart. The apparently simplest method is direct measurement of the mean blood flow velocity by the pulsed doppler technique and ot the cross-section area by ultrasonography, the product of these two values being the blood flow rate. Another method has been proposed, which consists of measuring the cardiac output before and after compression of the fistula, the difference between the two values being supposed to represent the blood flow rate in the fistula. A comparative study of these two methods was conducted in 17 patients aged from 2 to 21 years (mean: 14 years). The direct method gave a figure of 475 ml.min-1.m-2 (SD = 240), while the figure obtained with the indirect method was 471 ml.min-1.m-2 (SD = 227); the difference was statistically not significant. In terms of concept, however, the indirect method is open to much more severe criticism than the direct method, and whenever possible the latter should be preferred when measuring blood flow in arteriovenous fistulae.

[Nail-patella syndrome without extra-renal lesions. A new hereditary glomerular nephropathy]. / Syndrome de "nail-patella" sans atteinte extrarénale. Une nouvelle néphropathie héréditaire glomérulaire.

We observed an unusual glomerular disease in eight pediatric patients. Clinical features of this early, progressive renal disease included increased blood pressure in many cases, extrarenal hematologic and pulmonary symptoms, and, in one of our patients and two genetically related children, hemolytic uremic syndrome with thrombotic microangiopathy resulting in permanent renal failure. Histologic studies showed major and complex modifications of glomerular capillary walls and electron microscopy disclosed numerous bundles of fibrillar collagen within the extracellular glomerular matrix. Clinical and genetic investigations out-ruled hereditary osteo-onychodysplasia, a condition in which fibrillar collagen is found within glomerular basement membranes. In addition results of family studies were consistent with autosomal recessive transmission of this new entity.

Evaluation by immunoblotting of the criteria for tubular lesions diagnostic with urinary SDS-PAGE.

Immunoblotting methods have allowed the identification of 14 urinary proteins. From this study, it appears that some low-relative-molecular mass proteins are derived from disrupted high-relative-molecular mass proteins. The only reliable bands for the diagnosis of tubular lesions, on the polyacrylamide gel stained with Coomassie Blue, are retinol-binding-protein and beta 2-microglobulin. alpha 1-microglobulin can overlap with breakdown products of albumin. The visualization of Ig light chains and lysozyme is rather poor after Coomassie Blue stainings and the accurate identification of these bands may be only improved by the use of immunoblotting.

Berger's disease in children. Natural history and outcome.

The clinical course and outcome of 91 children less than 15 years of age at onset and followed for at least 1 year have been retrospectively analyzed. The course has been characterized by recurrent macroscopic hematuria in 74 patients, by proteinuria-microscopic hematuria and a single episode of macroscopic hematuria occurring either at onset or a few months later in 8, by proteinuria-microscopic hematuria in 7, and by proteinuria only in 1. Lastly, one patient showed rapidly progressive renal failure. Four groups were identified by light microscopy: minimal glomerular changes (26), focal and segmental glomerulonephritis (41), pure mesangial proliferation (3) and proliferative glomerulonephritis with crescents (21). A good correlation was found between the glomerular lesions observed by light microscopy and the outcome. In this series we have not observed a dramatic clinical deterioration suggesting a transformation from one histologic type to another, as reported by others. None of the 70 patients belonging to the first three groups has impaired renal function but two with focal and segmental glomerulonephritis have developed hypertension. Although the clinical course is benign, many patients have, at the last observation, an abnormal urinalysis characterized by microscopic hematuria and/or mild proteinuria; the proteinuria is over 1 g/24 h in six patients with focal and segmental glomerulonephritis. Ten patients remained in clinical remission for several years, but mesangial IgA deposits were still present in the only patient who had a repeat biopsy while in remission. In contrast, none of the patients with proliferative glomerulonephritis with crescents has had a prolonged remission. Six patients developed terminal renal failure 0.7, 0.11, 2, 4, 8 and 10 years after onset. Two additional patients are in moderate chronic renal failure with hypertension 10 and 12 years after onset. Most children show a persistent nephropathy, (in five proteinuria is over 1 g/24 h), and two of them have developed hypertension. Therapeutic trials using drugs with side-effects should, therefore, be used only in this group of patients.

[Comparison of left ventricular function in children hemodialyzed with bicarbonate and acetate. An echocardiographic study]. / Comparaison intra-individuelle de la fonction ventriculaire gauche chez les enfants hémodialysés sur bicarbonate et acétate. Une étude échocardiographique.

Left ventricular function before and after hemodialysis with acetate (A) and hemodialysis with bicarbonate (B) was investigated in 8 children on regular dialysis treatment. The decrease in left ventricular volume overload is similar in A and B. There is a significant increase in shortening fraction (B: 9,7 +/- 16,1%; p less than 0,05. A: 7,8 +/- 10,9%; p less than 0,05), in velocity of circumferential fiber shortening (Vcf) (B: 30,6 +/- 16,5%; p less than 0,0005. A: 23,5 +/- 16,6%; p less than 0,0025) and in corrected Vcf (B: 19,3 +/- 16,4%; p less than 0,0025. A: 13,6 +/- 11,4%; p less than 0,005). The ratio of pre-ejection period with the left ventricular ejection time (PEP/LVET) is increased significatively only after hemodialysis with bicarbonate. This difference may be due to a better left ventricular function in hemodialysis with bicarbonate than with acetate.