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In France, chronic diseases affect 3 million children. In children with chronic conditions, long-term somatic outcome has been well described, but little is known about the psychosocial aspects of well-being.Our aim was to build a self-administered questionnaire of global well-being in adults who had a chronic disease since or during childhood using a multidimensional and nonspecific approach. The questionnaire was constructed by a multidisciplinary group (epidemiologists, clinicians, sociologist, statistician). Items were built in compliance with reference data from the French general population (national surveys, free access) to allow comparative analysis adjusted for age and sex (and eventually other confounding factors) by indirect standardization (qualitative variables) or Z-scores (quantitative variables).The GEDEPAC-2 includes 108 items exploring 11 domains: education, employment, housing, material security, social links, civic engagement, leisure, environment, physical health/risky behavior, health-related quality of life and sex life. Factual questions and satisfaction scales jointly explore social well-being. Quality of life is analyzed in terms of physical quality of life, mental quality of life, fatigue and burden of treatment by 3 questionnaires validated in French (SF-12; MFI-20; Burden of Treatment Questionnaire). Experience of transition from pediatric to adult healthcare is described in 21 items. Paper and electronic versions were developed.Built in a multidimensional approach to well-being and in line with the available reference data, GEDEPAC-2 will facilitate the implementation of future studies on impact in adulthood of chronic disease in childhood.

A retrospective study was undertaken of 120 children with systemic lupus erythematosus (SLE) seen in Paris and its immediate suburbs who fulfilled at least four of the American College of Rheumatology diagnostic criteria for SLE, and in whom the disease was diagnosed before the age of 16 and between January 1975 and December 1987. Eleven of these children (eight girls and three boys) all more than 10 years of age (mean follow up 8.1 years; range 3-13) had thrombotic episodes (9%). Thrombosis was one of the presenting signs in seven patients; in five it was associated with typical symptoms of SLE, and in the other two the thrombotic episode was isolated and diagnosis of SLE was delayed one and three years. Of a total of 16 thrombotic episodes (six of which were recurrent), 14 involved the leg veins, and in four there was associated pulmonary embolism. There were two episodes that affected cerebral arteries. The American College of Rheumatology diagnostic criteria for SLE as well as the incidence of lupus anticoagulant, positive direct Coombs test, and vasculitis in this group of patients was compared with the incidence in patients with SLE but no thrombosis. Only lupus anticoagulant was significantly associated with thrombotic episodes: eight of 11 (73%) of patients with SLE and thrombotic (arterial or venous) episodes had lupus anticoagulant compared with only 10 of 74 patients (14%) with no history of thrombotic events in the same age group.

PURPOSE: Whereas gastrointestinal symptoms such as vomiting, diarrhea and abdominal pain are common in children suffering from the so-called post-diarrheal form (D+) of hemolytic uremic syndrome (HUS), more serious gastrointestinal complications are rare. We tried to define factors predictive of the severity of gastrointestinal complications post D+ HUS. METHODS: We reviewed the files of all children admitted to our hospital for D+ HUS between 1988 and 2000. We retained those cases with gastrointestinal complications and analyzed the consequences of these complications on the evolution of the children's conditions. RESULTS: Sixty-five children with D+ HUS were admitted to our hospital during this period. Sixteen children developed gastrointestinal complications involving one or more digestive organs: necrosis of the colon or ileum, hemorrhagic colitis, pancreatitis, transient diabetes, hepatic cytolysis and cholestasis, peritonitis and prolapse of the rectum. One child died. CONCLUSION: Gastrointestinal complications of D+ HUS are rare, but they can be lethal, and early surgery may sometimes prove necessary. However, we were not able to demonstrate a correlation between the severity of the gastrointestinal manifestations and the clinical or biological signs accompanying D+ HUS.

Although systematic vitamin D supplementation in adolescents remains debated, rickets is nevertheless a well recognized pathology in this age group. Adolescence is an at-risk period because of rapid growth, insufficient calcium intake and/or vitamin D status. Surveys have shown that calcium intake is insufficient (1000 mg a day) in 45% of boys and 71% of girls and that vitamin D status is deficient (25-OH-D10 ng/ml). The aims of the study carried out by the Calcium Group of the Société Française de Pédiatrie, were to evaluate the frequency of rickets, and to define the criteria for the adolescent population at risk. Forty-one adolescents with rickets were hospitalized between 1985 and 2000. Most of the cases were from the Northern France: 20 from Paris and suburbs, eight from the North-West, four from the North, four from the North-East; five were from the Center of France. The mean age was 13 years and two months for the 28 girls, and 14 years and four months for the 13 boys. Eighty per cent of the adolescents were from immigrant families (33/41): 15 were from sub-Saharan Africa, ten from North Africa, six from Pakistan and two from Turkey. Two thirds of the adolescents were hospitalized in the 2nd quarter of the year. Some adolescents suffered from lower limb pain, 16 had deformations of lower limbs, particularly genu valgum, associated with pain; seven others had either muscle spasms (4), tetany (3). Serum calcium level was low (average 1.84 mmol/l: [1.1-2.5]), and serum 25-OH D level was extremely low. Radiographic characteristics observed were metaphyseal strips on the knees, with condensed edges at times, with the presence of bone demineralization. The treatment combined calcium and vitamin D, and was often administered intravenously when a hypocalcemia was detected. Rickets is not frequent in adolescents, but nonetheless this pathology is not exceptional, and the number of cases is probably under-estimated. Rickets affects immigrant adolescents in particular but nevertheless could also present a certain risk period for the general population.

The diagnosis of chronic renal insufficiency (CRI) must be evoked in children with feeding difficulties, excessive thirst, insufficient weight and/or height gain. Complications of CRI in children are hydroelectrolytic desequilibrium (chronic dehydration, sodium depletion, acidosis), nutritional difficulties, osteodystrophy, growth retardation and psychologic and scolar difficulties. Human recombinant growth hormone treatment generally allows spectacular growth improvement. In children with end-stage renal failure, operation is performed by hemodialysis or, especially in children less than 2 years of age, peritoneal dialysis. Renal transplantation is the best treatment of end-stage renal failure in children, as it offers them a practically normal life, with the only obligation of absorbing medications every day.

To define prospectively the incidence of renal parenchymal lesions in the siblings of patients treated at one institution for primary vesico-ureteric reflux (VUR).From January 1997 to October 1998, a prospective study including renal scintigraphy (using dimercaptosuccinic acid, DMSA) and a radionuclide cystogram was proposed systematically to the asymptomatic siblings of children treated for primary VUR. The radionuclide cystograms were interpreted as showing the presence or absence of VUR and the DMSA scan as symmetrical or asymmetrical differential function, with or with no renal defect.Fifty-five families gave informed consent, of whom 46 completed the study (eight refused secondarily and one was omitted by exclusion criteria), representing 46 symptomatic patients and 65 siblings. There were 17 siblings with VUR (26%) including two of 13 infants and 15 of 52 children aged18 months. One radionuclide cystogram failed. Of the 17 refluxing siblings, four had a history of symptomatic urinary tract infection; 62 of the 65 siblings had a DMSA scan, of which 56 were normal and six (10%) showed abnormalities (five asymmetrical differential function and one parenchymal defect). Only one of these six patients had VUR at the time of the evaluation and only one had a small kidney detected by ultrasonography on one side (and no VUR). There were no adverse effects associated with screening.This study confirms a significant overall incidence of VUR (26%) in the asymptomatic siblings of patients treated for primary VUR. From the results of the DMSA scan (only one sibling had a parenchymal defect), the systematic screening of asymptomatic siblings does not appear to be beneficial.

We conducted a study to determine the incidence of haemolytic uraemic syndrome (HUS) in children in France and to assess the role of Shiga-toxin-producing Escherichia coli (STEC) infection in the aetiology of HUS. In collaboration with the Société de Néphrologie Pédiatrique we undertook a retrospective review of all cases of HUS hospitalized from January 1993 to March 1995 and a 1-year prospective study (April 1995-March 1996) of epidemiological and microbiological features of cases of HUS. The polymerase chain reaction (PCR) procedure was used to detect stx, eae, e-hlyA genes directly from case stool samples. Serum samples from cases were examined for antibodies to lipopolysaccharide (LPS) of 26 major STEC serogroups. Two hundred and eighty-six cases were reported. The average incidence per year was 0.7/10(5) children < 15 years and 1.8/10(5) children < 5 years. During the prospective study, 122/130 cases were examined for evidence of STEC infection using PCR and/or serological assays and 105 (86%) had evidence of STEC infection. Serum antibodies to E. coli O157 LPS were detected in 79 (67%) cases tested. In conclusion, this study showed that STEC infection is an important cause of HUS in children in France, with a high proportion related to the O157 serogroup.

To evaluate the reliability of Doppler-ultrasonography in identifying children with renal artery stenosis among those with hypertension, we compared Doppler ultrasonography results in 29 hypertensive children (mean age: 8.3 +/- 4.7 years) with angiography. Doppler-ultrasonography and arteriography were performed within a period less than two months. First, we established normal values in 40 normotensive children (mean age: 8.4 +/- 4.5 years). The diagnosis of renal artery stenosis was settled when maximal systolic velocities whereor = to 1.70 m.s-1. Peak systolic velocities values of Doppler-ultrasonography were significantly higher in patients with proven angiographic renal artery stenosis (3.56 +/- 0.70 m.s-1) than in hypertensive patients with normal renal arteries at angiography (1.02 +/- 0.29 m.s-1, p0.0001), and than in normotensive healthy children (1.05 +/- 0.33 m.s-1, p0.0001). We observed 3 false negative and 2 false positive diagnoses with Doppler-ultrasonography. Of the 3 false negatives, one had a stenosis on a right and posterior segmental artery and the other had bilateral and multiply intra-renal artery stenosis with few hemodynamic significance. The 2 false positives were due to a sinuous main renal artery and to a technical mistake, respectively. In another patient, Doppler-ultrasonography revealed a tight main renal artery stenosis, not detected by arteriography. Renal artery stenosis was subsequently confirmed by a second arteriography. Our results showed a sensitivity of 88%, a specificity of 93%, a positive predictive value of 92% and a negative predictive value of 89%, demonstrating the reliability of Doppler-ultrasonography for the diagnosis of renal artery stenosis in hypertensive children. With the use of a rigorous methodology and the increasing experience of the operators, diagnostic errors of renal artery stenosis are currently avoidable. Nevertheless, it is not excluded that intra-renal artery stenosis with few hemodynamic significance might be missed by Doppler-ultrasonography.

Intravenous recombinant human erythropoietin (Eprex Cilag) was used in 28 hemodialyzed children, treated in 3 French paediatric centers, from November 1989 to November 1990. Transfusion dependency disappeared in all cases: the number of transfusions decreased from 7.3 unit/patient/year to 0.6 unit/pt/year. The mean haemoglobin concentration for the whole group increased from 6.6 +/- 0.8 g/dl, to 9.2 +/- 1.2 at 6 months and 9.7 +/- 0.7 g/dl at 1 year. Twenty-two out of 28 children reached the target haemoglobin value of 9.6 g/dl (6 mmol/dL) within a mean time of 16.5 weeks. Poor responses were due to either a premature withdrawal of treatment because of renal transplantation, or too low a dosage for the age. The study showed indeed that the dose requirement was significantly dependent on physical development: the mean dosage required to maintain haemoglobin concentration at the target value was 300 U/kg/week in children weighing less than 20 kg, 222 U/kg/week in 20-30 kg children, and 135 U/kg/week in those weighing more than 30 kg (p = 0.02). The only complication was an increase in blood pressure, observed in 43% of cases. The increase of anti-hypertensive medication was always successful in controlling blood pressure, and hospitalization was required in only one case. The improvement in general condition was obvious, and in several cases, the cognitive abilities seemed to improve. The growth deficit remained unchanged.

A 16-year-old boy suffers from headaches. A bilateral adrenal pheochromocytoma is diagnosed and removed. After surgery, the image of bone sequestrum has disappeared. Osseous microthrombi secondary to hemoconcentration are responsible for the image of button sequestrum.

Arterial hypertension (HT) is a common complication after renal transplantation. We evaluated ambulatory blood pressure profiles during 24 h in 30 pediatric patients (pts) aged 6-22 years who had been transplanted 2 months to 7 years previously. Creatinine clearance was 14-121 (median 57) ml/min/1.73 m2. Twenty-three pts were being treated with ciclosporin. Sixteen pts received antihypertensive drugs. Using the monitor Nippon Collins 630 (Baxter), blood-pressure (BP) values were taken every 20 min during the day and overnight (10 p.m.-7 a.m.). Five out of 10 pts with elevated office BP readings were normotensive by ambulatory blood pressure monitoring (ABPM). Ambulatory hypertension was discovered in one child with normal office BP. Echocardiography was performed in 23 pts. Five of six pts with significant left-ventricular hypertrophy (LVH), but none of the 17 pts without LVH had ambulatory HT (p less than 0.01). The physiological decline of BP during the night was significantly reduced when compared to 21 subjects of similar age with essential HT (-9.2% vs. -15.4%; p less than 0.02); no correlation was found with renal function or prednisone dose. Ciclosporin tended to reduce the day-night gradient (-14.5% vs. -9.2%; p = 0.11). One child showed a severe nocturnal BP rise of 25 mmHg. We conclude that abnormalities of nyctohemeral BP rhythm, as described in transplanted adults, can be observed to a lesser extent in children. ABPM allows a better evaluation of BP compared to office BP and, thus, may contribute to a better management of these patients.

This study was undertaken to evaluate the reliability of ambulatory blood pressure monitoring (ABPM) in children and its diagnostic value in teenagers with borderline hypertension. The apparatus used was a Nippon Colin (NC) ABPM 630 which simultaneously obtains BP levels by the auscultatory and oscillometric methods. Among 61 children (mean age 12 +/- 3 years), proportion of measurement failures was 14% (470/3399) for oscillometry, 26.5% (893/3360) for auscultation, and 2.7% for both methods together. Comparison of conventional auscultatory blood pressure measurements (mercury manometer) and simultaneous NC-auscultatory BP values (50 pairs) showed slightly lower diastolic blood pressure values with the NC monitor (-3.8 +/- 4.9 mmHg, p less than 0.01). Comparison of blood pressure values provided by the two NC methods (1580 pairs) demonstrated slightly higher systolic blood pressure values (+2.5 +/- 4 mmHg; p less than 0.001) and slightly lower diastolic blood pressure values (-5 +/- 4 mmHg; p less than 0.001) with the oscillometric method. Thirty-four ambulatory blood pressure recordings were obtained in 28 children (mean age 13.3: range 8-17) with outpatient clinic blood pressure values suggestive of borderline hypertension. Numbers of ambulatory blood pressure readings were 44 +/- 5 during the day and 9 +/- 1 during the night. The mean of daytime blood pressure values was compared with reference blood pressure values obtained at rest in normal French children.(ABSTRACT TRUNCATED AT 250 WORDS)

Metaphyseal sclerotic bone changes associated with benign phaeochromocytoma are very rare in childhood. We report four cases, in each of which the radiographic changes returned to normal after removal of the tumour.

Recent studies have suggested that patients with neonatal onset of severe renal failure may be at risk for mental retardation. We studied the intellectual development of 13 pediatric patients with neonatal onset of severe renal failure who immediately received active medical therapy. The verbal, performance, and overall intelligence quotient (IQ) was determined using the Weschler-PPSI and the WISC-R at a mean age of 7 years and 4 months (4 years 5 months to 15 years 8 months). Mean overall IQ was 89 (73 to 106), mean verbal IQ was 94.7 (79 to 124) and mean performance IQ was 85.6 (69 to 112). Overall IQ was greater than 100 (101 to 106) in three patients (23%), and under 100 in ten patients (77%), with values of 87 to 92 in six cases (46%) and 73 to 84 in four cases (31%). A significant negative correlation (p less than 0.05) was found between the IQ and the length of hospital or medical institution stays during the first year of life. We found no significant correlation either between IQ and severity of renal failure, quality of growth or nutritional status before the age of two, or between IQ and dose of aluminum ingested. Our results suggest that early severe renal failure does not induce severe mental retardation. However, in 77% of studied children the IQ was within the lower portion of the normal range or under this normal range. This proportion is greater than that seen in normal children from similar socioeconomic backgrounds. Reducing hospital stays and separations from home, as well as even more active nutritional management, may help to improve these children's mental abilities.

Linear growth and bone maturation have been studied in 17 children treated with regular hemodialysis for one to three years. Linear growth was normal in four children and retarded in 13. The adolescent spurt when present was markedly decreased. Statural and skeletal development were parallel except in: (1) children under eight years of age, (2) pubertal patients, and (3) those with severe osteodystrophy. In these instances, linear growth was impaired in relation to bone maturation. Three factors involved in growth retardation have been documented in this series: insufficient purification, low caloric intake, and severe osteodystrophy.

The main coagulation and fibrinolysis proteins have been studied in 22 children with nephrotic syndrome. Some anomalies such as the increase of fibrinogen and of Factors II, V, VII + X concentrations and the decrease of antithrombin III concentration could induce an hypercoagulability. Other anomalies such as the high alpha 2 macroglobulin concentration and the frequently low plasminogen concentrations could induce a decrease of the plasma fibrinolytic activity. The consequences of the low alpha 1 antitrypsin levels are probably less important. The fast alpha 2 antiplasmin levels have been found variable in the different patients. Practically, it appears that most children with plasma albumin levels less than 15-17 g/l have antithrombin III levels less than 23 mg/dl (70% of normal), which appears to result in a high risk of thrombosis (11).

Mechlorethamine was administered at a low dose (0.8 mg/kg) to 27 children presenting with steroid dependent or partially responsive nephrotic syndrome, with signs of steroid toxicity. This drug induced a fast decrease of proteinuria (average delay: 7 days). It led to long lasting remission (average follow-up 34 months) in 16 cases (59%). Relapse occurred in 11 children (41%) most often (9 of 11 cases) in the first 7 months. However the evolutive pattern was clearly improved in 5 of these cases. Altogether, mechlorethamine allowed to stop corticosteroid therapy or, at least, to reduce the given dose, with a decrease of the signs of steroid toxicity, in 78% of the cases. In 6 cases (22%) evolution was almost not improved. One may hope that this dosage of mechlorethamine will not be gonadotoxic. This should be checked later on.

Our study concerned 147 children with chronic renal failure (CRF) (creatinine clearance less than 50 ml/min/1.73 m2). Its goal was to analyse the distribution of primary renal diseases, natural history of renal failure (RF) according to etiology, and long term survival. Renal diseases responsible for RF were: malformations of the urinary tract (38%), glomerular pathology (26%), hereditary renal diseases (20%), isolated renal hypoplasias (11%), and miscellaneous (5%). Corticoresistant nephrosis accounted for 34% of glomerular diseases and nephronophtisis 63% of hereditary renal diseases. On the whole, RF was related with an uropathy or renal hypoplasia in half of cases and with congenital renal disease in almost 3/4 of cases. The natural history varied according to primary renal disease: slow deterioration after a period of relative stability for uropathy or renal hypoplasias, slow and regular deterioration for nephronophtisis, rapid deterioration for glomerular diseases.

The results of a controlled therapeutic trial comparing 2 groups of patients presenting with hemolytic-uremic syndrome (HUS) are reported. Group A (15 children) was given urokinase (UK) and heparin; group B (18 children) received no treatment. Ages of patients, the delay before admission, the severity of anemia, thrombocytopenia and initial renal failure were similar in both groups. UK was responsible for bleedings in 12 children, minimal in 8, severe in 4. No child died in group A, 3 children died in group B (n.s.). Durations of hemolysis, thrombocytopenia and anuria were similar in both groups. Long-term evolutions of renal function and arterial pressure were comparable in both groups. Needle kidney biopsy (26 cases) showed cortical necrosis in 3 children of group A and in 2 of group B, and glomerular thrombotic microangiopathy in 10 children of group A and in 11 of group B. The average ratio of injured glomeruli was 40 (19 to 80) in group A, and 38 (21 to 75) in group B. Two children in group A and 3 children in group B presented with 50 to 80% of glomerular lesions. This trial suggests that UK is of no significant value in the treatment of HUS.

The effect of extractive haemodialysis and of postural changes on the plasma aldosterone concentration has been studied in 5 anephric children. The plasma aldosterone concentration tended to be lower after haemodialysis in spite of the extracellular volume depletion induced. No regular response occurred after orthostatism. There was no correlation between the plasma aldosterone and the plasma cortisol or the serum sodium concentration. A weak positive correlation was found between the plasma aldosterone and serum potassium concentration, suggesting that potassium may play a major role in aldosterone regulation in the anephric state.

Loirat, C., Buriot, D., Peltier, A. P., Berche, P., Aujard, Y., Griscelli, C. and Mathieu, H. (Clinique de Pediatre, Hopital Bretonneau and U 120 INSERM; U 18 INSERM, Centre Andre Lichtwitz, Hopital Lariboisiere; Unite d'Immunologie et d'Hematologie and Laboratoire Central de Bacterologie, Hopital des Enfants Malades, Paris, France). Fulminant meningococcemia in a child with hereditary deficiency of the seventh component of complement and proteinuria. Acta Paediatr Scand, 69:553, 1980.—A previously healthy 14-year-old boy presented with fulminant meningococcemia. He was found to have a total deficiency of C7. His serum totally lacked bactericidal activity against Neisseria meningitidis. Addition of purified C7 restored the serum hemolytic and bactericidal activity. Susceptibility to disseminated Neisseria infections has previously been reported in 3 patients with C7 deficiency, as well as in a few patients with deficiency of C5, C6 and C8. These findings emphasize the importance of intact complement mediated bactericidal activity in host defense against disseminated Neisseria infections. Evaluation of the complement system in individuals with Neisseria infections appears mandatory.

The measurement of the plasmatic level of C3 may easily be included in the record of all glomerular nephropathies. Its decrease is an important argument for the diagnosis of post-infectious acute glomerulonephritis, or renal involvement in sepsis supervening to a ventriculo-atrial diversion. Variations of C3 level are of major importance in the management of the nephropathies in systemic lupus. Besides these special etiological circumstances, a persisting low level of C3 most often cooresponds to a membrano-proliferative glomerulonephritis especially of the type characterized by intra-membranous dense deposits.

We studied glomerulonephritis in a child with a deficiency of the second component of complement (C2) who was without clinical or serologic evidence of systemic disease. The clinical course was severe, with malignant hypertension and terminal renal failure when the child was 14 years old. Results of histologic studies were typical of membranoproliferative glomerulonephritis with subendothelial deposits. Immunofluorescence microscopy showed diffuse and intense localization of IgG, C1q, and C4 as granular deposits along the glomerular capillary walls and within the mesanguim. Diffuse deposits of C3 were also found along the capillary walls. Nine months after transplantation, the graft biopsy specimen showed glomerular lesions with IgG, C1q, C4, and C3 deposits, which suggests the possibility of a recurrence. The analysis of the previously reported cases of glomerulonephritis with C2 deficiency showed variable, but generally mild, glomerular lesions. Progression of the glomerulonephritis to severe renal insufficiency, as in the present case, is exceptional.

Seventy-nine children with haemolytic uraemic syndrome (mean age 28 months) were randomly assigned either to a group receiving plasma infusions (plasma group, n = 39) or to a group treated conservatively (control group, n = 40). The duration of haemolysis, thrombocytopenia and anuria was similar in the two groups. Serum creatinine levels were similar in the two groups at the 1-month follow-up but were higher in the control group at 3 months (plasma group 49 +/- 14, control group 66 +/- 28 mumol/l; P less than 0.02) and at 6 months (plasma group 48 +/- 13, control group 63 +/- 21 mumol/l; P less than 0.005). The prevalence of proteinuria was also higher in the control group at the 6-month follow-up (plasma group 17%, control group 46%; P less than 0.02). However, differences were no longer significant after 1 year. Renal tissue was examined in 54 cases (plasma group, n = 27; control group, n = 27). Diffuse cortical necrosis was present in 7 cases in the control group but was absent in the plasma group (P less than 0.02). Taking into consideration the higher serum creatinine levels, the higher prevalence of proteinuria during the first 6 months of follow-up in the control group and the presence of diffuse cortical necrosis in this group compared with the plasma group, we conclude that plasma infusions should be regarded as beneficial. Further study is needed to determine which plasma fraction is involved.