[Five cases of trichobezoars in children]. / Trichobézoard: à propos de 5 observations.

Trichobezoars are made up of concretions of ingested hair and food. A history of occlusive syndrome in a context of trichotillomania and psychological problems must lead to this diagnosis. Bezoars can be fortuitously recognised by palpation of an epigastric abdominal mass while investigating anemia or esophageal reflux. This deviance is particularly dangerous. The first case of this series illustrates the Rapunzel syndrome with many perforations and necrosis of the small bowel. The 4 others are strict intragastric bezoars, quickly identified by echography. Treatment is exclusively surgical, digestion by papain or endoscopic extraction being impossible. Psychological assistance is mandatory.

[Neurologic signs revealing a Behçet's disease: two pediatric case reports]. / Atteinte neurologique révélatrice d'une maladie de Behçet: deux observations pédiatriques.

UNLABELLED: Behçet's disease can be revealed by neurologic signs. CASE REPORTS: We report two pediatric cases of Behçet's disease which initially presented with cerebral venous thrombosis. Glucocorticoïds, associated with anticoagulant treatment allowed a rapid recovery. One of the children presented three years later a thrombotic recurrence. CONCLUSION: A cerebral venous thrombosis may reveal Behçet's disease.

Mycoplasma pneumoniae-associated nephritis in children.

Mycoplasma pneumoniae infection is a rare cause of acute nephritis. Six children (2 girls) aged 5-10 years, admitted for nephritis, had serological tests showing recent Mycoplasma pneumoniae infection. The diagnosis of Mycoplasma pneumoniae infection was based on the presence of serum IgM, detected either by immunofluorescence (IF) (n = 1) or enzyme-linked immunosorbent assay (n = 5). Four children had a renal biopsy, with analysis of parenchymal Mycoplasma pneumoniae components by indirect IF and polymerase chain reaction. Extrarenal symptoms were: respiratory (n = 3), ear, nose and throat (n = 2), gastrointestinal (n = 3), hepatic (n = 1), neurological (n = 1), articular (n = 1), and hematological (n = 3). The patients presented with acute nephritis (1 had a nephrotic syndrome) or with acute renal failure and proteinuria. Pathological findings included type 1 membranoproliferative glomerulonephritis (MPGN, n = 1), proliferative endocapillary glomerulonephritis (n = 2) and minimal change disease (n = 1). The patient with type 1 MPGN progressed rapidly towards end-stage renal failure because of a congenital solitary kidney. Among the patients with endocapillary glomerulonephritis, 1 relapsed 6 months later and remained proteinuric, while the other recovered, as did the child with minimal change disease. The search for Mycoplasma pneumoniae antigens and nucleic acids in renal tissue was negative. However, the absence of the microorganism in the kidney is a common feature of post-streptococcal glomerulonephritis. We conclude that Mycoplasma pneumoniae is a rare yet potential cause of acute glomerulonephritis.

Body composition in children with renal disease: use of dual energy X-ray absorptiometry.

Dual energy X-ray absorptiometry (DEXA) is a non-invasive accurate method which estimates bone mineral content and density (BMD), as well as fat (FM) and lean (LM) body mass. This method was used in control children in order to establish normal values for BMD of lumbar spine and whole body composition ¿logistic curves, general equation E = k+K/[1+ alpha exp(- beta A)]¿. In children with chronic renal failure (CRF), LM correlated with the urinary excretion of creatinine (r = 0.97, P = 0.0001) independently from glomerular filtration rate. However, the assessment of LM by DEXA must take into account the hydration level, since there is a positive correlation between fluid loss and reduction in LM in children on hemodialysis (r = 0.98, P = 0.0001). After renal transplantation, a significant loss of BMD (median -9.2%) was observed at 6 months which returned to 95% of pretransplant values by the end of the 1st year. Maximal changes in LM and FM occurred during the first 3 months (-7.8% and +7.2%, respectively) and may be due to steroids; these should be influenced by physical activity since FM correlated inversely with maximal oxygen consumption (r = 0.69, P = 0.0001). Recombinant growth hormone treatment could also increase LM and decrease FM, as shown in 9 patients. DEXA appears therefore to be a reliable method for evaluating therapeutic interventions affecting nutritional status in children with CRF.

Bone mineral density after renal transplantation in children.

Longitudinal bone mineral changes after renal transplantation were studied in 14 children aged 8 +/- 4 years. Combination immunosuppressive therapy was given to all patients (prednisone, azathioprine, cyclosporine). Bone mineral density (BMD) measurements of the first through fourth lumbar vertebrae by dual-energy X-ray absorptiometry were performed within 1 year preceding renal transplantation and 6, 12, and 24 months afterward (M0, M6, M12, and M24, respectively). The results of BMD obtained in grams of hydroxyapatite per square centimeter of spine projected area were subsequently transformed to standard deviation scores for a normal pediatric population. In addition, we used a mathematical spine volume correction to give the results in grams per cubic centimeter. All patients had a well-functioning renal graft at M6, M12, and M24 and a normal serum creatinine level. Significant decreases in BMD, standard deviation score, and spine volume-corrected BMD were observed 6 months after renal transplantation (p < 0.05, p < 0.01, and p < 0.01 respectively); the median loss of BMD and spine volume-corrected BMD was 9.2% and 15.6% at M6, respectively, and the median serum parathyroid hormone level dropped from 125 to 34 pg/ml. Between M6 and M12, BMD increased significantly up to 95% (median) of pretransplantation values and reached 97.2% (median) at M24. Similar but less marked improvement was observed in spine volume-corrected BMD results, reaching 87.7% and 87.4% at M12 and M24, respectively. A negative correlation was found between the cumulative prednisone dose and BMD in grams per square centimeter at M6 (r2 = 0.603; p = 0.006), M12 (r2 = 0.532; p = 0.015), and M24 (r2 = 0.40; p = 0.014). There was no correlation between cumulative prednisone dose and spine volume-corrected BMD or standard deviation score. Mean 6-month cyclosporine levels did not correlate with any measure of BMD. We conclude that after renal transplantation children have a significant decrease of BMD during the first 6 months after the operation, despite normal graft function and growth improvement.

Outcome of cadaver kidney transplantation in small children.

Small children have often been reported to have poor outcome after kidney transplantation (KT). Recent reports from North America have shown that the use of living-related donors improves patient and graft survival. We report the experience in one centre of primary cadaveric KT using sequential immunosuppression in nine children aged 8-30 months and weighing 5.4-9.8 kg; donors were 0.7-12.3 years old. Four patients had pre-emptive KT and the other five were on peritoneal dialysis; the mean +/- SD waiting time was 2.0 +/- 2.4 months. Perioperative care has been published previously. The surgical approach was intraperitoneal if the aorta and vena cava were used (n = 7) and extraperitoneal for common iliac vessels anastomosis (n = 2); the duration of surgery was 3.5 +/- 0.9 h and the time for vascular anastomosis was 32 +/- 6 min. The recipients received ATG, azathioprine, prednisone and delayed administration of cyclosporin A. The patients were followed for 12-98 (median 41) months and showed good graft function (inulin clearance 63-100 ml/min/1.73 m2); only one child with recurrent haemolytic uraemic syndrome lost his graft three months post-transplantation and died after he had received a second graft. None of the recipients required post-transplant dialysis; arterial hypertension involved four children and was related to graft artery stenosis in two. Growth improved by 0.24 +/- 0.48 SD score of height per year.(ABSTRACT TRUNCATED AT 250 WORDS)

Recurrent nephrotic syndrome after transplantation: early treatment with plasmaphaeresis and cyclophosphamide.

Steroid-resistant nephrotic syndrome (NS) with focal glomerulosclerosis (FGS) and its recurrence after transplantation are mainly seen in children. The recurrence rate approximates 30% and the graft loss is about half this. Several therapeutic regimens have been proposed, giving conflicting results. In an attempt to remove a putative circulating factor and inhibit its production by lymphocytes, three patients with biopsy-proven FGS in the native kidney were included in a prospective uncontrolled trial using early plasmaphaeresis followed by substitutive immunoglobulins in association with methylprednisolone pulses and cyclophosphamide instead of azathioprine over a 2-month period. The patients were girls, aged 6.5, 13.3 and 15.8 years, who received a cadaveric transplant; concomitant immunosuppression included prednisone and cyclosporine A. All three patients exhibited early recurrence of the NS and were treated 5-10 days after the onset of proteinuria. Rapid and sustained remission was achieved in all patients within 12-24 days on therapy. One patient experienced a late acute but steroid-sensitive rejection episode; another suffered from septic ankle arthritis as a complication of reinforced immunosuppression. The latter girl had a second late recurrence of proteinuria that was controlled within 7 weeks. With a 18- to 27-month follow-up, all three patients have normal renal function, normal blood pressure and no proteinuria. We conclude that intensive therapy using plasmaphaeresis, steroid pulses and cyclophosphamide over a 2-month period can induce complete remission in children with early recurrence of NS after transplantation.

Familial infantile nephrotic syndrome with ocular abnormalities.

Two siblings born from consanguineous parents experienced infantile nephrotic syndrome with ocular and neurological abnormalities; the boy also had a micropenis; both patients died before age 1 year. An initial renal biopsy followed by a two-step binephrectomy allowed good histological assessment of disease progression in one patient. The progression of the lesions was characterized by mesangial involvement, then an extensive extracapillary proliferation and tubular dilatations with a high mitotic activity of the epithelium and nuclei of unequal size. The main features involved major ultrastructural changes of the glomerular basement membrane. These two patients may represent a new disease entity or a severe form of diffuse mesangial sclerosis, with autosomal recessive inheritance.

Measurement of bone mineral content of the lumbar spine by dual energy x-ray absorptiometry in normal children: correlations with growth parameters.

The bone mineral density (BMD) of the lumbar spine (L1-L4) was measured by dual energy x-ray absorptiometry (Hologic QDR 1000) in 135 healthy caucasian children, aged 1-15 yr, and values were correlated with age, height, weight, body surface, bone age, pubertal status, calcium intake, vitamin D supplementation, and serum bone gla protein. BMD increased with age in children of both sexes (r = 0.88; P less than 0.001) from 0.446 +/- 0.048 g/cm2 at 1 yr to 0.625 +/- 0.068 g/cm2 at 10 yr and 0.891 +/- 0.123 g/cm2 at 15 yr of age. The increase was steeper at the time of puberty, reaching values above 0.80 g/cm2 after puberty was achieved. There were no significant differences between boys and girls, except at the age of 12 yr when BMD was higher in girls than in boys (P = 0.007), probably because of the earlier onset of puberty in females. BMD was also highly correlated with height, weight, body surface, and bone age. BMD was not correlated with calcium intake when age was held constant, nor with vitamin D supplementation. Serum bone gla protein showed a steady increase during childhood, with peak values at 11-12 yr of age, and was weakly but significantly correlated with BMD (r = 0.27; P = 0.007). Because of low irradiation exposure, rapid scanning, and high precision, dual energy x-ray absorptiometry is a noninvasive method which is well adapted to the child. It should be helpful in the investigation and follow-up of children with diseases impairing bone metabolism.

[Omenn syndrome]. / Le syndrome d'Omenn.

Omenn's syndrome is a rare autosomal recessive disease characterized by the onset, from the first weeks of life, of an exsudative skin rash, alopecia, hepatosplenomegaly, diffuse lymph-nodes, diarrhea and increased susceptibility to infections with hypereosinophilia. The associated severe combined immunodeficiency (SCID) differs from the other SCID by the existence of lymphocytosis. The number of T lymphocytes is normal or elevated; they are sometimes immature; the distribution of their subsets (CD4/CD8) is variable. The B lymphocytes are quantitatively and functionally deficient. Pathological investigations reveal major lymphoid depletion with severe thymic hypoplasia associated with a proliferation of cells which have the whole immunohistochemical characteristics of Langerhans' cells but do not contain their specific granulations (Birbeck's granulations) on the ultrastructural examination. These cells are also found in the skin, lungs and liver. The outcome is usually fatal before 1 year of age. The pathogenesis of this disease is still discussed: it might be the result, in an immunodeficient child, either of a graft-versus-host reaction after materno-fetal transfusion of immunocompetent cells, or of an abnormal immunologic reaction after antigenic stimulation, or a deficit of molecules in lymphocyte ecotaxy.

[Familial infantile nephrotic syndrome with ocular abnormalities]. / Syndrome néphrotique infantile familial avec anomalies oculaires.

Two siblings born from consanguineous parents experienced infantile nephrotic syndrome with ocular and neurological abnormalities and a micropenis in the boy; both patients died before age 1. The opportunity to perform successively a renal biopsy and a two-step binephrectomy permitted a good histological follow-up. The lesions were characterized by mesangial involvement, followed by an extensive extracapillary proliferation and tubular dilatations with a high mitotic activity of the epithelium with anisocaryosis; the main features consisted in major ultrastructural changes of the glomerular basement membrane which were more significant and different from those previously described in the diffuse mesangial sclerosis. These 2 cases may either constitute a new entity or an extreme form of diffuse mesangial sclerosis, supported by recessive autosomal inheritance.