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Biomedical subjects

A Bensman

Publications and source records attributed to A Bensman.

At least 19 recordsLinked to original sources

Azathioprine-induced pulmonary haemorrhage in a child after renal transplantation.

We report a case of azathioprine-induced haemorrhagic alveolitis, in a 14-year-old boy, after renal transplantation. On day 25 the patient developed haemoptysis, fever and hypoxaemia. Chest X-ray showed diffuse reticulo-nodular shadows in both lung fields. Bronchoalveolar lavage samples were haemorrhagic and demonstrated a relative neutrophilia and a mild lymphocytosis, with a normal CD4/CD8 ratio. Azathioprine was discontinued on day 26. The patient required mechanical ventilation for 4 days. A positive leucocyte migration inhibition test and the recurrence of the symptoms after a second short course of azathioprine therapy suggested a cell-mediated mechanism. This patient is, to our knowledge, the first child to suffer from azathioprine-induced pulmonary haemorrhage.

Acute Disease

[Transient correction of partial congenital factor V deficiency in nephrotic syndrome].

Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.

Child, Preschool

[Acute urine retention: a rare mode of revelation of cervico-dorsal syringomyelia caused by cyproheptadine].

BACKGROUND: Syringomyelia is rare in children aged less than 10 years, and bladder dysfunction is an unlikely first manifestation. This report describes a case of repeated episodes of acute urinary retention in a young girl revealing syringomyelia and Arnold-Chiari malformation. CASE REPORT: A 2.5 year-old girl was admitted because she was suffering from acute urinary retention. Her poor appetite had been treated with cyproheptadine, a histamine type I blocking drug. Clinical investigation revealed no local cause for this bladder dysfunction except moderate spasticity of the legs. Cystography showed no vesicoureteral reflux. Because the episodes of urinary retention recurred each day, magnetic resonance imaging (MRI) was performed; this showed the typical features of syringomyelia extending from C5 to T11 plus Arnold-Chiari malformation. The cyproheptadine was discontinued and the urinary retention disappeared. CONCLUSION: Cyproheptadine may have revealed latent neurogenic bladder in this case, although urodynamic studies, performed 3 months later, detected no bladder dysfunction.

Acute Disease

[A rare cause of bone pain in children: primary hyperparathyroidism caused by adenoma].

BACKGROUND: Primary hyperparathyroidism is rare in children and adolescents. Bone changes may produce pain in the back or extremities, but this is rarely the first symptom of the disease. CASE REPORT: A 13 year-old girl suffered from pain in the back and lower extremities. Progressive bilateral genu valgum appeared. One year later, she became lame because of the deformity. ECG showed cardiac arrhythmia with atrial extra systoles. The serum calcium concentrations were 3.36 and 3.8 mmol/l, phosphate 0.76 mmol/l and alkaline phosphatases 6,612 U/I (N: 90-300). Urinary excretion of calcium was 17 and 26 mg/kg/day and the renal tubular reabsorption of phosphate was 77%. Radiological studies revealed resorption of subperiosteal bone, best seen along the margins of the phalanges, demineralization of the skull vault, bilateral coxa vara and zones of calcification on knee metaphyses. The serum concentration of parathyroid hormone (PTH) was 1,066 pg/ml (N: 10-55) and that of 1-25(OH)2D3 was 125 ng/ml (N: 20-80). Ultrasonography showed a heterogeneous mass, 23 x 15 mm, suggesting a parathyroid adenoma. This adenoma was independent of the left inferior parathyroid. It was removed and the biochemical findings gradually returned to normal. Bone demineralization also disappeared and the knee deformities were surgically corrected 9 months later. CONCLUSIONS: Bone changes may occasionally cause severe pain, indicating demineralization and hypercalcemia: hyperparathyroidism is one cause of such changes.

Adenoma

[Neurectodermal malignant tumor of the soft tissues after treatment with immunosuppressive agents of lipoid nephrosis].

Immunosuppressive drugs are known to increase the risk of inducing neoplasia, especially acute leukaemia when high doses are used. A case of nephrosis in a 10 year-old boy treated with chlormethine (cumulative dose: 0.8 mg/kg) and chlorambucil (cumulative dose: 10 mg/kg) is reported. Four years after the beginning of the treatment an extraskeletal Ewing's sarcoma occurred. Since the review of literature failed to find any malignancy induced by such an immunosuppressive treatment for nephrosis, the question whether or not this extraskeletal Ewing's sarcoma was attributable to this treatment remains unanswered.

Adrenal Cortex Hormones

[Renal transplantation in children. Surgical aspects].

The renal transplantation in children has some specificities: urologic anomalies (vesico ureteral reflux, posterior urethral valves) are frequently the cause of the renal failure, and necessitate a thorough surgical preparation before transplantation (nephrectomy, reconstitution of urinary tract). The child must have a sterile, compliant and continent urinary tract on the day of the operation. In small children (< 15 kg), it is often necessary to operate through a transperitoneal incision, especially if the donor is an adult: the anastomoses will then concern the aorta and vena cava. The results are good, even better than in adults, except for very young children (under six years of age). Transplantation with living related donor (LRD) give the best results. Currently, the graft survival is 87% to 90% after three years with LRD, versus 65 to 77% with cadaveric donors.

Age Factors

Renovascular hypertension: a rare cause of neonatal salt loss.

We report a case of severe hypertension in the newborn period due to obstruction of the right renal artery. The baby presented with polyuria leading to dehydration and was found to have hyponatraemia and severe renal salt loss. When sudden malignant hypertension is induced in experimental conditions, a high pressure diuresis and increased angiotensin II production are found. These findings could explain the renal salt loss, notwithstanding the effects of secondary hyperaldosteronism and hyper-reninaemia.

Dehydration

[Early tubular involvements in lead poisoning in children].

One of the main targets of lead poisoning is the kidney. Chronic poisoning can lead to kidney failure, and acute poisoning to tubulopathy with Toni-Debré-Fanconi syndrome. The primum movens of this tubulopathy is a distortion of mitochondrial energetic metabolism. We studied 27 children presenting with non symptomatic poisoning. Serum creatinine levels were normal. There was neither proteinuria nor glycosuria and only one child presented with hyperaminoaciduria. One third already presented with signs of tubulopathy, as shown by an increase in beta 2 microglobinuria in 36.5% of the cases studied (8 times in 22 samples) and enzymuria in 30.4% of the cases (23.5% if one excludes the children having had a chelation before the study). Kidney is therefore involved early in lead poisoning in children.

Acute Disease

[Atrial natriuretic factor in hemodialyzed children].

Indirect evidence suggest that volume overload is the major determinant of plasma atrial natriuretic factor (ANF) elevation in hemodialysis patients. Correlations between plasma ANF levels and extracellular volume (ECV) were investigated by simultaneously measuring both parameters in 30 pediatric hemodialysis patients (aged 1 to 17.5 years; 18 M, 12 F) 24 hours after a dialysis session. Plasma ANF was determined using a commercially available RIA (Amersham) after plasma extraction (SEP-Pack C18); ECV was estimated by determining the volume of distribution of inulin and expressing the result as the % of body weight. In hemodialysis children, ANF levels ranged from 43 to 427 pg/ml (versus 30-70 pmoles/ml in age-matched controls) and EVC ranged from 17 to 33% BW. A significant positive correlation was found between plasma ANF levels and ECV (r = 0.66; p less than 0.001). Patients who exhibited falls in blood pressure during the dialysis session had lower mean ANF and ECV values (133 +/- 90 pg/ml and 23 +/- 3% BW, respectively) than those who did not (211 +/- 123 pg/ml and 26 +/- 4% BW, respectively). Conversely, patients who needed chronic antihypertensive therapy had higher mean plasma ANF and ECV values (204 +/- 122 pg/ml and 26 +/- 4% BW, respectively) than those who did not (149 +/- 100 pg/ml and 23. 5 +/- 4.5% BW, respectively). In a small subgroup of patients who had repeated determinations, individual plasma ANF and ECV changes were closely matched and both parameters were well correlated.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent

Microsurgical creation and follow-up of arteriovenous fistulae for chronic haemodialysis in children.

Three hundred and eighty children underwent 434 angioaccesses. Of these angioaccesses, 113 were constructed in 74 children weighing under 10 kg. Most accesses (n = 340) were distal arteriovenous fistulae (AVF). After microsurgery there was a 96% immediate patency. Seventy percent of AVF, excluding distal ulnarbasilic AVF, were functional, sometimes after secondary superficialization of the vein. Eighty-five per cent of the distal radial-cephalic AVF are still patent after 2 years, 60% are still patent after 4 years. These radial-cephalic AVF required 65 repeat anastomoses, and 12 ligations of the proximal-radial artery in order to reduce to 50% the high blood flow (pre-reduction average index = 900 ml/min per m2. The patency rate of arteriovenous-bridge grafts was not encouraging. The severity of stenoses in the proximal-venous trunks, sometimes related to previous catheterization, is emphasized. Advantages of the radial-cephalic wrist AVF in children are highlighted.

Adolescent

[Nail-patella syndrome without extra-renal lesions. A new hereditary glomerular nephropathy].

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.

Adolescent

[Asymptomatic urinary infections in children].

Asymptomatic urinary tract infection is diagnosed on the usual criterion used for urinary tract infections in children (at least 10(5) organisms per ml). There are no clinical manifestations. These asymptomatic infections are due to organisms with little infective potential and no harmful effects on the urinary tract or renal parenchyma. In patients with no malformations of the urinary tract, antimicrobial treatment is unnecessary.

Bacteria

[Rheumatoid purpura and acute post-infectious glomerulonephritis].

The authors report the case of a 33 month-old child who presented some of the classical symptoms of the Henoch-Schoenlein purpura - arthritis, purpuric lesions of the lower extremities - associated with the full-blown picture of a post-infectious glomerulonephritis - low level of C3, proliferation of mesangial cells, exudation with large number of leukocytes, and C3 glomerular deposits. These findings and others previously described suggest that the Henoch-Schoenlein purpura is a syndrome and that some of its manifestations may occur in patients with post-infectious glomerulonephritis.

Acute Disease

[Nephrotic syndrome and B leukemia].

A boy, aged 14 1/2 years, presented with Burkitt leukemia. His renal status was normal before treatment. Chemotherapy (SFOP LMB 86 protocol) was begun Oct. 9, 1986. After the first 2 courses of chemotherapy, the patient had Gram negative sepsis treated with cefotaxime, netilmycine, Vancomycin and ornidazole. During sepsis, nephrotic syndrome developed (albumin 25 g/l, non selective proteinuria 15 g/24 h), with moderately high blood pressure, functional renal failure (creatinine 141 mumols/l, U/P urea = 20), polyuria and tubular damage. Kidney ultrasonography was normal. Needle biopsy showed minimal glomerular lesions, acute tubular lesions, and no deposits in immunofluorescence. The nephrotic syndrome disappeared within 3 weeks, with treatment of leukemia. He is at present in complete remission with a follow-up of 25 months.

Adolescent