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Biomedical subjects

P Cochat

Publications and source records attributed to P Cochat.

At least 145 records · Page 8Linked to original sources
PubMed1992

Alport syndrome and diffuse leiomyomatosis: deletions in the 5' end of the COL4A5 collagen gene.

Alport syndrome (AS) is an hereditary glomerulonephritis that is mainly inherited as a dominant X-linked trait. Structural abnormalities in the type IV collagen alpha 5 chain gene (COL4A5), which maps to Xq22, have recently been detected in several patients with AS. The association of AS with diffuse esophageal leiomyomatosis (DL) has been reported in 24 patients, most of them also suffering from congenital cataract. The mode of transmission and the location of the gene(s) involved in this association have not been elucidated. Southern blotting using cDNA probes spanning the whole COL4A5 and a 5' end COL4A5 genomic probe showed that three out of three patients with the DL-AS association had a deletion in the 5' part of the COL4A5 gene extending beyond its 5' end. This indicates that the same gene, COL4A5, is involved in classical AS and in DL-AS and that the transmission of DL-AS is X-linked dominant. These results also suggest that leiomyomatosis might be due to the alteration of a second gene involved in smooth muscle cell proliferation, which is located upstream of the COL4A5 gene, and that there might be a contiguous gene deletion syndrome, involving at least the genes coding for congenital cataract, DL and AS.

Adolescent↗
PubMed1992

[To break the isolation. Telecommunication to help the schooling of sick children].

A 2-way teleconferencing system has been described which links the sterile and pediatric units of the Center for Cancer Treatment in Lyon to a secondary school and the pediatric unit of the University Hospital Edouard Herriot where primary teaching is given. The Léon Bérard Center and Edouard Herriot Hospital are 300 m apart. Elie Vignal secondary school is 6 km from the first site; all three establishments are connected via hyperfrequency aerials. With the help of this system, communication (voice and image) between children in hospital and pupils or teachers in school is instantaneous. Children in bed can listen to the lesson, break in on the conversation in school, ask questions or answer those of teachers and play with children in the classroom. The aims of the system are: 1) to improve the education delivered to children and teenagers who have to spend long periods in hospital; 2) to establish a relationship between the worlds of healthy and sick children through teaching and playing in common; 3) to facilitate the social insertion of sick children after leaving the hospital.

Adolescent↗
PubMed1991

[Treatments of enuresis].

The current management of nocturnal enuresis is based on simple therapeutic schedules, available if the child, his parents and physician, are determined. The treatment first includes motivational therapy and bladder training exercises. If bedwetting is not controlled, conditioning alarm procedures can offer a high rate of positive results with a low risk of relapse. Tricyclic antidepressants should be avoided because of their severe cardiac and hepatic potential side-effects, and because they provide quite the same short and long-term results than desmopressin. Intranasal desmopressin (20 to 40 micrograms/day) offers a 40 to 60% rate of positive results, and can be administrated over a long period in relapsing patients.

Antidepressive Agents, Tricyclic↗
PubMed1991

Renal effects of continuous infusion of recombinant interleukin-2 in children.

Recombinant interleukin-2 (rIL-2) is a new promising treatment for cancer, but is associated with severe renal toxicity. This study is the first to analyse the renal effects of rIL-2 in children. Twenty-one cycles of continuous rIL-2 infusion were studied in 15 patients; mean age was 6.9 years and average weight 18.9 kg. Interstitial fluid retention and oliguria (baseline, 1.7 ml/kg per hour; nadir, 0.5 mg/kg per hour) were associated with hypotension (baseline, 101/56 mm Hg; nadir, 85/43 mm Hg) and decreased intravascular volume (plasma renin activity increased x 10). Weight gain (+7.9%) was observed in 13 cycles whereas weight loss (-6.3%) was shown in 8 cycles because of digestive and cutaneous losses, mainly in the youngest patients. This prerenal azotaemia was characterized by a decrease in creatinine clearance (from 101 to 36 ml/min per 1.73 m2) and a low fractional excretion of sodium (FENa) (from 0.70% to 0.09%). Hypotension and hypovolaemia needed vascular filling (n = 12), dopamine (n = 7) and interruption of rIL-2 (n = 2). Most abnormalities occurred as early as day 2 of therapy and were always reversible after a short period with sodium leakage (diuresis = 2.2 ml/kg per hour, FENa = 2.01%). Hypophosphataemia was associated with low urinary excretion of phosphorus, suggesting an increased uptake of inorganic phosphorus by rapidly proliferating lymphoid cells.

Adolescent↗
PubMed1991

Acute renal failure in a child after chewing of match heads.

A few days after ingestion of 40 match heads, a 3-year-old boy was admitted to hospital with oliguric acute renal failure (ARF) requiring peritoneal dialysis during 9 days. A renal biopsy showed acute tubulointerstitial nephritis; the outcome was rapidly favorable and the child recovered normal GFR. It seems to be the first published case of ARF after match poisoning, probably because of the presence of potassium bichromate.

Acute Kidney Injury↗
PubMed1991

Interleukin-2 and lymphokine-activated killer cells in 15 children with advanced metastatic neuroblastoma.

A phase II trial using interleukin-2 (IL2) and lymphokine-activated killer (LAK) cells was carried out in an attempt to treat children with end-stage neuroblastoma. Fifteen patients (median age, 7 years) were enrolled in the study. Twelve were in relapse after massive chemotherapy and autologous bone marrow transplantation (ABMT), and three had a primary refractory disease after conventional chemotherapy. IL2 was administered as an 18 x 10(6) IU/m2/d continuous infusion. One course consisted of a double 5-day treatment period separated by a 6-day break. Cytapheresis to harvest LAK progenitor cells was performed during the rest period. After a 4-day in vitro culture, LAK cells were reinjected during the second cycle of therapy. A phenotypic and functional analysis of immunologic parameters was conducted along with the therapeutic protocol. Toxicity was significant with two toxic deaths (cardiotoxicity and respiratory distress). The reinfusion of large amounts of LAK cells was clearly involved in one case, but this particularly severe toxicity has to be related to the patient's status (ie, heavy pretreatment). No significant clinical response was seen. The immunologic monitoring showed phenotypic and functional modifications in these patients before initiation of treatment and an unexpected absence of evolution of these parameters during IL2 therapy. Although the origin of these immune dysfunctions is not clear, they could be involved in the failure of IL2 therapy. Future studies of IL2 therapy in neuroblastoma should be undertaken earlier in the course of the disease.

Antibodies, Monoclonal↗
PubMed1991

[Abnormal implantation of the left coronary artery on the aorta: potentially lethal malformation in children].

The authors report on the case of a 10 year-old girl who died suddenly after exercise. The autopsy showed abnormal origin of the left coronary trunk from the right sinus of Valsalva, with proximal course of the vessel between the aorta and pulmonary trunk. We discuss on the mechanism of myocardial ischemia; the opportunity to diagnose such an anomaly when the patient is alive is rare, and cause of death is most frequently determined by necropsy.

Aorta, Thoracic↗
PubMed1991

[Organization of organ transplantation in France].

France was in tenth position in the field of kidney transplant in Europe in 1982, and is currently at the top of western countries for all transplant activities (per million inhabitants). Cyclosporin A was certainly a favourable factor for liver, heart and lung transplantations. Nevertheless the transplantation activity in France is twice than of Great Britain or Germany for those transplant specialties and the percentage of renal transplantations from living related donors is very low in France: 2.7% versus 30% in others. These data depend on a specific French organization which involves: a presume consent law; an emergency policy with mobile medical rescue teams and polyvalent intensive care units; a three level France Transplant organization: national, regional and local; hospital physicians as regional transplant coordinators who are independent from transplant teams; waiting list telematic computer system which promotes priorities: high emergencies, high-sensitized and pediatric recipients.

Child↗
PubMed1991

[Congenital mega-urethra. A case].

Congenital megalo-urethra is a malformation of the male urethra with a major dilatation of its penile part without obstruction downwards. It is either due to hypoplasia or agenesia of spongy tissue. Two types are described, scaphoid and fusiform types, according to the presence or the absence of erectile tissue. Megalo-urethra is a rare condition, and 56 cases were previously reported until 1987. A new case of this severe anomaly is reported and management discussed.

Humans↗
PubMed1991

[Urinary tract infections in infants: medical management].

The risks of acute pyelonephritis in infants concern microbial invasion and parenchymal damage. There is a high rate of septicemia and of urinary malformations in children under 1 yr of age; in addition, the kidney is a growing organ with a high risk of scarring. Such parenchymal injury may lead to arterial hypertension, and renal failure when bilateral. Vesicoureteric reflux must be evaluated by ultrasound and cystogram; long-term renal scarring needs to be assessed by scintigraphy or pyelography when children are 7 yr old. Short- and long-term risks could be limited by early intravenous biantibiotherapy.

Acute Disease↗
PubMed1991

[Resuscitation during renal transplantation in children].

Over a 4 yr-period, 60 children (aged 10 months to 17 yr) received 66 kidney transplants with the same surgical intensive care program, the fist 48 hr-period of which has been analysed in this study. Thirty percent of recipients were transplanted without previous dialysis and in 8%, body weight was below 10 kg at the time of surgery. The duration of anesthesia was 4.4 +/- 1.0 h and 32% received locoregional anesthesia. The mean duration for cold ischemia was 14.7 +/- 11.7 h and 26 +/- 7 min for warm ischemia; diuresis began during the operation in 79% of the patients. Routine vascular filling consisted of standard isotonic solute (11 +/- 4 ml/kg/h) associated with mannitol infusion; 59% of recipients required 20% human serum albumin and 42% blood transfusion. Post-operative diuresis was 7.4 +/- 6.0 ml/kg/h during the first 24 h, and sometimes resulted in hypovolemic episodes; 9% of the patients had primary non-functioning kidneys (4 transient acute tubular necrosis; 2 vascular thrombosis) and 4% required dialysis; the 1-yr survival rate was 82% for the grafts and 98% of the patients.

Adolescent↗
PubMed1990

[Resuscitation of children with brain death in view of organ procurement for therapeutic use].

Among the different stages from donor to recipient, the intensive care of children in a state of brain death (BD) is of paramount importance, the first step being having this goal in mind. As soon as procurement is contemplated and medico-legal formalities have been completed, all potentially involved persons and institutions (France-transplant, procurement and transplantation teams, immunology laboratory, recipients) must be contacted. Intensive care, started at the stage of irreversible coma, will be pursued near the place where procurement will be performed, geared to the obtention of multiple organs. Transportation is often required (for arteriography or procurement), but is carries a risk since BD creates an unstable situation which at any moment may turn into cardiac arrest. Under such conditions, intensive care has two main objectives: 1) to ensure adequate haemodynamics and tissue oxygen supplies, and 2) to avoid infection. After a 10 to 20 hours period of intensive care and after procurement a detailed report on both surgery and intensive care must be laid down.

Brain Death↗
PubMed1990

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.

Cataract↗
PubMed1990

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.

Absorptiometry, Photon↗