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Biomedical subjects

S Lortat-Jacob

Publications and source records attributed to S Lortat-Jacob.

At least 37 records · Page 2Linked to original sources
PubMed1997

Endothelial fibrinolytic reactivity and the risk of deep venous thrombosis after spinal cord injury.

Deep vein thrombosis (DVT) is a frequent event in patients with spinal cord injury, even with prophylactic anticoagulant therapy. Lower limb paralysis is a known major risk factor for venous thrombosis, supposedly due to the venostasis in relation with total immobility. The main goal of this study was to evaluate the endothelial response to anoxia to determine whether recovery of fibrinolytic potential occurs in patients subjected to forced bedrest because of a spinal cord injury and whether this recovery is related to the incidence and/or evolution of DVT. We evaluated vascular endothelium reactivity in the lower limbs no longer submitted to the hydrostatic pressure of the erected position in 15 patients with paraplegia or tetraplegia and in 10 normal volunteers after venous occlusion produced by the application of 10 cm Hg pressure to the lower limb for 15 min comparatively to the upper limb used as reference. Among the 15 patients, 10 whose spinal cord injury had occurred 1 to 6 months earlier were still receiving prophylactic anticoagulant therapy, whereas the five other patients were not receiving prophylactic anticoagulants because the injury dated back 6 months or more. After venostasis, tissue plasminogen activator (tPA) increased significantly in both patients and controls in the upper limb (tPA levels twofold and threefold respectively in controls and patients) but showed no significant changes in the lower limb; prolonged immobility did not allow recovery in the lower limbs of a level of fibrinolytic responsiveness identical to that in the upper limbs. The plasminogen activator inhibitor (PAI1) remained unchanged after anoxia, although wide interindividual variations were seen. Natural coagulation inhibitors and circulating blood stigmates of hypercoagulability were measured. None of the patients had abnormally low levels of coagulation inhibitors (ie, antithrombin III, protein C and protein S levels were normal). Seventy-five per cent of patients (prophylactically anticoagulated or not) had very high levels of fibrin degradation products (D. Dimer levels sevenfold to eightfold those of the controls), but all patients had normal levels of thrombin-antithrombin complexes and prothrombin fragments 1 + 2. The permanence of the thrombotic process characterized by an increase in D. Dimer levels without recovery of fibrinolytic potential suggests a proposal for the patients an indefinite antithrombotic treatment at curative doses.

Adolescent↗
PubMed1996

Serial transabdominal amnioinfusion in the management of gastroschisis with severe oligohydramnios.

Two fetuses with gastroschisis diagnosed in utero (at 19 weeks' gestation) had severe oligohydramnios at 30 to 31 weeks. Serial transabdominal amnioinfusions were performed to fill the amniotic cavity with saline, thereby avoiding the potential consequences of fetal exposure to severe oligohydramnios. In both cases, premature rupture of membranes occurred at 36 weeks, and the fetuses were delivered by cesarean section. There were minimal lesions of the extraabdominal bowel. After primary closure of the abdomen, the postoperative course was uneventful. These observations show that serial amnioinfusion is a feasible therapeutic approach for severe third-trimester oligohydramnios associated with gastroschisis.

Abdominal Muscles↗
PubMed1996

Artificial urinary sphincter implantation for neurogenic bladder: a multi-institutional study in 107 children.

OBJECTIVES: To evaluate the outcome of implantation of the AMS 800 artificial urinary sphincter in children with neurogenic bladder. PATIENTS AND METHODS: The records of 107 children (74 boys and 33 girls) treated for neurogenic urinary incontinence by implantation of the AMS 800 artificial sphincter between 1983 and 1993 were reviewed retrospectively. The underlying cause of incontinence in 92 patients was spina bifida, the mean age at implantation was 13.7 years and 30 patients had undergone previous bladder neck surgery. Implantation was carried out in combination with another surgical procedure in 24.3% of patients. The mean follow-up was 61 months (minimum 12). RESULTS: The mean operational life of the sphincter was 56 months. No deaths occurred but removal of the artificial sphincter was necessary in 20 patients (19%). Surgical revision was not required in 44 patients (41%) but at least one revision was performed in 63 and more than one in 42. There were 21 mechanical failures, 40 surgical complications and 39 cases involving changes in the dynamics of bladder function. Of the 87 children (81%) in whom the device is still in place, 72 are completely continent (83%), 10 are slightly wet, and five are incontinent. The overall success rate was 77%. CONCLUSIONS: The results emphasize that for successful implantation of an artificial urinary sphincter in children, the pre-operative bladder capacity must be sufficient and previous surgery should not have been performed on the bladder neck, the site of choice for implantation in children. Sphincterotomy is not always necessary in paediatric patients. Children and parents should be informed of the high complication rate and the need for long-term follow-up.

Adolescent↗
PubMed1996

Development of human renal function: reference intervals for 10 biochemical markers in fetal urine.

Evaluation of fetal renal function by analysis of fetal urine sampled in utero may improve perinatal care after a prenatal diagnosis of bilateral obstructive uropathy. We provide reference intervals for 10 fetal urinary compounds and examine their variation with gestational age. Forty-one fetuses with bilateral obstructive uropathy (urine sampled between 20 and 38 weeks of gestational age) had normal, healthy values for serum creatinine (< or = 50 mumol/L) at ages 1-2 years. These cases were thus assumed to represent a reasonable approximation to healthy values. Sodium and beta 2-microglobulin concentrations significantly decreased with gestational age; calcium, ammonia, and creatinine significantly increased; glucose, phosphorus, chloride, urea, and total protein concentrations did not vary. Our results provide reference values for prenatal evaluation of fetal renal function and suggest that glomerular filtration of macromolecules and tubular reabsorption of glucose and phosphorus are mature by 20 weeks of gestation, whereas tubular reabsorption of sodium and beta 2-microglobulin increases progressively during the second half of gestation.

Biomarkers↗
PubMed1994

True hermaphroditism: genetic variants and clinical management.

The diagnosis and management of 22 patients with true hermaphroditism are described. Sixteen of them were first seen before the age of 4 months. The initial manifestations were ambiguous genitalia in 20 cases (two of them identified prenatally by ultrasound examination), isolated clitoromegaly in one, and penile hypospadias plus unilateral cryptorchidism in one. All patients but one had at least one palpable gonad. Eleven of the twelve patients examined before the age of 6 months had basal plasma testosterone levels > 0.4 ng/ml. In older patients the stimulation test was necessary to demonstrate male testosterone secretion. The most common peripheral karyotype was 46,XX (17 cases); the other karyotypes were 47,XXY (1 case) and mosaicism 46,XX/46,XY (2 cases) or 46,XX/47,XXY (2 cases). One of the patients with the 46,XX karyotype had 46,XX/46,XY on fibroblast culture; four had the SRY gene in their leukocytes and one in the tissue taken at gonadal biopsy. A vagina was found in all patients at laparotomy, and a uterus was found in 17 cases (as a hemiuterus in 9). Genitography failed to demonstrate a uterus in only one case. The testicular tissue was dysgenetic but the ovarian tissue was normal. Sex assignment was male in 8 patients (reoriented by us in 2) and female in 14 patients (reoriented by us in 3). Spontaneous pubertal development occurred in the 4 patients (2 boys, 2 girls) with gonadal tissue who reached pubertal age. We conclude that true hermaphroditism is a heterogeneous condition in terms of its genetic background, with a prevalence of the 46,XX karyotype. There may be mosaicism with a Y-bearing cell line limited to the gonad (its frequency is probably underestimated), a paternal meiotic exchange between X and Y occurring in 46,XX cases with SRY, or a lack of the SRY gene, suggesting that other genes working independently of SRY may also determine testicular differentiation.

Child↗
PubMed1994

[Free amino acids in fetal urine and prognosis of renal function in bilateral obstructive uropathies].

In order to evaluate renal function, fetal urine was sampled in 27 fetuses with urinary tract obstruction diagnosed by ultrasonography. Amino acid concentrations were measured retrospectively. On histological examination performed after termination of pregnancy, five fetuses were found to have bilateral renal dysplasia (group 1). Eleven fetuses developed renal failure after birth: at one year, plasma creatinine concentration was over 50 mumol/l (group 2). Renal function was normal in the 11 other infants (group 3, plasma creatinine concentration at one year < 50 mumol-1). Statistically significant between-group differences were seen in fetal urine amino acids concentrations. However, there was an overlap of values in the three groups and individual amino acid concentrations could not be used predictively.

Amino Acids↗
PubMed1993

[Traumatic amputation of the lower limb and traumatic hemipelvectomy. Report of 2 cases in children and review of the literature].

Traumatic hemipelvectomy is rarely observed, mostly because very few patients survive the initial trauma. We describe 2 cases of children who survived this trauma. The first was a 12 year-old boy who accidentally fell between a metro train and the platform. He had a severe open trauma of the pelvis with important hemorrhage. After a first operation to stop hemorrhage and do a cystotomy and a colostomy, ischemia of the lower limb led to an inter-ilio-abdominal amputation. Two months were necessary to heal this. A prosthesis was made 12 months after the accident, postponed by urinary problems. The boy also had a traumatic amputation of the left arm and an upper metaphyseal fracture of the humerus. The second case was that of an 8 year-old girl who suffered a complete traumatic amputation of the hemipelvis after a road accident. A secondary colostomy was performed because of a local infection. Once healing was achieved, the child was low to follow-up. In the literature, we found 36 survivors to this dreadful lesion (4 of them children). We analysed the different problems for the treatment of this trauma caused, and their short term consequences. An early evaluation of the lesions, rapid and intensive resuscitation, early decision to complete the traumatic amputation if necessary, with urinary and digestive derivation, are important elements towards eventual successful outcome of the treatment.

Amputation, Traumatic↗
PubMed1991

Neonatal short bowel syndrome.

In this retrospective study the management of infants who had undergone resection of more than 50% of the small bowel as newborn infants between 1970 and 1988 was analyzed to define prognostic factors. Small bowel resections were performed for atresia (36 cases), volvulus (22 cases), gastroschisis (10 cases), necrotizing enterocolitis (11 cases), and other disorders (8 cases). Patients were classified into two groups depending on the length of residual small bowel: group 1 (n = 35) had less than 40 cm of small bowel and group 2 (n = 51) had 40 to 80 cm of residual small bowel. Patients in group 2 had significantly better survival rates than those in group 1 (92.0% vs 66.6%; p less than 0.001). The patients in group 1 who were born after 1980, when home parenteral nutrition was introduced, had better survival rates than those who were treated before 1980 (95.0% vs 65.0%; p less than 0.01). The time required for acquisition of intestinal adaptation depended on the intestinal length (average, 27.3 months for group 1 and 14 months for group 2; p less than 0.01) and on the presence or absence of the ileocecal valve. Parenteral or supportive enteral nutrition, or both, ensured normal growth in both groups. We conclude that more than 90% of infants now survive after extensive small bowel resection with parenteral nutrition and that the remaining small intestine will adapt with time. Home-based parenteral nutrition allowed children to be treated in the best psychosocial environment.

Adaptation, Physiological↗
PubMed1991

Achalasia of the esophagus in childhood. Surgical treatment in 35 cases, with special reference to familial cases and glucocorticoid deficiency association.

Achalasia of the esophagus is a relatively rare problem in children, but it may be the cause of severe lung disease, growth retardation and respiratory death in young infants. Surgical esophago-cardio-myotomy remains the treatment of choice, and this article details 25 years of experience with 35 children with achalasia of the esophagus and their late post-operative follow-up. The occurrence of achalasia in the first six months of life, the existence of a familial factor, the prevalent possible association with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem, somewhat different from the adult form, which is considered to be an acquired disease.

Child↗
PubMed1990

Home parenteral nutrition in children: 8 years of experience with 112 patients.

It is essential that children on prolonged parenteral nutrition for anatomical or functional loss of small bowel should enjoy a quality of life which is as normal as possible. Their return home is a major factor in this. Over the past 8 years, 112 children were able to remain at home on cyclic parenteral nutrition. Forty-nine of them are no longer on home parenteral nutrition (HPN), 45 are still on HPN, and 18 have died. Growth and quality of life were good in most cases. Most of the complications were from infection, 1 septicaemia per 594 days on HPN. In the light of these results, HPN seems to be the best option for children requiring prolonged parenteral nutrition, although it can only be considered within the framework of a specialized centre, which ensures patient follow-up, and provides the logistical support required for this high-technology treatment.

Journal Article↗
PubMed1990

Catheter related sepsis during long-term parenteral nutrition in paediatric gastroenterology patients: a study of 185 consecutive central venous catheters.

We report the results of a prospective study of catheter-related sepsis (CRS) in 154 gastroenterology paediatric patients treated for medical (n = 102) or surgical (n = 52) disorders. Over 2 years, 185 central venous catheters (CVC) were placed, including 155 Broviac and 30 Jonathan Shaw CVC. CVC remained in place 13,331 days (6,539 days of cyclic parenteral nutrition), with a mean catheter life of 72 days. Forty-eight episodes of CRS occurred, most of them due to coagulase-negative staphylococci (n = 29) and Staphylococcus aureus (n = 17). CRS rate was 0.26, with an overall incidence of 1 infection per 278 days of parenteral nutrition (PN). Factors significantly associated with CRS were: age 1-5 years (p < 0.01), a medical indication for treatment (p < 0.001) and summer months of PN (p < 0.01). Interestingly, CRS occurred predominantly (65%) within the first 2 months following CVC placement and the risk of infection correlated with local haemorrhage at the time of insertion (p < 0.01). Appropriate systemic therapy, without removing the catheter, permitted resolution of 90.9% (40/44) of all CRS and 100% (28/28) of CRS due to coagulase-negative staphylococci.

Journal Article↗
PubMed1990

[Urogenital abnormalities associated with anorectal malformations].

The authors presented various data with respect to the frequency (431 cases) of anorectal malformations associated to urogenital abnormalities (26 to 56%). They also indicated the frequent association of these anomalies with a lumbosacral vertebral malformation (28%). The higher the anomaly, the more serious the condition.

Abnormalities, Multiple↗
PubMed1989

Achalasia of the esophagus in childhood: surgical treatment in 35 cases with special reference to familial cases and glucocorticoid deficiency association.

Achalasia of the esophagus is a relatively rare problem in children, but it can result in severe lung disease, growth retardation, or respiratory death in young infants. Surgical esophagocardiomyotomy remains the treatment of choice, and this report details a 25-year experience with 35 children with achalasia of the esophagus and their long-term postoperative follow-up. Occurrence of achalasia in the first 6 months of life, the existence of familial cases, and the association of achalasia with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem.

Adolescent↗
PubMed1989

[Surgery of sexual ambiguity: experience of 298 cases].

From 1955 to 1988, 298 children suffering of ambiguous genitalia were operated upon in the Department of Pediatric Surgery, Hospital Necker-Enfants Malades, Paris. 187 were female pseudo-hermaphroditism (of which 85% congenital adrenal hyperplasia). 53 gonadal dysgenesis and 58 male pseudohermaphroditism--Feminizing genitoplasty, including relocation of the clitoris, published by us, in 1961, was made in 238 cases. It was for a long time the best procedure used, whatever be the etiology of intersex, because the results of masculinizing procedures to be used were poor. Using free bladder mucosa graft is a progress which allows an efficient one stage masculinizing genitoplasty, we have made 31 cases of it. Early and precise diagnosis, early medical treatment and psychological approach of intersex by a multidisciplinary team and adequate precocious one stage surgical repair of ambiguous genitalia, have dramatically improved this condition. Prenatal diagnosis of C21 hydroxylase deficiency and preventive control of adrenal hyperplasia of the foetus, by the supply of gluco-corticoïds to the mother in "a risk situation", will in the future allow to prevent virilisation of female foetuses--Surgery will become useless.

Adolescent↗
PubMed1987

Infantile myofibromatosis. Report of two cases in one family.

Two brothers with infantile myofibromatosis are reported. Both had cutaneous and skeletal myofibromas with spontaneous and complete healing of their cutaneous lesions. These cases suggest autosomal recessive inheritance of this rare disorder.

Bone Neoplasms↗
PubMed1984

Preservation of gonadal function in true hermaphroditism.

Twelve documented cases of true hermaphroditism have been studied since 1965; there was histological evidence of both ovarian and testicular tissue in all cases. The clinical, anatomical, and therapeutic procedures are reviewed with special emphasis on gonadal morphology, histology, and function. Seven patients were reared as females, 5 as males; 6 are now postpubertal and 3 are more than 30 years old. Five underwent bilateral castration; in seven cases, only the discordant gonadal tissue has been removed so that these patients may have endogenous gonadal secretion in keeping with their sex of rearing.

Adolescent↗