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Biomedical subjects

B Branger

Publications and source records attributed to B Branger.

At least 73 records · Page 4Linked to original sources

Hypoglossia-hypodactylia syndrome with jejunal atresia in an infant of a diabetic mother.

Despite advances in therapy for maternal diabetes, pregnancies of diabetic women remained at an increased risk of spontaneous abortion or delivery of an infant with major malformation. We report on an infant of a diabetic mother with hypoglossia-hypodactylia associated with complete jejunal atresia. A common pathogenesis for these 2 malformations could be a vascular disruptive mechanism with in utero arterial thrombosis.

Adult↗

Autoantibody to plasma fibrinopeptide A in a patient with a severe acquired haemorrhagic syndrome.

We describe a 50-year-old man with a severe acquired haemorrhagic syndrome. He had slightly prolonged clotting times using bovine thrombin, human thrombin and reptilase. His plasma contained a polyclonal IgG which interfered with the generation of fibrin monomers without inhibiting the aggregation of preformed monomers. The inhibitor delayed thrombin-induced fibrinopeptide A release. The IgG bound to insolubilized synthetic fibrinopeptide A (one binding site per molecule) and, with higher affinity, to fibrinogen (two binding sites per molecule). It did not bind to insolubilized fibrin monomers. The IgG did not impair the catalytic activity of thrombin toward a small synthetic substrate but inhibited the binding of thrombin to fibrinogen without binding to thrombin. The binding of the anti-fibrinopeptide A autoantibody to fibrinogen might have impaired thrombin-induced fibrinogen to fibrin conversion in vivo. This may have favoured the reported haemorrhagic syndrome which was associated with severe chronic renal insufficiency.

Autoantibodies↗

[Newborn infants with a birth weight of 1000 g and less. A regional study of 124 cases].

A retrospective and regional survey was performed in 1986 and 1987 in the neonatal centers of western France (9 districts of Pays de Loire and Bretagne with 156,134 births). The epidemiology of 124 neonates with a birth weight of 1,000 g or less was analysed and the follow-up was studied at the age of two. The incidence was 0.8 per thousand births. The mortality was 44.3%. There were no differences between surviving and dead infants concerning obstetrical circumstances, presentation and mode of delivery, multiple or single pregnancies and birth place. Seventy per cent were incubated in the delivery room. Forty-five per cent had respiratory distress syndrome (RDS): 60% of them died (vs 30% without RDS; P less than 0.001) 41% had ultrasound intracranial anomalies (vs 8.5% without RDS; P less than 0.003). There was significantly less death when the duration of rupture of membranes was greater than 12 hours (28% vs 51.7% for a duration less than 12 hours; P less than 0.03). Cerebral palsy was present in 19.3% of the survivors followed until the age of 2 years. The disabled infants differed significantly from normal infants in term of RDS and ultrasound intracranial anomalies. From this study, the incidence of infants with very low birth weight appears low in western France, possibly related to the fact that infants died before birth or before being sent to neonate units; survival and sequelae rate is comparable with other studies.

Follow-Up Studies↗

[Vertebral involvement and fetal alcohol syndrome].

Vertebral abnormalities observed in 8 children with fetal alcohol syndrome are described and discussed. They include scoliosis (4 cases), neural tube defects (4 cases) and complex malformations of the cervical spine (2 cases). The latter probably constitute a different entity from that of the Klippel-Feil syndrome. It is probable that alcohol plays a direct role in the genesis of these abnormalities.

Female↗

[Examination of the prepuce in 511 nursery school children. The role of retraction technics].

The foreskin of 511 nursery school children was examined. Among the 500 unoperated children, 69.4% had a freely retractable foreskin. Adhesions were found in 28% of children and phimosis in 2.6% (total proportion of children with phimosis including the operated cases was 4.5%). A negative history for forcible retraction was found in 63.6% of children; among these subjects, 64% had a freely retractile foreskin and 31.1% had adhesions, which were usually extensive. Among children who had undergone forcible retraction, 77.5% had a freely retractile foreskin and 22% had adhesions, which were minor in most instances (p less than 0.001). The person who performed forcible retraction (the family physician in 54% of cases) and the age of the child at the time of forcible retraction were recorded. This retrospective study in a pediatric population of mean age 3 years 7 months provides data on the outcome after forcible retraction. Mean age at forcible retraction was 13 months in 36.4% of patients. Among the 500 unoperated children, 204 (40.8%) were spontaneously free of foreskin adhesions.

Age Factors↗

[A severe epidemic of Streptococcus group G infection in hemodialysis: epidemiologic survey by molecular biology and preventive measures].

An outbreak of group G streptococci infection affected 6 patients of an hemodialysis unit. Group G streptococci were isolated from patients and from numerous atmospheric specimens, different parts of two dialysis machines, and two blankets, but from only one nurse on the hospital staff. Typing of group G streptococci by an improved method of DNA fingerprinting showed that the isolates from one patient, the nurse and the two blankets differed from one another. The group G streptococci were probably transmitted to patients by dialysis machines with defective microporous filters. No further case of group G streptococci infection was reported three years later since microporous guard filters were systematically doubled.

Adult↗

Two years experience of daily self-administered subcutaneous erythropoietin.

Recombinant human erythropoietin (EPO) has been used for 4 years in end-stage renal disease patients, administered intravenously 3 times a week. A study was undertaken to determine the optimal way of administration comparing 3 times weekly intravenous EPO to self-administered daily subcutaneous EPO (SADSCEPO). In a first group of 4 patients, we demonstrated that the change from 3 times weekly intravenous EPO to SADSCEPO permitted a dose reduction of 70%. In a second group of 20 patients who started the EPO therapy with the daily subcutaneous route at a median dose of 12 U/kg/day, the hematocrit increased from 20 to 30% in 4 months and remained over 30% in spite of a median dose reduction to 9 U/kg/day. The patients' acceptance of SADSCEPO was good. The mechanism allowing such a dose reduction is unknown. However, the significant reduction in median dosage requirement with the subcutaneous route should allow a greater number of patients to be treated more cost-effectively.

Adult↗

[Evaluation of perinatal policy. Result of a survey about birth in Pays de Loire].

A survey was undertaken in 3 French departments of the Pays de Loire from January 1, 1986 to December 31, 1986. Among the 32,876 neonate (NN) population, the authors studied the 2,753 NN who were hospitalized and a control group of 1,458 NN representative of the population of 30,123 NN who were not hospitalized. Socioeconomic patterns of mothers, of their pregnancies and deliveries were studied in both groups. The control group was compared with the data obtained in 1981 at the national level and in 1982 at the local level. This group was used as a reference for the study of hospitalized NN. Intrauterine growth retardation rate was 2.4%, prematurity rate 4.6% of live births, very premature rate (as defined by a gestational age less than 32 weeks) was 0.45% of births. Among the group of hospitalized NN, which represent 8.5% of the general population, the NN were regrouped according to Cullen's classification and their care load estimated according to the Omega score. Thus 2 subgroups were defined: the 2,298 ordinarily hospitalized (Cullen's classes I and II) and the 455 severely sick NN (classes III and IV) i.e. 1.3% of births. One third of the severely ill NN were very prematures. The 2 other thirds presented with respiratory (24%), infectious (24%), malformative (19%) or neurological (13%) diseases. Lengths of stay and Omega scores varied according to the causes for hospitalizations and were inversely correlated with gestational ages (p less than 0.001). Neonatal mortality was 3.3 p. 1,000 births and rate of stillbirths 6.5 p. 1,000.

Adult↗

[Injuries in children requiring hospitalization: a survey of 394 cases].

Over a 9 months period, 394 cases of injuries among 0 to 15 year-old children were recorded in the pediatrics department of a general hospital. This included traffic accidents, falls, and blows. There was a male prevalence (59,4%), and a maximum frequency in the afternoon (14-17 h) and during the summer holidays (July and August). Seventy per cent of the children were seen in the emergency ward within 3 hours following the injury. Traffic accidents were mostly related to bike accidents. The incidence of injuries among 0-15 year old children appears to be 90 for every 1,000 children in ambulatory medicine, 30 for 1,000 in emergency wards and 8,5 for 1,000 in the pediatrics wards with hospitalization lasting longer than 24 hours. From our study, the cost of the hospitalized children has been evaluated at 2,5 MF for one year in our district.

Accidents↗

Biocompatibility of blood tubings.

We studied hemocompatibility of various blood tubings with C3a anaphylatoxin measurement and comparative electron scanning microscopy. The following tubing materials were tested: polyvinylchloride (PVC) plasticised with phthalate (PVC), pvc plasticised with phthalate coextruded with polyurethane (PIV), and two phthalate-free lines: pvc plasticised with trimellitate coextruded with polyurethane (TRI) and pvc plasticised with LT 360 (LTP). Results of C3a generation rate showed a significant activation by all blood tubings, with a reduced rate with PIV when compared to all others. Electron scanning microscopy showed marked alterations of PIV surface on tubings stored for 6 months. Protein deposits on internal surfaces after dialysis were similar whatever tubing material was tested, but adhesive cell number was greater with TRI when compared to PVC and LTP. Hemocompatibility is unchanged with phthalate-free tubings when compared to phthalate plasticised ones. In contrast with phthalate plasticised PVC there is no beneficial effect of polyurethane coextrusion with trimellitate plasticised PVC in regard to C3a generation.

Anaphylatoxins↗

High-flux synthetic versus cellulosic membranes for beta 2-microglobulin removal during hemodialysis, hemodiafiltration and hemofiltration.

Efficient removal of beta 2 microglobulin (beta 2-M) in end-stage renal failure patients is a continuing preoccupation, as the incidence and severity of dialysis-associated amyloidosis are increasing. To evaluate comparative beta 2-M removal we studied six stable end-stage renal failure patients during high-flux 3-h haemodialysis, haemodia-filtration, and haemofiltration, using acrylonitrile, cellulose triacetate, polyamide and polysulphone capillary devices. The reduction of plasma beta 2-M, total removal in ultrafiltrate/dialysate, and beta 2-M sieving coefficients were measured by RIA. The results suggest that convection plays the major role in beta 2-M removal when high-flux synthetic membranes are used in combination with high blood flow rates. In contrast, using the cellulose triacetate membrane under investigation, beta 2-M removal is diminished when ultrafiltration rates are increased. Accordingly, in any future prospective study on the role of beta 2-M retention in the amyloidogenesis, it is recommended that high-flux synthetic membranes be employed rather than the type of high-flux cellulosic membranes used in this study. The modality with which these synthetic membranes are used is probably less important, as long as maximum convective transport rates are obtained. Under present conditions, this will imply haemofiltration or haemodiafiltration rather than haemodialysis.

Cellulose↗

[Tracheal agenesis].

A case of tracheal agenesis is reported. At present, this lethal and rare malformation cannot be recognized before birth. Circumstances of the pregnancy and the stages leading to the diagnosis at birth are reported. Autopsy allows to classify the type of malformation and to search for others malformations, as seen in the VATER syndrome. A tracheo-oesophageal fistula is always present. The mechanism is unknown and there is no chromosomal anomaly. Fifty cases have been published in literature.

Humans↗