[Inequity of compensation trajectories for victims of medical injuries. A survey in the French out-of-court settlement mechanism]. / Inégalités dans les parcours d'indemnisation des victimes d'accidents médicaux. Une enquête au sein du dispositif français de règlement amiable.

BACKGROUND: In France little is known about either the characteristics of people who take legal action because they believe themselves to be victims of harm caused by medical activity, or about their complaint trajectory. The law of 4th March 2002 created an out-of-court settlement mechanism which aims to reduce inequitable access to compensation experienced by victims faced with legal procedures that are both lengthy and costly. This mechanism now occupies a central position among the avenues of recourse available to patients and their families. METHODS: The study relates to the exhaustive database of 18,258 requests for compensation filed with the out-of-court settlement mechanism between 2003 and 2009. It takes 4 series of variables into account: (1) the characteristics of the people concerned by the request, (2) the recourse practices, (3) the result of the requests, (4) the characteristics of the commissions with whom they dealt. Univariate and multivariate analyses were performed, in particular to find factors relating to the different responses given to the requests. RESULTS: Of the requests filed with the out-of-court settlement mechanism, 34.5% led to compensation being awarded, 30.7% were deemed inadmissible and 34.8% were rejected on the basis of expert opinions. The risk of inadmissibility was greater when the victim was a woman, undeceased, or a minor aged between 1 and 17; it bore no relation to standard of living. Recourse to a lawyer (24%) depended on various characteristics, in particular the age and vital status of the victim. It is associated to the decrease of the risk of inadmissibility and to the increase of the chances of receiving compensation. There were significant differences in the ways requests were processed (depending on where they were filed), in the time it took to examine the case, and in the tendency to reject requests before or after expert medical opinion. CONCLUSION: This study offers the first ever description of the population of patients and families who accessed the out-of-court settlement mechanism for medical claims in France. It looks at how, within the mechanism, the diversity of practices impacts inequity and experiences during the compensation process.

[Acute renal failure secondary to paroxysmal cold hemoglobinuria]. / Insuficiencia renal aguda secundaria a hemoglobinuria paroxística al frío.

Paroxysmal cold hemoglobinuria is a type of hemolytic anemia that mainly affects children. Tubular renal injury induced by the heme pigment in intravascular hemolysis is a rare cause of renal failure. We describe the case of a 5-year-old boy who presented with dehydration and dark urine a few hours after exposure to cold. The child had had an upper respiratory infection the previous week. He developed anemia and acute renal failure. The direct antiglobulin test was positive with anti-C3c and C3d. The diagnosis of paroxysmal cold hemoglobinuria was confirmed by the presence of biphasic antibody in the Donath-Landsteiner test.

[The identification of multiple thrombophilic risk factors in an infant with cerebrovascular accident]. / Múltiples factores de riesgo trombofílicos identificados en un lactante con accidente vascular cerebral.

INTRODUCTION: Neonatal stroke (NNS) incidence appears to be increasing over the last years. This is believed to be a consequence of diagnostic accuracy rather than a real amplification of this entity. Nowadays, NNS incidence is estimated to be 1:4000 full newborns. CASE REPORT: Child with left middle cerebral artery territory infarction in which several thromboembolic risk factors were documented both in the child (neonatal sepsis and factor V Leiden) and his mother (lupus anticoagulant, pre-eclampsy and factor V Leiden). CONCLUSIONS: This case supports the increasing evidence in recent reports that association of multiple prothrombotic risk factors (maternal and foetal) is present in NNS genesis. This way the authors agree that wide prothrombotic study may be of crucial interest in identifying subjacent thrombophilic disease, even when an exogenous risk factor is present.

Reference values for reticulocyte parameters and hypochromic RBC in healthy children.

INTRODUCTION: New erythrocyte and reticulocyte parameters available on modern hematology analyzers have shown to be useful markers in children. However, pediatric method-specific reference ranges for these indices are sparse. We aim to establish pediatric reference values for reticulocyte parameters and hypochromic red blood cells in healthy children with the ADVIA 2120 hematology analyzer. METHODS: Prospective study of 311 healthy children aged from 6 months to 18 years old with normal hematological parameters. ADVIA 2120 hematology analyzer (Siemens Healthcare Diagnostics, Amadora, Portugal) was used for whole-blood hematological measures. The sample population was grouped according to age into three cohorts, and gender distribution was used for participants aged ≥12 years. RESULTS: Age- and gender-specific reference limits of reticulocyte hemoglobin content (CHr or Ret-He), difference between the reticulocyte and erythrocyte hemoglobin content (Delta-He), immature reticulocyte fraction (IRF) and percentage of hypochromic red blood cells (%Hypo) are provided. Delta-He, IRF and %Hypo showed statistically significant gender differences in the 12-17 years group. CHr presented no significant gender variation within all age groups (median 30.8 ± 1.7 pg). CONCLUSION: Establishing reliable pediatric reference intervals for these novel hematological parameters may offer valuable prospects for clinical practice and research in the pediatrics field.

A point mutation in the glycerol kinase gene associated with a deletion in the dystrophin gene in a familial X-linked muscular dystrophy: non-contiguous gene syndrome involving Becker muscular dystrophy and glycerol kinase loci.

We report a family with an X-linked recessive muscular dystrophy characterised by exercise-induced myalgia, recurrent pigmenturia and mild proximal muscle involvement. Immunocytochemical and immunoblotting analysis in muscle, using the antibody directed against the rod domain of dystrophin, revealed a loss of immunoreactivity, but the immunolabelling using the antibodies directed against the COOH and NH2 domains of dystrophin were almost normal. The immunoreactions for alpha-sarcoglycan, gamma-sarcoglycan and beta-dystroglycan were normal. In the five male patients of this family with increased serum creatine kinase levels (from x8 to x50), mass spectrometry screening of the urine revealed a large increase in glycerol elimination which was quantified by enzymatic assay (from x14 to x39). An in-frame deletion of the dystrophin gene (exons 13-29) was found in the same five males and in three carrier females. All the deleted chromosomes also carried a missense mutation at nucleotide 947 of the Xp glycerol kinase (GK) gene resulting in a Thr to Met substitution at codon 278. These findings indicate that the two mutations cosegregate on the same chromosome in this family. This is the first reported case of two physically independent mutations, within the DMD and GK genes, which are contiguous but several hundred kilobases apart.

Preparation and characterization of films from pea protein.

The conditions for protein film preparation from an alkaline dispersion of a pea protein isolate were investigated in the presence of polyols as plasticizers. Mechanical and barrier properties of resulting films were studied as a function of protein dispersion conditions, protein and plasticizer concentrations and ratios, chain length of the plasticizer, and pH and composition of the alkaline medium. Neither the mode of protein hydration nor the pea isolate origin had a significant effect on the mechanical properties of pea protein films. However, increasing the plasticizer chain length induced slightly higher surface hydrophobicity but poor mechanical properties. Addition of monoglycerides to film-forming solution allowed a significant improvement of the films during aging. Both tensile strength and surface hydrophobicity increased when ammonium hydroxide was used as protein dispersing agent instead of sodium hydroxide.

Molecular determinants of the influence of hydrophilic plasticizers on the mechanical properties of cast wheat gluten films.

The influence of a set of hydrophilic plasticizers varying in their chain length (ethyleneglycol and longer molecules) on the tensile strength and elongation at break of cast gluten films was studied. When considered on a molar basis (moles of plasticizer per mole of amino acid), the effect of the different plasticizers depended on their respective molecular weights for plasticizer/amino acid ratios in the range from 0.10 to 0.40. However, above a ratio of 0.40-0.50 mol/mol of amino acid, these differences were abolished and both stress and strain reached a plateau value, with all plasticizers studied. In fact, when a homologous series of molecules was considered, the ability for plasticizer to decrease stress and increase strain was closely related to the number of hydrogen bonds the molecule was able to share with the protein network. Ethyleneglycol's efficiency was, however, lower than expected from its hydrogen-bonding potential; a comparison with other diols demonstrated that this was due to the small size of this molecule. The particular effect of glycerol concentration on the films' mechanical properties suggested that other molecular features of the plasticizer, such as the number and position of hydroxide groups in the molecule, were involved in the plasticization mechanism.

Evolution of wheat gliadins conformation during film formation: a fourier transform infrared study.

The secondary structures of wheat gliadins (a major storage protein fraction from gluten) in film-forming solutions and their evolution during film formation were investigated by Fourier transform infrared spectroscopy. In the film-forming solution, wheat gliadins presented a mixture of different secondary structures, with an important contribution of beta-turns induced by proline residues. The presence of plasticizer did not have any influence on protein secondary structure in the film-forming solution. The evolution of protein conformation was followed during drying; the major feature of this evolution was a clear growing of the infrared band at 1622 cm(-1), characteristic of intermolecular hydrogen-bonded beta-sheets. This revealed the formation of protein aggregates during film drying. The influence of the drying temperature on film properties and gliadin secondary structures was also investigated. Higher drying temperatures induced an increase of both the tensile strength of the films and the amount of beta-sheets aggregates. Although the appearance of heat-induced disulfide bridge cross-links has already been described, there is clear evidence that hydrogen-bonded beta-sheets aggregates are also induced by thermal treatment. It was not possible, however, to determine whether there is a direct relationship between the occurrence of these aggregates and the increase of the tensile strength of the films.

Properties of deamidated gluten films enzymatically cross-linked.

Films were prepared at neutral pH from deamidated gluten by casting with or without enzymatic treatment by transglutaminase in the presence of various concentrations of diamines added to the film-forming solution. Variation in the glycerol/deamidated gluten ratio from 0.2 to 0.5 had a major effect on the film mechanical properties, which is characteristic of a plasticizing effect. A ratio of 0.35, producing a tensile strength of 1.14 +/- 0.12 MPa and an elongation at break of 376 +/- 62%, was chosen for most of the enzymatic modifications. The action of transglutaminase with or without the addition of external diamines induced a simultaneous increase in tensile strength and elongation at break of the films but tended to decrease the contact angle between the film surface and a water droplet. The presence of diamines in the film solution affected the elongation at break more than the tensile strength of the films. These diamines, able to react at their two extremities, probably acted as spacers between gluten proteins. The decrease in solubility was related to the formation of high molecular weight polymers in the film. The film properties were unaffected by the type of diamine added as secondary substrate in the transglutaminase reaction.

Hysteroscopy and hysterography.

Hysteroscopy and hysterography approach the uterine cavity in different ways and each has advantages and limitations. Hysteroscopy is more accurate for the diagnosis of abnormalities that project into the uterine cavity such as polyps, submucous myomas, endometrial hyperplasia, and endometrial adenocarcinoma. Contrast media used in hysterography enable the detection of lesions that penetrate the myometrium and congenital and acquired partitions of the uterine cavity. Diagnostic hysteroscopy, easily performed as an office procedure, should be the screening method of choice to search for uterine abnormalities except for the infertility patient who also requires a salpingogram. Hysterography should be considered as a complementary procedure only when the direct visual examination proves incomplete or inadequate. It is necessary for a thorough assessment of uterine malformations, adenomyosis, complex intrauterine adhesions, and uterine scars.

[Acting on clinical trials. Experience of organizations for the fight against AIDS]. / Agir sur les essais thérapeutiques. L'expérience des associations de lutte contre le SIDA en France.

This article analyses the action of organizations fighting against AIDS, from a sociological point of view, with regard to clinical trials conducted in France. It first refers to past work, and briefly describes how TRT5 was created (TRT5 is an interassociative group which defends the interests of persons suffering from HIV in clinical research). The organization of meetings, in 1993, between associative militants, methodologists and clinicians to discuss trial protocols with the support of the Agence Nationale de Recherches sur le Sida (ANRS) is an original experiment in France. Using a series of ethnographic observations made between 1994 and 1997, the article retraces the way in which, during these meetings, AIDS organizations took position on the methodology of controlled trials based on criteria of clinical effectiveness. It makes an analysis of the associative work done on this occasion on the information and consent sheets aimed at patients. Finally, it assesses the effect on associative mobilisation of current changes with regard to carrying out trials: the use of viral load as predictive markers of clinical effectiveness, and the wider access, outside trials, to molecules that are both more numerous and more effective.

[Management of drug addicts (heroin addicts) by general practitioners]. / La prise en charge des toxicomanes (héroïnomanes) par les médicins généralistes.

A telephone survey has been undertaken in a random sample of 150 General Practitioners (GPs) in four regions of France. 25% refused to answer. The study showed that drug addiction (use of heroin) is a serious issue for many of these physicians: 15% follow more than 20 drug addicts per year and only 12% see none; extrapolating the mean number of drug addicts followed yearly (9.4 per GP) to all GPs in the four regions (26,000, that is to say half of all french GPs) give an "active file" of 250,000 drugs addicts per year. Only 15% if the GPs feel they are educated for the management of drug addicts, although 70% say they prescribe medication such as hypnotics and anxiolytics (97%), the most cited being Tranxene 50 (Chlorazepate 50 mg), antalgics (84%), morphinic or morphine-like drugs (49%), mainly Temgesic, and at last antidepressants and neuroleptics (39%). GPs suffer from being isolated in their practices, as contacts with collegues or institutions specialized in drug addiction are few. If one third of the GPs wish an active participation in the management of drug addicts, and one third wish at least to "do something", they are all much ambiguous in their attitudes toward drug addiction. Their behaviors vary from medical pride to anxiety and even fear. They all mention a lot of practical problems with respect to reputation, practice, lack of time... Unanimous wishes concern opportuneness of setting up continuous medical education on this topic, of working out guidelines for prescription, of improving relationships with other institutions.

Symmetrical enchondromatosis of the hands and feet in two sisters.

We report two Portuguese sisters aged 9 and 12 years with symmetric well-circumscribed radiolucent cystic lesions on the long bone metaphysis of the hands and feet. The eldest also has soft tissue calcifications. They have no dysmorphic features and their growth is normal. Plasma values of parathyroid hormone (PTH), calcium, phosphorus, magnesium, and alkaline phosphatase are normal. Cerebral computed tomography (CT) scan shows no intracranial calcifications. A Raynaud phenomenon became evident during the last year in the eldest. The incisional biopsy of the left proximal metatarsial was performed through an area of typical radiographic appearance. The pathology specimen consisted of enchondroma tissue. The present cases are an extremely rare instance of this pathology, with symmetrical involvement of the hands and feet and a familial incidence.

[Progress in the treatment of tubal sterility: microsurgery (author's transl)]. / Un progrès dans le traitement de la stérilité tubaire: la microchirurgie.

During the last few years microsurgery has evoked general enthusiasm in several surgical specialities and especially in plastic surgery. This is also true of gynaecology, where it is used for the surgery of sterility. Tubal microsurgery improves the results of operations by ensuring that the anatomical reconstruction shall be more precise and by lessening the amount of adhesions and secondary fibrosis. The first results that have been published prove this. The superiority of these difficult techniques is especially demonstrated by tubal recanalisations effected after sterilisation when the anastomosis is made in healthy tissue, being from the isthmus to the isthmus or from the isthmus to the uterus. Microsurgery is also the sole hope for the surgery of tubes that are at present inoperable. It will effect transplantation of the tube as soon as the problem of rejection has been solved.

[Exploratory salpingotomy]. / Salpingotomie exploratrice.

One of the difficulties in tubal surgery concerning the distal portion of the tube is the accurate assessment of the value of the ampullary muscularis and mucosa on which depend the choice of the right operative procedure and the prognosis. The difficulties are most important in case of total terminal occlusion particularly when the site of the former ostium is no longer visible. An exploratory salpingotomy preceding any repair is made under the operating microscope. The simple technique that we describe allows: --an accurate assessment of the inner ampulla; --lysis of the mucosal adhesions inside the ampulla; --detection of the former ostium from the inside and the incision in the right place. The functional consequences of such an incision are difficult to appraise because the number of cases is so far too small and the follow up too short. However, those consequences seem slight when compared to the advantages, which are better precision in the investigation, the indication and the execution of the surgical repair.

[Contact embryoscopy (author's transl)]. / L'embryoscopie de contact.

Prenatal diagnosis based on anatomic definition of the fetus in utero is an difficult task, in the first trimester of pregnancy. The authors have recently introduced a new method : contact embryoscopy which consist in applying the optic piece of a contact hysteroscope (diameter of six millemeters) to the intact and translucent ovular membranes by the cervix. They have performed fifty embryoscopy prior to termination of pregnancy at 8 to 12 weeks. In 41 cases, they have seen the hands of fetus with the fingers. If this technic fails, it is possible, later, to use Echography and fetoscopy. The side-effects are discussed. Contact embryoscopy would be particularly useful in the detection of malformations of the extremities, for instance : chondro-ectodermal dysplasia (Ellis Van Creveld Syndrome) or ectrodactily.

[Vesico-uterine fistulae after Caesarean operation (author's transl)]. / Les fistules vésico-utérines après opération césarienne.

The vesico-uterine fistulae represent 4 per cent of all urogenital fistulae. They are rare and are found almost always after Caesarean operation. We have been able to find in the literature 110 vesico-uterine fistulae after Caesarean operation. They very rarely fail to be diagnosed if careful post-operative examination is made and the majority of them are discovered in the year following delivery. Urinary incontinence is the most common reason for consultation. More uncommon is amenorrhoea with menstruation through the bladder. Incontinent women are under constant threat of ascending urinary tract infection. Finally these women, whether they are incontinent or amenorrhoeic, are sterile. Their fertility remains diminished even after surgical cure of the fistula. The only treatment for those who complain of the condition is surgery. It should be undertaken after a careful gynaecological and urological assessment.

[Early control laparoscopy after tubal microsurgery (author's transl)]. / La coelioscopie de contrôle précoce apès microchirurgie tubaire.

It is essential to carry out laparoscopic control to determine the result of plastic tubal surgery. This is classically done 12 to 18 months after the operation, yet it has been suggested that this control should be carried out earlier between the 4th and the 8th weeks or even on the 8th day following the operation. The authors have carried out 63 early control laparoscopies after microsurgical operations on the tube at different dates: 33 times between 4 and 8 weeks after the operation, 5 times between the 10th and the 13th day, and 25 times on the 8th day. They are in favour of early laparoscopic control with the reserve that it will require much more experience to tell whether the procedure is harmless.