[The flexibility of family medicine]. / La flexibilité en médecine générale.

We are a European academic group of family doctors and we propose a definition of flexibility in family medicine. A review of the literature shows that flexibility and complexity are emerging concepts in the field of family practice. The outcomes of a workshop at the WONCA-Europe congress in 2014 are discussed. The flexibility is a capability of the general practitioner to deal with complex clinical situations in a biomedical and societal changing world. Flexibility is framed by ethics. It could improve the quality of care, be useful against burnout and used in medical research. In conclusion, family medicine should adopt a specific definition of the flexibility describing its specificity, a useful and teachable capacity.

[A new case of rare erythrocytosis due to EGLN1 mutation with review of the literature]. / Polyglobulie rare par mutation du gène EGLN1 : à propos d'un cas et revue de la littérature.

INTRODUCTION: The origin of polycythemia is often simple to detect. Sometimes it is necessary to look for hereditary forms, the decisive parameters being the dosage of erythropoietin and the measurement of the oxygen dissociation curve (P50). These rare diseases are related to high oxygen-affinity haemoglobins, abnormalities of the erythropoietin receptor or dysfunction of the HIF (hypoxia-inducible factor) pathway. CASE REPORT: We report the case of a 56-year-old patient with unexplained polycythemia associated with normal serum erythropoietin and normal P50, in whom the never previously described mutation c.400C>T(p.Gln134*) on exon 1 in the EGLN1 gene (encoding PHD2) was found. CONCLUSION: In the face of an unexplained polycythemia a good cooperation between clinicians and biologists is necessary to be able to characterize rare hereditary pathologies.

[Diagnostic workup in front of an atypical non hemolytic anemia]. / Démarche diagnostique devant une anémie « non hémolytique ¼ sans étiologie apparente.

The most potential causes of "non hemolytic" anemias are iron, folate or vitamin B12 deficiencies, severe renal impairment, endocrine diseases, inflammation and medullary disorders. In a non-exceptionnal way no cause is found, sometimes because of a wrong interpretation of analysis results and sometimes because of a little known etiology. The goal of this review is to point out analytical difficulties and to remember some rarer etiologies.

[A fatal septic shock due to Capnocytophaga canimorsus and review of literature]. / Infection fulminante fatale à Capnocytophaga canimorsus : à propos d'un cas et revue de la littérature.

INTRODUCTION: Capnocytophaga canimorsus is a Gram-negative bacterium present in normal dogs and cats mouths. It can be responsible for septic shocks after dog or cat bite, especially in asplenic patients. CASE REPORT: We report here a case of C. canimorsus infection rapidly presenting as a multiple organ dysfonction syndrome in an immunocompetent 54 years old patient, who lives near a dog, without any sign of bite. The evolution was a rapidly fatal fulminant septic shock. CONCLUSION: Among patients with a septic shock of unknown etiology, fatal evolution due to C. canimorsus should be avoided by systematic use of early antibiotherapy with amoxicilline and clavulanic acid, especially in patients who live near a pet, even if they are immunocompetent.

[Two cases of myeloid sarcomas with review of literature]. / Sarcome granulocytaire : à propos de 2 cas et revue de la littérature.

INTRODUCTION: Myeloid sarcomas are uncommon proliferations of immature myeloid cells occurring in any extramedullary organ. We report here two cases of myeloid sarcomas in patients with, respectively, a polycythemia vera and a myelodysplastic syndrome. CASE REPORTS: The first is an 81-year-old woman who presented with osteolytic lesions. Diagnosis has been highlighted using anatomopathological study after bone marrow biopsy, but it was delayed because of a very localized basin lesion and few positive myeloid markers. The second patient is an 86-year-old man who presented with pancytopenia and several lymph nodes. Lymph node cytology failed because of the rarity of blast cells. Diagnosis was done after anatomopathological study on lymph node biopsy which revealed a localized form of myeloid sarcoma. CONCLUSION: The diagnosis of myeloid sarcoma must be considered when unusual tumors occur in patients with a chronic myeloid disease. In that case, therapeutic options are those of an acute myeloid leukemia.

Comparative study of intravenous ketoprofen versus aspirin.

In order to assess the analgesic effect of an injectable preparation of ketoprofen, a double-blind comparative study was conducted against a soluble form of acetylsalicylic acid of known efficacy, widely used in the clinic. Only one indication was considered for inclusion in the trial, namely discogenic sciatica. We found no significant statistical difference between these two products, considering the criteria used. The time of onset of effect was, on average, 30 minutes and the mean duration of activity was 4 hours. The two products were well tolerated.

[Methods for evaluating non-steroidal anti-inflammatory agents in rheumatoid polyarthritis]. / Méthodes d'évaluation des anti-inflammatoires non stéroïdiens dans la polyarthrite rhumatoïde.

The study of the effectiveness of non-steroidal antiinflammatory drugs in rheumatoid arthritis requires only short term trials using simple, sensitive and reproducible tests based upon evaluation of pain. The estimation of spontaneous pain by the visual pain scale, number of nocturnal awakenings, Ritchie joint index, morning stiffness, grip strength and Lee functional index are the most informative parameters. The results of these quantitative tests of pain are usually in close agreement with patient judgement which remains the most important criterion.

[The cellularity of fibrillated articular cartilage. A comparative study of age-related and osteoarthrotic cartilage lesions from the human femoral head]. / Etude de la cellularité du cartilage articulaire fissuré. Comparison des lésions liées à l'âge des lésions arthrosiques de la tête fémorale humaine

The cellularity of human femoral head cartilage has been studied in age-related and osteoarthrotic fibrillated samples and control intact samples. Age-related fibrillated cartilage shows a marked increase of the cell density which is directly related to the proliferation of cell clusters and to cartilage thinning. In osteoarthrotic cartilage lesions multicellular clusters are less frequent and cell density is decreased. A smooth aspect of the exposed cartilage surface and an increased subchondral bone mass are only found in osteoar/hrotic samples. The findings suggest that age-related and osteoarthrotic cartilage lesions are due to different mechanical stresses governing the cellularity of the tissue.

[Cellular density of the femur head cartilage in relation to age]. / Etude de la densité cellulaire du cartilage de la tête fémorale en fonction de l'age

The authors studied the cell density in samples of cartilage taken from the superior and inferior poles of the femoral heads of 65 subjects between 16 and 100 years of age. This study showed that the average cell density of the cartilage decreased significantly with age and that the decrease was proportionally identical in the two poles of the femoral head. In absolute values, the decrease in superficial cell densty is much greater in the inferior than in the superior pole. The relation between the decrease in cell density with age, cartilaginous lesions of senescence, and arthrosis are discussed.

[Extensive apical pulmonary fibrosis complicating ankylosing spondylitis. 2 cases (author's transl)]. / Fibrose pulmonaire apicale extensive compliquant une spondylarthrite ankylosante. 2 cas.

The authors describe two cases of pulmonary fibrosis in patients suffering from ankylosing spondylitis. The diagnosis of tuberculosis was rejected on the basis of negative studies for tubercle bacilli and the total ineffectiveness of specific therapy. No evidence of microbial or parasitic infection was found. This fibrosis was associated with a considerable worsening in general condition. Nevertheless, the first patient remains alive 15 years after the onset of this pathological manifestation. The second patient died but autopsy was not possible. A wide review of the literature is undertaken in the light of these two cases.

Pilus formation and protein secretion by the same machinery in Escherichia coli.

The secreton (type II secretion) and type IV pilus biogenesis branches of the general secretory pathway in Gram-negative bacteria share many features that suggest a common evolutionary origin. Five components of the secreton, the pseudopilins, are similar to subunits of type IV pili. Here, we report that when the 15 genes encoding the pullulanase secreton of Klebsiella oxytoca were expressed on a high copy number plasmid in Escherichia coli, one pseudopilin, PulG, was assembled into pilus-like bundles. Assembly of the 'secreton pilus' required most but not all of the secreton components that are essential for pullulanase secretion, including some with no known homologues in type IV piliation machineries. Two other pseudopilins, pullulanase and two outer membrane-associated secreton components were not associated with pili. Thus, PulG is probably the major component of the pilus. Expression of a type IV pilin gene, the E.coli K-12 gene ppdD, led to secreton-dependent incorporation of PpdD pilin into pili without diminishing pullulanase secretion. This is the first demonstration that pseudopilins can be assembled into pilus-like structures.

[Cartilage of the head of the femoral in articular chondrocalcinosis (author's transl)]. / Le cartilage de la tête fémorale dans la chondrocalcinose articulaire.

A study of three femoral heads removed from patients suffering from articular chondrocalcinosis enabled the authors to identify the lesions of the cartilage in that disorder. They are mainly represented by crystals of calcium pyrophosphate. They appear within the cartilage which is macroscopically intact and histologically normal, but their location and the related tissue phenomena in their formation remain controversial and poorly understood. They accumulate in the various layers of the non-calcified cartilage within which they appear to hollow out genuine cavities by pushing back and then destroying the intercellular matrix and surrounding chondrocytes. In spite of their at times considerable size, the accumulations of crystals are not accompanied with fissures or erosion of cartilage tissue. This good histological tolerance appears to be explained by the distribution of calcification outside of the areas bearing pressure, which is in agreement with the often asymptomatic nature of chondrocalcinosis. These calcfications appear to be able to render cartilage fragile and to lead to pseudoarthrosic arthropathy when they are abundant and spread through the entire surface of a joint.

The cell density of human femoral head cartilage.

The human femoral head cartilage in 63 subjects with ages ranging from 16 to 100 years demonstrates that in the course of aging the cell density of the whole cartilage thickness decreases about 40 per cent. The reduction of the cellularity with age is more accentuated in the superficial than in the deeper zone and is even limited to the superficial zone during the latter part of aging. Proportionately the cell loss is similar in the superior and inferior poles of femoral head suggesting a uniform distribution of age related changes. In absolute value, the decrease of the superficial cell density is much higher in the inferior than in the superior pole; the decrease may be related to the propensity of this region to fibrillation.

[Quantitative histological study of the cartilage of human arthrotic femur head]. / Etude histologique quantitive du cartilage de la tëte fémorale humaine arthrosique

A quantitative histological study of the cartilage of the human arthrosic femoral head. In this work, samples of unsplit cartilage taken from 15 arthrosic femoral heads and 26 normal femoral heads from the same topographical areas, and of identical age, were studied by quantitative histological methods. This study was concerned with the thickness of the calcified and non-calcified cartilage, the cellular density, the average number of nuclei per lacuna, and the average surface area of the lacunae in the different layers of the cartilage. Comparison between the two groups of samples showed that the arthrosic cartilage differed from the normal cartilage by a diminution in the size of the superficial lacunae, a reduction in the number of nuclei per lacuna in the middle layer, a diminution in the cellularity of the deep layer and an augmentation in the layer of calcified cartilage. These modifications suggest that arthrosis of the hip is secondary to diffuse cellular alterations associating a diminution in metabolic activity and necrosis of the chondrocytes.

[Measurement of the variation in the thickness of lumbar disks over time in patients with lumbago]. / Mesure des variations avec le temps de l'épaisseur des disques lombaires chez les lombalgiques.

The average thickness of the last 3 lumbar disks was measured using an automatic image analyzer in 53 patients who were regularly followed-up both clinically and radiographically for an average period of 14.8 years (extremes: 6 and 39 years). This retrospective study showed that disks do not necessarily become pinched with age, even in patients with lumbago, that the rate of disk pinching is extremely variable from one patient to another, that disk collapse takes an average of 20 to 30 years, that there is a correlation between the severity of the lumbago and the rate at which the disk pinches, and that there are rapidly evolutive discopathies which are responsible for severe lumbago.

Multiple interactions between pullulanase secreton components involved in stabilization and cytoplasmic membrane association of PulE.

We report attempts to analyze interactions between components of the pullulanase (Pul) secreton (type II secretion machinery) from Klebsiella oxytoca encoded by a multiple-copy-number plasmid in Escherichia coli. Three of the 15 Pul proteins (B, H, and N) were found to be dispensable for pullulanase secretion. The following evidence leads us to propose that PulE, PulL, and PulM form a subcomplex with which PulC and PulG interact. The integral cytoplasmic membrane protein PulL prevented proteolysis and/or aggregation of PulE and mediated its association with the cytoplasmic membrane. The cytoplasmic, N-terminal domain of PulL interacted directly with PulE, and both PulC and PulM were required to prevent proteolysis of PulL. PulM and PulL could be cross-linked as a heterodimer whose formation in a strain producing the secreton required PulG. However, PulL and PulM produced alone could also be cross-linked in a 52-kDa complex, indicating that the secreton exerts subtle effects on the interaction between PulE and PulL. Antibodies against PulM coimmunoprecipitated PulL, PulC, and PulE from detergent-solubilized cell extracts, confirming the existence of a complex containing these four proteins. Overproduction of PulG, which blocks secretion, drastically reduced the cellular levels of PulC, PulE, PulL, and PulM as well as PulD (secretin), which probably interacts with PulC. The Pul secreton components E, F, G, I, J, K, L, and M could all be replaced by the corresponding components of the Out secretons of Erwinia chrysanthemi and Erwinia carotovora, showing that they do not play a role in secretory protein recognition and secretion specificity.