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von Hippel-Lindau disease is an inherited multisystemic familial cancer syndrome caused by mutations of the VHL gene. The spectrum of clinical manifestations is broad and includes central nervous system hemangioblastomas and visual benign and malignant tumors. The various manifestations can be demonstrated by means of different imaging techniques such as magnetic resonance imaging, computed tomography, and fluorescein retinal hemangiography. A systematic approach must be followed for repeated screening in patients at risk, since many lesions in VHL disease are treatable.

Identify the bacteria responsible for cutaneous infections observed in private practice and test their sensitivity to currently used antibiotics.The patients were examined by dermatologists. A bacteriological sample was taken and sent to a central laboratory for identification of the germ and antibiograms were performed and the minimal inhibiting concentrations (MIC) determined.Folliculitis, impetigo and furuncles were the three most frequent primary infections. Four hundred and forty-three patients were included and 442 samples were placed in culture. Cultures were positive in 265 cases (a single bacterial strain in 231 cases). Staphylococcus aureus was isolated in 208 cases, streptococci in 11 and enterobacteria in 3; occasionally several germs were present. Eight strains of staphylococci were meti-R (4 p. 100). All the strains were sensitive to pristinamycin and mupirocin and 90 p. 100 were sensitive to fusidic acid.The occurrence and diffusion of resistant strains is a daily concern in hospitals. In general practice, although care must be taken, the problem rarely occurs and the antibiotics used remain effective.

Pourfour du Petit syndrome, rarely reported, is the opposite of Claude Bernard-Horner syndrome.A 67 years old female is hospitalised for dysphagia, allowing the discovery of oesophagus carcinoma with mediastinal, pleural and costal extension. The discovery of left unilateral mydriasis associated with exophthalmos and eyelid retraction suggest Pourfour du Petit syndrome; this diagnosis is confirmed by CT-scan, finding pedicular lysis of high dorsal vertebras and intra-canalar tumoral extension.Pourfour du Petit syndrome has the same localisation value and the same aetiologies as Claude Bernard-Horner syndrome, but its mechanism proceeds byan exciting lesion of cervical sympathetic nervous system. The recognition of this entity can allow the diagnosis of pathologies that need emergency treatment.

In France, immediate breast reconstruction (IBR) for infiltrating carcinoma remains controversial. Many teams advocate the possible event of a post mastectomy radiotherapy and its negative effect on IBR. In our Institute we do not exclude infiltrating breast cancer patient from IBR. In the poor prognostic patients who wish IBR, we recommend autologous IBR to obtain the best aesthetic result with minimum revision procedures and best tolerance to adjuvant radiotherapy. From January 1993 to December 1997, we performed 687 IBR with myocutaneous flap for infiltrating carcinomas. In this group only 68 patients needed postoperative chest wall radiotherapy (45 Gy): 27 TRAM flap, 41 latissimus flap. Only one of the TRAM but 39 of latissimus flaps were associated with a prosthesis. The mean follow-up was 24 months. Fourteen patients developed metastatic disease, and ten were dead at the time of the chart revue. The autogenous TRAM flap tolerate radiation quite well and remain soft and mobile. The latissimus flap associated with a prosthesis developed capsular contracture (BAKER II or III) in 71% of cases. In all cases the cosmetic impairment was not important and the result after capsulectomy remained soft. We concluded that IBR could be offered to motivated patients in all stages of the disease regardless of the subsequent chest wall radiotherapy, and we recommend its use for possible autologous reconstruction.

The genotype-phenotype relationship of Lynch syndrome displays many enigmatic features which cannot be explained satisfactorily by the prevailing concepts of carcinogenesis and genetic predisposition to cancer. We propose here a new model of carcinogenesis divided into two and only two evolutive phases: a) a preliminary phase starting with the counter-selective loss of mismatch repair function, in which most clones with the RER mutator phenotype are eliminated through apoptosis or an accelerated ageing process; b) an explosive phase that is initiated only if mutations blocking apoptosis and senescence, rapidly acquired during the short life span of the non-transformed RER+ clones, eventually rescue one mismatch repair-deficient cell that gives rise to the malignant clone. Carcinogenesis is proposed here to progress irreversibly and very rapidly once initiated. We shall show how this model provides a meaningful etiologic and pathogenic interpretation of all the curious features of Lynch syndrome.

In the last twenty years, our understanding of carcinogenesis has evolved from a description of the natural history of cancer development, a conception inherited from Darwin, to the dissection of the underlying molecular mechanisms leading to phenotypic changes in cells, a neodarwinian interpretation. Most scientists agree that random germ cell mutations enhancing cell survival and reproduction capacities play a major role in the evolution of species and that carcinogenesis progresses from acquisition of genetic anomalies which alter the normal function of oncogens or antioncogens. Since the discovery of src, the first oncogen to be identified twenty years ago, and more recently the discovery of antioncogens such as p53, hopes and expectations have grown steadily, and passionately, often far beyond reason dietated by the real world of clinical medicine. Yet curiously, many clinicians hesitate to express one essential point: advances in science and technology, is not equivalent to advances in medicine. It is our responsibility to resist the illusion of progress, for this illusion turns out to have a negative impact in our patients. To paraphrase the French philosopher André Comte-Sponville, the power conferred by the exercise of medicine is not based solely on scientific knowledge, but also on human compassion.

This review focuses on the genetics of inborn predisposition to the development of cancer. The gene defects responsible for the majority of the most common syndromes of inherited predisposition to cancer have been deciphered during the last five years. These discoveries have shown that the genetics of most of these inherited forms of cancer is remarkably simple: the mechanism underlying the risk of cancer is precisely the one proposed by Knudson to explain the inherited forms of retinoblastoma. Only a small minority of the cancers are imputable to inherited mutations causing a very high risk of cancer. However, low penetrance susceptibility genes, mainly acting as environmental risk modifiers, are probably involved in the development of the majority of cancers. As much as possible, we will consider the immediate and long term possible repercussions on everyday medical practice of these advances in basic understanding of genetic cancer risks.

Cancers are the most frequent and at the same time the most complicated of somatic genetic diseases. Technical progress in the last fifteen years has enabled to analyse the acquired genetic abnormalities found in the vast majority of cancers. This molecular dissection of cancer has led to an understanding of this disease that can basically be viewed as a rupture of the balance between two class of genes, the oncogenes and the antioncogenes. This article defines the properties of these cancer genes and gives through a few examples an insight into the various mechanisms of cancerogenesis.

A 15 years old boy was admitted to the hospital for high fever, and a four month history of abdominal pain and weight loss. Clinical examination showed painful swelling of the left lumbar region. A retro peritoneal mass was revealed by tomodensitometry. There was a marked biological inflammatory syndrome without bacteriological evidence of infectious disease. Final diagnosis was performed by surgery showing a big abscess. Bacteriological culture of pus was positive for a Haemophilus actinomycetemcomitans.

Thirty-one infants and children aged 1 month to 15 years 3 months were treated with ceftriaxone once a day for the treatment of a meningitis related to Neisseria meningitidis (19 cases), haemophilus influenzae (7 cases), streptococcus pneumoniae (1 case), not identified bacteria (4 cases). All identified bacteria were sensitive to ceftriaxone. Twenty children were treated with 100 mg/kg/day, 11 with 50 mg/kg/day. CSF was sterile at the first control-generally performed 30 h after the onset of treatment-in all cases. Despite a great number of severe forms (fulminans purpura and septic shock; 11 cases; severe neurologic disturbances: 6 cases), all patients survived and recovered after a treatment of 9 to 22 days. Two infants exhibited neurologic sequelae: deafness, delayed development and hydrocephalus. Tolerance to ceftriaxone appeared to be good. With a 100 mg/kg/day dosage, mean CSF level at 6 h was 3.3 mg/l (0.8-7.7), on the first day of treatment. At the end of treatment, mean CSF level at 24h was 0.47 (0.15-2.5). With a 50 mg/kg/day dosage, mean CSF level at 6 h was 2,1 mg/l (1.1-3.9) in the first day of treatment. At the end of the treatment, mean CSF level at 24h was 0.22 mg/l (0.08-0.5). Once a day administration of ceftriaxone is adequate for the treatment of meningitis in infants and children. Though a 50 mg/kg/day dosage is probably sufficient in most cases, it seems to be more secure to use a 100 mg/kg/day dosage.