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The improvement of ultrasound scan techniques is enabling ever earlier prenatal diagnosis of developmental anomalies. In France, apart from cases where the mother's life is endangered, the detection of 'particularly serious' conditions, and conditions that are 'incurable at the time of diagnosis' are the only instances in which a therapeutic abortion can be performed, this applying up to the 9th month of pregnancy. Thus numerous conditions, despite the fact that they cause distress or pain or are socially disabling, do not qualify for therapeutic abortion, despite sometimes pressing demands from parents aware of the difficulties in store for their child and themselves, in a society that is not very favourable towards the integration and self-fulfilment of people with a disability. Cleft lip and palate (CLP), although it can be completely treated, is one of the conditions that considerably complicates the lives of child and parents. Nevertheless, the recent scope for making very early diagnosis of CLP, before the deadline for legal voluntary abortion, has not led to any wave of abortions. CLP in France has the benefit of a exceptional care plan, targeting both the health and the integration of the individuals affected. This article sets out, via the emblematic instance of CLP, to show how present fears of an emerging 'domestic' or liberal eugenic trend could become redundant if disability is addressed politically and medically, so that individuals with a disability have the same social rights as any other citizen. [ABSTRACT FROM AUTHOR]

Competing Interests: Declaration of Competing Interest None.

The particular situation of the French administrative detention center (ADC) medical units appears to be an exemplary case to study the difficulties facing medical practice. Indeed, the starting point of our inquiry was an amazing observation that needed to be addressed and understood: why are professional interpreters so seldom requested in ADC medical units, where one would expect that they would be "naturally" present? Aiming to fully explore the meanings of the "absent interpreter", this article takes into account the possible meanings of this situation: the recourse to professional interpreters in France is far from expected given cumulative evidence of its benefits; perceptions of illegal immigrants and medical habitus itself may both hamper the use of a third party; the ADCs are a very stressful place for healthcare professionals, with conflicting missions, political issues enmeshed with medical goals, and heavy affective burden that may lead to self-protection. Silencing voices of suffering others might be seen as the hidden indecent truth of the "absent interpreter". These reflections open a window to a larger issue with regard to the full range of medicine: what are the place, the role and the function of patient's words and narratives in contemporary medicine? The highly invested somatic perspective and its political corollary giving primacy to bare life harbor potential risks of obscuring speeches and undervaluing narratives.

Background: Little data is currently available in the literature on neutropenia and agranulocytosis in the elderly, and, to our knowledge, idiosyncratic drug-induced agranulocytosis is particularly poorly covered, or not at all., Objective: We herein describe the clinical picture and outcome of patients aged ≥75 years with established idiosyncratic drug-induced agranulocytosis., Patients and Methods: Data from 61 patients over 75 years old with idiosyncratic drug-induced agranulocytosis were retrospectively reviewed. All cases were extracted from a cohort study on agranulocytosis (n = 203) in the Strasbourg University Hospitals (Strasbourg, France), a referral center., Results: The mean age was 84.9 years (range 75-95), the gender ratio (F/M) was 2.4. Underlying diseases were present in 74 %. The most frequent causative drugs were antibiotics (43.8 %), antithyroid drugs (15.8 %), neuroleptic and anti-epileptic agents (12.3 %), and antiaggregant platelet agents (10.5 %). The primary clinical features during hospitalization included isolated fever (27.6 %), septicemia or septic shock (24.1 %), and pneumonia (20.7 %). The mean neutrophil count at nadir was 0.15 × 10 9 /L (range 0-0.4). All febrile patients were treated with broad-spectrum antibiotics and 36 with hematopoietic growth factors. Outcome was favorable in 85.3 % of patients; nine patients died. Two elderly patients (3.3 %) died of uncontrolled septic shock relating to the depth of the neutropenia. Comparison of mortality between <75- and ≥75-year-old patients revealed a statistical difference: 4.2 % versus 14.8 % (p = 0.023)., Conclusions: Our study demonstrates that 30 % of idiosyncratic drug-induced agranulocytosis concerned elderly patients. Antibiotic, antithyroid, neuroleptic, anti-epileptic, and antiaggregant platelet agents are the primary causative drug classes. Idiosyncratic drug-induced agranulocytosis is typically serious in this frail population of elderly patients, with at least 50 % suffering from severe sepsis and with a mortality rate of approximately 15 %. Modern management of agranulocytosis may reduce the infection-related mortality (3.3 %)., Competing Interests: Compliance with Ethical StandardsFundingNo sources of funding were used to assist the preparation of this manuscript.Conflicts of interestAll authors (R. Mourot-Cottet, F. Maloisel, O. Keller, F. Séverac, T. Vogel, M. Tebacher, JC. Weber, G. Kaltenbach, JE. Gottenberg, B. Goichot, J. Sibilia, AS. Korganow, R. Herbrecht and E. Andrès) declare that they have no conflicts of interest directly relevant to the content of this manuscript. F. Maloisel and E. Andrès are recipients of a grant from Chugai, Amgen, Roche, and GSK, but these sponsors had no part in the research or writing of the present manuscript.

Ileal neuroendocrine tumors are slow-growing grade 1 or, more rarely, grade 2 neuroendocrine tumors which, however, are frequently metastatic to regional lymph nodes and the liver. A few cases of ileal neuroendocrine tumors that are metastatic to the breast have also been reported in the medical literature. The knowledge of this uncommon clinical presentation is of great importance because it needs to be differentiated from primary breast carcinomas with neuroendocrine features, which represent completely different entities with a different therapeutic approach. The diagnosis of a breast metastasis from an ileal neuroendocrine tumor and its distinction from a well-differentiated primary neuroendocrine tumor of the breast is a challenging task for clinicians and pathologists. This workup is particularly difficult when the breast lesion is the first sign of malignancy. In the present paper, we describe the clinicopathological features of an ileal neuroendocrine tumor first presenting with a breast metastasis in a 50-year-old woman and we discuss the key diagnostic features for the differential diagnosis with primary well-differentiated neuroendocrine tumor of the breast. Moreover, we have reviewed the medical literature to give the reader a comprehensive overview on this topic.

The improvement of ultrasound scan techniques is enabling ever earlier prenatal diagnosis of developmental anomalies. In France, apart from cases where the mother's life is endangered, the detection of "particularly serious" conditions, and conditions that are "incurable at the time of diagnosis" are the only instances in which a therapeutic abortion can be performed, this applying up to the 9th month of pregnancy. Thus numerous conditions, despite the fact that they cause distress or pain or are socially disabling, do not qualify for therapeutic abortion, despite sometimes pressing demands from parents aware of the difficulties in store for their child and themselves, in a society that is not very favourable towards the integration and self-fulfilment of people with a disability. Cleft lip and palate (CLP), although it can be completely treated, is one of the conditions that considerably complicates the lives of child and parents. Nevertheless, the recent scope for making very early diagnosis of CLP, before the deadline for legal voluntary abortion, has not led to any wave of abortions. CLP in France has the benefit of a exceptional care plan, targeting both the health and the integration of the individuals affected. This article sets out, via the emblematic instance of CLP, to show how present fears of an emerging "domestic" or liberal eugenic trend could become redundant if disability is addressed politically and medically, so that individuals with a disability have the same social rights as any other citizen.

The evolution of our health care system strengthens multidisciplinary collaboration in medical practice. Today, the tumor board meeting (TBM) has become mandatory for treatment decision-making in oncology. Experts recommend that such a multidisciplinary tool be extended to prophylactic mastectomy. We will discuss the relevance of this extension on the basis of a survey with female patients and health professionals confronted with prophylactic mastectomy. Health professionals find the TBM necessary because it allows more reasoned and consensual proposals to be made, reduces the burden of difficult decisions, and promotes a comprehensive view of those who receive care. However, this approach has also drawn criticism. Unlike medical consultation, which focuses on a patient's singularity, the TBM seems poorly suited to take into account psychosocial factors governing the decision-making process. It may be easier for a group to reach a complex decision, but it does not mean that the TBM allows for decisions to be adapted on a singular basis. Therefore, we suggest to modify the TBM so that it better suits the needs of patients susceptible to breast cancer. The referring physician, who knows the patient best, should guide the content of multidisciplinary deliberations and put the focus on individual criteria raised during medical consultations.

It is time to challenge the issue of pleasure associated with the core of medical practice. Its importance is made clear through its opposite: unhappiness--something which affects doctors in a rather worrying way. The paper aims to provide a discussion on pleasure on reliable grounds. Plato's conception of techne is a convenient model that offers insights into the unique practice of medicine, which embraces in a single purposive action several heterogeneous dimensions. In Aristotle's Ethics, pleasure appears to play a central role for action's assessment and intensification. Pleasure is also tightly associated with the Kantian faculty of reflective judgment, which operates at the heart of clinical reasoning. Indeed, practicing medicine means to deal with the particular and the manifold, requiring clinical judgment, but also relying on embodied habitus. With Bourdieu's notion of habitus, pleasure is the mark of a happy practice, which presupposes a deep involvement in one's field. Throughout our inquiry, the question of pleasure comes to offer a critical reappraisal of real medical practice and leads to consider ethics more as a component of techne than as a separate realm of concern.

Chronic meningococcaemia is an unusual clinical presentation of Neisseria meningitidis infection. We describe the case of a patient, who presented with total IgA deficiency and partial IgM deficiency with a low switched memory B cells count, suggestive of a borderline form of common variable immunodeficiency (CVID). The role of IgA in the protection against Neisseria meningitidis, and the link between IgA deficiency and CVID are discussed.

Aim: We studied the pathogenesis of puffy hand syndrome of intravenous drug use. We hypothesized that injections of high-dose sublingual buprenorphine, instead of the recommended sublingual administration, could play an important role in lymphatic obstruction and destruction., Design and Participants: We set up a case-control study in substitution centres, recruiting intravenous drug addicts with and without puffy hands, respectively. The subjects were asked to answer anonymously a questionnaire of 40 items comprising social and demographic status, history of illicit drugs use, buprenorphine misuse and injection practices., Findings: We included 33 cases and 33 controls, mean age of 34 years. They were past heroin users, mainly methadone-substituted. In multivariate analysis, sex (women) (OR = 8.9, P = 0.03), injections in the hands (OR = 5.9, P = 0.03), injections in the feet (OR = 6.5, P = 0.01) and the absence of tourniquet (OR = 7.0, p = 0.02) were significant risk factors for puffy hand syndrome. In 69.7% of the cases and 59.4% of the controls, respectively, there was a high-dose sublingual buprenorphine misuse, although it appeared not to be a significant risk factor for puffy hand syndrome., Conclusions: Injection practices are likely to cause puffy hands syndrome, but buprenorphine misuse should not be considered as a significant risk factor. However, intravenous drug users must still be warned of local and systemic complications of intravenous drug misuse.

Surgical resection remains the curative procedure for liver tumors, but even with improvements in method it is still a major operation with significant morbidity and mortality in experts' hands, and a long learning curve for those surgeons who undertake it. Recently radiofrequency ablation has gained some credibility as an alternative method of dealing with liver tumors deemed unresectable. A novel technique of liver resection assisted by the application of radiofrequency is described here. A patient with colorectal liver metastases underwent a segment II/III liver resection with this technique. Following laparotomy, the tumor was identified with intraoperative ultrasound and a 'cooled-tipped' radiofrequency probe was used to ablate liver parenchyma 2cm away from the edge of the tumor. To achieve full thickness of radiofrequency ablation, several insertions were applied. The effect of radiofrequency on liver parenchyma was monitored with an intraoperative ultrasound by micro-bubbles generated by radiofrequency ablation. The length of the resection was 45 min with a blood loss of 30mL. The patient was discharged on the 6th postoperative day without complications. In this report we indicate how the use of radiofrequency ablation can be combined with standard surgical resection of liver cancers to provide a quick, and relatively bloodless operation that is likely to reduce morbidity and mortality and is easy for its practitioners to learn.

Purpose: To determine the role of the catalytic subunit of human telomerase reverse transcriptase (hTERT) in predicting survival after resection of hepatic colorectal metastases (CRM)., Patients and Methods: Two hundred one patients who underwent curative resection of hepatic CRM between 1990 and 2000 were identified from a multicenter database. The CRM were analyzed for hTERT nucleolar expression by standard immunohistochemical techniques. hTERT expression and known clinicopathologic factors of survival were examined., Results: With a median follow-up of 80 months, 152 patients (75.6%) had died; the 5-year overall survival was 30.7%. On univariate analysis, number of metastases greater than two (P = .0005), extrahepatic disease (P = .0054), disease-free interval less than 12 months (P = .006), carcinoembryonic antigen level greater than 200 ng/mL (P = .0071), and positive hTERT nucleolar staining (P < .0001) were associated with decreased survival. On multivariate analysis, three factors independently predicted survival: number of metastases (relative risk [RR] = 1.74; P = .0011); disease-free interval (RR = 1.70; P = .0035); and positive hTERT nucleolar staining (RR = 2.03; P < .0001). Patients with none or one of these factors had a 5-year survival rate of 48%, whereas those with two or three of these factors had a 5-year survival of 15% (P < .0001)., Conclusion: hTERT nucleolar expression is associated with worse survival after resection of hepatic CRM. hTERT expression in conjunction with number of hepatic metastases and disease-free interval may permit more accurate prediction of survival after resection of hepatic CRM.

Hypothesis: Pancreaticogastrostomy (PG) is associated with a lower relaparotomy rate following pancreaticoduodenectomy (PD) than pancreaticojejunostomy (PJ)., Design: Retrospective clinical trial., Setting: Department of digestive surgery and transplantation., Patients: Between 1987 and 2001, 250 consecutive patients underwent PD in our institution. Among them, 83 patients underwent PJ and 167, PG., Main Outcome Measures: Preoperative clinicopathological features, intraoperative parameters, in-hospital mortality, postoperative morbidity, pancreatic fistula (PF), relaparotomy rates, and length of hospital stay were analyzed and compared between 2 reconstructive methods, PJ and PG, after PD., Results: The morbidity rate, including PF, was lower in the PG group (38.3%) than in the PJ group (53.0%; P =.02). The mortality rate did not differ between the PG group (2.9%) and PJ group (2.4%). Conversely, the incidence of PF and the mean +/- SD length of hospital stay were significantly lower in the PG group (2.3% and 17.2 +/- 7.7 days) than in the PJ group (20.4% and 23.3 +/- 11.7 days; P<.001 for both variables). Moreover, the overall relaparotomy rate was significantly lower in the PG group (4.7%) than in the PJ group (18.0%; P =.001). Nine (52.9%) of 17 patients with PF in the PJ group underwent relaparotomy. These 9 patients underwent subsequent completion pancreatectomy (n = 7) or removal of peripancreatic necrotized tissue (n = 2) with a postoperative mortality rate of 22.2%. However, no patient required relaparotomy for PF in the PG group because medical therapy succeeded in all 4 patients with PF. Moreover, no mortality related to PF occurred in the PG group., Conclusion: The PG procedure is a safe method of reconstruction after PD, with a significantly lower rate of PF and relaparotomy.

Hepatic resection (HR) is the treatment of choice for small hepatocellular carcinoma (HCC) in a noncirrhotic liver, whereas liver transplantation (LT) offers better results in patients with impaired hepatic function (Child B and C). However, it is still debated whether HR or LT is the best strategy for patients with Child A cirrhosis. We conducted a retrospective study on 37 consecutive patients with Child A cirrhosis and small HCC, treated between 1991 and 1999. Seventeen of these patients, who underwent LT, were compared with 20 patients who underwent HR, and prognostic factors for survival and tumor recurrence were analyzed. The primary endpoints were the intention-to-treat, 3- and 5-year survival, and 3- and 5-year recurrence-free survival. Three- and 5-year patient survival rate both were significantly (P =.04) higher in the LT group (87% and 71%, respectively) than in the HR group (67 and 36% respectively). Similarly, the 3- and 5- year recurrence-free survival rates were 87% and 80% for the LT group, and 52% and 40% for the HR group (P =.03). Absence of microscopic vascular invasion was the only other prognostic factor correlated with significantly better recurrence-free survival (P =.02). Therefore, we concluded that in patients with Child A cirrhosis and small HCC, liver transplantation resulted in better overall and disease-free survival than HR.

Background: Determination of tumor clonality has implications for molecular characterization and the optimal treatment of cancer. Allelotyping allows detection of the two alleles, maternal and paternal, and provides additional information regarding clonal genetic defects. The presence of allelic imbalances (AI) in tumors is a general event, but is not necessary at the same allele (alternative AI). The authors' goal was to determine whether the presence of alternative AI (AA) was a marker of heterogeneity and prognosis., Methods: To further analyze the heterogeneity of lung tumors, tumor DNA released in the plasma was compared with primary tumor DNA from 24 lung carcinoma patients. The comparison was performed by allelotyping using 12 microsatellites targeting 9 chromosomal regions, taking in each case leukocyte DNA as reference. To extend and confirm these observations, 26 primary colorectal carcinomas with paired synchronous liver metastasis were analyzed using an enlarged panel of 33 microsatellites., Results: AA were observed in 40% (20 of 50) of all patients, in 25% (6 of 24) of lung carcinoma patients but at a higher level, and in 54% (14 of 26) of colorectal carcinoma patients. They affected different chromosome localizations and each tumor stage. In both types of cancer, patients with AA had a higher AI mean frequency in their primary tumor., Conclusions: Detection of AA is an original marker of heterogeneous tumors, demonstrating that independent events occurred on specific genetic sites required for cancer progression., (Copyright 2003 American Cancer Society.DOI 10.1002/cncr.11324)

Background: Portal vein embolization (PVE), proposed to induce contralateral hepatic hypertrophy before major hepatectomy, carries some negative side effects since growth rate of metastases in the future remnant liver (RL) can be more rapid than that of nontumoral liver parenchyma. Therefore, metastases in the RL should be ideally resected before PVE, and a major hepatectomy can then be performed after PVE in patients with multiple bilobar colorectal liver metastases (MBLM). The aim of this study was to assess feasibility and outcome in patients with initially unresectable colorectal liver metastases treated by a one- or two-stage hepatectomy procedure (TSHP) combined with PVE., Patients and Methods: From December 1996 to December 1999, 180 patients with colorectal liver metastases underwent hepatectomy. During the same period, 18 were initially considered as unresectable. TSHP combined with PVE was attempted for 7 patients (group A) among those with MBLM, and a one-stage hepatectomy after PVE was attempted in another group of 11 patients (group B) among those with non-MBLM., Results: Nonanatomical resections for left liver metastases were performed as a first stage without any complications in group A. A right hepatectomy (RH) was performed in 5 patients in group A (feasibility = 71%). In group B, 7 of the 11 patients underwent a RH or an extended RH after PVE (feasibility = 64%). Postoperative complications rate did not differ between group A and B. Mortality was nil. Three-year survival rate was 53% in group A and 100% in group B., Conclusions: These results suggest that one- or two-stage hepatectomy combined with PVE can be applied safely to selected patients initially considered as unresectable. Three-year survival was similar to that observed in patients with initially resectable liver metastases.

Systematic allelotyping of colorectal cancers and liver metastases is realized since 1996 in a close collaboration between the surgery, pathology departments and the molecular biology laboratory. Using amplification of 35 different microsatellites allelic imbalances and microsatellites instabilities were recorded. Beside the well documented increasing amount of genomic rearrangements we identified a subset of early stages tumors presenting a profile of rearrangement corresponding to high grades, and high grades cancers with a molecular profile corresponding to low grade. The comparison of synchrones liver metastases and colorectal tumors allowed us to propose for a more extensive studies the existence of sensitive structural chromosomic regions affecting separately both chromosomes and that it should be possible to identify a limited number of specific genes concerned by most of the advanced stages.

Liver resection is the only curative option offering long-term survival in patients with colorectal liver metastases (25 to 40% five-year survival). It can be achieved with low mortality and low morbidity. However, this surgical approach can be offered only for approximately 10 to 20% of patients with colorectal liver metastases. Therefore, 80 to 90% of patients are excluded from liver surgery and will receive palliative therapies. Recent advances have selected subgroups of patients presenting initially unresectable disease to achieve curative resection. These new multidisciplinary strategies were developed in order to increase safely the resecability in patients with initially non-resectable liver metastases and to improve treatment of recurrences in patients with isolated liver metastases either by repeat hepatectomies or local tumor destruction. These strategies offer the same survival than observed in patients with initially resectable liver metastases. Our series includes 438 patients operated on for colorectal liver metastases between 1987 and 2002. Overall mortality was 1.1%, morbidity was 26%. Actuarial 5-year and 10-year survival were respectively 29.6% and 20.1%.

Background: We investigated whether hepatic pedicle lymph node (HP-LN) involvement is a more significant prognostic factor and whether HP-LN dissection could be efficient in patients with positive HP-LN involvement., Methods: From 1988 to 1998, HP-LN dissection was prospectively performed in 160 patients undergoing hepatectomy for colorectal liver metastases. Survival of patients with HP-LN involvement limited to the hepatoduodenal ligament and retropancreatic portion (area 1) was compared with that of patients with HP-LN involvement spreading over the common hepatic artery and celiac axis (area 2)., Results: HP-LN involvement was detected in 17 patients. The survival rate was significantly lower in patients with HP-LN involvement. HP-LN involvement was the most significant prognostic factor. Survival was significantly higher in patients with HP-LN involvement limited to area 1 than in those with HP-LN involvement spreading over area 2., Conclusions: HP-LN involvement was the most significant prognostic indicator in patients with colorectal liver metastases. Positive LNs of area 1 should no longer be considered an absolute contraindication to liver resection, but in case of area 2 lymph node involvement, liver resection does not seem justified.

Purpose: Elderly patients with nonchemotherapy drug-induced agranulocytosis present commonly with severe infections, and have a mortality of at least 20%. We studied whether granulocyte colony-stimulating factor (G-CSF), a hematopoietic growth factor that shortens the duration of neutropenia, is useful in these patients., Subjects and Methods: We studied 54 patients > or =65 years of age who had drug-induced agranulocytosis, some of whom had been treated with G-CSF. We determined the times until hematologic recovery (defined as a neutrophil count >1.5 x 10(9)/L), tolerance of G-CSF, and clinical outcomes., Results: Of the 54 patients, 20 received G-CSF. Two patients who had not been treated with G-CSF died of uncontrolled septic shock and extensive pneumonia. The mean (+/- SD) time until hematologic recovery was significantly less in patients treated with G-CSF (6.6 +/- 3.9 days vs. 8.8 +/- 4.9 days, P <0.04). Compliance with G-CSF therapy was good; only mild flu-like symptoms and transient bone pain were reported in 12 patients., Conclusion: Our findings suggest that G-CSF therapy may be beneficial in the management of drug-induced agranulocytosis in elderly patients.

Background/aims: To obtain a margin-negative resection and increase the indication for resection of periampullary malignancies, pancreaticoduodenectomy with a SM-PVR (superior mesenterico-portal vein resection) has been performed. However, an arterial resection, other vascular resections except SM-PVR (e.g., an inferior vena caval resection), or a metastatic tumor resection combined with pancreaticoduodenectomy has yet to be fully elucidated because of the high risk of postoperative complications and extremely poor long-term survival in patients undergoing these exceptional procedures. The present report focused on highly selected patients undergoing an arterial resection or a vena caval resection associated with pancreaticoduodenectomy., Methodology: Besides 31 patients with periampullary tumors undergoing pancreaticoduodenectomy associated with SM-PVR in our department, a group of 4 patients underwent arterial resections and another patient underwent pancreaticoduodenectomy combined with a resection of liver metastasis together with an inferior vena caval resection. These five patients were reported in the present study., Results: A 27 year-old-woman presented pancreatic ductal adenocarcinoma of the pancreatic head and a liver metastasis in which involvements of the superior mesenterico-portal vein and the inferior vena cava were shown. Pancreaticoduodenectomy was performed with SM-PVR associated with a left hemihepatectomy combined with a segment 1 resection and an inferior vena caval resection. The patient did not present severe postoperative complications and experienced a good quality of life during 16 months after surgery. Four other patients underwent arterial resections. These arterial resections were performed only when a margin-negative resection was feasible. The superior mesenteric artery was resected and reconstructed with a Goretex graft in one patient. The right hepatic artery was resected and reconstructed with a saphenous graft in two patients. The other patient underwent a resection of the common hepatic artery and reconstruction was performed with the splenic artery. Three of the four patients presented postoperative complications but were conservatively treated. Two patients are still alive 25 months and 8 months after surgery. One patient died of sepsis 5 months after surgery, and the other died of cancer progression 19 months after surgery., Conclusions: The indication for retropancreatic arterial resection associated with pancreaticoduodenectomy should be carefully evaluated only when a margin-negative resection can be achieved. An appropriate bypass method of arterial reconstruction should be selected because a direct end-to-end anastomosis is not always feasible. Hepatectomy for metastases of pancreatic ductal carcinoma should be also regarded as an exceptional procedure.