[Right paraduodenal hernia: a rare cause of intestinal obstruction]. / Hernie paraduodénale droite: une cause rare d'occlusion intestinale.

A 17 year old male was admitted emergently with acute small bowel obstruction. An urgent laparotomy revealed a loop of gangreous ileum herniated through a right paraduodenal hernia. The compromised bowel was resected and a primary anastomosis was performed. This case report allows us to discuss the diagnostic and therapeutic features of this rare condition.

Health related quality of life in people with hereditary neuromuscular diseases: an investigation of test-retest agreement with comparison between two generic questionnaires, the Nottingham health profile and the short form-36 items.

The present work attempts to define reproducibility, test-retest and internal consistencies of two standardised tools that measure health related quality of life (HRQoL), specifically as they apply to hereditary neuromuscular disease (HNMD): the Nottingham health profile (NHP) and the medical outcome study 36-item short-form questionnaire (MOS SF-36). A cross sectional survey of 108 hereditary neuromuscular disease patients completed the questionnaires consecutively in the course of multidisciplinary consultations in Reims between April 2002 and February 2005. The results of the study confirm the acceptability of using generic questionnaires such as the Nottingham health profile and the SF-36, and show good reliability for these instruments. For both instruments, reproducibility (test-retest) appears excellent for the physical dimensions explored, and satisfactory for the mental dimensions. There is nonetheless a need for health related quality of life measures validated for neuromuscular disease patients. Health related quality-of-life (HRQoL) measures provide information on how patients assess their health and the care provision they are offered.

[Family caregivers and hereditary muscular disorders: association between burden, quality of life and mental health]. / Les aidants familiaux des personnes myopathes: liaisons entre charge de soins, qualité de vie et santé mentale.

OBJECTIVE: The analysis of caregiver difficulties seems important to define the life of the patient. The aim of the study was to evaluate the burden of perceived care, health-related quality of life, and mental health of family caregivers who care for patients with hereditary neuromuscular disease. MATERIALS AND METHOD: Fifty-nine family caregivers were investigated in the course of multidisciplinary consultations in Reims, France, between April 2002 and February 2005. Burden of perceived care, mental functioning and mental health were measured by answers to an burden interview instrument (Zarit Burden Inventory, ZBI), a health-related quality of life questionnaire (the SF-36 and General Health Questionnaire-12 items [GHQ-12]), and an instrument quantifying anxiety and depression (Hospital Anxiety and Depression scale [HAD]). The domain scores of the SF-36 were compared with those of the general population. Non-parametric correlations between scores were calculated. RESULTS: Thirty-five parents, 20 spouses, a sister, a grandmother and two friends cared for patients with hereditary myopathy. The average age of caregivers was 50+/-11 years; 81% were women. Ten percent of the caregivers had above-normal scores on the HAD scale, One-third perceived a significant reduction in health-related quality of life, and more half expressed a moderate or important burden according to their answers on the ZBI. Depression occurred in a great proportion of the caregivers, and they showed reduced health-related quality of life as compared with the general population. CONCLUSION: Taking into account these results, it appears useful to set up a specific support for family caregivers of patients with hereditary myopathy.

In vitro study of THP-doxorubicin retention in human leukemic cells using confocal laser microspectrofluorometry.

Microspectrofluorometry allows the analysis of fluorescent molecules such as anthracyclines in the nucleus of isolated living cells. Using this technique, we confirmed that the amount of doxorubicin or THP-doxorubicin incorporated into the nucleus was related to the resistant or sensitive character of K562 cells. It was then extended to the study of fresh leukemic cells and kinetic studies were performed allowing the calculation of the retention rate (RR) of anthracycline (THP-doxorubicin) into the cell nucleus. A reproducibility study confirmed the accuracy of the method. Blast cells collected in patients with acute myeloid (n = 22) or lymphoid (n = 8) leukemia, at diagnosis (n = 26), or in relapse (n = 4) have been studied. RR varied from 8 to 98% independently of the type of leukemia or the clinical status. RR did not correlate either with P-glycoprotein or with CD34 expression although this latter result should be confirmed on a higher number of subjects. Among 18 patients presenting with AML at diagnosis, 14 have been treated with intensive chemotherapy including anthracyclines; the only one who had resistant disease had the lowest RR value. In conclusion, the results obtained here show that microspectrofluorometry allows the performance of kinetic studies on fresh leukemic cells in order to quantify chemo-resistance phenomena related to drug transport.

Intranuclear concentration measurements of doxorubicin in living leucocytes from patients treated for a lympho-proliferative disorder.

The accumulation of doxorubicin (DOX) in white blood cells of treated patients has been studied by quantitative microspectrofluorometry. From blood samples of treated patients, leucocyte subpopulations were separated by the gradient method. Emission fluorescence spectra from a microvolume of a single living cell nucleus were analysed in terms of spectral shape and fluorescence yield between free and DNA-bound doxorubicin. With this non-destructive analysis technique, intranuclear doxorubicin concentrations were determined within +/- 10%. Doxorubicin concentrations were measured in patients treated with bolus injection. After an accumulation of DOX in leucocytes during the first 30 min, intranuclear doxorubicin concentration did not vary significantly for 24 h, whereas its concentration in plasma decreased. Despite large differences between patients, monocytes accumulated significantly more doxorubicin than granulocytes or lymphocytes did.

Combination chemotherapy with cisplatin, etoposide and gallium chloride for lung cancer: individual adaptation of doses.

Twelve inoperable lung cancer patients were treated with a combination chemotherapy of cisplatinum (CDDP) and etoposide (VP16), as a continuous infusion for 5 days, every 21 days, and with a daily oral administration of GaCl3. Dosages of CDDP and VP16 were adapted in order to obtain an area under the curve (AUC) of 80,000 micrograms l-1.h for plasma total platinum and of 200 mumol.l-1 h for plasma VP16 during each 120 h infusion. GaCl3 was given at the dosage of 400 mg/24h from the time of diagnosis at least until the evaluation after 3 courses of chemotherapy. An objective response was observed in 5 non small cell (NSCLC) lung cancer patients (group 1) and 3 small cell (SCLC) lung cancer patients (group 2). In the other 4 patients with a NSCLC no partial response was noted (group 3). No significant difference in area under the curve (AUC) was noted between the 3 groups, either for plasma total platinum (group 1 = 89,598 +/- 20,843 micrograms l-1.h; group 2 = 88,081 +/- 15,431 micrograms l-1.h; group 3 = 83,820 +/- 13,455 micrograms l-1.h), or for VP16 (group 1 = 227 +/- 41 mumol.l-1 h; group 2 = 217 +/- 29 mumol.l-1.h and group 3 = 211 +/- 30 mumol.l-1.h). The maximal plasma Ga concentrations were 244 +/- 34 micrograms/l in group 1, 112 +/- 57 micrograms/l in group 3 (p less than 0.005) and 243 +/- 132 micrograms/l in group 2. It was then decided to increase the dose of GaCl3 in the further non-responding patients. In 6 responders, 3 additional courses of this combination chemotherapy could have been given without major toxicity, allowing a much more important decrease in the tumor volume in 4 of them. This schedule of treatment should permit the chemotherapy to continue for longer than 6 courses, in order to improve the survival time.

A new "no-touch" technique for the laparoscopic treatment of gastric stromal tumors.

BACKGROUND: Gastric stromal neoplasms are rare, accounting for < 2% of gastric tumors. Definite criteria for the malignant nature of such tumors are difficult to establish. Although their laparoscopic management has been described, there is still debate as to how to handle these tumors intraoperatively. METHODS: We report a new technical modification of laparoscopic resection used in two gastric stromal tumors, with special precautions taken to avoid the operative dissemination of unsuspected malignancy. RESULTS: The operative course and postoperative follow-up were uneventful. In both cases, histology showed no features of malignancy. CONCLUSION: To avoid tumor seeding during the resection of gastric stromal tumors, preventive measures--including absence of manipulation of the tumor, elimination of direct contact with the abdominal wall, and avoidance of disruption of the mucosa--should be implemented.

Expression of stromelysin 3 and tissue inhibitors of matrix metallo-proteinases, TIMP-1 and TIMP-2, in rheumatoid arthritis.

Rheumatoid hyperplastic synovial lining cells and sublining fibroblasts are known to produce, in vivo and in vitro, matrix metallo-proteinases which degrade extracellular matrix components of joints. We have studied by immunohistochemistry and in situ hybridization the presence of matrix metallo-proteinase stromelysin 3 and its potential inhibitors TIMP-1 and TIMP-2 in 5 cases of normal synovia, 5 cases of chronic synovitis and 12 cases of rheumatoid arthritis. Few hyperplastic synoviocytes and some sparse fibroblasts have been found to produce stromelysin 3 in all rheumatoid arthritis and 2 chronic synovitis. Stromelysin 3 seems to have a limited role in the destructive process of extracellular matrix. TIMP-1 and TIMP-2 were largely expressed principally in hyperplastic synoviocytes and in endothelial cells of all rheumatoid synovitis and 2 chronic synovitis. These findings plead for a balance between matrix metallo-proteinases and their inhibitors in these inflammatory lesions.

[Treatment of tumors of the small intestine in Peutz-Jeghers' syndrome. Value of a combined endoscopic and surgical approach. Apropos of 2 familial cases, one of which with a 15-year follow-up]. / Traitement des tumeurs du grêle du syndrome de Peutz-Jeghers. Intérêt d'une approche combinée, endoscopique et chirurgicale. A propos de 2 cas familiaux, dont l'un suivi 15 ans.

We report two cases of Peutz-Jeghers syndrome who underwent intraoperative enteroscopy. In the first case, the fiberscope was sterilized with ethylene oxide, while in the second, an electronic colonoscope was decontaminated with glutaraldehyde plus phenate. The endoscope was introduced through an enterotomy. This led to the discovery of several hamartoma greater than 10 mm which had gone undetected by the surgeon and guided the surgical procedure. In a case with approximately 30 hamartoma, associated endoscopic polypectomy and surgical removal of polyps by eversing the mucosa through enterotomies allowed the medicosurgical team to obtain a "clean small bowel" without resection.

[Invalidating polyarthritis in chronic pancreatitis. Recovery by pancreaticojejunostomy]. / Polyarthrite invalidante au cours d'une pancréatite chronique. Guérison par wirsungo-jéjunostomie.

A 38 year-old man presented with migratory joint arthropathy. He complained of abdominal pain, diarrhea and weight loss for 2 years. Periarticular needle aspiration yielded cytosteatonecrosis. The diagnosis of chronic pancreatitis was based on the results of ultrasound, CT scan, and endoscopic retrograde pancreatography. The latter showed a dilated and moniliform main pancreatic duct. Failure of symptomatic medical treatment of arthritis led to perform pancreaticojejunostomy which was followed immediately by complete relief of arthritic symptoms. During pancreatic disease, whether malignant or benign, joint involvement is often associated with bone, cutaneous, serosal, and multiorgan involvement. The pathogenesis and therapy of joint lesions in pancreatic disease are controversed. Surgical treatment of the causative disease, and especially pancreaticojejunostomy should undoubtedly be considered more often.

Dysphagia after laparoscopic 360 repair for gastroesophageal reflux disease.

In comparison to medical treatment, antireflux surgery is recognized as an effective, efficient and longlasting therapy, as well as the only treatment that is able to modify the natural history of gastroesophageal reflux disease (GERD). The 360 fundoplication is the most widely used surgical procedure for GERD. Although performed in the era of H2-blockers and open surgery, comparison of the so-called Nissen repair to both symptomatic and continuous medical therapies concluded that surgery was superior to medical therapy in every outcome measure used.

[Evaluation of multidrug resistance phenotype on medullary specimens from patients with acute leukemia by determination of nuclear efflux of tetrahydropyranyl-doxorubicin. Approach by confocal laser microspectrofluorometry]. / Evaluation du phénotype de la résistance pléiotropique sur des prélèvements médullaires de patients présentant une leucémie aiguë par mesure de l'efflux nucléaire de la tétrahydropyranyl-doxorubicine. Approche par microspectrofluorimétrie confocale laser.

Confocal microspectrofluorometry allows the analysis of fluorescent molecules such as anthracylines in isolated living cells. An optical microscope fitted with a phase-contrast 100 X water-immersion objective enables simultaneous observation of the sample, focusing of the laser beam on the selected cell fraction (nucleus) and collection of the fluorescence emitted from the sample. The resulting intranuclear spectra are interpreted according to a quantitative model of the fluorescence spectra of both free and DNA-bound anthracycline. The intranuclear drug concentration can thus be determined. This technique has been applied to blast cells collected in patients with acute leukemia. Leukemic cells are aspirated from bone marrow, separated by Ficoll sedimentation and resuspended in RPMI-1640 containing 10% fetal calf serum and 200 nM tetrahydropyranyl-doxorubicin (THP-DOX). After one hour, 20 cells are analyzed and the mean nuclear drug content is determined (C1). Cells are then resuspended in the same medium but without anthracycline for 3 hours and the mean intranuclear drug concentration is then also determined (C3). From C1 and C3 the retention rate (RR) is calculated. Firstly, the accuracy of the method was checked. In 4 AML patients, two different samples aspirated on the same day were divided into two portions. Thus, two measurements were made on each one (4 values per patient). Coefficients of variation were satisfactory (4, 6, 12, and 12%). Secondly, blast cells collected in patients with AML and ALL at diagnosis or in relapse were studied. P-glycoprotein (P-gp) and CD34 expression was also studied using respectively immunohistochemistry land flow cytometry. Results obtained from the first 21 patients showed that there was no correlation between RR and either P-gp or CD34 expression. This could result from the efflux of THP-DOX by other mechanisms and/or low sensitivity of the staining technique.

[Chronic urticaria-macroglobulinemia (Schnitzler syndrome): developing to IgM myeloma. Apropos of a case]. / Urticaire chronique-macroglobulinémie (syndrome de Schnitzler): évolution vers un myélome à IgM. A propos d'un cas.

Schnitzler's syndrome has been described in 1972. It associates a chronic urticaria with leukocytoclastic vasculitis, a macroglobulinemia, bone pains, hyperostosis. In the twenty three cases reported, the evolution is benign but three patients including the princeps case developed a Waldenström's disease or a lymphoplasmocytic lymphoma. We present the case of one patient, aged 65, with Schnitzler's syndrome, who, after 6 years evolution, presents a IgM myeloma. Schnitzler's syndrome appeared as a pre-hemopathic state which has to be taken care of regularly and for a long time.

[Influence of seasons on risk of flare-up of systemic lupus: retrospective study of 66 patients]. / Influence de la saison sur le risque de poussée systémique du lupus: étude rétrospective de 66 patients.

PURPOSE: To establish the possible connection between visceral, arthro-cutaneous and biological spreading of systemic lupus (SL) and hours of sunlight. MATERIAL AND METHODS: Retrospective study of 66 SL patients, consisting of 52 visceral and 14 arthro-cutaneous cases taking into account the chronological pattern of each new aggravation, based on 480 clinical records. RESULTS: Increased frequency in visceral aggravation was observed in the post-summer period (August-January) (n = 57), as compared with the pre-summer period (February-July) (n = 25) (RR = 1.75, P = 0.006). This post-summer visceral aggravation was correlated with cutaneous affection (RR = 4.18) and absence of previous corticotherapy (RR = 3.97). Visceral and arthro-cutaneous aggravations taken together revealed a more disturbed immune balance pattern in the post-summer period (anti-dsDNA: 30 versus 25.1 IU/L [P = 0.07]; C3: 0.83 vs 0.921 IU/L [P = 0.05]; C4: 0.146 vs 0.183 [P = 0.05]), providing evidence of greater severity. Moderate thrombopenia (50-120 10(9)/L) accompanying visceral SL with antiphospholipids (n = 33) was more frequent during the post-summer period, even in the absence of aggravation (P = 0.03). The quarterly distribution of visceral aggravations was correlated with average hours of sunlight in the preceding quarter (P = 0.01). CONCLUSION: There is a post-summer increase in the frequency and severity of visceral SL spreading correlated to cutaneous exacerbation and sunlight.

[Pulmonary manifestations of primary Gougerot-Sjögren syndrome. Apropos of 8 cases in a series of 35 patients]. / Manifestations pulmonaires du syndrome de Gougerot-Sjögren primitif. A propos de huit observations dans une série de 35 patients.

Clinical, roentgenologic, functional and broncho-alveolar lavage features of lung involvement in primary Sjögren's syndrome were assessed in a retrospective study of 35 cases. Diffuse interstitial patterns on chest radiography were present in six patients and alveolar patterns were suggestive of lymphoid interstitial pneumonitis or pseudolymphoma in two. Acute and febrile onset mimicked infectious pneumonitis in three patients when dyspnea was the most common clinical feature in others. Patients with primary Sjögren's syndrome and pulmonary disease were older (65 vs 56 years) (P = 0.025), have more frequently extra-glandular manifestations (P = 0.03), keratoconjunctivis sicca (P = 0.018) and biological perturbations (hypergammaglobulinemia (P = 0.03), antinuclear antibodies (P = 0.01) than those without lung involvement. Low diffusion capacity was present in seven patients associated twice to small airways obstruction. Bronchoalveolar lavage revealed in all cases an increased total cells count (mean: 6.96 10(5)/mm3) and a lymphocytic alveolitis (range: 11 to 66%; mean: 38%) associated with an elevated percentage of alveolar neutrophils in four patients. A low CD4/CD8 ratio was related to a pejorative issue. Treatment consisted in corticotherapy combined with oral cyclophosphamide in case of pseudolymphoma.

[Ileo-anal anastomosis: various types of ileal reservoir]. / Anastomoses iléo-anales: les différents types de réservoir iléal.

The creation of an ileal reservoir ensures an improvement in the functional result obtained after ileo-anal anastomosis. As a result of its storage function, the reservoir allows a reduction in the number of stools per day. Several types of reservoir have been described. The S-shaped reservoir consists of three aniso-peristaltic limbs and an efferent loop with end-to-end ileo-anal anastomosis. The W reservoir is equivalent to a double J reservoir. The side-to-side iso-peristaltic reservoir consists of two iso-peristaltic limbs and an efferent loop with end-to-end ileo-anal anastomosis. After analysing the specific characteristics of each type of reservoir, the authors conclude that the necessity of a reservoir no longer needs to be demonstrated, but that there is no formal argument in favour of one particular model.

[Traumatic rupture of the pericardium with left intrapleural luxation of the heart in a patient with multiple trauma]. / Rupture traumatique du péricarde avec luxation intra-pleurale gauche du coeur chez un polytraumatisé.

Traumatic rupture of the pericardium is a rare lesion usually associated with violent thoracic trauma and sudden deceleration. The case described herein is one of left intra-pleural luxation of the heart secondary to trauma which was asymptomatic for nine hours and was revealed by acute symptoms due to mobilisation of the patient. Diagnosis of this entity is difficult and is usually made in the acute multiple trauma setting. Although usually negative, lung films may be contributive; sonocardiogram, which should be obtained routinely in this setting, is often non-specific but usually provides the indication for thoracotomy. The most severe complication is extra-pericardic luxation of the heart with strangulation or volvulus, depending on the site and size of the pericardial tear. While all proven cases of pericardial rupture should be explored surgically, treatment varies for different teams according to the site and the size of the rupture.

[Benign mucus-secreting tumors of the appendix. Six cases]. / Tumeurs mucosécrétantes bénignes de l'appendice. Six observations.

The authors report six cases of mucus-secreting tumor of the vermicular appendix, in four women and two men (mean age 59 years). All were benign. Pain in the right lower quadrant was the initial sign in 4 cases, while the mucus-secreting tumor was an incidental finding in the other two cases. A mass was palpated in the right lower quadrant in one case. A barium enema was performed in four cases and showed an extrinsic compression of the caecal base in one case, and of the right colon in another case. Four appendectomies, one associated with removal of the adjacent caecal tissues and two right colectomies were performed. Analysis of these six cases and a review of the literature allow us to recall the histologic classification of these tumors, which determines the prognosis and to emphasize the sonographic and CT scan findings which might lead to earlier preoperative diagnosis in the future. Management depends upon the circumstances under which the tumor is discovered, the local anatomy, and the type of tumor. Appendectomy is the treatment most often performed. Faced with a tumor of the appendix, without histologic proof or when the local anatomic conditions increase the risk of opening an abscess, right colectomy is recommended.

[Cardiac anomalies in disseminated lupus erythematosus diagnosed by Doppler ultrasonography. Prevalence and association with antiphospholipid syndrome]. / Anomalies cardiaques dans le lupus érythémateux disséminé diagnostiquées par échographie Doppler. Prévalence et association au syndrome des antiphospholipids.

OBJECTIVES: Antiphospholipid antibodies in patients with systemic lupus erythematosus (SLE) are widely associated with thrombosis, recurrent abortions and a thrombocytopenia. The purpose of this study was first to evaluate the prevalence of cardiac abnormalities in patients with SLE and secondly to establish the relationship between the findings and the presence of an antiphospholipid syndrome. SUBJECTS AND METHODS: First of all, a total of 52 consecutive patients with SLE and 52 healthy sex-and-age-matched control subjects were evaluated in a cross sectional study. All underwent M mode and two dimensional echocardiography, color flow imaging, pulsed and continuous wave Doppler. Secondly, in the SLE group, subjects exposed to antiphospholipid syndrome (n = 20) were compared to the control subjects. RESULTS: Compared with the control group, patients with SLE had significantly more pericardial abnormalities [(p = 0.0006) RR [3.36-infinity], mitral regurgitation [(p = 0.032) RR 2.48 [1.25-5.6]], tricuspid regurgitation [(p = 0.0016) RR 2.41 [1.58-8.85]. There was no significant difference between both groups for the left ventricular mass (p = 0.07), posterior wall (p = 0.25) and interventricular septum dimension (p = 0.16), and mitral valve thickness (p = 0.66). The antiphospholipid syndrome was significantly associated with increased left ventricular mass (p = 0.0054), posterior wall (p = 0.022) and interventricular septum dimension (p = 0.026). The relative risk increased for tricuspid and pulmonary regurgitation in SLE patients who had the antiphospholipid syndrome. CONCLUSION: The prevalence of left ventricular hypertrophy and right heart valvular regurgitation is significantly raised in SLE patients who have the antiphospholipid syndrome. However, antiphospholipid syndrome does not modify the prevalence of pericardial abnormalities which seems to be due to SLE alone.

[Perineal eventration after amputation of the rectum. Apropos of 3 cases]. / Eventrations périnéales après amputation du rectum. A propos de trois observations.

Three cases of perineal eventration after amputation of rectum are reported. Defined as the issue of intraperitoneal contents at the perineum secondary to surgery, perineal eventration occurs after 1% of abdominoperineal amputations, more frequently in women. Predisposing factors are a wide pelvis, extensive excision of levator muscles, hysterectomy, factors of postoperative increased abdominal pressure, absence of postoperative adhesions between intestinal loops and infection of perineal scar. The influence of primary or secondary wound closure is more difficult to determine. The eventration is manifest by pain and perineal bulging, sometimes marked and preventing walking, but asymptomatic forms are frequent. Intestinal loops are visible below a thinned skin, cutaneous ulcers, urinary disorders and rupture of sac being the common complications. Surgical treatment is necessary for patients free from neoplastic recurrence, those inconvenienced by the eventration and those with large eventrations, the most effective procedure being uterine fixation to promontory, and use of a plate to close lower region of pelvis, using an abdominal approach alone.