[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.

Hereditary neuromuscular disease and multicomposite subjective health status: feasibility, internal consistency and test-retest reliability in the French version of the Nottingham Health Profile, the ISPN.

OBJECTIVE: To evaluate the feasibility, internal consistency and reproducibility of the French version of the Nottingham Health Profile (NHP) completed by adults with hereditary neuromuscular disease. DESIGN: Cross-sectional study with evaluation at 15 +/- 7 days for NHP test-retest. SETTING: Multidisciplinary rehabilitation consultations in Reims. SUBJECTS: Sixty-four neuromuscular disease outpatients completed the NHP consecutively between April 2002 and December 2003. MAIN MEASURES: French version of the Nottingham Health Profile (NHP), Barthel Index and sociodemographic characteristics. RESULTS: The average completion percentages for the different dimensions was 84.2% (range 72-97%). With respect to the completion feasibility of the physical mobility subscale, 7-20% of neuromuscular disease patients failed to complete four items out of eight. In the pain subscale, the same difficulty was encountered for three items out of eight. Internal consistency as assessed by Cronbach's alpha was acceptable for the subscales physical mobility (0.88), emotional reaction (0.74), sleep (0.77), and pain (0.81); it was less reliable for the subscale social isolation (0.61), and poor for the subscale energy (0.47). Test-retest agreement measured by intraclass correlation coefficient was in all instances greater than 0.70. CONCLUSION: Some items in the pain and physical mobility subscales pose problems related to the relevance of the wording for patients confined to wheelchairs. Recoding of the measure makes it possible to avoid missing data from these dependent patients. Scores differ statistically according to the coding used. In study reports, details of such procedures should be provided for comparison of ISPN results with those from other studies in the literature.

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.

[Appendicitis or non-specific pain in the right iliac fossa?]. / Appendicite ou douleur non spécifique de la fosse iliaque droite?

Nearly 300,000 appendectomies are performed yearly in France. The negative laparotomy rate for suspected acute appendicitis is about 20%. Appendiceal perforation increases the risk of postoperative complications and may lead to death. Differential diagnosis of right lower quadrant abdominal pain is a major public health problem. Reported means of increasing diagnostic accuracy have included in-hospital observation with clinical and biological structured data collection with or without computer-assisted diagnosis, ultrasonography, CT, and laparoscopy. The ideal diagnostic test is yet to be defined. However, structured clinical examination is the cornerstone of any management algorithm. "When in doubt, take it out" is no longer acceptable.

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.

Effect of chlorambucil on bone mineral density in the course of chronic lymphoid leukemia.

The purpose of this work was to study the effects of chronic lymphoid leukemia (CLL) and its treatments on bone mineral density (BMD). Lumbar and femoral BMD was measured by X-ray absorptiometry in 50 (32 M, 18 F, median age 65, range age: 47-87 yr) CLL patients. In order to gauge the respective effects of CLL and corticoids on bone mass, 31 CLL patients under treatment were compared with 31 controls on cortisone. Nineteen untreated patients with CLL were compared with controls devoid of osteopenia risk factor. There was no significant difference regarding lumbar and femoral BMD between the untreated patients with CLL and the healthy controls. An increase in lumbar and femoral BMD was noted in the treated CLL group compared with the controls on cortisone (lum BMD: 1.018 vs. 0.861 g/cm2, p=6.10(-4); fem BMD: 0.773 vs. 0.699 g/cm2, p=0.037). This increase was observed only in patients who had received chlorambucil (lum BMD: 1.066 vs. 0.861 g/cm2, p=0.10(-4); fem BMD: 0.806 vs. 0.699 g/cm2, p=4.10(-3)), whereas there was no difference between the CLL patients treated without chlorambucil and the controls on cortisone. Multiple linear regression analysis confirmed the marked effect of chlorambucil (r=0.3715, p<10(-3)) on BMD increase in the course of CLL.

Laparoscopic antireflux surgery--technique and results.

Although gastroesophageal reflux disease (GERD) can be effectively treated by proton-pump inhibitors, surgery is still the only means of definitive cure of the disease. After introduction of laparoscopic surgery, there has been a clear trend to surgical repair of the incompetent cardia. The indications for surgical treatment are: endoscopically proven esophagitis, persistent or recurrent complaints under medical treatment, esophageal stricture and/or pH-metrically proven acid reflux as well as reflux-induced coughing (chronic aspiration). Although the laparoscopic antireflux operations is a technically demanding procedure, it can be performed with similar results as compared to conventional surgery. The operative technique is reported in detail. From January 1992 to March 1997, 146 consecutive patients with GERD have been operated on laparoscopically. The overall conversion rate was 8.2% (n = 12). 133 patients were operated on according to the Nissen procedure including hiatoplasty. The Toupet operation was performed in only one case. 84 men and 42 women had a mean age of 49 years (20-76). The median duration of symptoms was 48 months (1-600). Except five patients all had medical treatment for at least 2 years. Twice pneumatic balloon dilatation of an esophageal stricture was necessary preoperatively. The median operation time was 210 minutes (70-660). Conversion to open surgery because of intraoperative complications was necessary in 6 patients. Postoperative complications occurred in 14 patients, all of them being successfully treated conservatively. No patient died. 121 patients (90.3%) had follow up examinations for at least 6 months. Retreatment was necessary in 5 cases: 1x slipped Nissen (laparoscopic repair), 1x intrathoracic hernia (conventional reoperation), 2x dysphagia > 4 months postoperatively (endoscopic balloon dilatation) and 1x recurrent ulcer (conventional operation). With a correct indication, laparoscopic Nissen repair for GERD is a suitable, safe and definitive treatment.

Rheumatic manifestations of scurvy. A report of two cases.

Bleeding into the muscles and joints can be the presenting manifestation of scurvy, as illustrated by two case-reports. One patient presented with hemarthrosis of the tibiotalar joint due to an insufficiency fracture and was suspected to have scurvy based on the presence of purpura and hypertrophy of the gums with loss of teeth. In the other patient, multiple hematomas in the lower limbs were found at presentation and the presence of coiled hairs suggested the diagnosis. Both patients had completely eliminated fruit and vegetables from their diet. Low levels of ascorbic acid were found in serum and urine. A full recovery was achieved in both cases under ascorbic acid supplementation.

[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.