Pilonidal sinus disease: an intergluteal localization of hidradenitis suppurativa/acne inversa: a cross-sectional study among 2465 patients.

BACKGROUND: Hidradenitis suppurativa (HS), also referred to as acne inversa, is a debilitating skin disease characterized by inflammatory nodules, chronic abscesses and tunnels (fistulae and sinuses). The association with pilonidal sinus disease (PSD) is frequently reported but not well documented. OBJECTIVES: To determine the prevalence and characteristics of inflammatory skin lesions located in the intergluteal fold (IGF) of patients with HS. METHODS: This was an international multicentre retrospective cross-sectional study based on data collection from a large cohort of patients with HS with and without histopathology. Results From a total of 2465 patients with HS included in the study, 661 (27%) reported lesions in the IGF. These patients were significantly more often smokers and had more severe HS. Of the 238 patients with an available clinical diagnosis, intergluteal-HS (IG-HS) was diagnosed in 52 patients (22%) and PSD was diagnosed in 186 patients (78%). IG-HS was associated with the localization of HS in the proximity of the IGF, including the buttocks, genitals and the anus. There was a possibility of misclassification bias in this study as a clinical/image-based diagnosis or histopathology of the IGF lesions was not always available. CONCLUSIONS: The high prevalence of PSD suggests a strong link between both entities. Therefore, it may be useful to identify common pathophysiological mechanisms and develop common therapeutic strategies. What's already known about this topic? The occurrence of pilonidal sinus disease has not been clearly reported among patients with hidradenitis suppurativa/acne inversa. What does this study add? This is the first study that investigated the prevalence of pilonidal sinus disease among a large cohort of patients and identified the patient characteristics. Risk factors that might help to improve the management of patients were identified.

[Misdiagnosis of hidradenitis suppurativa continues to be a major issue. The R-ENS Verneuil study]. / Errance médicale des patients atteints d'hidradénite suppurée : un problème majeur et persistant. Étude « R-ENS Verneuil ¼.

OBJECTIVE: To provide physicians with an understanding of the factors behind significant delays in the diagnosis of hidradenitis suppurativa (HS) in France. PATIENTS AND METHODS: This prospective multicentre national study conducted from October 2015 to March 2016 included all patients consulting for HS. Patient data were collected by means of a standardized questionnaire. Univariate and multivariate analyses were conducted to collect factors associated with a significant time to diagnosis of at least 5.5years, defined as the period between the onset of initial clinical signs and the time of formal diagnosis. RESULTS: The 16 participating centres enrolled 312 patients (62% women), of average age 35years. The average age at onset of HS was 22years. Before formal diagnosis by a dermatologist (64% of cases), 170 (54%), 114 (37%) and 45 (15%) patients had previously consulted at least 3, 5 and 10 general physicians, respectively. The average time between the initial clinical signs of HS, the first dermatology visit and the definitive diagnosis was 6.2 and 8.4 years, respectively. Active smoking (OR adjusted 1.85; P=0.027) and disease onset at a younger age (adjusted OR 0.92; P<0.001) were both associated with significant delays in diagnosis. CONCLUSION: These results emphasized misdiagnosis among HS patients but did not evidence any association between either sociodemographic or economic characteristics and the existence of significant times to diagnosis.

Low and high body mass index in hidradenitis suppurativa patients-different subtypes?

INTRODUCTION: Overweight is a well-established risk factor for hidradenitis suppurativa (HS). In this cross-sectional study, we compare HS patients with a high body mass index (BMI) with HS patients with a low BMI to investigate differences in disease characteristics. MATERIALS AND METHOD: Patients were recruited from 17 dermatological centres from four continents. A total of 246 patients with a BMI below 25 were compared to 205 patients with a BMI of above 35. RESULTS: Patients with a high BMI suffered more severe disease (Hurley, physician global assessment, number of areas affected and patient-reported severity (PRS), P < 0.001 for all). There was no difference in smoking (P = 0.783) nor in family history (P = 0.088). In both low and high BMI patients, early onset of HS was a predictor of positive family history (P < 0.001, for each). For low BMI patients, an increase in BMI significantly increased PRS (P < 0.001). For patients with a high BMI, number of pack-years significantly increased PRS (P = 0.001). Cluster analysis of eruption patterns was location specific for low BMI patients but severity specific for high BMI patients. DISCUSSION: Patients with a low and high BMI could represent two clinically different subtypes. We suggest a non-linear relationship between BMI and impact of HS. As patients go from a low BMI patient to a high BMI patient (or from high to low), eruption patterns and risk factors may change.

Primary lung adenocarcinoma: characteristics by smoking habit and sex.

The incidence of adenocarcinoma is increasing, particularly among females. We sought to assess the role of tobacco consumption in clinical presentation according to sex. In this retrospective study, 848 patients diagnosed between 1997 and 2006 at Grenoble University Hospital (Grenoble, France) were stratified into four groups according to smoking habits. Differences between sexes and two contrasting female profiles emerged. Female current smokers were younger than female never-smokers (median 51 versus 69 yrs; p < 0.001), more often had surgery (62.7% versus 39%; p = 0.01) and had a median (95% CI) estimated survival of 26.2 (18.1-49.2) versus 15.1 (12.8-22.2) months (p = 0.002). Both groups had similar survival when taking treatment into account. Among males, smoking did not influence presentation. Male current smokers were older than female current smokers (median 59 yrs; p < 0.001) and fewer had surgery (48.8%; p = 0.015), although the percentage of stage IIIb-IV disease was similar (53% and 46%; nonsignificant) and they had a poorer estimated survival of 14.3 (13.0-18.5) months (p = 0.0024). Males smoked more than females (median 41 versus 30 pack-yrs; p < 0.001). Quitting smoking delayed age at diagnosis by 11 yrs for females (p = 0.0035) and 8 yrs for males (p < 0.001). Our results support the hypothesis that carcinogenesis differs between males and females, and between female smokers and never-smokers.

[Assessment of cancer RCP meetings in Rhône-Alpes: a survey on the ground]. / Évaluation des réunions de concertation pluridisciplinaire (RCP) en cancérologie dans la région Rhône-Alpes: une enquête de terrain.

The results of a local survey sent to urologists, oncologists and radiotherapeutists working in Rhône-Alpes have been reported to assess the value of multidisciplinary oncological meetings (RCP) in Urology. The results of this short study have been analyzed and compared to the national results published by the Inspection Générale des Affaires Sociales report. Meanwhile, we have created a professional electronic directory collecting all RCP of Rhône-Alpes, which will be accessible soon.

Rare diseases in disabled children: an epidemiological survey.

AIM: To estimate the contribution of rare diseases (RD) to severe impairment in 7-year-old children. METHODS: Data from a morbidity register of childhood impairments in a single French region were used. Impairments were classified as a mental, sensorial, neuromuscular (skeletal or movement-related) impairment (MSN_I) according to the International Classification of Functioning. Details of children born from 1980 to 1994 and resident in the county under study when they were 7 years old were recorded. A rare disease was defined as a prevalence rate of <1 per 2000 general population. RESULTS: 26% of children with severe MSN_I had a rare disease; in 36% the MSN_I was of unknown origin. The proportion of impairments that were due to a rare disease varied according to the type of impairment: 3.3% for severe psychiatric disorders; 16.0% for intellectual impairment; 37.2% for hearing impairment; 41.2% for neuromuscular, skeletal and movement impairment; and 81.1% for visual impairment. The overall prevalence rate of rare diseases was 2.1 per 1000 (459/218 283), and it increased significantly over time (p = 0.003). The latter increase was not associated with a decrease in the proportion of impairments of unknown origin, indicating an improvement in the survival of the children with a rare disease. CONCLUSIONS: In this study, a rare disease was at the origin of 26% of cases of severe MSN_I. This proportion remained stable over time, whereas the prevalence rate, as well as the prevalence rate of MSN_I disability, increased over time.

Favorable outcome of liver transplantation despite a high hepatitis B virus replication: beyond the limits?

Patients with end-stage liver disease due to chronic hepatitis B virus (HBV) infection with a persistent viral replication are generally denied liver transplantation (LT). We report the case of a patient who presented with the emergence of a YMDD escape mutant virus under lamivudine treatment, and developed terminal liver failure requiring LT. Pre-LT introduction of adefovir led to only a mild decrease in replication. The patient was treated with a combination of intravenous hepatitis B immune globulin (HBIG) that was started perioperatively, and also continued lamivudine and adefovir after LT. One year after LT, there was no evidence of HBV infection recurrence. This observation suggests that persistent high HBV replication might not be a contra-indication to LT, providing adequate and effective prophylaxis is given, using HBIG and antiviral drug combination therapy.

[Are preoperative examinations useful in the management of patients with renal hyperparathyroidism?]. / L'imagerie préopératoire des hyperparathyroïdies des insuffisants rénaux a-t-elle un intérêt en pratique clinique?

AIM OF THE STUDY: To evaluate the efficiency of preoperative parathyroid ultrasonography and scintigraphy in the management of renal hyperparathyroidism. PATIENTS AND METHODS: The charts of the last consecutive 200 patients who underwent surgery for renal hyperparathyroidism from 1998 to 2003 were retrospectively reviewed to collect data concerning parathyroid gland function, results of preoperative ultrasonography and scintigraphy, as well as modalities and results of surgical exploration. RESULTS: Ultrasonography and scintigraphy sensibilities were 36.4% and 49.3%, respectively. Efficiency of both examinations was improved when they were combined (sensibility of 64.7%) and in those patients managed for recurrent hyperparathyroidism. Were more often detected by preoperative examinations glands with high weight and/or greatest diameter, orthotopic and inferior glands as well as glands exhibiting nodular hyperplasia content upon pathological examination. CONCLUSION: Parathyroid ultrasonography and scintigraphy are of poor interest in the management of renal hyperparathyroidism. In a preoperative setting, they should be performed only in patients with recurrent disease.

Elevated serum levels of C-reactive protein are indicative of a poor prognosis in patients with esophageal cancer.

SUMMARY: Despite improved results gained by the combination of neoadjuvant chemotherapy and radiotherapy with surgery, the prognosis for the esophageal carcinoma patient remains poor. There is a need for prognostic markers that can help improve patient selection. The aim of this study was to evaluate whether serum levels of C-reactive protein (CRP) have any influence on the patient prognosis in terms of prediction of chemoradiosensibility, and overall and disease-free survival. Blood samples were obtained at the time of diagnosis in 67 patients with biopsy-proven primary carcinoma of the esophagus. Although not significantly, the mean CRP level was higher than that of 20 patients with benign esophageal pathology who served as controls. No significant correlation could be found between CRP levels and baseline characteristics such as age, gender, histology, cancer localization, tumor depth, lymph node status or metastatic spread. However, patients with CRP levels higher than 6 mg/L were more frequently non-responders to chemoradiotherapy (P = 0.035), had a shorter overall survival (P = 0.061) and a shorter disease-free survival (P = 0.016). It is concluded from these results that pretreatment measurement of serum CRP levels in patients with esophageal cancer could to be used in routine practice as indicators of chemoradiosensibility and prognosis.

[Gastric carcinoid tumours. Is there a place for antrectomy?]. / Les tumeurs carcinoïdes de l'estomac. Quelle place pour l'antrectomie ?

UNLABELLED: Gastric carcinoid tumours are classified in 3 types depending on whether they are sporadic (type III), or they are associated with a chronic atrophic gastritis (type I) or a multiple endocrine neoplasia type I-associated Zollinger-Ellison syndrome (type II). For type I tumours, the role of antrectomy, which aims to suppress the causative hypergastrinemia, has not been determined. AIM: To determine, from literature review the role of antrectomy in the management of type I gastric carcinoid tumours. METHODS: Bibliographic study searching for published observations of antrectomy for type I gastric carcinoid tumours. Data regarding postoperative evolution of gastrinemia and carcinoid tumours were collected. RESULTS: Thirty-eight published cases were identified. Preoperative gastrinemia was elevated in the 32 patients in whom it was measured. It came to normal ranges in the 19 patients in whom it was postoperatively assessed. With a mean follow-up of 34 months (1 to 120), disappearance of carcinoid tumours was observed in 27 of 38 patients (71%), the 11 others having tumour recurrence or persistence. When postoperatively assessed, hyperplasia of fundic enterochromaffine-like cells persisted in 7 patients, regressed in 4 and disappeared in the 6 others. No antrectomy-related complication was reported. CONCLUSION: Antrectomy can be considered as a worthwhile alternative for the treatment of gastric carcinoid tumours related to chronic atrophic gastritis and hypergastrinemia.

[Small bowel metastases from head and neck cancers]. / Métastases intestinales des cancers de la sphère oropharyngée.

AIM OF THE STUDY: Small bowel metastases represent less than 10% of malignant tumours of the small bowel. Among primitive cancers with small bowel metastases, head and neck cancers are exceptional. The aim of the study was to evaluate clinical and evolutive characteristics of patients with small bowel metastases from head and neck cancers. PATIENTS AND METHODS: Analyse of a reported case and bibliographic study by asking the Medline database and gathering anamnestic, clinical and evolutive data from observations of small bowel metastases from head and neck cancers. RESULTS: We report the case of a 63 years-old male patient with a small bowel metastasis from a head and neck cancer. Nine other observations have been published in the international literature. Analysis of this 10 patient-series shows that small bowel metastases of head and neck cancers occur rather in old male patients (mean age: 71 years). They are usually discovered after primitive cancer diagnosis, always because of occlusive, perforative or bleeding complications. Jejunal metastases are rather complicated by perforation whereas ileal ones rather induce occlusion or digestive bleeding. Discovering a small bowel metastasis in the context of head and neck cancer is highly indicative of a poor prognosis. In these 10 patients, deaths occurred within the 8 months following the diagnosis, because of postoperative complication or neoplastic evolution. CONCLUSIONS: Small bowel metastases from head and neck cancers usually occur as emergent complications. They are indicative of an advanced disease and the prognosis is extremely poor. The aim of the management is to afford the best quality of life as possible.

Retained appendicolith after laparoscopic appendectomy: the need for systematic double ligature of the appendiceal base.

Appendicoliths are considered to be strong indicators of appendicitis and the complications of appendicitis. We report the case of a 29-year-old woman who underwent a laparoscopic appendectomy for appendicitis with an appendicolith. The appendix was divided with a single ligature at the appendiceal base, and an appendicolith escaped into the pelvis. Thereafter, the patient suffered recurrent pelvic abscess. The diagnosis of retained appendicolith was made by repeated CT scans that revealed a mobile spontaneous calcification within the abscess. This postoperative complication could have been avoided if a systematic division of the appendix had been performed between double ligatures.

Concurrent aberrant right gastric vein directly draining into the liver and variations of the hepatic artery.

We report an autopsy on a 46-year-old man, a case that presented the concurrence of two rare vascular variations of the lesser omentum: aberrant right gastric vein draining directly into the liver, and multiple hepatic arteries. Although the left gastric vein emptied into the left aspect of the portal vein, the right one was found to ascend from the gastric lesser curvature along the right aspect of the common bile duct and to reach directly the porta hepatis. A left hepatic artery originating from the left gastric artery entered the porta hepatis in conjunction with the left ramus of the portal vein. A predominant right hepatic artery arose from the superior mesenteric artery and entered the porta hepatis in conjunction with the right ramus of the portal vein. The proper hepatic artery originating from the celiac artery entered the porta hepatis in conjunction with the aberrant right gastric vein. The possibility of a common underlying mechanism for these rare vascular variations is discussed.

Aortoesophageal fistula of uncommon origin: perforation of a Barrett's ulcer.

Aortoesophageal fistulas are life-threatening conditions of which over half are secondary to thoracic aortic aneurysms. Four cases related to perforation of a Barrett's ulcer have been described so far, accounting for less than 1% of published aortoesophageal fistulas. We report a fifth case, which presented with severe hypotension, anemia and hematemesis. The patient underwent emergency esophagectomy and aortic closure but postoperatively required aortic endoprosthesis for residual bleeding. This case highlights the great diagnostic and therapeutic challenge associated with perforated Barrett's ulcer.

Trends in elective terminations of pregnancy between 1989 and 2000 in a French county (the Isère).

OBJECTIVES: This study was performed in order to provide a description of indications for induced elective terminations of pregnancy (ETOP), their characteristics (e.g. gestational age), and their evolution over time. DESIGN OF THE STUDY: This is an epidemiological study. The geographic area covered is the French county of 'Isère', which represents a mean of 14 000 births per year over the study period. MATERIALS AND METHODS: Data on ETOPs were collected actively from medical records by a register of childhood deficiencies and adverse perinatal events in this county. Between 1989 and 2000, 996 ETOPs were notified. RESULTS: Four main grounds for ETOPs were identified: (1) morphological anomalies with normal karyotype (39%), (2) chromosomal anomalies (35%), (3) other fetal grounds (16%), and (4) maternal indications (10%). Prevalence rates for the first two grounds increased significantly over the study period respectively from 2.0 to 2.9 and from 1.4 to 2.7 per 1000. Among the ETOPs carried out because of fetal indications, the percentage of late ETOPs (from 24 weeks of gestation) was 34.6%, and remained stable over the studied period. In some cases, a medical consensus was not reached with respect to indications for termination (sex chromosome anomalies, limb defects). We estimated the percentage of these cases as being 2.7% of the figure for fetal indications, without any variation in prevalence over the whole period (p = 0.59). The increasing number of ETOPs that occurred in the chromosomal aberrations group during the study period is thought to be due to an increase in diagnostic sensitivity. The increase that occurred in the morphological anomalies group is thought to be due both to an increase in sensitivity and to a widening of the field with respect to indications, some of which have an uncertain prognosis (e.g. agenesis of the corpus callosum). CONCLUSION: This study provides useful data for monitoring medical practice consistency within the field of prenatal diagnosis, and for the drive to keep medical practice within ethically acceptable limits.

[Evaluation of maternal morbidity after drug-induced termination of pregnancy (TOP) after 12 gestation weeks]. / Evaluation de la morbidité maternelle après interruption médicamenteuse de grossesse à partir de 12 semaines d'aménorrhée.

OBJECTIVE: The number of TOP for medical indications has increased regularly over the last ten years. At the same time, the methods used for TOP have evolved. The purpose of this study was to assess the frequency of complications after drug-induced termination of pregnancy in order to determine whether using this method for interrupting pregnancy during the second or third trimester adds further danger for the mother in terms of early severe risk (uterine rupture, hysterectomy) or less severe long-term risk (infection). MATERIAL AND METHODS: This metaanalysis included all articles devoted to pregnancy interruption from 12 gestation weeks retrieved from the Medline database and published between 1989 and 1999 in the United States, Canada, Australia, New Zealand, or the European Union. After excluding articles that included in utero death (n=8), isolated case reports and series involving a high-risk of maternal somatic complications (n=16), and surgical methods for pregnancy termination (n=4), we retained 23 articles for analysis. These articles had included 58,891 drug-induced terminations of pregnancy. For each article, we recorded the following complications: bleeding requiring transfusion, uterine rupture, ovular or placentar retention, and infection. A classical homogenicity test was performed for each type of complication. When this test was not significant, a mean rate, weighing by size of the study, was calculated. RESULTS: One study reported maternal deaths (3/143000). The weighted mean rate for late retention (>24 hr) was 1.5 [CI95: 1.1%-1.9%]. For infections, the rates were very variable between studies (from 0.7% to 3.6% with one study reporting 8%). For bleeding with transfusion, the weighted mean rate was 0.7% [CI95: 0.5%-0.9%]. This rate was significantly higher than the rate observed in 1999 in France after delivery excepting medically terminated pregnancy (p<10(-3)) but probably is a reflection of the variable transfusion practices during the eighties in these different countries. Th rate of uterine rupture after medically terminated pregnancy was 0.1% [CI95: 0.07%-0.17%] and would be higher after delivery (excepting terminations) but not significantly (p=0.07). CONCLUSION: This metaanalysis demonstrates that the risk of severe complications (uterine rupture and bleeding requiring transfusion) are rare but are more prevalent than after delivery except pregnancy termination. The metaanalysis approach is justified due to the low incidence of these severe complications. A prospective multicentric study of the complications using a geographical base would be useful to obtain unbiased data on risk level. A risk analysis by gestational age, maternal age, parity, and product used would thus be possible, as would long-term monitoring of maternal outcome.