Integration and activity of hospital-based palliative care consultation teams: the INSIGHT multicentric cohort study.

BACKGROUND: Hospital-based Palliative Care Consultation Teams (PCCTs) have a consulting role to specialist services at their request. Referral of patients is often late. Early palliative care in oncology has shown its effectiveness in improving quality of life, thereby questioning the "on request" model of PCCTs. Whether this evidence changed practice is unknown. This multicentre prospective cohort study aims to describe the activity and integration of PCCTs at the patient level. METHODS: For consecutive patients newly referred to participating PCCTs, the team collected the following data: circumstances of first referral, problems identified, number of interventions, patient's survival after first evaluation and place of death. RESULTS: Seventeen PCCTs based in university hospitals in Paris area, recruited 744 newly referred adult patients, aged 72 ± 15 years, 52% males, and 504(68%) with cancer as primary diagnosis. After 6 months, 548(74%) had died. At first evaluation, 12% patients were outpatients, 88% were inpatients. Symptoms represented the main reasons for referral and problems identified; 79% of patients had altered performance status; 24% encountered the PCCT only once. Median survival (1st-3rd quartile) after first evaluation by the PCCT was 22 (5-82) days for overall patients, and respectively 31 (8-107) days and 9 (3-34) days for cancer versus noncancer patients (p < 0.0001). Place of death was acute care hospital for 51.7% patients, and home or Palliative Care Unit for 35%. Patients referred earlier died more often in PCU. CONCLUSION: The study provides original data showing a still late referral to the PCCTs in France. Cancer patients represent their predominant activity. The integrated palliative care model seems to emerge besides the "on request" model which originally characterised their missions.

Computed tomography evaluation of high-grade esophageal necrosis after corrosive ingestion to avoid unnecessary esophagectomy.

BACKGROUND: Esophagectomy is the standard of care for high-grade corrosive esophageal necrosis as assessed endoscopically. However, the inaccuracy of endoscopy in determining the depth of intramural necrosis may lead to unnecessary esophageal resection, with devastating consequences. Our aim was to evaluate the use of computed tomography (CT) for the emergency diagnostic workup of endoscopic high-grade corrosive esophageal necrosis. METHODS: In a before (2000-2007)/after (2007-2012) study of patients with grade 3b endoscopic esophageal necrosis, we compared outcomes after routine emergency esophagectomy versus selection for emergency esophagectomy based on CT evidence of transmural necrosis, defined as at least two of the following: esophageal-wall blurring, periesophageal-fat blurring, and the absence of esophageal-wall enhancement. Survival estimated using the Kaplan-Meier method was the primary outcome. RESULTS: Compared to the routine-esophagectomy group (n = 125), the CT group (n = 72) had better overall survival in the crude analysis (hazard ratio [HR], 0.43; 95 % confidence interval [95 %CI], 0.21-0.85; P = 0.015) and in the analysis matched on gender, age, and ingested agent (HR, 0.36; 95 %CI, 0.16-0.79; P = 0.011). No deaths occurred among patients managed without emergency esophagectomy based on CT findings, and one-third of CT-group patients had their functioning native esophagus at last follow-up. Self-sufficiency for eating and breathing was more common (84 % vs. 65 %; relative risk [RR], 1.27; 95 %CI, 1.04-1.55; P = 0.016) and repeat suicide less common (4 % vs. 15 %; RR, 0.27; 95 %CI, 0.09-0.82; P = 0.019) in the CT group. CONCLUSION: The decision to perform emergency esophagectomy for endoscopic high-grade corrosive esophageal injury should rely on CT findings.

Upper digestive tract reconstruction for caustic injuries.

OBJECTIVE: The aim of the study was to compare the short- and long-term outcomes of colopharyngoplasty and esophagocoloplasty for caustic injuries of the upper digestive tract. BACKGROUND: Simultaneous esophageal and pharyngeal reconstruction by colopharyngoplasty allows regaining nutritional autonomy in patients with severe pharyngoesophageal caustic injuries. METHODS: Patients who underwent upper digestive tract reconstruction for caustic injuries by colopharyngoplasty (n = 116) and esophagocoloplasty (n = 122) between 1993 and 2012 were included. Survival and functional outcomes were analyzed. Success was defined as nutritional autonomy after removal of the jejunostomy and tracheotomy tubes. Quality of life was assessed using the QLQ-OG25 and SF12v2 questionnaires. RESULTS: Overall Kaplan-Meyer survival at 1, 5, and 10 years after colopharyngoplasty and esophagocoloplasty were 92%, 74%, 67% and 92%, 83%, 73%, respectively (P = 0.56). Quality of life and functional results (success: 57% vs 95%, P < 0.0001) were impaired after colopharyngoplasty. On multivariate analysis, older age (odds ratio [OR]: 0.94; confidence interval [CI]: 0.91-0.97 P < 0.0001) and pharyngeal reconstruction (OR: 0.05; CI: 0.02-0.13, P < 0.0001) were associated with failure. The decline in success with age was more pronounced after colopharyngoplasty with only 1 (7%) of 15 patients operated after the age of 55 being self-sufficient for eating and breathing. Laryngeal resection during colopharyngoplasty had no influence on success (54% vs 58%, P = 0.67) CONCLUSIONS:: The need to associate pharyngeal reconstruction during esophageal reconstruction for caustic injuries has a long-term negative impact on functional outcome.

Black patients of African descent and HLA-DRB1*15:03 frequency overrepresented in epidermolysis bullosa acquisita.

Epidermolysis bullosa acquisita (EBA) is a rare autoimmune bullous disease (AIBD). However, higher EBA incidence and predisposing genetic factor(s) involving an HLA haplotype have been suspected in some populations. This retrospective study assessed the overrepresentation of black patients with EBA, its link with HLA-DRB1*15:03, and their clinical and immunological characteristics. Between 2005 and 2009, 7/13 (54%) EBA and 6/183 (3%) other-AIBD patients seen consecutively in our department were black (P=10(-6)); moreover 7/13 (54%) black patients and 6/183 (3%) white patients had EBA (P=10(-6)). In addition, between 1983 and 2005, 12 black patients had EBA. Finally, among the 19 black EBA patients, most of them had very atypical clinical presentations, 9 were natives of sub-Saharan Africa, 1 from Reunion Island, 7 from the West Indies, and 2 were of mixed ancestry. HLA-DRB1*15:03 allelic frequencies were 50% for African patients, significantly higher than the control population (P<10(-3)), and 21% for the West Indians (nonsignificant). High EBA frequencies have already been reported in American blacks significantly associated with the HLA-DR2. In conclusion, black-skinned patients developing EBA seem to have a genetic predisposition, and EBA should be suspected systematically for every AIBD seen in this population.

Rituximab for patients with refractory mucous membrane pemphigoid.

BACKGROUND: Mucous membrane pemphigoid (MMP) still represents a potentially life- and sight-threatening disease. In a subset of patients with severe MMP, conventional immunosuppressants are ineffective or contraindicated. OBSERVATIONS: Twenty-five patients with severe refractory MMP, including 5 with mucous membrane-dominant epidermolysis bullosa acquisita, received 1 or 2 cycles of rituximab (375 mg/m(2) weekly for 4 weeks). Twenty-one of the patients were receiving concomitant therapy with dapsone and/or sulfasalazine therapy, which was maintained during rituximab cycles. Complete responses in all affected sites (ocular and/or extraocular) were obtained in 17 patients (68%) by a median time of 12 weeks after the first cycle, and 5 additional patients responded completely after a second cycle, yielding an 88% complete response rate. In all but 1 of the 10 patients with ocular lesions, their eyes became noninflammatory within a mean of 10 weeks. Among the 3 patients (12%) who developed severe infectious complications, 2 (8%) died; they had been receiving concomitant conventional immunosuppressants and high-dose corticosteroids and were hypogammaglobulinemic. Treatment with immunosuppressants was discontinued for all other patients, and no other infection was observed. Ten patients experienced relapse after a mean of 4 (range, 1-16) months after achieving complete responses. CONCLUSIONS: Rituximab appears to have rapid and dramatic efficacy in patients with severe, refractory MMP. The occurrence of severe infections in patients receiving concomitant conventional immunosuppressants supports using rituximab without other immunosuppressants. Controlled prospective studies are warranted to define an optimal treatment protocol.

Colopharyngoplasty for the treatment of severe pharyngoesophageal caustic injuries: an audit of 58 patients.

OBJECTIVE: The aim of this study was to describe the technique of colopharyngoplasty for the reconstruction of concomitant esophageal and pharyngeal caustic injuries and to evaluate the postoperative course and late functional outcomes. SUMMARY BACKGROUND DATA: Surgical treatment of esophageal and pharyngeal strictures is a difficult challenge because reconstruction at this level interferes with the mechanisms of deglutition and respiration. Several techniques have been described for the treatment of this condition but none is accepted as the gold standard. METHODS: Fifty-eight patients (34 men, median age 37 years) underwent colopharyngoplasty for caustic injuries between 1993 and 2005. Forty patients (69%) had a previous psychiatric history of depression (n = 30) or schizophrenia (n = 10). After removal of all scar tissues, the pharyngeal reconstruction was performed with the cervical end of the colic transplant employed for esophageal replacement. Laryngeal resection was associated in half of the patients. Success of the procedure was defined as recovery of nutritional autonomy and airway patency. RESULTS: Operative mortality was 2%. Postoperative complications required reoperation in 16 patients (28%). The functional outcome was evaluated in 46 patients with a follow-up of more than 6 months. The tracheostomy was withdrawn in 42 (91%) patients after a median of 42 days (range, 20-1020). The jejunostomy was removed in 32 patients (70%) after a median of 12 months (range, 2-54). Finally, the procedure was successful in 31 patients (67%). Logistic regression analysis showed that advanced age, a previous history of psychiatric disease, and early reoperation had an adverse impact on fuctional outcome. Seven patients (12%) repeated the suicide attempt. CONCLUSIONS: Colopharyngoplasty is a simple and reliable procedure that can be successfully employed to restore the digestive continuity in patients with concomitant esophageal and pharyngeal caustic injuries. Control of the underlying psychiatric disease before reconstruction is a key factor for success.

A prospective study of upper aerodigestive tract manifestations of mucous membrane pemphigoid.

We conducted a prospective study between 1995 and 2002 to investigate nose and throat (NT) manifestations of mucous membrane pemphigoid (MMP). One hundred ten consecutive patients with clinical, histologic, and immunologic criteria of MMP were seen in 2 referral centers for bullous diseases. They were systematically asked about the existence of persistent NT symptoms. Patients who had any were examined with a flexible nasopharyngolaryngoscope by the same otorhinolaryngologist. When possible, NT mucous membrane (MM) biopsies were taken for direct immunofluorescence (IF) assays to determine lesion specificity. Thirty-eight (35%) patients (23 F/15 M; mean age, 58.5 yr) had the following NT symptoms: 35 (92%) nasal, 19 (50%) pharyngeal, and 10 (26%) laryngeal. Five (13%) had acute dyspnea. Thirty-three (87%) of the 38 symptomatic patients had lesions at physical examination: 30 (79%) nasal, 6 (16%) pharyngeal, and 19 (50%) laryngeal. Laryngeal involvement was asymptomatic in 11 patients. Lesions were mainly atrophic rhinitis and oropharyngeal and epiglottal erosions. Nasal valves, choanae, pharynx, and/or larynx were severely scarred in 7 (18%) patients, causing the death of 3. Direct IF showed malpighian epithelium associated with linear immune deposits (IgG, IgA, or C3) along the chorioepithelial junction in all 18 biopsies performed, including those of 4 symptomatic patients without lesions at physical examination. The presence of severe ophthalmologic lesions (p = 0.02) and > or =3 sites involved other than NT (p = 0.02) were predictive of laryngeal involvement. In contrast, laryngeal symptoms, disease duration, HLA DQB1*0301, and smoking were not significantly associated with laryngeal lesions. In conclusion, at least 35% of MMP patients had NT involvement. Atrophic rhinitis was the most frequent lesion. The most severe were the laryngeal lesions that were significantly associated with severe ocular involvement and disseminated disease, and could be fatal. Our results highlight the necessity of a multidisciplinary approach to MMP management to assure early diagnosis of NT involvement, to guide therapeutic choices, and to improve patient survival and functional outcomes.