Supportive care in the acute phase of Stevens-Johnson syndrome and toxic epidermal necrolysis: an international, multidisciplinary Delphi-based consensus.

BACKGROUND: Supportive care is the cornerstone of management of adult and paediatric Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). However, consensus on the modalities of supportive care is lacking. OBJECTIVES: Our aim in this international multicentric Delphi exercise was to establish a multidisciplinary expert consensus to standardize recommendations regarding supportive care in the acute phase of SJS/TEN. METHODS: Participants were sent a survey via the online tool SurveyMonkey, consisting of 103 statements organized into 11 topics: multidisciplinary team composition, suspect drug management, infection prevention, fluid resuscitation and prevention of hypothermia, nutritional support, pain and psychological distress management, management of acute respiratory failure, local skincare, ophthalmological management, management of other mucosa, and additional measures. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). The results were analysed according to the RAND/UCLA Appropriateness Method. RESULTS: Forty-five participants from 13 countries (on three continents) participated. After the first round, a consensus was obtained for 82.5% of the 103 initially proposed statements. After the second round, a final consensus was obtained for 102 statements. CONCLUSIONS: We have reached an international Delphi-based consensus on best supportive care practice for SJS/TEN. Our expert consensus should help guide physicians in treating patients with SJS/TEN and thereby improve short-term prognosis and the risk of sequelae.

[The process of meaning-making in adjustment to multiple sclerosis]. / Le processus de construction de sens dans l'adaptation à la sclérose en plaques.

INTRODUCTION: Progressive neurological disorders require continual adaptation. People who maintain a better psychological balance in the face of the disease are those who find meaning in their experience. The ability to find a positive meaning in having multiple sclerosis (MS) is more common in people who are at the beginning of their disease or those who have few functional limitations (Pakenham, 2008). This qualitative study examines the process of meaning-making by people who are moderately to severely affected by MS. METHODS: Eight participants told the story of their experiences in individual semi-structured interviews. The verbatim transcripts were subjected to phenomenological analysis. RESULTS: The results allowed the identification of the essential elements that shape the experience of meaning-making. The adaptation happens in two main areas: (1) limiting the impact of specific symptoms, and (2) investing in activities that combine meaningful relationships and the feeling of being useful. Meaning is developed in the search for a new existential balance that reconciles creative tensions, particularly in the oscillation between letting go and determination. The process of meaning-making is distributed along a continuum of strategies that allows one to act as if nothing had happened, act within the limits of the disease, act despite the disease, act in new ways because of the disease, or act thanks to the disease. CONCLUSIONS: Understanding the process of meaning-making while adjusting to MS can allow the identification of possible interventions to better accompany people who are most severely affected by MS in their adaptation to the disease.

Post-acute phase and sequelae management of epidermal necrolysis: an international, multidisciplinary DELPHI-based consensus.

BACKGROUND: Long-term sequelae are frequent and often disabling after epidermal necrolysis (Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN)). However, consensus on the modalities of management of these sequelae is lacking. OBJECTIVES: We conducted an international multicentric DELPHI exercise to establish a multidisciplinary expert consensus to standardize recommendations regarding management of SJS/TEN sequelae. METHODS: Participants were sent a survey via the online tool "Survey Monkey" consisting of 54 statements organized into 8 topics: general recommendations, professionals involved, skin, oral mucosa and teeth, eyes, genital area, mental health, and allergy workup. Participants evaluated the level of appropriateness of each statement on a scale of 1 (extremely inappropriate) to 9 (extremely appropriate). Results were analyzed according to the RAND/UCLA Appropriateness Method. RESULTS: Fifty-two healthcare professionals participated. After the first round, a consensus was obtained for 100% of 54 initially proposed statements (disagreement index < 1). Among them, 50 statements were agreed upon as 'appropriate'; four statements were considered 'uncertain', and ultimately finally discarded. CONCLUSIONS: Our DELPHI-based expert consensus should help guide physicians in conducting a prolonged multidisciplinary follow-up of sequelae in SJS-TEN.

A pilot study on upper esophageal sphincter dilatation for the treatment of dysphagia in patients with oculopharyngeal muscular dystrophy.

Upper esophageal sphincter (UES) dilatation was done for the treatment of moderate to severe dysphagia with a Maloney bougie in 14 patients with oculopharyngeal muscular dystrophy (OPMD) or with an achalasia dilator in three patients. The severity of dysphagia prior to UES dilatation was evaluated by a 15-point dysphagia score, a pharyngeal and esophageal manometry and a radionuclide pharyngoesophageal transit study. Using actuarial life table, the improvement rate after dilatation with Maloney bougie was 64.3% (95% CI 39.2-89.4) at 3- and 6-month follow-ups, and was 55.7% (95% CI 28.9-82.5) at 12- and 18-month follow-ups. At 3-month post-dilatation, we observed a significant reduction of the mean dysphagia score from 9.6 to 7.2 (P = 0.05). No significant manometric or radionuclide factors were found to predict effective dilatation. The results of this pilot study showed that UES dilatation with Maloney bougie or achalasia dilator may be an effective treatment of moderate dysphagia in patients with OPMD. However, further studies with larger sample sizes are needed to corroborate these results and to assess long-term outcome.

False-positive captopril renography in patients taking calcium antagonists.

UNLABELLED: Captopril-enhanced renography is the noninvasive test of choice for the diagnosis of renovascular hypertension. Previous studies have shown that bilateral symmetrical changes are associated with many renal conditions. However, patients with normal renal angiography occurred in our institutions despite this scintigraphic pattern, and no known conditions could explain these results. The purpose of this study was to evaluate the diagnostic implications of bilateral symmetrical renal function deterioration on captopril renography. METHODS: Eighty-six captopril renal scintigraphies performed at two centers to exclude renovascular hypertension (50 consecutive patients after the observation of a bilateral symmetrical renal function deterioration despite a normal angiogram at one institution and 36 patients with both captopril renography and renal angiography at the other institution) were retrospectively reviewed. Baseline and captopril-enhanced renograms were obtained with 99mTc-mercaptoacetyltriglycine and a 1-day protocol in 50 patients; 36 patients were studied using 99mTc-diethylenetriamine pentaacetic acid and a 2-day protocol. Bilateral symmetrical renal function deterioration was detected. RESULTS: Ten patients presented with bilateral symmetrical renal function deterioration on their captopril renograms; 9 of them were taking calcium antagonists (p=0.015). Control studies performed in 5 patients without these medications demonstrated normal captopril renograms in 4 and persistent renal dysfunction in 1. No explanation was found for the patient who was not taking any medication. Angiograms performed in 5 patients showed normal renal arteries. An 11th patient who was taking a calcium antagonist showed dysfunction of his one kidney on the captopril renogram but no artery stenosis on the renal angiogram. CONCLUSION: Calcium antagonists can cause false-positive captopril renograms. These medications should be stopped before captopril renography, and physicians should be aware of this possible drug interaction if bilateral symmetrical renal function deterioration is seen on a patient's captopril renogram.

The role of Apligraf in the treatment of venous leg ulcers.

A cultured, allogeneic, bi-layered human skin equivalent has recently become available to help clinicians manage difficult-to-heal venous ulcers. This skin equivalent has an epidermis and dermis similar to human skin. Its living keratinocytes and fibroblasts are from cultured cell banks derived from human neonatal foreskin. Because the skin equivalent is made up of viable human cells, it cannot be terminally sterilized. Safety concerns, which have been addressed, include the risk of possible transmission of infection, immunogenicity, immunological graft rejection, and tumor formation. However, the maternal blood of the neonatal donor and the master cell banks are screened for infectious agents. Additionally, the human skin equivalent is produced under strict aseptic control, with sterility continuously monitored by the Good Manufacturing Processes. This paper reviews the characteristics of this human skin equivalent and provides practice guidelines.