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.

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.

Significant linkage for Tourette syndrome in a large French Canadian family.

Family and twin studies provide strong evidence that genetic factors are involved in the transmission of Gilles de la Tourette syndrome (TS) and related psychiatric disorders. To detect the underlying susceptibility gene(s) for TS, we performed linkage analysis in one large French Canadian family (127 members) from the Charlevoix region, in which 20 family members were definitely affected by TS and 20 others showed related tic disorders. Using model-based linkage analysis, we observed a LOD score of 3.24 on chromosome 11 (11q23). This result was obtained in a multipoint approach involving marker D11S1377, the marker for which significant linkage disequilibrium with TS recently has been detected in an Afrikaner population. Altogether, 25 markers were studied, and, for level of significance, we derived a criterion that took into account the multiple testing arising from the use of three phenotype definitions and three modes of inheritance, a procedure that yielded a LOD score of 3.18. Hence, even after adjustment for multiple testing, the present study shows statistically significant evidence for genetic linkage with TS.

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.

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.

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.

Health patterns of cardiac surgery clients using home health care nursing services.

The purpose of this study was to examine the health patterns of cardiac surgical patients in the home health care population and their relationships to outcomes and duration of home health care using Gordon's Functional Health Pattern framework. Home health care records of 96 cardiac surgical clients were reviewed. Admission health pattern data, reasons for admission, duration and outcomes of home care services, characteristics of hospital experience, and demographic data were analyzed. Dysfunctional health patterns were primarily in the area of activity/exercise. The most common reasons for admission were monitoring of cardiopulmonary status, wound care, and instruction on diet, medications, and cardiac regimen. The mean duration of home care was 28.8 days. Thirty percent of the sample were readmitted to the hospital. Duration of home care was shorter for those who were married and for those who reported weakness, tiredness, or fatigue as a chief complaint. Readmission to the hospital was more likely for those who had complications during their initial hospital stay and those who required at least partial assistance with bathing, dressing, feeding, or toileting. Implications for practice and research are discussed.

The bicycle ergometer for muscle power testing.

The force-velocity relationship implies that the faster a contracting muscle is permitted to shorten, the weaker it becomes or the slower a contracting muscle is permitted to shorten, the stronger it becomes until it reaches maximal isometric tension. The relationship is hyperbolic for a denervated muscle but is inverse and linear for functionally innervated muscle groups. When the force of contraction is multiplied by the velocity of contraction, a power production curve is obtained. The purposes of the study were to examine the force-velocity relationship on a standard bicycle ergometer, to deduce a power-velocity curve, to compare the results of young men and women and to see if the measures were reproducible. Fifty-eight young men and women volunteered for the study. Testing consisted of pedalling as quickly as possible for five seconds at resistance settings from 2 to 7-kg. In order to evaluate the reproducibility of the scores, retesting was done one hour later. It was shown that the relationship between the resistance settings and the number of revolutions completed by the male and female subjects was inverse and linear. The higher the resistance setting was, the larger was the difference in the scores of the young men and women. When a power curve was derived for each group, a peak power was only identified in the female subjects and this was encountered at a 5-kg resistance setting. The scores obtained at a resistance setting of 5 and 7 kg for female and male subjects respectively showed the best reproducibility. The bicycle ergometer may not be used as an alternative but as a complementary tool to an isokinetic dynamometer.

[Segmental replacement of thoracic aorta with autologous pericardium during growth (author's transl)]. / Le remplacement de l'isthme aortique par autogreffe péricardique durant la croissance.

Surgical treatment of coarctation of the aorta in the newborn often recurs during childhood. As a means of avoiding this complication segmental replacement of the thoracic aorta with autologous pericardium was evaluated. The isthmic aorta of seven mongrel puppies was replaced by a tubular live pericardial autograft. Between 5 and 36 months (average 15) after surgery, the status of the graft was evaluated by hemodynamic, angiographic, macroscopical and histological studies. Growth of the graft was appreciated by comparing its diameter to that of the descending aorta DG/DA. No pressure gradient across the graft was noted. No significant structure of the graft was observed. The internal surface of the graft was smooth, and the wall, slightly thinner than that of the aorta showed no aneurysm. The diameter of the graft was increased (DG mean 37 %) proportionally to the increase of the aorta (DA mean 36 %). Histologically the graft was endothelialized, the wall was thickened and made up of concentric collagen fibers, live fibroblasts and muscular cells. These results show that during the first months of life, the isthmus of the aorta can be replaced by an autologous pericardial graft which will follow the growth of the normal aorta and adapt to its hemodynamic regimen.

Immunochemical and enzymatic studies on the soluble antigens of normal human kidney.

The antigenic composition of the soluble extract from normal human kidney was determined with the combined techniques of immunological absorption, immuno-diffusion and immunoelectrophoresis. The kidney extract contained at least eleven non-serum, tissue antigens; some of them were found in the extracts of other organs, but three were kidney-specific. None of the soluble tissue antigens from human kidney was characterized as a lipoprotein; however, three of them stained with the reagent for glycoproteins. Esterase activities were also demonstrated in four non-serum, renal antigens, one of which was present only in the kidney extract and absent in the other organs extracts.

Blood compatibility of silicone rubber chemically coated with cross-linked albumin.

A new method is reported for chemically coating cross-linked albumin onto silicone rubber in an attempt to improve the blood compatibility of the material. Promising results were obtained in an evaluation using both vena cava and renal embolus antithrombogenicity tests ondogs. Coated silicone rubber rings induced much less renal emboli than uncoated ones after one week implantation.