Pain care for patients with epidermolysis bullosa: best care practice guidelines

BMC Medicine

Danette Stanko-Lopp⁶  Lynne G Maxwell²  Anna E Martinez⁷  Anne Lynch-Jordan⁸  Christina Liossi⁴  Emily Harrop³  Julie Good⁵  Kenneth R Goldschneider¹ 

[1] Pain Management Center, Department of Anesthesiology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA;Department of Anesthesiology and Critical Care, Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA;Helen and Douglas Hospices, Oxford and John Radcliffe Hospital, Oxford, USA;Great Ormond Street Hospital for Children NHS Trust, London, UK;Lucille Packard Children’s Hospital, Department of Anesthesia (by courtesy, Pediatrics), Stanford University, Stanford, California, USA;James M. Anderson Center for Health Systems Excellence, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA;National Paediatric Epidermolysis Bullosa Centre, Great Ormond Street Hospital NHS Foundation Trust, London, UK;Pain Management Center and Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children’s Hospital Medical Center, Cincinnati, Ohio, USA

关键词: Dystrophic Epidermolysis Bullosa Research Association International;    Recessive dystrophic epidermolysis bullosa;    Chronic pain;    Acute pain;    DEBRA;    RDEB;    Practice guidelines;    Pain;    Epidermolysis bullosa;   

Others  :  1121403
DOI  :  10.1186/s12916-014-0178-2

 received in 2013-12-19, accepted in 2014-09-09,  发布年份 2014

【 摘 要 】

Background

Inherited epidermolysis bullosa (EB) comprises a group of rare disorders that have multi-system effects and patients present with a number of both acute and chronic pain care needs. Effects on quality of life are substantial. Pain and itching are burdensome daily problems. Experience with, and knowledge of, the best pain and itch care for these patients is minimal. Evidence-based best care practice guidelines are needed to establish a base of knowledge and practice for practitioners of many disciplines to improve the quality of life for both adult and pediatric patients with EB.

Methods

The process was begun at the request of Dystrophic Epidermolysis Bullosa Research Association International (DEBRA International), an organization dedicated to improvement of care, research and dissemination of knowledge for EB patients worldwide. An international panel of experts in pain and palliative care who have extensive experience caring for patients with EB was assembled. Literature was reviewed and systematically evaluated. For areas of care without direct evidence, clinically relevant literature was assessed, and rounds of consensus building were conducted. The process involved a face-to-face consensus meeting that involved a family representative and methodologist, as well as the panel of clinical experts. During development, EB family input was obtained and the document was reviewed by a wide variety of experts representing several disciplines related to the care of patients with EB.

Results

The first evidence-based care guidelines for the care of pain in EB were produced. The guidelines are clinically relevant for care of patients of all subtypes and ages, and apply to practitioners of all disciplines involved in the care of patients with EB. When the evidence suggests that the diagnosis or treatment of painful conditions differs between adults and children, it will be so noted.

Conclusions

Evidence-based care guidelines are a means of standardizing optimal care for EB patients, whose disease is often times horrific in its effects on quality of life, and whose care is resource-intensive and difficult. The guideline development process also highlighted areas for research in order to improve further the evidence base for future care.

【 授权许可】

   
2014 Goldschneider et al.; licensee BioMed Central Ltd.

【 图 表 】

Figure 1.

【 参考文献 】

• [1]Fine JD, Johnson LB, Weiner M, Suchindran C: Assessment of mobility, activities, and pain in different subtypes of epidermolysis bullosa. Clin Exp Dermatol 2004, 29:122-127.
• [2]Tabolli S, Sampogna F, Di Pietro C, Paradisi A, Uras C, Zotti P, Castiglia D, Zambruno G, Abeni D: Quality of life in patients with epidermolysis bullosa. Br J Dermatol 2009, 161:869-877.
• [3]Margari F, Lecce PA, Santamato W, Ventura P, Sportelli N, Annicchiarico G, Bonifazi E: Psychiatric symptoms and quality of life in patients affected by epidermolysis bullosa. J Clin Psychol Med Settings 2010, 17:333-339.
• [4]Van Scheppingen C, Lettinga AT, Duipmans JC, Maathius CG, Jonkman MF: Main problems experienced by children with Epidermolysis Bullosa: a qualitative study with semi-structured interviews. Acta Derm Venerol 2008, 88:143-150.
• [5]Fine J, Bruckner-Tuderman L, Eady RA, Bauer EA, Bauer JW, Has C, Heagerty A, Hintner H, Hovnanian A, Jonkman MF, Leigh I, Marinkovich P, Martinez AE, McGrath JA, Mellerio JE, Moss C, Murrell DF, Shimizu H, Uitto J, Woodley D, Zambruno G: Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification. J Am Acad Dermatol 2014, 70:1103-1126.
• [6][http://www.debra-international.org/homepage.html] webcite Debra International []
• [7]Clark E, Burkett K, Stanko-Lopp D: Let Evidence Guide Every New Decision (LEGEND): an evidence evaluation system for point-of-care clinicians and guideline development teams. J Eval Clin Pract 2009, 15:1054-1060.
• [8][http:/ / www.cincinnatichildrens.org/ service/ j/ anderson-center/ evidence-based-care/ legend/ ] webcite Cincinnati Children’s Hospital Medical Center: LEGEND documents. 2013. .
• [9]Brozek JL, Akl EA, Alonso-Coello P, Lang D, Jaeschke R, Williams JW: Grading quality of evidence and strength of recommendations in clinical practice guidelines. Part 1 of 3. An overview of the GRADE approach and grading quality of evidence about interventions. Allergy 2009, 64:669-677.
• [10]Guyatt GH, Oxman AD, Vist GE, Kunz R, Falck-Ytter Y, Alonso-Coello P, Schünemann HJ: GRADE: an emerging consensus on rating quality of evidence and strength of recommendations. BMJ 2008, 336:924-926.
• [11][http:/ / www.debra-international.org/ med-professionals/ best-clinical-practice-guidelines-b cpg/ pain-management.html] webcite Debra International pain management guidelines page []
• [12]Gatchel RJ, Peng YB, Peters ML, Fuchs PN, Turk DC: The biopsychosocial approach to chronic pain: scientific advances and future directions. Psychol Bull 2007, 133:581-624.
• [13]Gerik SM: Pain management in children: developmental considerations and mind-body therapies. South Med J 2005, 98:295-302.
• [14]Green JP, Barabasz AF, Barrett D, Montgomery GH: Forging ahead: the 2003 APA Division 30 definition of hypnosis. Int J Clin Exp Hypn 2005, 53:259-264.
• [15]Palermo TM, Chambers CT: Parent and family factors in pediatric chronic pain and disability: an integrative approach. Pain 2005, 119:1-4.
• [16]Eccleston C, Palermo TM, Williams AC, Lewandowski A, Morely S: Psychological therapies for the management of chronic and recurrent pain in children and adolescents. Cochrane Database Syst Rev 2009., 2
• [17]Palermo TM, Eccleston C, Lewandowski AS, Williams AC, Morley S: Randomized controlled trials of psychological therapies for management of chronic pain in children and adolescents: an updated meta-analytic review. Pain 2010, 148:387-397.
• [18]Kashikar-Zuck S, Swain NF, Jones BA, Graham TB: Efficacy of cognitive-behavioral intervention for juvenile primary fibromyalgia syndrome. J Rheumatol 2005, 32:1594-1602.
• [19]Eccleston C, Williams AC, Morley S: Psychological therapies for the management of chronic pain (excluding headache) in adults. Cochrane Database Syst Rev 2009., 2
• [20]Richardson J, Smith JE, McCall G, Pilkington K: Hypnosis for procedure-related pain and distress in pediatric cancer patients: a systematic review of effectiveness and methodology related to hypnosis interventions. J Pain Symptom Manage 2006, 31:70-84.
• [21]Uman LS, Chambers CT, McGrath PJ, Kisely S: Psychological interventions for needle-related procedural pain and distress in children and adolescents. Cochrane Database Syst Rev 2006., 4
• [22]Chambers CT, Taddio A, Uman LS, McMurtry CM: Psychological interventions for reducing pain and distress during routine childhood immunizations: a systematic review. Clin Ther 2009, 31:S77-S103.
• [23]Kleiber C, Harper DC: Effects of distraction on children's pain and distress during medical procedures: a meta-analysis. Nurs Res 1999, 48:44-49.
• [24]Liossi C, White P, Hatira P: A randomized clinical trial of a brief hypnosis intervention to control venepuncture-related pain of paediatric cancer patients. Pain 2009, 142:255-263.
• [25]Malloy KM, Milling LS: The effectiveness of virtual reality distraction for pain reduction: a systematic review. Clin Psychol Rev 2010, 30:1011-1018.
• [26]Hanson MD, Gauld M, Wathen CN, Macmillan HL: Nonpharmacological interventions for acute wound care distress in pediatric patients with burn injury: a systematic review. J Burn Care Res 2008, 29:730-741.
• [27]Liossi C, Franck L: Psychological interventions for acute pediatric pain. In Clinical Pain Management Acute Pain. Edited by Macintyre P, Rowbotham D, Walker S. Hodder Arnold, London; 2008:308-323.
• [28]Hagermark O, Wahlgren C: Treatment of itch. Semin Dermatol 1995, 14:320-325.
• [29]Chida Y, Steptoe A, Hirakawa N, Sudo N, Kubo C: The effects of psychological intervention on atopic dermatitis. A systematic review and meta-analysis. Int Arch Allergy Immunol 2007, 144:1-9.
• [30]Azrin NH, Nunn RG: Habit-reversal: a method of eliminating nervous habits and tics. Behav Res Ther 1973, 11:619-628.
• [31]Rosenbaum MS, Ayllon T: The behavioral treatment of neurodermatitis through habit-reversal. Behav Res Ther 1981, 19:313-318.
• [32]Ehlers A, Stangier U, Gieler U: Treatment of atopic dermatitis: a comparison of psychological and dermatological approaches to relapse prevention. J Consult Clin Psychol 1995, 63:624-635.
• [33]Hopton A, MacPherson H: Acupuncture for chronic pain: is acupuncture more than an effective placebo? A systematic review of pooled data from meta-analyses. Pain Pract 2010, 10:94-102.
• [34]Cepeda MS, Carr DB, Lau J, Alvarez H: Music for pain relief. Cochrane Database Syst Rev 2006., 2
• [35]Bussing A, Ostermann T, Ludtke R, Michalsen A: Effects of yoga interventions on pain and pain-associated disability: a meta-analysis. J Pain 2012, 13:1-9.
• [36]Cordier W, Steenkamp V: Herbal remedies affecting coagulation: a review. Pharm Biol 2012, 50:443-452.
• [37]Kashikar-Zuck S, Lynch AM: Psychological interventions for chronic pain. In Pain in Children: A Practical Guide for Primary Care. Edited by Walco GA, Goldschneider KR. Humana Press, Totowa, NJ; 2008:145-152.
• [38]Power N, Liossi C, Franck L: Helping parents to help their child with procedural and everyday pain: practical, evidence-based advice. J Spec Pediatr Nurs 2007, 12:203-209.
• [39]von Baeyer CL, Spagrud LJ: Systematic review of observational (behavioral) measures of pain for children and adolescents aged 3 to 18 years. Pain 2007, 127:140-150.
• [40]Stinson JN, Kavanagh T, Yamada J, Gill N, Stevens B: Systematic review of the psychometric properties, interpretability and feasibility of self-report pain intensity measures for use in clinical trials in children and adolescents. Pain 2006, 125:143-157.
• [41]Goldschneider K, Lucky AW, Mellerio JE, Palisson F, del Carmen Viñuela Miranda M, Azizkhan RG: Perioperative care of patients with epidermolysis bullosa: proceedings of the 5th international symposium on epidermolysis bullosa, Santiago Chile, December 4–6, 2008. Pediatr Anaesth 2010, 20:797-804.
• [42]Goldschneider KR, Lucky AW: Pain management in epidermolysis bullosa. Dermatol Clin 2010, 28:273-282.
• [43]Steffel J, Luscher TF, Ruschitzka F, Tanner FC: Cyclooxygenase-2 inhibition and coagulation. J Cardiovasc Pharmacol 2006, 47:S15-S20.
• [44]Herod J, Denyer J, Goldman A, Howard R: Epidermolysis bullosa in children: pathophysiology, anaesthesia and pain management. Paediatr Anaesth 2002, 12:388-397.
• [45]Manjushree R, Lahiri A, Ghosh BR, Laha A, Handa K: Intranasal fentanyl provides adequate postoperative analgesia in pediatric patients. Can J Anaesth 2002, 49:190-193.
• [46]Borland M, Jacobs I, King B, O'Brien D: A randomized controlled trial comparing intranasal fentanyl to intravenous morphine for managing acute pain in children in the emergency department. Ann Emerg Med 2007, 49:335-340.
• [47]Desjardins PJ, Norris LH, Cooper SA, Reynolds DC: Analgesic efficacy of intranasal butorphanol (Stadol NS) in the treatment of pain after dental impaction surgery. J Oral Maxillofac Surg 2000, 58:19-26.
• [48]Kelly RE: Regional anesthesia in children with epidermolysis bullosa dystrophica. Anesthesiology 1988, 68:469.
• [49]Sopchak AM, Thomas PS, Clark WR: Regional anesthesia in a patient with epidermolysis bullosa. Reg Anesth 1993, 18:132-134.
• [50]Yee LL, Gunter JB, Manley CB: Caudal epidural anesthesia in an infant with epidermolysis bullosa. Anesthesiology 1989, 70:149-151.
• [51]Diwan R, Vas L, Shah T, Raghavendran S, Ponde V: Continuous axillary block for upper limb surgery in a patient with epidermolysis bullosa simplex. Paediatr Anaesth 2001, 11:603-606.
• [52]Englbrecht JS, Langer M, Hahnenkamp K, Ellger B: Ultrasound-guided axillary plexus block in a child with dystrophic epidermolysis bullosa. Anaesth Intensive Care 2010, 38:1101-1105.
• [53]Doi S, Horimoto Y: Subcutaneous tunnelling of an epidural catheter in a child with epidermolysis bullosa. Acta Anaesth Scand 2006, 50:394-395.
• [54]Viscusi ER, Martin G, Hartrick CT, Singla N, Manvelian G: Forty-eight hours of postoperative pain relief after total hip arthroplasty with a novel, extended-release epidural morphine formulation. Anesthesiology 2005, 102:1014-1022.
• [55]Mhuircheartaigh RJ, Moore RA, McQuay HJ: Analysis of individual patient data from clinical trials: epidural morphine for postoperative pain. Br J Anaesth 2009, 103:874-881.
• [56]Hartrick CT, Hartrick KA: Extended-release epidural morphine (DepoDur): review and safety analysis. Expert Rev Neurother 2008, 8:1641-1648.
• [57]Denyer JE: Wound management for children with epidermolysis bullosa. Dermatol Clin 2010, 28:257-264.
• [58]Chou R, Fanciullo GJ, Fine PG, Adler JA, Ballantyne JC, Davies P, Donovan MI, Fishbain DA, Foley KM, Fudin J, Gilson AM, Kelter A, Mauskop A, O'Connor PG, Passik SD, Pasternak GW, Portenoy RK, Rich BA, Roberts RG, Todd KH, Miaskowski C: Clinical guidelines for the use of chronic opioid therapy in chronic noncancer pain. J Pain 2009, 10:113-130.
• [59]Noble M, Treadwell JR, Tregear SJ, Coates VH, Wiffen PJ, Akafomo C, Schoelles KM: Long-term opioid management for chronic noncancer pain. Cochrane Database Syst Rev 2010., 1
• [60]Katz N, Mazer NA: The impact of opioids on the endocrine system. Clin J Pain 2009, 25:170-175.
• [61]Davies D, Vlaming D, Haines C: Methadone analgesia for children with advanced cancer. Pediatr Blood Cancer 2008, 51:393-397.
• [62]Huh B, Park CH: Retrospective analysis of low-dose methadone and QTc prolongation in chronic pain patients. Korean J Anesthesiol 2010, 58:338-343.
• [63]Cruciani RA: Methadone: to ECG or not to ECG…That is still the question. J Pain Symptom Manage 2008, 36:545-552.
• [64]Martin JA, Campbell A, Killip T, Kotz M, Krantz MJ, Kreek MJ, McCarroll BA, Mehta D, Payte JT, Stimmel B, Taylor T, Haigney MC, Wilford BB: QT interval screening in methadone maintenance treatment: report of a SAMHSA expert panel. J Addict Dis 2011, 30:283-306.
• [65]Nicholson AB: Methadone for cancer pain. Cochrane Database Syst Rev 2009., 4
• [66]Camilleri M: Opioid-induced constipation: challenges and therapeutic opportunities. Am J Gastroenterol 2011, 106:835-842.
• [67]Moore RA, Wiffen PJ, Derry S, McQuay HJ: Gabapentin for chronic neuropathic pain and fibromyalgia in adults. Cochrane Database Syst Rev 2011., 3
• [68]Chiu YK, Prendiville JS, Bennett SM, Montgomery CJ, Oberlander TF: Pain management of junctional epidermolysis bullosa in an 11-year-old boy. Pediatr Dermatol 1999, 16:465-468.
• [69]Gray P, Williams B, Cramond T: Successful use of gabapentin in acute pain management following burn injury: a case series. Pain Med 2008, 9:371-376.
• [70]Lara-Corrales I, Mellerio JE, Martinez AE, Green A, Lucky AW, Azizkhan RG, Murrell DF, Agero AL, Kantor PF, Pope E: Dilated cardiomyopathy in epidermolysis bullosa: a retrospective, multicenter study. Pediatr Dermatol 2010, 27:238-243.
• [71]Taibjee SM, Ramani P, Brown R, Moss C: Lethal cardiomyopathy in epidermolysis bullosa associated with amitriptyline. Arch Dis Child 2005, 90:871-872.
• [72]Twillman RK, Long TD, Cathers TA, Mueller DW: Treatment of painful skin ulcers with topical opioids. J Pain Symptom Manage 1999, 17:288-292.
• [73]LeBon B, Zeppetella G, Higginson IJ: Effectiveness of topical administration of opioids in palliative care: a systematic review. J Pain Symptom Manage 2009, 37:913-917.
• [74]Watterson G, Howard R, Goldman A: Peripheral opioids in inflammatory pain. Arch Dis Child 2004, 89:679-681.
• [75]Than MP, Smith RA, Cassidy S, Kelly R, Marsh C, Maderal A, Kirsner RS: Use of a keratin-based hydrogel in the management of recessive dystrophic epidermolysis bullosa. J Dermatol Treat 2013, 24:290-291.
• [76]Lander JA, Weltman BJ, So SS: EMLA and amethocaine for reduction of children’s pain associated with needle insertion. Cochrane Database Syst Rev 2006., 3
• [77]Cepeda MS, Tzortzopoulou A, Thackrey M, Hudcova J, Arora Gandhi P, Schumann R: Adjusting the pH of lidocaine for reducing pain on injection. Cochrane Database Syst Rev 2010., 12
• [78]Arbuckle HA: Bathing for individuals with Epidermolysis bullosa. Dermatol Clin 2010, 28:265-266.
• [79]Cerio R, Dohil M, Jeanine D, Magina S, Mahé E, Stratigos AJ: Mechanism of action and clinical benefits of colloidal oatmeal for dermatologic practice. J Drugs Dermatol 2010, 9:1116-1120.
• [80]Peterson B, Berman S, Arbuckle A: The effectiveness of saltwater baths in the treatment of patients with epidermolysis bullosa.Poster presented at the 37th Annual Meeting of the Society of Pediatric Dermatology, Baltimore July 7–10, 2011.
• [81]Wolfe TR, Braude DA: Intranasal medication delivery for children: a brief review and update. Pediatrics 2010, 126:532-537.
• [82]Ezike HA, Odiakosa MC: Oral ketamine for wound care procedures in adult patients with burns. South Afr J Anaesth Analg 2011, 17:242-248.
• [83]Humphries Y, Melson M, Gore D: Superiority of oral ketamine as an analgesic and sedative for wound care procedures in the pediatric patient with burns. J Burn Care Rehabil 1997, 18:34-36.
• [84]Blonk MI, Koder BG, van den Bemt PM, Huygen FJ: Use of oral ketamine in chronic pain management: a review. Eur J Pain 2010, 14:466-472.
• [85]Bell RF, Eccleston C, Kalso EA: Ketamine as an adjuvant to opioids for cancer pain. Cochrane Database Syst Rev 2012., 11
• [86]Ugur F, Gulcu N, Boyaci A: Oral ketamine for pain relief in a child with abdominal malignancy. Pain Med 2009, 10:120-121.
• [87]Watterson G, Denyer J, Hain R: Skin Symptoms. In Oxford Textbook of Palliative Care for Children. Edited by Goldman A, Hain R, Liben S. Oxford University Press, Oxford, UK; 2005:448-459.
• [88]Burton JH, Auble TE, Fuchs SM: Effectiveness of 50% nitrous oxide/50% oxygen during laceration repair in children. Acad Emerg Med 1998, 5:112-117.
• [89]Rowland AS, Baird DD, Shore DL, Weinberg CR, Savitz DA, Wilcox AJ: Nitrous oxide and spontaneous abortion in female dental assistants. Am J Epidemiol 1995, 141:531-538.
• [90]Nunn JF, Chanarin I, Tanner AG, Owen ER: Megaloblastic bone marrow changes after repeated nitrous oxide anaesthesia. Reversal with folinic acid. Br J Anaesth 1986, 58:1469-1470.
• [91]Sanders RD, Weimann J, Maze M: Biologic effects of nitrous oxide: a mechanistic and toxicologic review. Anesthesiology 2008, 109:707-722.
• [92]Lourenço-Matharu L, Ashley PF, Furness S: Sedation of children undergoing dental treatment. Cochrane Database Syst Rev 2012., 3
• [93]Tyagi P, Dixit U, Tyagi S, Jain A: Sedative effects of oral midazolam, intravenous midazolam and oral diazepam. J Clin Pediatr Dent 2012, 36:383-388.
• [94]Patterson DR, Ptacek JT, Carrougher GJ, Sharar SR: Lorazepam as an adjunct to opioid analgesics in the treatment of burn pain. Pain 1997, 72:367-374.
• [95]Freeman EB, Köglmeier J, Martinez AE, Mellerio JE, Haynes L, Sebire NJ, Lindley KJ, Shah N: Gastrointestinal complications of epidermolysis bullosa in children. Br J Dermatol 2008, 158:1308-1314.
• [96]Fine JD, Mellerio JE: Extracutaneous manifestations and complications of inherited epidermolysis bullosa: part I. Epithelial associated tissues. J Am Acad Dermatol 2009, 61:367-384.
• [97]Travis SP, McGrath JA, Turnbull AJ, Schofield OM, Chan O, O'Connor AF, Mayou B, Eady RA, Thompson RP: Oral and gastrointestinal manifestations of epidermolysis bullosa. Lancet 1992, 340:1505-1506.
• [98]Ergun GA, Lin AN, Dannenberg AJ, Carter DM: Gastrointestinal manifestations of epidermolysis bullosa. A study of 101 patients. Medicine 1992, 71:121-127.
• [99]Marini I, Vecchiet F: Sucralfate: a help during oral management in patients with epidermolysis bullosa. J Periodontol 2001, 72:691-695.
• [100]Buchsel PC: Gelclair oral gel. Clin J Oncol Nurs 2003, 7:109-110.
• [101]Buchsel PC: Polyvinylpyrrolidone-sodium hyaluronate gel (Gelclair): a bioadherent oral gel for the treatment of oral mucositis and other painful oral lesions. Expert Opin Drug Metab Toxicol 2008, 4:1449-1454.
• [102]Cingi C, Songu M, Ural A, Yildirim M, Erdogmus N, Bal C: Effects of chlorhexidine/benzydamine mouth spray on pain and quality of life in acute viral pharyngitis: a prospective, randomized, double-blind, placebo-controlled, multicenter study. Ear Nose Throat J 2010, 89:546-549.
• [103]Vandenplas Y, Rudolph CD, Di Lorenzo C, Hassall E, Liptak G, Mazur L, Sondheimer J, Staiano A, Thomson M, Veereman-Wauters G, Wenzl TG: Pediatric gastroesophageal reflux clinical practice guidelines: joint recommendations of the North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition (NASPGHAN) and the European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN). J Pediatr Gastroenterol Nutr 2009, 49:498-547.
• [104]Nwokediuko SC: Current trends in the management of gastroesophageal reflux disease: a review. ISRN Gastroenterol 2012, 2012:391631.
• [105]Fine JD, Johnson LB, Weiner M, Suchindran C: Gastrointestinal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. J Pediatr Gastroenterol Nutr 2008, 46:147-158.
• [106]Lai-Cheong JE, McGrath JA: What is Kindler syndrome? Skinmed 2011, 9:145-146.
• [107]Azizkhan RG, Stehr W, Cohen AP, Wittkugel E, Farrell MK, Lucky AW, Hammelman BD, Johnson ND, Racadio JM: Esophageal strictures in children with recessive dystrophic epidermolysis bullosa: an 11-year experience with fluoroscopically guided balloon dilatation. J Pediatr Surg 2006, 41:55-60.
• [108]Anderson SH, Meenan J, Williams KN, Eady RA, Prinja H, Chappiti U, Doig L, Thompson RP: Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa. Gastrointest Endosc 2004, 59:28-32.
• [109]Mortell AE, Azizkhan RG: Epidermolysis bullosa: management of esophageal strictures and enteric access by gastrostomy. Dermatol Clin 2010, 28:311-318.
• [110]Dohil R, Aceves SS, Dohil MA: Oral viscous budesonide therapy in children with epidermolysis bullosa and proximal esophageal strictures. J Pediatr Gastroenterol Nutr 2011, 52:776-777.
• [111]Hsieh CH, Huang CJ, Lin GT: Death from colonic disease in epidermolysis bullosa dystrophica. BMC Dermatol 2006, 6:2.
• [112]Belsey JD, Geraint M, Dixon TA: Systematic review and meta analysis: polyethylene glycol in adults with non-organic constipation. Int J Clin Pract 2010, 64:944-955.
• [113]Hanson S, Bansal N: The clinical effectiveness of Movicol in children with severe constipation: an outcome audit. Paediatr Nurs 2006, 18:24-28.
• [114]Shah N, Freeman E, Martinez A, Mellerio J, Smith VV, Lindley KJ, Sebire NJ: Histopathological features of gastrointestinal mucosal biopsy specimens in children with epidermolysis bullosa. J Clin Pathol 2007, 60:843-844.
• [115]Gupta PJ, Heda PS, Shrirao SA, Kalaskar SS: Topical sucralfate treatment of anal fistulotomy wounds: a randomized placebo-controlled trial. Dis Colon Rectum 2011, 54:699-704.
• [116]Alpsoy E, Er H, Durusoy C, Yilmaz E: The use of sucralfate suspension in the treatment of oral and genital ulceration of Behҫet disease: a randomized, placebo-controlled, double-blind study. Arch Dermatol 1999, 135:529-532.
• [117]Nelson RL, Thomas K, Morgan J, Jones A: Non surgical therapy for anal fissure. Cochrane Database Syst Rev 2012., 2
• [118]Poh A, Tan KY, Seow-Choen F: Innovations in chronic anal fissure treatment: a systematic review. World J Gastrointest Surg 2010, 27:231-241.
• [119]Khan MT: Podiatric management in epidermolysis bullosa. Dermatol Clin 2010, 28:325-333.
• [120]Thastum M, Herlin T, Zachariae R: Relationship of pain-coping strategies and pain-specific beliefs to pain experience in children with juvenile idiopathic arthritis. Arthritis Rheum 2005, 53:178-184.
• [121]Terrill PJ, Mayou BJ, McKee PH, Eady RA: The surgical management of dystrophic epidermolysis bullosa (excluding the hand). Br J Plast Surg 1992, 45:426-434.
• [122]Maritsi D, Martinez AE, Mellerio JE, Eleftheriou D, Pilkington CA: An unusual case of epidermolysis bullosa complicated by persistent oligoarticular juvenile idiopathic arthritis; lessons to be learned. Pediatr Rheumatol Online J 2011, 9:13.
• [123]Gubinelli E, Angelo C, Pacifico V: A case of dystrophic epidermolysis bullosa improved with etanercept for concomitant psoriatic arthritis. Am J Clin Dermatol 2010, 11:53-54.
• [124]Fewtrell MS, Allgrove J, Gordon I, Brain C, Atherton D, Harper J, Mellerio JE, Martinez AE: Bone mineralization in children with epidermolysis bullosa. Br J Dermatol 2006, 154:959-962.
• [125]Martinez AE, Mellerio JE: Osteopenia and osteoporosis in epidermolysis bullosa. Dermatol Clin 2010, 28:353-355.
• [126]Bruckner AL, Bedocs LA, Keiser E, Tang JY, Doernbrack C, Arbuckle HA, Berman S, Kent K, Bachrach LK: Correlates of low bone mass in children with generalized forms of epidermolysis bullosa. J Am Acad Dermatol 2011, 65:1001-1009.
• [127]Lacativa PG, Farias ML: Osteoporosis and inflammation. Arq Bras Endocrinol Metabol 2010, 54:123-132.
• [128]Tilg H, Moschen AR, Kaser A, Pines A, Dotan I: Gut, inflammation and osteoporosis: basic and clinical concepts. Gut 2008, 57:684-694.
• [129]Levis S, Theodore G: Summary of AHRQ’s comparative effectiveness review of treatment to prevent fractures in men and women with low bone density or osteoporosis: update of the 2007 report. J Manag Care Pharm 2012, 18:S1-15. discussion S13
• [130]Noguera A, Ros JB, Pavía C, Alcover E, Valls C, Villaronga M, González E: Bisphosphonates, a new treatment for glucocorticoid-induced osteoporosis in children. J Pediatr Endocrinol Metab 2003, 16:529-536.
• [131]Gandrud LM, Cheung JC, Daniels MW, Bachrach LK: Low-dose intravenous pamidronate reduces fractures in childhood osteoporosis. J Pediatr Endocrinol Metab 2003, 16:887-892.
• [132]Falcini F, Trapani S, Ermini M, Brandi ML: Intravenous administration of alendronate counteracts the in vivo effects of glucocorticoids on bone remodeling. Calcif Tissue Int 1996, 58:166-169.
• [133]Chou R, Qaseem A, Snow V, Casey D, Cross JT Jr, Shekelle P, Owens DK: Diagnosis and treatment of low back pain: a joint clinical practice guideline from the American College of Physicians and the American Pain Society. Ann Intern Med 2007, 147:478-491.
• [134]Fine JD, Johnson LB, Weiner M, Stein A, Cash S, Deleoz J, Devries DT, Suchindran C: Eye involvement in inherited epidermolysis bullosa: experience of the National Epidermolysis Bullosa Registry. Am J Ophthalmol 2004, 138:254-262.
• [135]Figueira EC, Murrell DF, Coroneo MT: Ophthalmic involvement in inherited epidermolysis bullosa. Dermatol Clin 2010, 28:143-152.
• [136]Watson SL, Lee MH, Barker NH: Interventions for recurrent corneal erosions. Cochrane Database Syst Rev 2012., 9
• [137]Turner A, Rabiu M: Patching for corneal abrasion. Cochrane Database Syst Rev 2006., 2
• [138]Calder LA, Balasubramanian S, Fergusson D: Topical nonsteroidal anti-inflammatory drugs for corneal abrasions: meta-analysis of randomized trials. Acad Emerg Med 2005, 12:467-473.
• [139]Gibbins S, Stevens BJ, Yamada J, Dionne K, Campbell-Yeo M, Lee G, Caddell K, Johnston C, Taddio A: Validation of the Premature Infant Pain Profile-Revised (PIPP-R). Early Hum Dev 2014, 90:189-193.
• [140]Stevens BJ, Gibbins S, Yamada J, Dionne K, Lee G, Johnston C, Taddio A: The Premature Infant Pain Profile-Revised (PIPP-R): initial validation and feasibility. Clin J Pain 2014, 2014:238-243.
• [141]Hummel P, Puchalski M, Creech SD, Weiss MG: Clinical reliability and validity of the N-PASS: neonatal pain, agitation and sedation scale with prolonged pain. J Perinatol 2008, 28:55-60.
• [142]Krechel SW, Bildner J: CRIES: a new neonatal postoperative pain measurement score. Initial testing of validity and reliability. Paediatr Anaesth 1995, 5:53-61.
• [143]Lawrence J, Alcock D, McGrath P, Kay J, MacMurray SB, Dulberg C: The development of a tool to assess neonatal pain. Neonatal Netw 1993, 12:59-66.
• [144]Manworren RC, Hynan LS: Clinical validation of FLACC: preverbal patient pain scale. Pediatr Nurs 2003, 29:140-146.
• [145]Gill AM, Cousins A, Nunn AJ, Choonara IA: Opiate-induced respiratory depression in pediatric patients. Ann Pharmacother 1996, 30:125-129.
• [146]Purcell-Jones G, Dormon F, Sumner E: The use of opioids in neonates. A retrospective study of 933 cases. Anaesthesia 1987, 42:1316-1320.
• [147]Lynn AM, Slattery JT: Morphine pharmacokinetics in early infancy. Anesthesiology 1987, 66:136-139.
• [148]Kart T, Christrup LL, Rasmussen M: Recommended use of morphine in neonates, infants and children based on a literature review: part 1–pharmacokinetics. Paediatr Anaesth 1997, 7:5-11.
• [149]Yamada J, Stinson J, Lamba J, Dickson A, McGrath PJ, Stevens B: A review of systematic reviews on pain interventions in hospitalized infants. Pain Res Manag 2008, 13:413-420.
• [150]Harrison D, Stevens B, Bueno M, Yamada J, Adams-Webber T, Beyene J, Ohlsson A: Efficacy of sweet solutions for analgesia in infants between 1 and 12 months of age: a systematic review. Arch Dis Child 2010, 95:406-413.
• [151]Cignacco EL, Sellam G, Stoffel L, Gerull R, Nelle M, Anand KJ, Engberg S: Oral sucrose and “facilitated tucking” for repeated pain relief in preterms: a randomized controlled trial. Pediatrics 2012, 129:299-308.
• [152]Mellerio JE, Weiner M, Denyer JE, Pillay EI, Lucky AW, Bruckner A, Palisson F: Medical management of epidermolysis bullosa: Proceedings of the IInd International Symposium of Epidermolysis Bullosa, Santiago, Chile, 2005. Int J Dermatol 2007, 46:795-800.
• [153]Tremlett M, Anderson BJ, Wolf A: Pro-con debate: is codeine a drug that still has a useful role in pediatric practice? Paediatr Anaesth 2010, 20:183-194.
• [154]MacDonald N, MacLeod SM: Has the time come to phase out codeine? CMAJ 2010, 182:1825.
• [155]Allegaert K, Naulaers G: Gabapentin as part of multimodal analgesia in a newborn with epidermolysis bullosa. Paediatr Anaesth 2010, 20:972-973.
• [156]Saroyan JM, Tresgallo ME, Farkouh C, Morel KD, Schecter WS: The use of oral ketamine for analgesia with dressing change in an infant with epidermolysis bullosa: report of a case. Paediatr Dermatol 2009, 26:764-766.
• [157]Jevtovic-Todorovic V: Anesthesia and the developing brain: are we getting closer to understanding the truth? Curr Opin Anaesthesiol 2011, 24:395-399.
• [158]Fine JD, Johnson LB, Weiner M, Suchindran C: Cause-specific risks of childhood death in epidermolysis bullosa. J Pediatr 2008, 152:276-280.
• [159]Yuen WY, Duipmans JC, Molenbuur B, Herpertz I, Mandema JM, Jonkman MF: Long-term follow-up of patient with Herlitz-type junctional epidermolysis bullosa. Br J Dermatol 2012, 167:374-382.
• [160]Kho YC, Rhodes LM, Robertson SJ, Su J, Varigos G, Robertson I, Hogan P, Orchard D, Murrell DF: Epidemiology of epidermolysis bullosa in the antipodes: the Australasian epidermolysis bullosa registry with a focus on Herlitz junctional epidermolysis bullosa. Arch Dermatol 2010, 146:635-640.
• [161]Fine JD, Johnson LB, Weiner M, Li KP, Suchindran C: Epidermolysis bullosa and the risk of life-threatening cancers: the National EB Registry experience, 1986–2006. J Am Acad Dermatol 2009, 60:203-211.
• [162]Fine JD, Johnson LB, Weiner M, Stein A, Cash S, DeLeoz J, Devries DT, Suchindran C: Inherited epidermolysis bullosa and the risk of death from renal disease: experience of the National Epidermolysis Bullosa Registry. Am J Kidney Dis 2004, 44:651-660.
• [163]Fine JD, Hall M, Weiner M, Li KP, Suchindran C: The risk of cardiomyopathy in inherited epidermolysis bullosa. Br J Dermatol 2008, 159:677-682.
• [164]Fine JD, Johnson LB, Weiner M, Suchindran C: Tracheolaryngeal complications of inherited epidermolysis bullosa: cumulative experience of the National Epidermolysis Bullosa Registry. Laryngoscope 2007, 117:1652-1660.
• [165]Craig F, Abu-Saad Huijer H, Benini F, Kuttner L, Wood C, Feraris PC, Zernikow B: IMPaCCT: standards of paediatric palliative care. Eur J Pall Care 2007, 14:1-7.
• [166]Palliative care for children Pediatrics 2000, 106:351-357.
• [167][http://www.who.int/cancer/palliative/definition/en/] webcite World Health Organization Definition of Palliative Care. Available at: .
• [168]Mallipeddi R: Epidermolysis bullosa and cancer. Clin Exp Dermatol 2002, 27:616-623.
• [169]WHO Guidelines on the Pharmacological Treatment of Persisting Pain in Children with Medical Illnesses. WHO Press, World Health Organization, Geneva, Switzerland; 2012.
• [170]Mercadante S: Opioid titration in cancer pain: a critical review. Eur J Pain 2007, 11:823-830.
• [171]Bruera E, Pereira J, Watanabe S, Belzile M, Kuehn N, Hanson J: Opioid rotation in patients with cancer pain: a retrospective comparison of dose ratios between methadone, hydromorphone, and morphine. Cancer 1996, 78:852-857.
• [172]Quigley C: Opioid switching to improve pain relief and drug tolerability. Cochrane Database Syst Rev 2004., 3
• [173]Gagnon B, Almahrezi A, Schreier G: Methadone in the treatment of neuropathic pain. Pain Res Manag 2003, 8:149-154.
• [174]McNicol ED, Carr DB, Eisenberg E: Opioids for neuropathic pain. Cochrane Database Syst Rev 2006., 8
• [175]Clements JA, Nimmo WS, Grant IS: Bioavailability, pharmacokinetics and analgesic activity of ketamine in humans. J Pharm Sci 1982, 71:539-542.
• [176]O’Neil B, Lucas C, Gannon C: Use of a subcutaneous syringe driver in epidermolysis bullosa. Palliat Med 2001, 15:77-78.
• [177]Elsner F, Radbruch L, Loick G, Gaertner J, Sabatowski R: Intravenous versus subcutaneous morphine titration in patients with persisting exacerbation of cancer pain. J Palliat Med 2005, 8:743-750.
• [178]Reymond L, Charles MA, Bowman J, Treston P: The effect of dexamethasone on the longevity of syringe driver subcutaneous sites in palliative care patients. Med J Aust 2003, 178:486-489.
• [179][http://www.appm.org.uk/resources/APPM_Master_Formulary_2012__2nd_edition.pdf] webcite Association of Paediatric Palliative Medicine: Master Formulary. Available at: .
• [180]Mercadante S, Intravaia G, Villari P, Ferrera P, Riina S, Mangione S: Intravenous morphine for breakthrough (episodic-) pain in an acute palliative care unit: a confirmatory study. J Pain Symptom Manage 2008, 35:307-313.
• [181]Mercadante S, Villari P, Ferrera P, Mangione S, Casuccio A: The use of opioids for breakthrough pain in acute palliative care unit by using doses proportional to opioid basal regimen. Clin J Pain 2010, 26:306-309.
• [182]Zeppetella G, Davies AN: Opioids for the management of breakthrough (episodic) pain in cancer patients. Cochrane Database Syst Rev 2013., 10
• [183]Nischler E, Diem A: Pruritis in epidermolysis bullosa. In Life with Epidermolysis Bullosa: Etiology, Diagnosis, Multidisciplinary Care and Therapy. Edited by Fine JD, Hinter H. Springer-Verlag/Wein, Austria; 2008:233-240.
• [184]Jeffry J, Seungil K, Chen ZF: Itch signalling in the nervous system. Physiology 2011, 26:286-292.
• [185]Raap U, Stander S, Metz M: Pathophysiology of itch and new treatments. Curr Opin Allergy Clin Immunol 2011, 11:420-427.
• [186]Goutos I, Eldardiri M, Khan AA, Dziewulski P, Richardson PM: Comparative evaluation of antipruritic protocols in acute burns. The emerging value of gabapentin in the treatment of burns pruritis. J Burn Res 2010, 31:57-63.
• [187][http://www.ebnurse.org/index.php?id=64] webcite EB Nurse. Itching []
• [188]Ahuja RB, Gupta R, Gupta G, Shrivastava P: A comparative analysis of cetirizine, gabapentin and their combination in the relief of post-burn pruritus. Burns 2011, 37:203-207.
• [189]Hundley JL, Yosipovitch G: Mirtazapine for reducing nocturnal itch in patients with chronic pruritus: a pilot study. J Am Acad Dermatol 2004, 50:889-891.
• [190]Davis MP, Frandsen JL, Walsh D, Andresen S, Taylor S: Mirtazapine for pruritus. J Pain Symptom Manage 2003, 25:288-291.
• [191]Shaw RJ, Dayal S, Good J, Bruckner AL, Joshi SV: Psychiatric medications for the treatment of pruritis. Psychosom Med 2007, 69:970-978.
• [192]Zylicz Z, Krainik M: Serotonin reuptake inhibitors and other antidepressants in the treatment of chronic pruritus. Pruritus 2010, 3:301-306.
• [193]Calikoglu E, Anadolu R: Management of generalized pruritus in dominant dystrophic epidermolysis bullosa using low-dose oral cyclosporin. Acta Derm Venereol 2002, 82:380-382.
• [194]Jones EA: Pruritus and fatigue associated with liver disease: is there a role for ondansetron? Expert Opin Pharmacother 2008, 9:645-651.
• [195]Deshpande PV: Ondansetron for paediatric uraemic pruritus: a case report. Pediatr Nephrol 2004, 19:694-696.
• [196]Murphy M, Reaich D, Pai P, Finn P, Carmichael AJ: A randomized, placebo-controlled, double-blind trial of ondansetron in renal itch. Br J Dermatol 2003, 148:314-317.
• [197]Tey HL, Yosipovitch G: Targeted treatment of pruritus: a look into the future. Br J Dermatol 2011, 165:5-17.
• [198]Ahuja RB, Gupta GK: A four arm, double blind, randomized and placebo controlled study of pregabalin in the management of post-burn pruritus. Burns 2013, 39:24-29.