期刊论文详细信息
BMC Gastroenterology
Outcome of gastric emptying and gastrointestinal symptoms after liver transplantation for hereditary transthyretin amyloidosis
Ole B Suhr1  Intissar Anan1  Pontus Karling1  Torbjörn Sundström2  Jonas Wixner1 
[1] Departments of Public Health and Clinical Medicine, Umeå University, Umeå, S-90187, Sweden;Radiation Sciences, Umeå University, Umeå, S-90187, Sweden
关键词: Transthyretin;    Liver;    Transplantation;    Gastric emptying;    Functional gastrointestinal disorders;    Hereditary;    Amyloidosis;   
Others  :  1211537
DOI  :  10.1186/s12876-015-0284-4
 received in 2014-09-26, accepted in 2015-04-17,  发布年份 2015
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【 摘 要 】

Background

Hereditary transthyretin amyloid (ATTR) amyloidosis is a rare but fatal autosomal dominant condition that is present all over the world. A liver transplantation has been shown to halt the progress of the disease in selected patients and is currently considered to be the standard treatment. Gastrointestinal manifestations are common in hereditary ATTR amyloidosis and are important for the patients’ morbidity and mortality. The aim of this study was to evaluate the long-term outcome of gastric emptying, gastrointestinal symptoms and nutritional status after liver transplantation for the disease.

Methods

Swedish patients with hereditary ATTR amyloidosis transplanted between 1990 and 2012 were included. A standardized method for measuring gastric emptying with a Tc99m-labelled meal followed by scintigraphy was utilized. Validated questionnaires were used to assess gastrointestinal symptoms and the modified body mass index (mBMI), in which BMI is multiplied by s-albumin, was used to evaluate nutritional status. Non-parametrical statistical tests were used.

Results

Gastric emptying rates and nutritional statuses were evaluated approximately eight months before and two and five years after liver transplantation, whereas gastrointestinal symptoms were assessed in median nine months before and two and nine years after transplantation. No significant change was found in gastric emptying (median half-time 137 vs. 132 vs. 125 min, p = 0.52) or nutritional status (median mBMI 975 vs. 991 vs. 973, p = 0.75) after transplantation. Gastrointestinal symptom scores, however, had increased significantly over time (median score 7 vs. 10 vs. 13, p < 0.01).

Conclusions

Gastric emptying and nutritional status were maintained after liver transplantation for hereditary ATTR amyloidosis, although gastrointestinal symptom scores had increased over time.

【 授权许可】

   
2015 Wixner et al.; licensee BioMed Central.

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【 参考文献 】
  • [1]Ando Y, Nakamura M, Araki S: Transthyretin-related familial amyloidotic polyneuropathy. Arch Neurol 2005, 62:1057-62.
  • [2]Saraiva MJ: Transthyretin amyloidosis: a tale of weak interactions. FEBS Lett 2001, 498:201-3.
  • [3]Sousa MM, Saraiva MJ: Neurodegeneration in familial amyloid polyneuropathy: from pathology to molecular signaling. Prog Neurobiol 2003, 71:385-400.
  • [4]Merlini G, Westermark P: The systemic amyloidoses: clearer understanding of the molecular mechanisms offers hope for more effective therapies. J Intern Med 2004, 255:159-78.
  • [5]Pepys MB: A molecular correlate of clinicopathology in transthyretin amyloidosis. J Pathol 2009, 217:1-3.
  • [6]Suhr O, Danielsson A, Holmgren G, Steen L: Malnutrition and gastrointestinal dysfunction as prognostic factors for survival in familial amyloidotic polyneuropathy. J Intern Med 1994, 235:479-85.
  • [7]Suhr O, Danielsson A, Rydh A, Nyhlin N, Hietala SO, Steen L: Impact of gastrointestinal dysfunction on survival after liver transplantation for familial amyloidotic polyneuropathy. Dig Dis Sci 1996, 41:1909-14.
  • [8]Steen L, Ek B: Familial amyloidosis with polyneuropathy. A long-term follow-up of 21 patients with special reference to gastrointestinal symptoms. Acta Med Scand 1983, 214:387-97.
  • [9]Suhr OB, Svendsen IH, Andersson R, Danielsson A, Holmgren G, Ranlov PJ: Hereditary transthyretin amyloidosis from a Scandinavian perspective. J Intern Med 2003, 254:225-35.
  • [10]Holmgren G, Ericzon BG, Groth CG, Steen L, Suhr O, Andersen O, et al.: Clinical improvement and amyloid regression after liver transplantation in hereditary transthyretin amyloidosis. Lancet 1993, 341:1113-6.
  • [11]Suhr OB, Ericzon B-G: Selection of hereditary transthyretin amyloid patients for liver transplantation: the Swedish experience. Amyloid 2012, 19(Suppl 1):78-80.
  • [12]Parrilla P, Ramirez P, Andreu LF, Bueno SF, Robles R, Miras M, et al.: Long-term results of liver transplantation in familial amyloidotic polyneuropathy type I. Transplantation 1997, 64:646-9.
  • [13]Pomfret EA, Lewis WD, Jenkins RL, Bergethon P, Dubrey SW, Reisinger J, et al.: Effect of orthotopic liver transplantation on the progression of familial amyloidotic polyneuropathy. Transplantation 1998, 65:918-25.
  • [14]Suhr OB, Wiklund U, Ando Y, Ando E, Olofsson BO: Impact of liver transplantation on autonomic neuropathy in familial amyloidotic polyneuropathy: an evaluation by spectral analysis of heart rate variability. J Intern Med 1997, 242:225-9.
  • [15]Jonsén E, Suhr OB, Tashima K, Athlin E: Early liver transplantation is essential for familial amyloidotic polyneuropathy patients’ quality of life. Amyloid 2001, 8:52-7.
  • [16]De Carvalho M, Conceição I, Bentes C, Luís MLS: Long-term quantitative evaluation of liver transplantation in familial amyloid polyneuropathy (Portuguese V30M). Amyloid 2002, 9:126-33.
  • [17]Ohya Y, Okamoto S, Tasaki M, Ueda M, Jono H, Obayashi K, et al.: Manifestations of transthyretin-related familial amyloidotic polyneuropathy: long-term follow-up of Japanese patients after liver transplantation. Surg Today 2011, 41:1211-8.
  • [18]Okamoto S, Zhao Y, Lindqvist P, Backman C, Ericzon B-G, Wijayatunga P, et al.: Development of cardiomyopathy after liver transplantation in Swedish hereditary transthyretin amyloidosis (ATTR) patients. Amyloid 2011, 18:200-5.
  • [19]Lång K, Wikström L, Danielsson A, Tashima K, Suhr OB: Outcome of gastrointestinal complications after liver transplantation for familial amyloidotic polyneuropathy. Scand J Gastroenterol 2000, 35:985-9.
  • [20]Suhr OB, Anan I, Ahlström KR, Rydh A: Gastric emptying before and after liver transplantation for familial amyloidotic polyneuropathy, Portuguese type (Val30Met). Amyloid 2003, 10:121-6.
  • [21]Grybäck P, Hermansson G, Lyrenäs E, Beckman KW, Jacobsson H, Hellström PM: Nationwide standardisation and evaluation of scintigraphic gastric emptying: reference values and comparisons between subgroups in a multicentre trial. Eur J Nucl Med 2000, 27:647-55.
  • [22]Wixner J, Karling P, Rydh A, Hörnsten R, Wiklund U, Anan I, et al.: Gastric emptying in hereditary transthyretin amyloidosis: the impact of autonomic neuropathy. Neurogastroenterol Motil 2012, 24:1111-e568.
  • [23]Jonsen E, Suhr O, Athlin E, Wikström L: Quality of life after liver transplantation in patients with familial amyloidotic polyneuropathy. Amyloid 1996, 3:124-9.
  • [24]Gnjidic D, Hilmer SN, Blyth FM, Naganathan V, Waite L, Seibel MJ, et al.: Poly-pharmacy cutoff and outcomes: five or more medicines were used to identify community-dwelling older men at risk of different adverse outcomes. J Clin Epidemiol 2012, 65:989-95.
  • [25]Moen J, Antonov K, Larsson CA, Lindblad U, Nilsson JLG, Råstam L, et al.: Factors associated with multiple medication use in different age groups. Ann Pharmacother 2009, 43:1978-85.
  • [26]Berk JL, Suhr OB, Obici L, Sekijima Y, Zeldenrust SR, Yamashita T, et al.: Repurposing diflunisal for familial amyloid polyneuropathy: a randomized clinical trial. JAMA 2013, 310:2658-67.
  • [27]Coelho T, Maia LF, da Silva AM, Cruz MW, Planté-Bordeneuve V, Suhr OB, et al.: Long-term effects of tafamidis for the treatment of transthyretin familial amyloid polyneuropathy. J Neurol 2013, 260:2802-14.
  • [28]Suhr OB, Holmgren G, Steen L, Wikström L, Norden G, Friman S, et al.: Liver transplantation in familial amyloidotic polyneuropathy. Follow-up of the first 20 Swedish patients. Transplantation 1995, 60:933-8.
  • [29]Tashima K, Ando Y, Terazaki H, Yoshimatsu S, Suhr OB, Obayashi K, et al.: Outcome of liver transplantation for transthyretin amyloidosis: follow-up of Japanese familial amyloidotic polyneuropathy patients. J Neurol Sci 1999, 171:19-23.
  • [30]Adams D, Samuel D, Goulon-Goeau C, Nakazato M, Costa PM, Feray C, et al.: The course and prognostic factors of familial amyloid polyneuropathy after liver transplantation. Brain 2000, 123(Pt 7):1495-504.
  • [31]Yamamoto S, Wilczek HE, Nowak G, Larsson M, Oksanen A, Iwata T, et al.: Liver transplantation for familial amyloidotic polyneuropathy (FAP): a single-center experience over 16 years. Am J Transplant 2007, 7:2597-604.
  • [32]Ginsburg PM, Thuluvath PJ: Diarrhea in liver transplant recipients: etiology and management. Liver Transpl 2005, 11:881-90.
  • [33]Herrero JI, Benlloch S, Bernardos A, Bilbao I, Castells L, Castroagudin JF, et al.: Gastrointestinal complications in liver transplant recipients: MITOS study. Transplant Proc 2007, 39:2311-3.
  • [34]Sgourakis G, Radtke A, Fouzas I, Mylona S, Goumas K, Gockel I, et al.: Corticosteroid-free immunosuppression in liver transplantation: a meta-analysis and meta-regression of outcomes. Transpl Int 2009, 22:892-905.
  • [35]Lankarani KB, Eshraghian A, Nikeghbalian S, Janghorban P, Malek-Hosseini SA: New onset diabetes and impaired fasting glucose after liver transplant: risk analysis and the impact of tacrolimus dose. Exp Clin Transplant 2014, 12:46-51.
  • [36]Chang L, Toner BB, Fukudo S, Guthrie E, Locke GR, Norton NJ, et al.: Gender, age, society, culture, and the patient’s perspective in the functional gastrointestinal disorders. Gastroenterology 2006, 130:1435-46.
  • [37]Rayner CK, Horowitz M: Physiology of the ageing gut. Curr Opin Clin Nutr Metab Care 2013, 16:33-8.
  • [38]Conceição I, De Carvalho M: Clinical variability in type I familial amyloid polyneuropathy (Val30Met): comparison between late- and early-onset cases in Portugal. Muscle Nerve 2007, 35:116-8.
  • [39]Koike H, Sobue G: Late-onset familial amyloid polyneuropathy in Japan. Amyloid 2012, 19(Suppl 1):55-7.
  • [40]Okamoto S, Wixner J, Obayashi K, Ando Y, Ericzon B-G, Friman S, et al.: Liver transplantation for familial amyloidotic polyneuropathy: impact on Swedish patients’ survival. Liver Transpl 2009, 15:1229-35.
  • [41]Agréus L, Svärdsudd K, Nyrén O, Tibblin G: The epidemiology of abdominal symptoms: prevalence and demographic characteristics in a Swedish adult population. A report from the Abdominal Symptom Study. Scand J Gastroenterol 1994, 29:102-9.
  • [42]Corazziari E: Definition and epidemiology of functional gastrointestinal disorders. Best Pract Res Clin Gastroenterol 2004, 18:613-31.
  • [43]Abell TL, Camilleri M, Donohoe K, Hasler WL, Lin HC, Maurer AH, et al.: Consensus recommendations for gastric emptying scintigraphy: a joint report of the American Neurogastroenterology and Motility Society and the Society of Nuclear Medicine. J Nucl Med Technol 2008, 36:44-54.
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