PMID- 27132122
OWN - NLM
STAT- MEDLINE
DCOM- 20180123
LR  - 20180131
IS  - 1873-2364 (Electronic)
IS  - 0960-8966 (Linking)
VI  - 26
IP  - 6
DP  - 2016 Jun
TI  - Parenteral nutrition improves nutritional status, autonomic symptoms and quality 
      of life in transthyretin amyloid polyneuropathy.
PG  - 374-7
LID - S0960-8966(15)30097-3 [pii]
LID - 10.1016/j.nmd.2016.03.006 [doi]
AB  - Transthyretin familial amyloid polyneuropathy (TTR-FAP) is an inherited 
      amyloidosis, leading to death in about ten years in most cases due to cardiac 
      failure or wasting syndrome. Previous studies showed that modified body mass 
      index was related to time before death, duration of gastrointestinal 
      disturbances, malabsorption and functional capacity. We report two patients in 
      whom nutritional status worsened despite diet modification, hypercaloric 
      supplement and two relevant therapeutic approaches such as liver transplant and 
      tafamidis meglumine, respectively. The first patient, a 52-year-old lady carrying 
      Thr49Ala mutation, had a disease duration of twelve years and had lost weight up 
      to 35 kg because of daily diarrhea. The second patient, a 63-year-old man with 
      Glu89Gln mutation and a disease duration of fifteen years, was in the New York 
      Heart Association (NYHA) Functional Classification class III and his weight was 
      39 kg. In both cases, a peripherally inserted central catheter was placed for 
      parenteral nutrition. It allowed to improve their nutritional status and clinical 
      conditions, with body weight gains of 11 and 8 kg in a one year follow-up, 
      respectively. Moreover, reduction of autonomic symptoms including postural 
      hypotension, nausea and diarrhoea was recorded with ameliorated quality of life. 
      Our experience suggests that parenteral nutrition may be useful in reducing 
      complications and disabilities in TTR-FAP patients, even when all dietary 
      adjustments have been ineffective. Reasonably, the improvement in nutritional 
      status may prolong survival in TTR-FAP patients.
CI  - Copyright (c) 2016 Elsevier B.V. All rights reserved.
FAU - Russo, Massimo
AU  - Russo M
AD  - Nemo Sud Clinical Centre, AOU Policlinico, Messina, Italy.
FAU - Vita, Gian Luca
AU  - Vita GL
AD  - Nemo Sud Clinical Centre, AOU Policlinico, Messina, Italy.
FAU - Stancanelli, Claudia
AU  - Stancanelli C
AD  - Unit of Neurology, Department of Clinical and Experimental Medicine, University 
      of Messina, Messina, Italy; Biomedical Department of Internal and Specialistic 
      Medicine, University of Palermo, Palermo, Italy.
FAU - Mazzeo, Anna
AU  - Mazzeo A
AD  - Unit of Neurology, Department of Clinical and Experimental Medicine, University 
      of Messina, Messina, Italy.
FAU - Vita, Giuseppe
AU  - Vita G
AD  - Nemo Sud Clinical Centre, AOU Policlinico, Messina, Italy; Unit of Neurology, 
      Department of Clinical and Experimental Medicine, University of Messina, Messina, 
      Italy. Electronic address: giuseppe.vita@unime.it.
FAU - Messina, Sonia
AU  - Messina S
AD  - Nemo Sud Clinical Centre, AOU Policlinico, Messina, Italy; Unit of Neurology, 
      Department of Clinical and Experimental Medicine, University of Messina, Messina, 
      Italy.
LA  - eng
PT  - Case Reports
PT  - Journal Article
DEP - 20160330
PL  - England
TA  - Neuromuscul Disord
JT  - Neuromuscular disorders : NMD
JID - 9111470
RN  - Amyloidosis, Hereditary, Transthyretin-Related
SB  - IM
MH  - Amyloid Neuropathies, Familial/complications/*diet 
      therapy/genetics/physiopathology
MH  - Autonomic Nervous System Diseases/*diet therapy/etiology/genetics/physiopathology
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - *Nutritional Status
MH  - *Parenteral Nutrition
MH  - *Quality of Life
MH  - Treatment Outcome
OTO - NOTNLM
OT  - Familial amyloid polyneuropathy
OT  - Nutritional status
OT  - Parenteral nutrition
OT  - TTR-FAP
OT  - modified body mass index (mBMI)
EDAT- 2016/05/02 06:00
MHDA- 2018/01/24 06:00
CRDT- 2016/05/02 06:00
PHST- 2015/10/31 00:00 [received]
PHST- 2016/03/24 00:00 [revised]
PHST- 2016/03/28 00:00 [accepted]
PHST- 2016/05/02 06:00 [entrez]
PHST- 2016/05/02 06:00 [pubmed]
PHST- 2018/01/24 06:00 [medline]
AID - S0960-8966(15)30097-3 [pii]
AID - 10.1016/j.nmd.2016.03.006 [doi]
PST - ppublish
SO  - Neuromuscul Disord. 2016 Jun;26(6):374-7. doi: 10.1016/j.nmd.2016.03.006. Epub 
      2016 Mar 30.