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Abdominal pain related to mitochondrial neurogastrointestinal encephalomyopathy syndrome may benefit from splanchnic nerve blockade
Author(s) -
ÇELEBI NALAN,
ŞAHIN ALTAN,
CANBAY ÖZGÜR,
ÜZÜMCÜGIL FILIZ,
AYPAR ULKÜ
Publication year - 2006
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2006.01918.x
Subject(s) - medicine , blockade , abdominal pain , weakness , sensory loss , surgery , anesthesia , receptor
Summary Patients diagnosed with abdominal pain related to mitochondrial neurogastrointestinal encephalopathy (MNGIE) may benefit from splanchnic nerve blockade. MNGIE, varying in age of onset and rate of progression, is caused by loss of function mutation in thymidine phosphorylase gene. Gastrointestinal dysmotility, pseudo‐obstruction and demyelinating sensorimotor peripheral neuropathy (stocking‐glove sensory loss, absent tendon reflexes, distal limb weakness, and wasting) are the most prominent manifestations. Patients usually die in early adulthood (mean 37.6 years; range 26–58 years). We report a case of an 18‐year‐old patient with MNGIE. Our patient's abdominal pain was relieved after splanchnic nerve blockade.