Abstract
#Carcinoid syndrome, characterized by symptoms such as diarrhea,
flushing or #wheezing, is caused by an excessive production of serotonin.
Diarrhea and excessive bowel movement negatively affects the quality of
life of these patients. #Somatostatin analogues are efficacious but
cannot optimally control symptoms. A review of clinical efficacy data in
supporting the therapeutic indication for orphan drug designation is
presented focused on the new approved drug, telotristat ethyl, an
inhibitor of the #tryptophan hydroxylase type I enzyme involved in the
synthesis of serotonin. By depleting serotonin in the gastrointestinal
tract, telotristat ethyl interrupts the pathological chain process of
the diarrhea and demonstrated efficacy in patients with carcinoid
syndrome. Carcinoid tumors are a subset of functional #neuroendocrine tumors which
can develop in lungs, thymus, small intestine, ovary, thyroid glands
[1]. The carcinoid syndrome, caused by an excessive production of
serotonin, occurs in less than 10% of the neuroendocrine tumors and
manifest with typical symptoms of diarrhea, abdominal pain and flushing.
These tumors can also secrete other hormones such as growth hormone,
insulin, glucagon, #vasoactive intestinal peptide or #parathyroide hormone
related peptide. The incidence is increasing, in part due to
accessibility to diagnosis and on physician awareness about these forms
of tumors [1], but also to environmental factors, such as smoking or
asbestos exposure [2-4] that have been recently linked to the
development of the neuroendocrine tumors. The diagnosis is based on
symptoms, biological markers (hormones, serotonin or metabolites),
imaging identification and histologic confirmation. The WHO
classification requires histology, mitotic count and proliferation index
determined
by the percent of cells staining positively with a monoclonal
antibody directed against a nuclear antigen in the proliferating cells
(KI-67/MIB-1) [5].
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For more Open Access Medical Articles on BJSTR
Novel Therapy of the #CarcinoidSyndrome Related Diarrhea by Marina Ruxandra Oțelea in BJSTR
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