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Bronchial hyperreactivity in patients with familial amyloidotic polyneuropathy and autonomic neuropathy. 1997

O Kawano, and H Kohrogi, and Y Ando, and H Iwagoe, and K Fujii, and J Hamamoto, and T Yamaguchi, and H Okabe, and M Ando
The Department of Laboratory Medicine, Kumamoto University School of Medicine, Japan.

To investigate the role of autonomic regulation on airway reactivity, we performed bronchial inhalation tests of methacholine (MCh) and histamine (Hist) in Japanese patients with familial amyloidotic polyneuropathy (FAP) and autonomic neuropathy. First we examined the FEV1 and Raw in seven patients with FAP and in six normal subjects, then we administered aerosols of increasing concentrations of MCh (0.075 to 25 mg/ml) at about 5-min intervals via a nebulizer controlled by a dosimeter. We measured the FEV1 until either the concentration of MCh producing a 20% reduction from the basal value (PD20) or the maximal concentration was reached. Five of the seven patients with FAP showed bronchial hyperreactivity to MCh, and PD20 to MCh was significantly lower than that of the normal subjects (p < 0.01). Furthermore the PD20 tended to correlate inversely with the severity of autonomic neuropathy (p = 0.052). The bronchial hyperreactivity to MCh was completely blocked by pretreatment inhalation of ipratropium bromide, suggesting the muscarinic receptor-mediated mechanism. Of these five patients with hyperreactivity to MCh, three with low PD20 to MCh (< 50 units) did not respond to Hist, but two with high PD20 (> 50 units) to MCh did, suggesting different mechanisms of hyperreactivity to MCh and Hist in FAP. The PD20 to Hist significantly correlated inversely to the PD20 to MCh (p < 0.05). Histochemical examination revealed marked amyloid deposition in the vagus nerves and tracheal wall in an autopsied patient with FAP and severe autonomic symptoms. These data suggest that patients with FAP and advanced autonomic neuropathy have bronchial hyperreactivity to MCh and/or Hist, probably because of denervation supersensitivity resulting from amyloid deposition in the peripheral autonomic nerves of the airways.

UI MeSH Term Description Entries
D008297 Male Males
D009241 Ipratropium A muscarinic antagonist structurally related to ATROPINE but often considered safer and more effective for inhalation use. It is used for various bronchial disorders, in rhinitis, and as an antiarrhythmic. N-Isopropylatropine,(endo,syn)-(+-)-3-(3-Hydroxy-1-oxo-2-phenylpropoxy)-8-methyl-8-(1-methylethyl)-8-azoniabicyclo(3.2.1)octane,Atrovent,Ipratropium Bromide,Ipratropium Bromide Anhydrous,Ipratropium Bromide Monohydrate,Ipratropium Bromide, (endo,anti)-Isomer,Ipratropium Bromide, (exo,syn)-Isomer,Ipratropium Bromide, endo-Isomer,Itrop,Sch-1000,Sch-1178,N Isopropylatropine,Sch 1000,Sch 1178,Sch1000,Sch1178
D001985 Bronchial Provocation Tests Tests involving inhalation of allergens (nebulized or in dust form), nebulized pharmacologically active solutions (e.g., histamine, methacholine), or control solutions, followed by assessment of respiratory function. These tests are used in the diagnosis of asthma. Allergen Bronchial Provocation Tests,Allergen Challenge, Endobronchial,Antigen Bronchial Provocation Tests,Bronchial Allergen Challenge,Bronchial Challenge Tests,Inhalation Provocation Tests,Provocation Tests, Bronchial,Endobronchial Challenge Tests,Allergen Challenge, Bronchial,Allergen Challenges, Bronchial,Allergen Challenges, Endobronchial,Bronchial Allergen Challenges,Bronchial Challenge Test,Bronchial Provocation Test,Challenge Test, Bronchial,Challenge Test, Endobronchial,Challenge Tests, Bronchial,Challenge Tests, Endobronchial,Challenge, Bronchial Allergen,Challenge, Endobronchial Allergen,Challenges, Bronchial Allergen,Challenges, Endobronchial Allergen,Endobronchial Allergen Challenge,Endobronchial Allergen Challenges,Endobronchial Challenge Test,Inhalation Provocation Test,Provocation Test, Bronchial,Provocation Test, Inhalation,Provocation Tests, Inhalation,Test, Bronchial Challenge,Test, Bronchial Provocation,Test, Endobronchial Challenge,Test, Inhalation Provocation,Tests, Bronchial Challenge,Tests, Bronchial Provocation,Tests, Endobronchial Challenge,Tests, Inhalation Provocation
D005260 Female Females
D006632 Histamine An amine derived by enzymatic decarboxylation of HISTIDINE. It is a powerful stimulant of gastric secretion, a constrictor of bronchial smooth muscle, a vasodilator, and also a centrally acting neurotransmitter. Ceplene,Histamine Dihydrochloride,Histamine Hydrochloride,Peremin
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000336 Aerosols Colloids with a gaseous dispersing phase and either liquid (fog) or solid (smoke) dispersed phase; used in fumigation or in inhalation therapy; may contain propellant agents. Aerosol
D001342 Autonomic Nervous System Diseases Diseases of the AUTONOMIC NERVOUS SYSTEM, including sympathetic, parasympathetic, and enteric nervous systems. Autonomic Disorders,Central Autonomic Nervous System Diseases,Disorders of the Autonomic Nervous System,Dysautonomia,Nervous System Diseases, Autonomic,Nervous System Diseases, Parasympathetic,Nervous System Diseases, Sympathetic,Non-Familial Dysautonomia,Parasympathetic Nervous System Diseases,Peripheral Autonomic Nervous System Diseases,Sympathetic Nervous System Diseases,ANS (Autonomic Nervous System) Diseases,ANS Diseases,Autonomic Central Nervous System Diseases,Autonomic Diseases,Autonomic Nervous System Disorders,Autonomic Peripheral Nervous System Diseases,Segmental Autonomic Dysfunction,ANS Disease,Autonomic Disease,Autonomic Disorder,Autonomic Dysfunction, Segmental,Autonomic Dysfunctions, Segmental,Disorder, Autonomic,Dysautonomia, Non-Familial,Dysautonomias,Non Familial Dysautonomia,Non-Familial Dysautonomias,Segmental Autonomic Dysfunctions
D016085 Bronchoconstrictor Agents Agents causing the narrowing of the lumen of a bronchus or bronchiole. Bronchial-Constricting Agents,Bronchoconstrictor Effect,Bronchoconstrictor Effects,Bronchoconstrictors,Agents, Bronchial-Constricting,Agents, Bronchoconstrictor,Bronchial Constricting Agents,Effect, Bronchoconstrictor,Effects, Bronchoconstrictor

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