Lower urinary tract dysfunction in type 1 familial amyloidotic polyneuropathy in Kumamoto, Japan.
OBJECTIVE
To evaluate lower urinary tract dysfunction of type 1 familial amyloidotic polyneuropathy (FAP) patients in Kumamoto, Japan.


METHODS
Lower urinary tract symptoms were evaluated in FAP patients. Urodynamic studies were evaluated in FAP patients as compared to those in control subjects. The location and distribution of amyloid deposits were evaluated in the urinary bladder in an autopsy case.


RESULTS
In lower urinary symptoms, 86%, 19% and 38% patients showed difficulty in urination, urinary frequency and urinary incontinence. In detrusor function during filling cystometry, 14% patients showed detrusor overactivity. Moreover, 43% patients showed low compliance bladder, 62% and 38% patients showed normal and reduced bladder sensation, respectively. First desire to void (FDV), strong desire to void (SDV) and post-voided residual urine (PVR) were increased in FAP patients as compared to those in control subjects. In the urethral pressure profilometry, 71%, 10% and 19% patients showed incompetent, normal functional and overactive urethral closure mechanism, respectively. Maximum urethral pressure (MUP), maximum urethral closure pressure (MUCP) and functional profile length (FPL) were decreased in FAP patients compared to those in control subjects.


CONCLUSIONS
Autonomic, somatic nerve systems and bladder detrusor musculature might be impaired in lower urinary tract of type 1 FAP patients in Kumamoto, Japan.
Wada
Yoshihiro
Y
Department of Urology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan. pullpullachan326@bridge.ocn.ne.jp
Ando
Yukio
Y
Kai
Nobuyuki
N
Takahashi
Wataru
W
Kikukawa
Hiroaki
H
Nakanishi
Juro
J
Honda
Jiro
J
Jinnouchi
Yoshiteru
Y
Ueda
Shoichi
S
D000328:Adult; D028227:Amyloid Neuropathies, Familial / Q000150:complications* / Q000453:epidemiology; D005260:Female; D006801:Humans; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012720:Severity of Illness Index; D014505:Urban Population; D053201:Urinary Bladder, Overactive / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D014549:Urinary Incontinence / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D016055:Urinary Retention / Q000150:complications* / Q000453:epidemiology / Q000503:physiopathology; D014563:Urodynamics