Impaired gallbladder motility and delayed orocecal transit contribute to pigment gallstone and biliary sludge formation in beta-thalassemia major adults.
OBJECTIVE
Gallbladder and gastrointestinal motility defects exist in gallstones patients and to a lesser extent in pigment gallstone patients. To investigated the role of gallbladder and gastrointestinal motility disorders in pigment gallstone formation in beta-thalassemia major.


METHODS
Twenty-three patients with beta-thalassemia major (16 females; age range 18-37 years) and 70 controls (47 females, age range 18-40 years) were studied for gallbladder and gastric emptying (functional ultrasonography), orocecal transit (OCTT, H(2)-breath test), autonomic dysfunction (sweat-spot, cardiorespiratory reflex tests), bowel habits, gastrointestinal symptoms and quality of life (all with questionnaires). Gallbladder content (ultrasonography) was examined before and during 8-12 mo follow-up.


RESULTS
Gallstones and/or biliary sludge were found in 13 (56%) patients. beta-thalassemia major patients had increased fasting (38.0+/-4.8 mL vs 20.3+/-0.7 mL, P = 0.0001) and residual (7.9+/-1.3 mL vs 5.1+/-0.3 mL, P = 0.002) volume and slightly slower emptying (24.9+/-1.7 min vs 20.1+/-0.7 min, P = 0.04) of the gallbladder, together with longer OCTT (132.2+/-7.8 min vs 99.7+/-2.3 min, P = 0.00003) than controls. No differences in gastric emptying and bowel habits were found. Also, patients had higher dyspepsia (score: 6.7+/-1.2 vs 4.9+/-0.2, P = 0.027), greater appetite (P = 0.000004) and lower health perception (P = 0.00002) than controls. Autonomic dysfunction was diagnosed in 52% of patients (positive tests: 76.2% and 66.7% for parasympathetic and sympathetic involvement, respectively). Patients developing sludge during follow-up (38%, 2 with prior stones) had increased fasting and residual gallbladder volume.


CONCLUSIONS
Adult beta-thalassemia major patients have gallbladder dysmotility associated with delayed small intestinal transit and autonomic dysfunction. These abnormalities apparently contribute together with haemolytic hyperbilirubinemia to the pathogenesis of pigment gallstones/sludge in beta-thalassemia major.
Portincasa
Piero
P
Section of Internal Medicine, Department of Internal and Public Medicine, University Medical School of Bari, P.zza G. Cesare 11, 70124 Bari, Italy. p.portincasa@semeiotica.uniba.it
Moschetta
Antonio
A
Berardino
Massimo
M
Di-Ciaula
Agostino
A
Vacca
Michele
M
Baldassarre
Giuseppe
G
Pietrapertosa
Anna
A
Cammarota
Rosario
R
Tannoia
Nunzia
N
Palasciano
Giuseppe
G
D000293:Adolescent; D000328:Adult; D001342:Autonomic Nervous System Diseases / Q000453:epidemiology; D001646:Bile / Q000502:physiology*; D002432:Cecum; D004068:Digestive System Physiological Phenomena; D005260:Female; D005704:Gallbladder / Q000502:physiology* / Q000503:physiopathology*; D042882:Gallstones / Q000453:epidemiology* / Q000209:etiology; D005746:Gastric Emptying / Q000502:physiology; D005772:Gastrointestinal Transit / Q000502:physiology*; D006801:Humans; D008297:Male; D009055:Mouth; D012016:Reference Values; D017086:beta-Thalassemia / Q000503:physiopathology*