AIM: 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...AIM: 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 gallstoneformation in β-thalassemia major.METHODS: Twenty-three patients with β-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, H2-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. β-thalassemia major patients had increased fasting (38.04-4.8 mL vs 20.3±0.7 mL, P= 0.0001) and residual (7.94-1.3 mL vs 5.1±0.3 mL, P= 0.002) volumeb and slightly slower emptying (24.94±2.7 min vs20.2±0.7 min,P = 0.04) of the gallbladder, together with longer OCTT(232.24±7.8 rain vs99.7±2.3 rain, P= 0.00003) than controls.No differences in gastric emptying and bowel habits were found. Also, patients had higher dyspepsia (score: 6.74-1.2vs 4.94-0.2, 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.CONCLUSION: Adult β-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 β-thalassemia major.展开更多
文摘AIM: 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 gallstoneformation in β-thalassemia major.METHODS: Twenty-three patients with β-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, H2-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. β-thalassemia major patients had increased fasting (38.04-4.8 mL vs 20.3±0.7 mL, P= 0.0001) and residual (7.94-1.3 mL vs 5.1±0.3 mL, P= 0.002) volumeb and slightly slower emptying (24.94±2.7 min vs20.2±0.7 min,P = 0.04) of the gallbladder, together with longer OCTT(232.24±7.8 rain vs99.7±2.3 rain, P= 0.00003) than controls.No differences in gastric emptying and bowel habits were found. Also, patients had higher dyspepsia (score: 6.74-1.2vs 4.94-0.2, 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.CONCLUSION: Adult β-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 β-thalassemia major.