Aged ; Aged, 80 and over ; Fatal Outcome ; Female ; Glycogen Storage Disease Type I ; complications ; Hepatitis E ; complications ; Hong Kong ; Humans ; Male ; Middle Aged ; Risk Factors

OBJECTIVETo study the status of iron deposition in patients with β-thalassemia intermedia and major in mainland China.

METHODSThe status of transfusion and chelation was examined in 39 patients with β-thalassemia intermedia or major. Serum ferritin levels were measured. MRI T2* technique was used to detect cardiac and hepatic iron deposition.

RESULTSSerum ferritin levels ranged from the minimum of 1500 ng/mL up to a maximum of 11491 ng/mL. From liver MRI T2* measurement, 15 cases had severe hepatic iron deposition (38%) and moderate deposition was found in 15 cases (38%), mild in 7 cases (18%), and normal in 2 cases (5%). Heart MRI T2* showed severe heart iron deposition in 7 cases (18%), mild in 5 cases (13%), and normal in 27 cases (69%). One case had cardiac arrhythmia. Four cases were over 20 years of age, and presented with gonadal function hypoplasia. The majority of patients did not receive regular transfusion and they had delayed, suboptimal chelation due to financial problems. Serum ferritin level was closely related with timing and dosage of chelation.

CONCLUSIONSIn patients with β-thalassemia who do not receive early regular transfusion and iron chelation therapy, iron deposition may occur at an early age. Important organs and tissue functional lesions and related complications also result. Relevant agencies and family members should be aware of this trend and develop appropriate strategies to improve the medical condition and quality of life of patients with this disorder.

Adolescent ; Adult ; Blood Transfusion ; Child ; Female ; Ferritins ; blood ; Humans ; Iron ; metabolism ; Liver ; metabolism ; Magnetic Resonance Imaging ; methods ; Male ; Myocardium ; metabolism ; beta-Thalassemia ; metabolism ; therapy

Age-related sporadic cerebral small vessel disease (CSVD) has gained increasing attention over the past decades because of its increasing prevalence associated with an aging population. The widespread application of and advances in brain magnetic resonance imaging in recent decades have significantly increased researchers' understanding in the in vivo evolution of CSVD, its impact upon the brain, its risk factors, and the mechanisms that explain the various clinical manifestation associated with sporadic CSVD. In this review, we aimed to provide an update on the pathophysiology, risk factors, biomarkers, and the determinants and spectrum of the clinical manifestation of sporadic CSVD.
Aged ; Aging ; Brain/diagnostic imaging* ; Cerebral Small Vessel Diseases/epidemiology* ; Humans ; Magnetic Resonance Imaging ; Pandemics