镰刀状红细胞贫血患儿的右心室异常改变:进展性肺循环血管阻力增高的临床证据

Objective:To assess the effects of sickle cell anemia (SCA)-on the right ventricle (RV). Study design:Echocardiograms of 32 children with SCA were compared with age-matched healthy controls. RV measurements included diastolic area index,fractional area change,free-wall mass index,ejection time corrected for heart rate (ETc),and tricuspid regurgitation(TR) gradient. Results:SCA subjects had elevated RV ETc(mean ±standard deviation,0.369 ±0.030 sec vs 0.351 ±0.022 sec; P < 0.01),diastolic area index (19.9 ±2.4 cm2/m2 vs13.2 ±2.1 cm2/m2; P < 0.01) and free-wall mass index (33.2 ±4.4 g/m2 vs 23.9 ±4.3 g/m2; P < 0.01),whereas RV fractional area change (37 ±8%vs 36 ±4%) was not different from controls. Although RV diastolic area index in SCA paralleled the normal range over time,RV free-wall mass index continued to gradually rise throughout childhood (r = 0.42; P < 0.05). TR gradients>2.5m/sec,consistent with pulmonary hypertension,were found in 5 (16%) of SCA subjects,all older than 9 years.Conclusions:RV preload and systolic function do not worsen during childhood in SCA; however,RV mass index and the prevalence of pulmonary hypertension increase consistent with rising pulmonary vascular resistance.

急性胸部综合征对于镰刀状红细胞贫血患儿肺功能的影响

Objective:To test the hypothesis that children with sicklecell disease (SCD) who experienced an acute chest syndrome(ACS) hospitalization episode would have worse lung function than children with SCD without ACS episodes. Study design:Forced expiratory volume in 1 second (FEV1); forced vitalcapacity (FVC); FEV1/FVC ratio; peak expiratory flow (PEF);forced expiratory flow at 25%(FEF25),50%(FEF50),and 75%(FEF75) of FVC; airway resistance (Raw); and lung volumes were compared in 20 children with ACS and 20 aged-matched children without ACS (median age,11 years; range,6 to 16 years). Fourteen age-matched pairs were assessed before and after bronchodilator use. Results:The mean Raw (P = 0.03),TLC (P = 0.01),and RV (P = 0.003) were significantly higher in the group with ACS than in the group without ACS. There were no significant differences in the changes in lung function test results in response to bronchodilator administration between the 2 groups,but the children with ACS had a lower FEF25 (P = 0.04) and FEF75 (P = 0.03) pre-bronchodilator use and a lower mean FEV1/FVC ratio (P = 0.03) and FEF75 (P =0.03) post-bronchodilator use. Conclusions:Children with SCD who experienced an ACS hospitalization episode had significant differences in lung function compared with those who did not experience ACS episodes. Our results are compatible with the hypothesis that ACS episodes predispose children to increased airway obstruction.

RETINAL ARTERIOLAR OBSTRUCTION IN A PATIENT WITH SICKLE CELL TRAIT AND GLAUCOMA SECONDARY TO TRAU-MATIC HYPHEMA

Purpose: To report an acute monocular retinal vascular occlusion in a patient with sickle cell trait and glaucoma secondary to traumatic hyphema. Methods and Results: Case report. A 35 year old Arab man who sustained blunt trauma to his right eye was hospitalized with hyphema, increased intraocular pres-