Hijazi Z, Onadeko B O, Khadadah M, Haider M Z, Adekile A D, Al-Habashi H
Department of Pediatrics, Kuwait University, Safat 13110, Kuwait.
Int J Clin Pract. 2005 Feb;59(2):163-7. doi: 10.1111/j.1742-1241.2004.00216.x.
Conflicting ventilatory defects have been reported in children with sickle cell disease (SCD). In Kuwait, the disease is relatively mild with a low incidence of acute chest syndrome and other complications, presumably due to the Arab-Indian haplotype chromosomal background and elevated Hb F levels. There have been no previous studies of pulmonary function in patients with this haplotype. Pulmonary function test (PFT) was carried out on 28 steady state children with SCD (21 homozygous sickle cell (SS), seven S beta(o) thal) and two group of controls: 17 age- and sex-matched healthy children and 10 children with HbH disease. The charts of the SCD patients were reviewed for frequency of acute chest syndrome and vaso-occlusive crisis. The mean values of forced vital capacity (FVC) (83.2 +/- 11.9 vs. 91.2 +/- 11.7) and vital capacity (VC) (81.5 +/- 11.8 vs. 90.5 +/- 10.9) were significantly lower in the SS patients compared with healthy controls (p < 0.05). Similarly, these values were significantly lower than in those of the HbH group (p < 0.001 for VC and p < 0.01 for FVC). The mean forced expiratory volume in 1 s (FEV1) was lower in SS patients (86.4 +/- 11.5) compared with healthy controls (94.2 +/- 14.2), but the difference was not significant (p = 0.07). Also, the FEV1 was significantly lower in SS patients than in the HbH group (p < 0.001). There was no significant difference in the PFT parameters between SS patients with acute chest syndrome and those without. Although patients with frequent vaso-occlusive crisis had lower PFT parameters, the differences were not significant in comparison to those with infrequent crisis. This study revealed an early restrictive and obstructive pulmonary function pattern in steady state children with SCD. The finding also indicates that the changes of PFT parameters in SS patients could not be attributed to anaemia per se as patients with HbH who also have chronic anaemia did not show similar changes. This observation underscores the early occurrence of pulmonary involvement, even in patients with an otherwise relatively mild SCD.
据报道，镰状细胞病（SCD）患儿存在相互矛盾的通气功能缺陷。在科威特，该病相对较轻，急性胸综合征和其他并发症的发生率较低，这可能归因于阿拉伯 - 印度单倍型染色体背景和较高的Hb F水平。此前尚无关于具有这种单倍型患者肺功能的研究。对28例处于稳定期的SCD患儿（21例纯合子镰状细胞（SS），7例Sβ⁰地中海贫血）以及两组对照进行了肺功能测试：17例年龄和性别匹配的健康儿童以及10例HbH病患儿。回顾了SCD患者的病历，统计急性胸综合征和血管闭塞性危机的发生频率。与健康对照组相比，SS患者的用力肺活量（FVC）平均值（83.2±11.9 vs. 91.2±11.7）和肺活量（VC）平均值（81.5±11.8 vs. 90.5±10.9）显著降低（p < 0.05）。同样，这些值也显著低于HbH组（VC的p < 0.001，FVC的p < 0.01）。SS患者的1秒用力呼气量（FEV1）平均值（86.4±11.5）低于健康对照组（94.2±14.2），但差异不显著（p = 0.07）。此外，SS患者的FEV1显著低于HbH组（p < 0.001）。有急性胸综合征的SS患者与无急性胸综合征的患者在肺功能测试参数上无显著差异。尽管频繁发生血管闭塞性危机的患者肺功能测试参数较低，但与不频繁发生危机的患者相比，差异不显著。本研究揭示了处于稳定期的SCD患儿存在早期限制性和阻塞性肺功能模式。该发现还表明，SS患者肺功能测试参数的变化不能归因于贫血本身，因为同样患有慢性贫血的HbH病患者并未表现出类似变化。这一观察结果强调了即使在其他方面病情相对较轻的SCD患者中，肺部受累也会早期出现。
