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儿童镰状细胞病:第一部分。实验室诊断、病理生理学与健康维护。

Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance.

作者信息

Wethers D L

机构信息

St Luke's--Roosevelt Hospital Center, New York, New York, USA.

出版信息

Am Fam Physician. 2000 Sep 1;62(5):1013-20, 1027-8.

PMID:10997528
Abstract

Over the past 25 years, morbidity and mortality have decreased significantly in children with sickle cell disease, and screening tests are now available to diagnose the disease in newborns. The incidence of sepsis caused by pneumococcal and Haemophilus influenzae infections has declined because of the prophylactic administration of penicillin soon after birth and the timely administration of pneumococcal and H. influenzae type b vaccines. Optimal nutrition can maximize growth in children with sickle cell disease, and timely screening can identify complications such as retinal damage and chronic renal involvement, thereby ensuring prompt treatment. Family physicians and parents who have been educated about sickle cell disease can detect acute, life-threatening complications such as splenic sequestration crisis and acute chest syndrome at their onset, thereby allowing treatment to be instituted without delay.

摘要

在过去25年里,镰状细胞病患儿的发病率和死亡率显著下降,现在已有筛查测试可用于诊断新生儿疾病。由于出生后不久预防性使用青霉素以及及时接种肺炎球菌和b型流感嗜血杆菌疫苗,由肺炎球菌和流感嗜血杆菌感染引起的败血症发病率有所下降。最佳营养可使镰状细胞病患儿的生长最大化,及时筛查可发现诸如视网膜损伤和慢性肾脏受累等并发症,从而确保及时治疗。接受过镰状细胞病相关教育的家庭医生和家长能够在急性、危及生命的并发症(如脾梗死危机和急性胸部综合征)发作时就及时发现,从而能够立即进行治疗。

相似文献

1
Sickle cell disease in childhood: Part I. Laboratory diagnosis, pathophysiology and health maintenance.儿童镰状细胞病:第一部分。实验室诊断、病理生理学与健康维护。
Am Fam Physician. 2000 Sep 1;62(5):1013-20, 1027-8.
2
Update: new guidelines for the treatment of infants with sickle cell disease. Agency for Health Care Policy and Research.更新:镰状细胞病婴儿治疗新指南。医疗保健政策与研究机构。
Pediatr Nurs. 1993 Nov-Dec;19(6):600-5.
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The young child with sickle cell disease.患有镰状细胞病的幼儿。
Adv Nurse Pract. 1998 Jun;6(6):32-9.
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Acute splenic sequestration crisis in children with sickle cell disease.镰状细胞病患儿的急性脾潴留危象
Saudi Med J. 2001 Dec;22(12):1076-9.
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Newborn screening for sickle cell disease: effect on mortality.镰状细胞病的新生儿筛查:对死亡率的影响。
Pediatrics. 1988 Jun;81(6):749-55.
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Care of the child with sickle cell disease: acute complications.镰状细胞病患儿的护理:急性并发症
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Sickle cell disease in childhood: Part II. Diagnosis and treatment of major complications and recent advances in treatment.儿童镰状细胞病:第二部分。主要并发症的诊断与治疗及治疗的最新进展
Am Fam Physician. 2000 Sep 15;62(6):1309-14.
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[Sickle cell disease: from childhood to adolescence. Management in 2001].[镰状细胞病:从儿童期到青少年期。2001年的管理]
Bull Soc Pathol Exot. 2001 May;94(2):85-9.
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Initial presentation of unscreened children with sickle cell disease: the Toronto experience.镰状细胞病未筛查儿童的初次就诊情况:多伦多的经验
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Management of acute splenic sequestration crisis in sickle cell disease.镰状细胞病急性脾滞留危象的管理
J Assoc Acad Minor Phys. 1990;1(3):67-70.

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Patients with sickle cell disease have increased sensitivity to cold and heat.镰状细胞病患者对冷热更加敏感。
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