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Effect of splenectomy on destructive bone changes in children with chronic (Type I) Gaucher disease.

作者信息

Ashkenazi A, Zaizov R, Matoth Y

出版信息

Eur J Pediatr. 1986 Apr;145(1-2):138-41. doi: 10.1007/BF00441877.

DOI:10.1007/BF00441877
PMID:3732318
Abstract

The incidence and severity of osteolytic bone changes in patients with chronic (Type I) Gaucher disease splenectomized in the first decade of life were compared to those in patients of the same age group and similar degree of severity of the disease in whom the spleen remained intact at least until the second half of the second decade. The size of the spleen, measured by palpation, was used as an index of severity. In the splenectomized group osteolytic changes appeared within a few months following splenectomy in six out of eight cases. The changes were severe in five cases and moderate in one. In contrast, in the non-splenectomized group, evidence of bone destruction was found in two out of eight patients and classified as mild in both cases. Furthermore, in three patients in this group, who remained free of bone destruction until splenectomy in the second half of the second decade, osteolytic lesions appeared soon after the operation. Children with chronic Gaucher disease can be spared a great deal of suffering caused by bone disease, if splenectomy is avoided or postponed as far as possible.

摘要

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本文引用的文献

1
METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.葡萄糖脑苷脂的代谢。II. 戈谢病中酶缺乏的证据。
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1 型戈谢病中的高血铁黄素症和铁过载。
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Management of non-neuronopathic Gaucher disease with special reference to pregnancy, splenectomy, bisphosphonate therapy, use of biomarkers and bone disease monitoring.非神经元型戈谢病的管理,特别涉及妊娠、脾切除术、双膦酸盐治疗、生物标志物的使用及骨病监测。
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Paediatric non-neuronopathic Gaucher disease: recommendations for treatment and monitoring.小儿非神经元型戈谢病:治疗与监测建议
Eur J Pediatr. 2004 Feb;163(2):67-75. doi: 10.1007/s00431-003-1363-z. Epub 2003 Dec 16.
7
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Eur J Pediatr. 2004 Feb;163(2):58-66. doi: 10.1007/s00431-003-1362-0. Epub 2003 Dec 16.
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Remaining problems in the management of patients with Gaucher disease.
J Inherit Metab Dis. 2001;24 Suppl 2:122-6; discussion 87-8. doi: 10.1023/a:1012452715079.
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Arch Dis Child. 1996 Sep;75(3):218-22. doi: 10.1136/adc.75.3.218.
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