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西伯利亚哈士奇的良性家族性高磷酸酶血症

Benign familial hyperphosphatasemia in Siberian huskies.

作者信息

Lawler D F, Keltner D G, Hoffman W E, Nachreiner R F, Hegstad R L, Herndon P A, Fischer B J

机构信息

Department of Pet Nutrition Research, Ralston Purina Co, St Louis, MO 63264, USA.

出版信息

Am J Vet Res. 1996 May;57(5):612-7.

PMID:8723869
Abstract

OBJECTIVE

To evaluate benign familial hyperphosphatasemia involving serum alkaline phosphatase (SAP) in pups.

DESIGN

Pups with markedly increased SAP activity were evaluated and compared with unaffected siblings, and with other unaffected Siberian Husky pups from the same colony.

ANIMALS

8 related litters of Siberian Husky pups (n = 56).

PROCEDURE

At ages 11 and 16 weeks, pups were given physical examinations and blood was obtained for hematologic and serum biochemical analyses (including electrolytes and isoenzymes of alkaline phosphatase), ionized calcium concentration, and serum parathyroid hormone concentration. Diet, growth and health performance, skeletal radiographs, and genealogical data also were evaluated.

RESULTS

Of 42 pups tested, 17 had markedly high total SAP values. Mean total SAP activity of affected pups at ages 11 and 16 weeks was over 5 times greater than mean total SAP activity of unaffected siblings and other unaffected Siberian Husky pups of the same age (P < 0.001). Clinical, radiologic, and biochemical evaluation of the subjects revealed no other abnormal findings. The source of the increased SAP activity was characterized in 5 affected pups as bone isoenzyme. The mode of inheritance could be deduced from the data, but the trait clearly is familial and autosomal.

CONCLUSION

The condition described in the family of Siberian Huskies bears similarity to human benign, persistent, familial hyperphosphatasemia.

CLINICAL RELEVANCE

Benign familial hyperphosphatasemia should be considered in the differential diagnosis of markedly increased SAP activity in young dogs.

摘要

目的

评估幼犬中涉及血清碱性磷酸酶(SAP)的良性家族性高磷酸酶血症。

设计

对SAP活性显著升高的幼犬进行评估,并与未受影响的同胞以及来自同一犬群的其他未受影响的哈士奇幼犬进行比较。

动物

8窝相关的哈士奇幼犬(n = 56)。

程序

在11周龄和16周龄时,对幼犬进行体格检查,并采集血液进行血液学和血清生化分析(包括电解质和碱性磷酸酶同工酶)、离子钙浓度和血清甲状旁腺激素浓度检测。还对饮食、生长和健康状况、骨骼X光片以及系谱数据进行了评估。

结果

在42只接受检测的幼犬中,17只的总SAP值显著偏高。11周龄和16周龄时受影响幼犬的平均总SAP活性比未受影响的同胞以及同龄的其他未受影响的哈士奇幼犬的平均总SAP活性高出5倍以上(P < 0.001)。对这些幼犬的临床、放射学和生化评估未发现其他异常结果。在5只受影响的幼犬中,升高的SAP活性来源被确定为骨同工酶。虽然可以从数据中推断遗传模式,但该性状显然是家族性的且为常染色体遗传。

结论

哈士奇家族中描述的这种情况与人类良性、持续性、家族性高磷酸酶血症相似。

临床意义

在对幼犬SAP活性显著升高进行鉴别诊断时,应考虑良性家族性高磷酸酶血症。

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