Bone Fractures in Children with Chronic Cholestasis

骨質變化在長期膽汁鬱積病童身上雖不少見，但經治療後仍未有顯著改善的卻不多。本文回顧四位長期膽汁鬱積併發骨折病童（兩人爲Alagille syndrome，另兩人爲膽道閉鎖）的臨床表現、生化檢驗結果、骨的放射線檢查，以及肝髒切片等。在放射線學的檢查方面，只有一位病童符合佝僂病的表現（骨折部位在前臂），其他病童僅有骨密度降低及各別位元於上臂、前臂及腕部的骨折。至於生化檢查的結果方面，符合佝僂症骨折病變的病童顯示低血鈣及低血磷，其中一位病童有副甲狀腺素過低及低血鈣和疑似高血磷，另一位僅有輕微高血磷而已。另外一位病童則顯示血鈣和血磷都在正常範圍之內。經過治療之後，除了臨床診斷爲佝僂病的病童在骨病變方面略有起色之外，其他三位病童並未獲得預期中的改善。所有病童都已經去世。我們認爲長期膽汁鬱積導致末期肝髒病變的病童與骨病變之間的關係仍待釐清，然而就目前已知的知識，至少有佝僂病的問題介入，故在確定長期膽法鬱積的診斷後，持續投與丁種維生素及鈣質以避免更嚴重的骨病變是必要的。

關鍵字

骨折；膽汁鬱積；佝僂病

並列摘要

The clinical features, biochemical data, roentgenographical bone changes and liver histological studies of four children with fractures related to chronic cholestasis are described. Two of them had Alagille syndrome and the other two, biliary atresia. Only one patient presented with forearm fracture associated with typical radiographic evidence of rickets. The others revealed generalized osteopenia together with fractures in arms, forearms, and wrists respectively. Of these, one with rickets had hypocalcemia and hypophosphatemia; one had decreased parathyroid hormone with mild hypocalcemia and hyperphosphatemia; one had hyperphosphatemia solely. Eucalcemia and euphosphatemia were noted in the remaining patient. Their bone lesions deteriorated in spite of treatment, except for the one with clinical rickets. All the patients succumbed eventually. It was concluded that the real pathogenesis of bone lesions in end-stage liver disease of chronic cholestatic children remains unclear. However, because rickets may intervene, continuing efforts to supply vitamin D and calcium since diagnosis of chronic cholestasis on a long-term basis, to prevent fracture, is still mandatory.

並列關鍵字

fracture ； cholestasis ； rickets

國際替代計量

Bone Fractures in Children with Chronic Cholestasis

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