소아기 일과성 고포스파타아제혈증(Hyperphosphatasemia)에 대한 임상고찰 = Benign Transient Hyperphosphatasemia of Infancy and Childhood

다국어 초록 (Multilingual Abstract)

Purpose:The aim of study is to investigate the clinical manifestations and the etiology of markedly-elevated serum alkaline phosphatase(ALP) levels in children without accompanying liver or bone disease.
Methods:The serum ALP and other biochemical laboratory data of 4,989 patients attending the pediatric department of Ewha Womans University Mokdong Hospital between January 2002 to December 2002 were studied. The subjects' ages ranged from 4 months to 14 years. Among them, serum ALP levels were markedly elevated over 1,000 IU/L in 114 children. Among those 114 children, 97 children without liver or bone disease were reviewed retrospectively.
Results:Of the 114 children with serum ALP activity of over 1,000 IU/L, 97(85.8 percent) children had neither liver or bone disease. The average ALP activity level was 1,539±948　IU/L. The male:female ratio was 1:0.7. Forty-four children(45.3 percent) were between 4 months to 1-year-old, 19 children(19.3 percent) were between 1- to 2-year-old. We observed a seasonal clustering of cases in during October with 10 cases(10.2 percent) and in during December with 11 cases(11.3 percent). The most common clinical presentation was respiratory tract infection in 25 cases(26.9 percent), and acute gastroenteritis in 17 cases(17.5 percent). Forty-four cases had follow-up ALP activity level, 1 month later and the activity decreased under 1,000 IU/L in 37 cases(84percent).
Conclusion:Hyperphosphatasemia is a benign and transient phenomenon. If there are no clinical and laboratory abnormalities of liver and bone, we suggest monitoring monitor the high serum ALP level 1 month later. If decreased, it seems that it is not necessary for specific examinations to exclude other diseases of malignant condition.

국문 초록 (Abstract)

목 적 : 본 연구에서는 간이나 골격계 질환 없이 알카라인 포스파타아제만 증가된 환아들의 임상적 특징을 알아보고 원인과 의의를 알아보고자 하였다. 방 법 : 2002년 1월부터 2002년 12월까지 1년 동안 본원에서 알카라인 포스파타아제를 포함한 생화학 검사를 시행한 4개월부터 14세까지의 소아 4,989명에서 알카라인 포스파타아제가 1,000 IU/L 이상으로 측정된 환자 114명 중 골격계나 간질환이 없었던 환아를 대상으로 하여 의무기록을 이용하여 후향적인 조사를 하였다.결 과 : 알카라인 포스파타아제가 1,000 IU/L 이상으로 측정된 114명 중 97명(85.8%)이 골격계나 간질환 없이 알카라인 포스파타아제가 상승하였으며 남녀 성비는 1 : 0.7로 남아가 많았다. 평균 알카라인 포스파타아제는 1,539±948 IU/L이었으며, 연령 분포는 4개월에서 1세 미만이 44명(45.3%), 1세에서 2세가 19명(19.3%)으로 가장 많았으며, 월별 분포는 9월에서 12월까지 비교적 발병률이 높았다. 동반 질환은 기관지염 및 폐렴, 천식, 크룹 등의 호흡기계 질환이 25명(26.9%), 급성 장염이 17명(17.5 %)으로 가장 많았다. 1개월 후 알카라인 포스파타아제가 추적검사가 시행되었던 44명 중 37명(84%)에서 1,000 IU/L 이하로 떨어졌다.결 론:소아기에 알카라인 포스파타아제 상승하는 것은 골격이나 간의 질환이 없는 경우 비특이적 지표로 생각된다. 일과성 고포스파타아제혈증은 양성적이고 일시적인 현상으로써 임상적으로 간이나 골격계의 이상이 없으며, 1-2개월 후에 다시 검사하여 알카라인 포스파타아제가 감소하는 경우에는, 악성 종양이나 기타 질환을 배제하기 위하여 불필요한 특수 검사들을 시행할 필요가 없을 것으로 보인다.

참고문헌 (Reference)

1 "unexplained elevation of serum alkaline phosphatase" 3289-92, jama1984

2 "What is the cause of benign transient hyperphosphatasemia a Study of 35 Cases" 335-40,

3 "Viral infection of infants and children with benign transient hyperphosphatasemia" 33 : 215-8, 2002

4 "Transient hyperphosphatsemia of infancy:four new cases and a suggested etiology" 26 : 1109-10, 1980

5 "Transient hyperphosphatsemia of infancy" 69 : 235-8, 1980

6 "Transient hyperphosphatasemia:an important condition to recognize" 37 : 359-62, 2001

7 "Transient hyperphosphatasemia of infancy:an insufficiently recognized syndrome" 23 : 292-4, 1977

8 "Transient hyperphosphatasemia of infancy following rotavirus infection" 9 : 283-5, 1984

9 "Transient hyperphosphatasemia of infancy and early childhood:clinical and biochemical features of 21 cases and literature review" 33 : 313-8, 1987

10 "Transient hyperphosphatasemia of infancy and childhood:study of 194 cases" 46 : 1868-9, 2000

11 "Serum bone alkaline phosphatase isoenzyme levels in normal children and children with growth hormone a potential marker for bone formation and response to GH therapy" 82 : 2056-61, 1997

12 "Separation and identification of alkaline phosphatase isoenzymes and isoforms in the serum of healthy persons by isoelectric focusing" 33 : 2171-7, 1987

13 "Recognition of benign transient hyperphosphatasemia" 96 : 108-9, 2003

14 "Isoenzyme studies in transient hyperphosphatasemia of infancy ten new cases and a review of the literature" 736-40,

15 "Follow-up study on effects of height velocity and puberty onset on biochemical markers of bone turnover" 46 : 47-60, 1999

16 "Benign transient hyperphosphatasemia of infancy and childhood" 93 : 1162-4, 2000

17 "Behavior of serum alkaline phosphatase in prematurity" 593-609, zkinderheilkd1954

18 "A study of transient hyperphosphatasemia of infancy and childhood" 16 : 888-96, 1996

19 "A case of transient hyperphosphatasemia" 43 : 856-60, 2000

20 "6 Cases of transient hyperphosphatasemia of infancy and early childhood" 43 : 105-10, 2000