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우연히 발견된 무증상 구루병 8예

Eight cases of incidentally diagnosed as subclinical rickets

  • 서지영 (을지대학교 의과대학 소아과학교실) ;
  • 김규리 (을지대학교 의과대학 소아과학교실) ;
  • 이희우 (을지대학교 의과대학 소아과학교실) ;
  • 안영민 (을지대학교 의과대학 소아과학교실)
  • Seo, Ji-Young (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Kim, Curie (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Lee, Hee-Woo (Department of Pediatrics, School of Medicine, Eulji University) ;
  • Ahn, Young-Min (Department of Pediatrics, School of Medicine, Eulji University)
  • 투고 : 2008.01.21
  • 심사 : 2008.07.11
  • 발행 : 2008.08.15

초록

목 적 : 비타민 D는 뼈의 무기질화에 필수적인 역할을 하는 호르몬으로 부족하게 되면 구루병이 생길 수 있다. 본 연구는 비타민 D 결핍증이 잘 발생하는 위험군을 알아보고 그 예방 대책에 대한 관심을 고지하고자 계획하게 되었다. 방 법 : 2003년 3월부터 2007년 7월까지 노원을지병원 소아과에 내원한 환아들중에서 검사 상 우연히 알칼리성인산분해효소가 증가해 있으면서 방사선사진으로 무증상 구루병으로 진단받은 환아들에 대하여 후향적으로 그 특징과 치료 경과를 조사하였다. 결 과 : 남아 6명과 여아 2명의 총 8명이 무증상 구루병으로 진단되었다. 이들의 진단 시 연령은 $12.6{\pm}5.8$개월이었으며, 1월부터 7월 사이에 진단되었고 동반질환으로 폐렴, 요로감염, 장염, 철결핍성 빈혈이 있었다. 이들은 모두 모유 수유를 위주로 하고 있었고, 진단당시 2명의 환아가 몸무게가 3백분위수 미만으로 성장 장애를 보이고 있었다. 진단 시 평균 혈중 알칼리성인산분해효소는 $1,995.8{\pm}739.5IU/L$로 증가되어 있었고, 평균 혈중 칼슘은 $9.5{\pm}0.6mg/dL$, 평균 혈중 인은 $3.6{\pm}1.5mg/dL$였다. 평균 intact-PTH는 $214.8{\pm}155.9pg/mL$로 증가되어 있었고 25-hydroxyvitamin D는 2명을 제외한 6명의 환아에서 30 ng/mL 미만으로 감소되어 있었다. 모든 환자들은 손목 방사선 촬영에서 구루병양 변화를 보이고 있었으나 외관상 골기형이나 발달 장애는 없었다. 이들 중 6명은 3개월간 calcitriol을 투여한 후 생화학적인 검사와 손목 방사선 사진 상의 변화는 호전되었고 정상적으로 성장하였다. 2명은 치료하지 않은 채 저절로 호전되었으며 역시 외관상 성장장애를 보이지는 않았으나 2년간의 추적 관찰 후 현재 1명은 무릎 관절면의 골간단 이형성을 보이고 있으며 다른 1명은 일시적인 척추 측만증을 보였다가 호전되고 있다. 결 론 :무증상 구루병으로 진단된 환아들이 비타민 D 결핍이나 부족으로 인한 것으로 생각되며 모유 수유아에서 비타민 D 결핍증의 빈도가 높으므로 알칼리성인산분해효소가 높은 환자에서는 이를 의심하여 25-hydroxyvitamin D와 손목 방사선 사진을 검사하여 확인하여야 하며 이들 중 일부는 비타민 D 결핍성 구루병으로 진행할 수 있으므로, 모유 수유아에서 비타민 D 보충에 대한 교육이 필요하리라 생각된다.

Purpose : Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. Methods : We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. Results : Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was $12.6{\pm}5.8months$, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was $1995.8{\pm}739.5IU/L$, the mean calcium count was $9.5{\pm}0.6mg/dL$, and the mean phosphorus content was $3.6{\pm}1.5mg/dL$. The mean intact parathyroid hormone was $214.8{\pm}155.9pg/mL$ (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was $82.4{\pm}49.3pg/mL$ (reference range, 2070), and the mean 25-hydroxyvitamin D was $29.6{\pm}10.6ng/mL$ (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. Conclusion : Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

키워드

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피인용 문헌

  1. 비타민 D 결핍성 구루병 영.유아의 임상적 특징 vol.53, pp.2, 2008, https://doi.org/10.3345/kjp.2010.53.2.152
  2. 모유수유아의 비타민 D 섭취에 대한 한국형 가이드라인 제안 vol.26, pp.1, 2012, https://doi.org/10.7778/jpkm.2012.26.1.070
  3. The Study in Vitamin D Concentration in the Blood for Infants with High Level of Alkaline Phosphatase vol.27, pp.1, 2008, https://doi.org/10.7180/kmj.2012.27.1.17