Alos Nathalie, Eugène Dardye, Fillion Marc, Powell Julie, Kokta Victor, Chabot Gilles
Endocrinology Service, Sainte-Justine Hospital and Research Center, Université de Montréal, Montréal, Québec, Canada.
Horm Res. 2006;65(6):289-94. doi: 10.1159/000092602. Epub 2006 Apr 6.
Subcutaneous fat necrosis (SCFN) of the newborn is an uncommon disorder that occurs in the first weeks of life after foetal distress. It can be complicated by potentially life-threatening hypercalcemia. Treatments of hypercalcemia have included hydration, furosemide and corticosteroids. Only one report has described the use of intravenous bisphosphonates for this condition. We propose that pamidronate could be the first line therapy for severe hypercalcemia in SCFN.
Four newborns presented between 2001 and 2004 with SCFN complicated by severe hypercalcemia. At diagnosis, ionized calcium levels were higher than 1.4 mmol/l and were associated with high urinary calcium/creatinine ratios and high 1,25-dihydroxyvitamin D levels. Despite treatment with IV fluids, low calcium diet and furosemide, calcium levels remained high. The patients were given 3-4 doses (0.25-0.50 mg/kg/dose) of pamidronate. Urinary calcium/creatinine ratios and calcium levels decreased within 48-96 h. 1,25-dihydroxyvitamin D levels normalized with resolution of the skin lesions. No persistent nephrocalcinosis was observed.
Pamidronate is effective, well-tolerated in the short-term and obviates the need for prolonged treatment with furosemide and corticosteroids. To prevent nephrocalcinosis, pamidronate might be considered as first line treatment for severe hypercalcemia in SCFN.
新生儿皮下脂肪坏死（SCFN）是一种罕见的疾病，发生于胎儿窘迫后的出生后最初几周。它可能并发有潜在生命危险的高钙血症。高钙血症的治疗方法包括补液、呋塞米和皮质类固醇。仅有一篇报告描述了静脉使用双膦酸盐治疗这种情况。我们提出帕米膦酸可能是SCFN严重高钙血症的一线治疗药物。
2001年至2004年间，有4例新生儿出现SCFN并伴有严重高钙血症。诊断时，离子钙水平高于1.4 mmol/L，且伴有高尿钙/肌酐比值和高1,25 - 二羟维生素D水平。尽管采用了静脉补液、低钙饮食和呋塞米治疗，钙水平仍居高不下。这些患者接受了3 - 4剂（0.25 - 0.50 mg/kg/剂）的帕米膦酸治疗。尿钙/肌酐比值和钙水平在48 - 96小时内下降。随着皮肤病变的消退，1,25 - 二羟维生素D水平恢复正常。未观察到持续性肾钙质沉着症。
帕米膦酸有效，短期耐受性良好，避免了长期使用呋塞米和皮质类固醇治疗的需要。为预防肾钙质沉着症，帕米膦酸可被视为SCFN严重高钙血症的一线治疗药物。
