Ozalla M D, Herrero C, Ventura P J, Lecha M, Alvarez L, Mascaro J M
Department of Dermatology, Hospital Clínic, Faculty of Medicine, University of Barcelona, Spain.
J Dermatol. 1999 Jul;26(7):409-15. doi: 10.1111/j.1346-8138.1999.tb02018.x.
The objective of this study was to establish normal reference ranges for porphyrins in healthy neonates. There is little information about urinary porphyrin excretion in this age group. This knowledge may provide an early diagnostic tool for detecting subtle alterations or latent forms in disorders of heme biosynthesis. Fifty healthy neonates were selected from the Department of Obstetrics. Total urinary porphyrins in random specimens were analysed by a spectrofluorometry method. The measurement of porphyrin fractions was made by fluorometric high-performance liquid chromatography (HPLC). The results were adjusted to urinary creatinine excretion to correct any imprecision and interindividual variation in body mass. The urinary total porphyrin had a median value of 331.50 (nmol/L). A statistically significant relationship between total porphyrin (nmol/L) and creatinine (mmol/L) was found (p < 0.01). The porphyrin/creatinine ratio showed a median value of 56.30 nmol/mmol creatinine. The study of individual porphyrins revealed that coproporphyrin and uroporphyrin were the major porphyrins excreted in neonates (coproporphyrin represents 81.98% and uroporphyrin 16.64% of total porphyrin); in both cases, isomer I was predominant with median values of 22.36 and 6.25 nmol/mmol creatinine, respectively. No significant relationships were found between porphyrin excretion and sex, gestational age, weight, or height. Our data provide the reference limits for porphyrins in neonates as a diagnostic guideline for evaluation of subtle alterations in heme biosynthesis.
本研究的目的是建立健康新生儿卟啉的正常参考范围。关于该年龄组尿卟啉排泄的信息很少。这些知识可能为检测血红素生物合成障碍中的细微改变或潜在形式提供一种早期诊断工具。从产科选取了50名健康新生儿。随机样本中的尿总卟啉通过荧光分光光度法进行分析。卟啉组分的测定采用荧光高效液相色谱法(HPLC)。将结果根据尿肌酐排泄量进行校正,以纠正体重方面的任何不精确性和个体间差异。尿总卟啉的中位数为331.50(nmol/L)。发现总卟啉(nmol/L)与肌酐(mmol/L)之间存在统计学显著关系(p < 0.01)。卟啉/肌酐比值的中位数为56.30 nmol/mmol肌酐。对单个卟啉的研究表明,粪卟啉和尿卟啉是新生儿排泄的主要卟啉(粪卟啉占总卟啉的81.98%,尿卟啉占16.64%);在这两种情况下,异构体I均占主导,中位数分别为22.36和6.25 nmol/mmol肌酐。未发现卟啉排泄与性别、胎龄、体重或身高之间存在显著关系。我们的数据提供了新生儿卟啉的参考限值,作为评估血红素生物合成细微改变的诊断指南。