Pierson M, Malaprade D, Fortier G, Belleville F, Lasbennes A, Wuilbereq L
Arch Fr Pediatr. 1978 Feb;35(2):151-64.
Five children from 3 different families presented with severe dwarfism and the morphological and biological features described by Laron: familial occurrence, small stature, peculiar facies, high levels of plasma HGH and resistance to treatment by GH. This therapeutic inefficiency is expressed by an absence of physical growth and unchanged nitrogen balance, during a prolonged treatment. The plasma levels of somatomedine were very low (K. Hall's biological method) and not influenced by administration of exogenous HGH. These findings suggest that the fundamental disorder is not an abnormal structure of the molecules of GH. This hypothesis seems further confirmed by the structural analysis of plasma HGH, which gave the same results as those of the reference GH.
来自3个不同家庭的5名儿童表现出严重侏儒症以及拉伦所描述的形态学和生物学特征：家族性发病、身材矮小、特殊面容、血浆生长激素水平高以及对生长激素治疗有抵抗性。这种治疗无效表现为在长期治疗期间身体没有生长且氮平衡未改变。生长调节素的血浆水平非常低（K. 霍尔的生物学方法），并且不受外源性生长激素给药的影响。这些发现表明根本障碍并非生长激素分子结构异常。血浆生长激素的结构分析结果与参考生长激素相同，这一假说似乎进一步得到了证实。
