Kinoshita Kaori, Minagawa Masanori, Anzai Michiko, Sato Yumiko, Kazukawa Itsuro, Shimohashi Kyoko, Ota Setsuo, Kohno Yoichi
Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan.
Department of Pediatrics, Graduate School of Medicine, Chiba University, Chiba, Japan ; Environmental Health Science Project for Future Generations, Graduate School of Medicine, Chiba University, Chiba, Japan.
Clin Pediatr Endocrinol. 2007;16(1):31-6. doi: 10.1297/cpe.16.31. Epub 2007 Feb 7.
Pseudohypoparathyroidism (PHP) is a metabolic disorder characterized by organ resistance to the action of parathyroid hormone. PHP type 1 is subclassified into two apparent disorders, type 1a (PHP1a) and type 1b (PHP1b). Patients with PHP1a show Albright hereditary osteodystrophy including short stature. Patients with PHP1b have no such skeletal defects, however, literature regarding the growth of PHP1b is not currently available. We evaluated growth charts of PHP patients, including four PHP1a patients and six PHP1b patients. Growth patterns were different between PHP type 1a and 1b. Adult height was abnormally low in all PHP1a patients. The growth pattern of PHP1a was characterized by mild growth impairment in the prepubertal period, a blunted growth spurt and premature cessation of the growth spurt. The adult height of male PHP1b was slightly lower than average. An early growth spurt was observed only in male patients with PHP1b and it may reduce the adult height of male patients with PHP1b. This warrants further investigation into the growth and pubertal development of PHP1b patients.
假性甲状旁腺功能减退症(PHP)是一种代谢紊乱疾病,其特征为机体对甲状旁腺激素的作用产生抵抗。1型PHP可细分为两种明显的疾病类型,即1a型(PHP1a)和1b型(PHP1b)。PHP1a患者表现出包括身材矮小在内的奥尔布赖特遗传性骨营养不良。然而,PHP1b患者没有此类骨骼缺陷,目前尚无关于PHP1b患者生长情况的文献。我们评估了PHP患者的生长图表,其中包括4例PHP1a患者和6例PHP1b患者。1a型和1b型PHP的生长模式有所不同。所有PHP1a患者的成年身高均异常偏低。PHP1a的生长模式特点是青春期前生长轻度受损、生长突增减弱以及生长突增过早停止。男性PHP1b患者的成年身高略低于平均水平。仅在男性PHP1b患者中观察到早期生长突增,这可能会降低男性PHP1b患者的成年身高。这值得对PHP1b患者的生长和青春期发育进行进一步研究。