Pniewska-Siark Barbara, Jeziorowska Anna, Bobeff Irena, Lewiński Andrzej
Department of Endocrinology and Metabolic Diseases, Medical University of Lodz, Polish Mother's Memorial Hospital-Research Institute, Lodz, Poland.
Endocr Regul. 2006 Mar;40(1):7-14.
Evaluation of clinical and biochemical differences between various forms of thyroid dysgenesia in children.
The study involved 102 children at the age between 4.8 and 14.2 years who were born with congenital hypothyroidism (CH), as diagnosed by neonatal screening examinations. In all the children diagnosis was settled and the levothyroxine (L-T4) administration was started by the 19th day of life. Out of the examined children, 79 were selected with following three forms of developmental thyroid disorders: Group I--athyroidism (thyroid aplasia or agenesis), Group II--thyroid hypoplasia, Group III--thyroid ectopy. On the basis of neonatal TSH (nTSH) levels obtained by screening and serum TSH, FT4 and Tg concentrations, the severity of hypothyroidism was determined at the time of diagnosis. Physical and mental development of the children was evaluated on the basis of growth and bone age indices and Wechsler's scale, respectively.
Developmental disorders were diagnosed in 79 cases (77.4% CH) which included 45 cases (44.1%) of athyroidism, 31 cases (30.4%) of thyroid hypoplasia and 3 cases (2.9%) of thyroid ectopy. The physical and mental development in the studied groups was evaluated as normal.
In the group of children with athyroidism, significantly lower growth indices and IQ values were found in comparison with respective values observed in the other study groups. However, the indices of physical and mental development in all the studied groups were within the normal values for children population. An early diagnosis and early administration of hormonal replacement therapy by L-T4 ensure normal development of children with CH, regardless of underlying causes and associated with them severity of congenital hypothyroidism.
评估儿童各种形式甲状腺发育异常的临床和生化差异。
该研究纳入了102名年龄在4.8至14.2岁之间的儿童，这些儿童经新生儿筛查诊断为先天性甲状腺功能减退症（CH）。所有儿童均确诊，并在出生后第19天开始服用左甲状腺素（L-T4）。在这些接受检查的儿童中，选取了79名患有以下三种形式甲状腺发育障碍的儿童：第一组——无甲状腺症（甲状腺缺如或发育不全），第二组——甲状腺发育不全，第三组——甲状腺异位。根据筛查获得的新生儿促甲状腺激素（nTSH）水平以及血清促甲状腺激素、游离甲状腺素（FT4）和甲状腺球蛋白（Tg）浓度，在诊断时确定甲状腺功能减退的严重程度。分别根据生长和骨龄指标以及韦氏量表评估儿童的身体和智力发育情况。
79例（77.4%的CH）被诊断为发育障碍，其中包括45例（44.1%）无甲状腺症、31例（30.4%）甲状腺发育不全和3例（2.9%）甲状腺异位。研究组的身体和智力发育评估为正常。
与其他研究组观察到的相应值相比，无甲状腺症儿童组的生长指标和智商值显著较低。然而，所有研究组的身体和智力发育指标均在儿童人群的正常范围内。早期诊断并通过L-T4进行早期激素替代治疗可确保CH儿童的正常发育，无论其潜在病因以及与之相关的先天性甲状腺功能减退的严重程度如何。
