Landing B H, Swanson V L, Shankle W R
Pediatr Pathol. 1985;4(3-4):213-7. doi: 10.3109/15513818509026895.
The weights of the spleens of series of patients with various disorders of children dating from birth or early infancy and causing splenomegaly, with or without cirrhosis of the liver, were analyzed. The linear regression equation for spleen weight versus age in months for each disease was derived, and the rate constants from these equations were adjusted for the age range of the patients in each group. The original data of Coppoletta and Wolbach were used for normal values. The rates of splenic growth of appropriate entities for which the regression equation could be computed fell into three groups, with adjusted rate constants (growth of spleen in grams per month) of 6.53-6.95 (biliary atresia, thalassemia, and cirrhosis following neonatal hepatitis), 2.30-2.62 (cirrhosis of alpha-1-antitrypsin deficiency, infantile polycystic disease, and spherocytosis), and 1.06-1.11 (cystic fibrosis and idiopathic thrombocytopenic purpura). These classes of splenic growth rates are approximately 10, 3.7, and 1.6 times the normal growth rate (0.67 g/mo). Rate constants could not be computed for the categories cirrhosis following viral hepatitis and hemolytic anemia other than spherocytosis and sickle cell anemia, and the numbers of patients with splenic vein obstruction, cirrhosis with the cholestatic syndrome of parenteral alimentation, hypoplastic anemia with hemosiderosis, tyrosinemia, Byler's disease, congenital hepatic fibrosis, and Wilson's disease were too few for analysis. The significance of the finding of classes or "quantum groups" of splenic growth rates in disorders of children, dating from birth or early infancy and causing splenomegaly, is uncertain. Comparable data on adequate series of patients with other appropriate disorders will be necessary.
对一系列患有各种儿童疾病的患者脾脏重量进行了分析，这些疾病自出生或婴儿早期起病，导致脾肿大，伴有或不伴有肝硬化。得出了每种疾病脾脏重量与月龄的线性回归方程，并针对每组患者的年龄范围对这些方程的速率常数进行了调整。使用了科波莱塔和沃尔巴赫的原始数据作为正常值。能够计算回归方程的相应实体的脾脏生长速率分为三组，调整后的速率常数（脾脏每月生长克数）分别为6.53 - 6.95（胆道闭锁、地中海贫血和新生儿肝炎后的肝硬化）、2.30 - 2.62（α-1抗胰蛋白酶缺乏症肝硬化、婴儿多囊病和球形红细胞增多症）以及1.06 - 1.11（囊性纤维化和特发性血小板减少性紫癜）。这些脾脏生长速率类别分别约为正常生长速率（0.67克/月）的10倍、3.7倍和1.6倍。对于病毒性肝炎后的肝硬化以及除球形红细胞增多症和镰状细胞贫血外的其他溶血性贫血类别，无法计算速率常数，且脾静脉阻塞、肠外营养胆汁淤积综合征肝硬化、含铁血黄素沉着性再生障碍性贫血、酪氨酸血症、比勒氏病、先天性肝纤维化和威尔逊病患者数量过少，无法进行分析。在出生或婴儿早期起病并导致脾肿大的儿童疾病中发现脾脏生长速率类别或“量子组”这一发现的意义尚不确定。需要有其他适当疾病的足够系列患者的可比数据。
