Niiazova M Kh, Kapranov N I, Makatsariia A D
Pediatriia. 1989(2):27-30.
The results of the clinical observations over 127 patients with mucoviscidosis and chronic (primary and secondary) pneumonia are presented. The children were aged from 1 to 15 years. Study of the plasma and platelet links of hemostasis permitted one to reveal considerable disorders seen during exacerbation in all the groups. The intensity of the disorders correlated well with the gravity of the patient's condition and with the bronchopulmonary process spreading as well as with the presence of complications on the part of the cardiovascular system (cor pulmonale) and the hepatobiliary system (liver cirrhosis). The chronic form of the disseminated intravascular coagulation (DIC) syndrome was diagnosed in 50 percent of the patients with mucoviscidosis, in 1/3 of the patients with secondary and in 18 percent of the patients with primary chronic pneumonia. The subacute form of the DIC syndrome was diagnosed in 15 percent of the patients with mucoviscidosis and in 4.5 percent of the patients with secondary chronic pneumonia. The multimodality treatment without heparin made it possible to correct the hemostatic disturbances in the majority of the patients under observation. In some children with mucoviscidosis and secondary chronic pneumonia, the use of heparin in the form of intravenous injections or ultrasonic inhalations permitted making such a correction more rapidly and more completely.
本文介绍了对127例患有黏液黏稠病及慢性（原发性和继发性）肺炎患儿的临床观察结果。这些患儿年龄在1至15岁之间。对止血的血浆和血小板环节进行研究后发现，所有组在病情加重期间均出现了明显紊乱。紊乱的程度与患者病情的严重程度、支气管肺部病变的扩散情况以及心血管系统（肺心病）和肝胆系统（肝硬化）并发症的出现情况密切相关。50%的黏液黏稠病患者、1/3的继发性慢性肺炎患者以及18%的原发性慢性肺炎患者被诊断为慢性弥散性血管内凝血（DIC）综合征。15%的黏液黏稠病患者和4.5%的继发性慢性肺炎患者被诊断为亚急性DIC综合征。在大多数观察对象中，不使用肝素的多模式治疗能够纠正止血障碍。在一些患有黏液黏稠病和继发性慢性肺炎的儿童中，通过静脉注射或超声雾化吸入肝素能够更快、更彻底地实现这种纠正。
