Pansatiankul B J, Ruengsuwan S, Lektrakul J
Children's Hospital, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1993;24 Suppl 1:121-6.
Twenty infants aged 2 weeks to 3 months with the diagnosis of bleeding disorder secondary to low prothrombin complex level were studied. Sixty children of the control group were matched to the cases by age +/- 2 weeks, sex and race. The ratio of boys to girls was 2.3:1. The median, mean, and range of age of the cases and controls were 43.5 days, 43.7 days, 21-73 days and 43.5 days, 46.8 days, 26-28 days respectively. Most of them were pale with a mean hematocrit of 23.55%. The partial thromboplastin time and prothrombin time were markedly prolonged. The means of vitamin K dependent coagulation factors II, VII, IX and X were 1.10%, 5.87%, 2.86%, and 4.47% of adult activity, respectively. The clinical manifestations related to the bleeding of the cases were drowsiness and convulsion (95%), pallor (85%), and apparent bleeding (10%). The sites of the bleeding were demonstrated in the cranial cavity (95%), gastrointestinal tract and oral cavity (15%), and skin (5%). Nineteen patients with intracranial hemorrhage had bleeding in the subdural space (79%), intracerebral (42%), intraventricular (32%), and subarachnoid space (5.2%). The mortality rate and permanent brain damage occurred in 10% and 45%, respectively. Only 45% of the cases recovered normally. The permanent neurological sequelaes were hemiparesis (44.4%), microcephaly (33.3%), convulsive disorder (33.3%), mental retardation (33.3%), spasticity (22.2%), and hydrocephalus (11.1%). Breast feeding alone up to the day of study (OR = 7.0, p < 0.005) was found to be a significant risk factor for bleeding in these infants.(ABSTRACT TRUNCATED AT 250 WORDS)
对20名年龄在2周龄至3月龄、诊断为低凝血酶原复合物水平继发出血性疾病的婴儿进行了研究。对照组的60名儿童在年龄(±2周)、性别和种族方面与病例组相匹配。病例组中男女性别比为2.3:1。病例组和对照组的年龄中位数、平均数及范围分别为43.5天、43.7天、21 - 73天以及43.5天、46.8天、26 - 28天。他们大多面色苍白,平均血细胞比容为23.55%。部分凝血活酶时间和凝血酶原时间显著延长。维生素K依赖的凝血因子II、VII、IX和X的活性分别为成人活性的1.10%、5.87%、2.86%和4.47%。病例组与出血相关的临床表现为嗜睡和惊厥(95%)、面色苍白(85%)以及明显出血(10%)。出血部位见于颅腔(95%)、胃肠道和口腔(15%)以及皮肤(5%)。19例颅内出血患者的出血部位位于硬膜下间隙(79%)、脑内(42%)、脑室内(32%)以及蛛网膜下腔(5.2%)。死亡率和永久性脑损伤发生率分别为10%和45%。仅45%的病例正常康复。永久性神经后遗症包括偏瘫(44.4%)、小头畸形(33.3%)、惊厥障碍(33.3%)、智力发育迟缓(33.3%)、痉挛(22.2%)以及脑积水(11.1%)。研究发现,仅母乳喂养至研究当日(比值比=7.0,p<0.005)是这些婴儿出血的一个显著危险因素。(摘要截断于250字)