Roach E S, Sinal S H
Section of Pediatric Neurology, Bowman Gray School of Medicine of Wake Forest University, Winston-Salem, North Carolina.
Clin Pediatr (Phila). 1989 Aug;28(8):371-3. doi: 10.1177/000992288902800807.
Four infants with newly diagnosed cystic fibrosis developed a bulging anterior fontanel within days of starting enzyme replacement treatment. In the same time period, 41 hospitalized patients less than 1 year of age were diagnosed as having cystic fibrosis and treated, suggesting that increased intracranial pressure is common in this setting (9.7%). The clinical course of the four infants was similar: the bulging fontanel became apparent 1-6 days after initiation of pancreatic enzyme replacement and resolved within 1 week in three patients and within 4 months in the other patient. Two patients became mildly irritable. Computed cranial tomography and lumbar puncture were done in three patients, with completely normal findings except elevated cerebrospinal fluid pressure in two infants. There was no apparent difference in this treatment or clinical course before diagnosis between the four infants who developed a transient bulging fontanel compared with the overall group of cystic fibrosis patients.
四名新诊断为囊性纤维化的婴儿在开始酶替代治疗后的几天内出现前囟门隆起。在同一时期，41名1岁以下住院患者被诊断为患有囊性纤维化并接受治疗，这表明在这种情况下颅内压升高很常见（9.7%）。这四名婴儿的临床病程相似：前囟门隆起在开始胰腺酶替代治疗后的1至6天出现，三名患者在1周内消退，另一名患者在4个月内消退。两名患者变得轻度烦躁。三名患者进行了头颅计算机断层扫描和腰椎穿刺，除两名婴儿脑脊液压力升高外，其余结果完全正常。与整个囊性纤维化患者组相比，出现短暂前囟门隆起的四名婴儿在诊断前的这种治疗或临床病程没有明显差异。
