PDF(Pubmed)
Abstract：
BACKGROUND: Here we present the case of a newborn baby boy with severe plasminogen deficiency causing occlusive hydrocephalus and ligneous conjunctivitis.
METHODS: Shortly after birth, the hydrocephalus was treated with a ventriculoperitoneal shunt implantation. However, the child had to be readmitted soon afterward because of shunt obstruction. Subglottic microtrauma caused by the necessary intubations then led to another life-threatening complication - subglottic stenosis with pseudomembrane formation. Microsurgical removal had to be performed to secure the airway. Initially, regular plasma transfusions achieved slightly elevated plasminogen activity levels and short-term improvement of the respiratory situation. However, shunt dysfunction reoccurred, and alternative treatment options were needed. Since therapy with plasminogen concentrate is already available in the USA with encouraging results, this treatment option was organized in hopes of equally good results for this patient. Fortunately, under short-term substitution with plasminogen concentrates, the implantation of a new ventriculoperitoneal shunt was successful, and respiratory problems resolved.
CONCLUSIONS: Plasminogen concentrates are critically needed in Europe and other parts of the world to improve the care of and prevent complications among patients with plasminogen deficiency.
METHODS: Shortly after birth, the hydrocephalus was treated with a ventriculoperitoneal shunt implantation. However, the child had to be readmitted soon afterward because of shunt obstruction. Subglottic microtrauma caused by the necessary intubations then led to another life-threatening complication - subglottic stenosis with pseudomembrane formation. Microsurgical removal had to be performed to secure the airway. Initially, regular plasma transfusions achieved slightly elevated plasminogen activity levels and short-term improvement of the respiratory situation. However, shunt dysfunction reoccurred, and alternative treatment options were needed. Since therapy with plasminogen concentrate is already available in the USA with encouraging results, this treatment option was organized in hopes of equally good results for this patient. Fortunately, under short-term substitution with plasminogen concentrates, the implantation of a new ventriculoperitoneal shunt was successful, and respiratory problems resolved.
CONCLUSIONS: Plasminogen concentrates are critically needed in Europe and other parts of the world to improve the care of and prevent complications among patients with plasminogen deficiency.
摘要：
背景：在这里，我们介绍一例新生儿严重纤溶酶原缺乏导致闭塞性脑积水和结膜炎的病例。
方法：出生后不久，脑积水采用脑室-腹腔分流术治疗.然而,由于分流阻塞，这个孩子不得不很快再次入院。必要的插管引起的声门下微创伤随后导致另一种危及生命的并发症-声门下狭窄并形成假膜。必须进行显微外科手术切除以固定气道。最初，定期血浆输注可实现纤溶酶原活性水平的轻度升高和呼吸状况的短期改善。然而,分流功能障碍复发,需要替代治疗方案.由于纤溶酶原浓缩物的治疗在美国已经有了令人鼓舞的结果，组织这种治疗方案是为了希望对该患者有同样好的结果.幸运的是,在用纤溶酶原浓缩物短期替代下，新的脑室-腹腔分流术成功植入,并解决了呼吸问题。
结论：欧洲和世界其他地区迫切需要纤溶酶原浓缩物，以改善纤溶酶原缺乏症患者的护理和预防并发症。
方法：出生后不久，脑积水采用脑室-腹腔分流术治疗.然而,由于分流阻塞，这个孩子不得不很快再次入院。必要的插管引起的声门下微创伤随后导致另一种危及生命的并发症-声门下狭窄并形成假膜。必须进行显微外科手术切除以固定气道。最初，定期血浆输注可实现纤溶酶原活性水平的轻度升高和呼吸状况的短期改善。然而,分流功能障碍复发,需要替代治疗方案.由于纤溶酶原浓缩物的治疗在美国已经有了令人鼓舞的结果，组织这种治疗方案是为了希望对该患者有同样好的结果.幸运的是,在用纤溶酶原浓缩物短期替代下，新的脑室-腹腔分流术成功植入,并解决了呼吸问题。
结论：欧洲和世界其他地区迫切需要纤溶酶原浓缩物，以改善纤溶酶原缺乏症患者的护理和预防并发症。
