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[儿童囊性纤维化中铜绿假单胞菌的治疗]

[Treatment of Pseudomonas aeruginosa in cystic fibrosis in a child].

作者信息

Selimović Amina, Saracević Ediba, Mujicić Ermina, Dizdarević Aida

机构信息

Pedijatrijska klinika, Klinicki centar Univerziteta u Sarajevu, Bosna i Hercegovina.

出版信息

Med Arh. 2006;60(1):68-9.

Abstract

The report deals with the case of a 10-year-old girl with chronic cystic fibrosis. She has been repeatedly treated at the hospital. She has been hospitalized due to respiratory deterioration. Cystic fibrosis is a rare disease, inherited autosomaly recessively, but is very complex in terms of diagnostic and treatment. Fibrosis is the formation of scar tissue due to injury or long term inflammation. The diagnosis is confirmed based on a clinical picture of the child, measure of Chloride in the sweat, chest X-ray, CT thorax, laboratory findings--genetic confirmation CFTR genes. The diagnosis is originally set when she was 4 years old. She is now admitted due to a deterioration of the main disease. Five days before the admission, the girl had a higher bodily temperature, cough and difficult breathing. Due to the deteriorated general condition and the respiratory insufficiency and respiratory acidosis in blood gas analysis, the girl was intubated and put on the complete mechanical ventilation (IPPV). Since the girl is a chronic patient with bronchiectasie chronic walls of bronchi changes full of the mucus, who is not responding to conservative treatment (antibiotics), therapeutic and diagnostic flexible bronchoscopy had to be performed, resulting in a gram-negative bacteri Pseudomonas aeruginosa--a typical bacteri for chronically sick C. F. patient. Pseudomonas aeruginosa is typically acquired in early childhood. This bacteria is giving progressive lung disease and often aggravates morbidity and mortality. So the main thing as a respiratory management is prevention of lung infection with this bacteria. A Pseudomonas therapy was prescribed according to the sensitive antibiogram, (Garamycin). Antibiotics are crucial to treating cystic fibrosis lung infections. Therapy with an amynoglicoside in combination with a B-lactam or a quinolone antibiotic is standard. It is a difficult to deliver a high doses at these antibiotics via the iv. route without significant systemic adverse events (otoxicity and nephrotoxicity). A reformulation of the aminoglycoside antibiotic tobramicin or garamycin therapy is solution for inhalation. To be well established infections the suppression of Pseudomonas aeruginosa has been shown to lead to decreasing same bacteria and benefits lung function from antibiotic therapy in a way that can be maintained over extended period. During bronchoscopy was given locally on changes mucous pulmozyme (to destroy a very hard mucous) and garamycin. So, after taking out a lot of mucus, it was later continued spontaneously. Control chest x ray and blood gas analysis are now very improved.

摘要

该报告讲述了一名患有慢性囊性纤维化的10岁女孩的病例。她曾多次在该医院接受治疗。此次因呼吸功能恶化而住院。囊性纤维化是一种罕见的常染色体隐性遗传病,但其诊断和治疗都非常复杂。纤维化是由于损伤或长期炎症导致瘢痕组织形成。根据患儿的临床表现、汗液氯化物检测、胸部X光、胸部CT、实验室检查结果——CFTR基因的基因确诊来明确诊断。最初在她4岁时确诊。她此次因主要疾病病情恶化而入院。入院前五天,女孩体温升高、咳嗽且呼吸困难。由于全身状况恶化以及血气分析显示呼吸功能不全和呼吸性酸中毒,女孩接受了插管并进行完全机械通气(IPPV)。由于该女孩是一名患有支气管扩张且支气管壁慢性改变并充满黏液的慢性病患者,对保守治疗(抗生素)无反应,因此必须进行治疗性和诊断性可弯曲支气管镜检查,结果发现革兰氏阴性菌铜绿假单胞菌——这是囊性纤维化慢性病患者的典型病菌。铜绿假单胞菌通常在儿童早期感染。这种细菌会导致进行性肺部疾病,且常常加重发病率和死亡率。所以作为呼吸管理的主要事项是预防这种细菌引起的肺部感染。根据药敏试验结果开出了针对铜绿假单胞菌的治疗方案(庆大霉素)。抗生素对于治疗囊性纤维化肺部感染至关重要。氨基糖苷类药物与β-内酰胺类或喹诺酮类抗生素联合治疗是标准方案。通过静脉途径给予这些抗生素高剂量时很难不出现明显的全身不良反应(耳毒性和肾毒性)。将氨基糖苷类抗生素妥布霉素或庆大霉素改为吸入疗法是解决办法。对于已确诊的感染,已证明抑制铜绿假单胞菌可减少该细菌数量,并在较长时间内维持抗生素治疗对肺功能的益处。在支气管镜检查期间,在肺部黏液病变部位局部给予了派罗欣(用于破坏非常黏稠的黏液)和庆大霉素。因此,在清除大量黏液后,黏液分泌后来自行持续减少。目前胸部X光对照和血气分析结果有了很大改善。

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