Clinical Analysis Of The Complete Kartagener Syndrome With Respiratory Tract Complication As Initial Presenting Symptom:2 Cases Report

Purpose: Kartagener Syndrome, Bronchiectasis-nasosinusitis—situs inversus visverum syndrome, characters as “triad”: chronic nasosinusitis, Bronchiectasis,mirror situs inversus. It’s a kind of congenital autosomal invisiable genetic disease.Patients are easier to bring out respiratory and other infectious disease than other normal people because of the abnormal of congenital physiological structure. AS the disease is rare, less attention were paid by most doctors which cause the disease can’t be treated at early stage, progressed and even threaten life. With the continuous improvement of medical level, more and more clinicians take the pathogenesis clinical diagnosis of Kartagener Syndrome as a new research subject.Based on the research of Kartagener Syndrome in medical and other domain, the thesis aims at discuss the clinical characteristics, pathogenesis, clinical diagnosis of KS, especially the treatment to complicated airway infectious disease, analyzing the reason of missing diagnosis, early screening and premonition.Method: The clinical diagnosis documents 2 cases of Kartagener Syndrome which were collected by respiration department of The Second Hospital of Dalian Medical University from 2000 to 2014 were retrospectively analyzed to research the pathogenesis, clinical diagnosis and clinical treatment effect. The 2 cases were diagnosis as complete KS after apical auscultation, X-ray film and CT plain scan.The thesis further combine with the material of another 7 patients of situs inversus,which were collected by other department of hospital in the same period. In 7patients, 1case was diagnosed incomplete KS, 6 cases were diagnosed situs inversus.Comparative analysis their common point, relativity and main difference, through pathogenesis, clinical diagnosis and clinical treatment effect.Result: The chest and abdominal organs of the 2 cases of KS which with respiratory tract complication as initial presenting symptom are completely mirror situs inverse. The chest CT shows dextrocardia; bronchiectasis and infected with“track sign”, “cylindrical expansion ” and diffuse bronchitis in different degree. Thetwo cases are all have nasopharynx with anaphylactis rhinobyon and shed tears.Paranasal sinus CT showed: soft tissue density is visible on bilateral maxillary sinus, liquid surface can be seen to the right maxillary sinus. As the history of case NO.1, he did “nasal polypectomy surgery”. With the investigation of the two cases, we found that the parents of the No.1 case are cousins, known as intermarriage.After the surgery, frequent nasal obstruction and snotty nose got released. These two were treated with antibiotics, mucus and improve ciliary activity diluted drugs and other symptomatic treatment. Patients with bronchiectasis infection symptoms were relieved.If the cases have regular follow-up, the infections and the degree of recurrence can be reduced. Another seven cases are presented in patients with congenital visceral transposition collection, including one case of incomplete KS. 7 patients were all shown without respiratory system infection, visiting with acute appendicitis, erysipelas, acute renal failure, gynecological infections and other diseases respectively. According to the disease history and radiological data, the 7 cases are all confirmed that they don’t have the typical "triad", excluding the diagnosis of complete Kartagener syndrome.According to the congenital physical predisposition caused by structural abnormalities of the common characteristics, these patients were positive for antiinfection treatment, effectively control the development of the disease. The follow-up has not found a case of further development of KS.Conclusion: The complete and incomplete Kartagener syndrome are both belong to the rare disease in clinic, mostly occurring as a single exhibit symptoms of respiratory infection. It can be easily missed, but with more and more knowledge of the disease gathered, diagnose patients who used to be highly suspicious, combining with other material, miss diagnosis can be minimized. In a controlled phase of the disease given a positive symptomatic treatment can alleviate the symptoms of respiratory and systemic infections, reducing the chances of infection, which is conducive to the disease lapse. For the genetic factors of Kartagener syndrome, popularize sound child rearing and pre-pregnancy health checks is effective in reducing the incidence of KS, helping to avoid adverse pregnancy and birth defects, improving population quality.