• Title/Summary/Keyword: kartagener syndrome

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A Case of Kartagener's Syndrome Presenting as Respiratory and Right Heart Failures (호흡부전과 우심부전을 동반한 Kartagener 증후군 1예)

  • Yang, Suck-Chul;Lee, Kyung-Sang;Yoon, Ho-Joo;Shin, Dong-Ho;Park, Sung-Soo;Lee, Jung-Hee;Park, Choong-Ki
    • Tuberculosis and Respiratory Diseases
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    • v.43 no.2
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    • pp.251-256
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    • 1996
  • Kartagener's syndrome, a congenital disease transmitted as an autosomal recessive illness with a prevalence of approximately 1:20,000 persons, is characterized by the triple association of situs inversus, bronchiectasis, and sinusitis. Affected persons have an incoordination of ciliary motility that leads to defective mucociliary transport, chronic bronchial infections. Kartagener's syndrome is a subset of the immotile cilia syndrome and therefore all patients with Kartagener's syndrome have immotile cilia with obvious ultrastructural defects in the ciliary axoneme. In the respiratory tract this inability presumably causes impaired clearance of mucus and inhaled particles and results in the chronic infections of the sinuses and bronchial trees that are characterized of the disease. The end-stage phenomenon in Kartagener's syndrome, respiratory or heart failure is a less common event and heart-lung transplantation is becoming an accepted therapy for patients with end-stage pulmonary disease in Kartagener's syndrome in many institutes. We report one case of Kartagener's syndrome in a 25-year-old young woman who was presented as respiratory and right heart failures, with review of literatures.

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Kartagener's Syndrome ; A Report of Two Cases (Surgical Treatment of Bronchiectasis in Kartagener's Syndrome.) (Kartagener's Syndrome ; 2례 보고 (Kartagener 증후군의 기관지확장증의 와과적 치료))

  • Kwack, Sang-Ryong;Rho, Joon-Rhyang
    • Journal of Chest Surgery
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    • v.12 no.3
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    • pp.159-164
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    • 1979
  • Kartagener`s syndrome is a clinical entity comprising a combination of situs inversus, bronchiectasis, and sinusitis or nasal polyposis. This syndrome is rare and is usually seen in a young age group. The syndrome is punctated by recurrent upper respiratory tract infection and pneumonia. This is a report of Kartagener`s syndrome found in 18 years old male and 21 years old female patients who were received surgical treatment of bronchiectasis. The male patient was performed right transposed lingular segmentectomy and lower lobectomy and female patient was perforated left transposed middle lobectomy and lower lobectomy. Both patients were discharged with good results.

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Siewert-Kartagener's syndrome in a dog

  • Rankyung Jung;Jihye Choi;Hyeona Bae ;Dong-In Jung ;Kyoung-Oh Cho;DoHyeon Yu
    • Journal of Veterinary Science
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    • v.24 no.4
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    • pp.57.1-57.8
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    • 2023
  • Siewert-Kartagener's syndrome, a type of primary ciliary dyskinesia, is a complex disease comprising situs inversus, rhinosinusitis, and bronchiectasis. Situs inversus totalis is a condition in which all organs in the thoracic and abdominal cavities are reversed. Furthermore, primary ciliary dyskinesia, an autosomal genetic disease, may coexist with situs inversus totalis. Reports on Siewert-Kartagener's syndrome in veterinary medicine are limited. We report a rare case of primary ciliary dyskinesia with Siewert-Kartagener's syndrome in a dog, concurrently infected with canine distemper virus and type-2 adenovirus. This case highlights that situs inversus totalis can cause primary ciliary dyskinesia, and concurrent infections are possible.

Surgical Treatment of Immotile Cilia Syndrome Associated with Kartagener`s Syndrome (Report of one case) (Kartagener 증후군을 동반한 Immotile Cilia Syndrome 의 외과적 치험 1례)

  • Kim, Ju-Hyeon;Park, Seung-Il
    • Journal of Chest Surgery
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    • v.21 no.2
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    • pp.383-388
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    • 1988
  • Immotile cilia syndrome is a congenital structural abnormality of cilia. The structural abnormality is lack of dynein arm or defective radial spoke or microtubular transposition. In this syndrome, ciliary movement is completely absent or dyskinetic and half of this syndrome shows Kartagener`s triad. We report a 13-year-old girl who had immotile cilia syndrome with Kartagener`s triad. She had been suffering from frequent respiratory infection, hemoptysis, large amount of sputum, and sinusitis. Bronchography revealed tubular bronchiectasis in right lower lobe and that lobe was resected for treatment of bronchiectasis. Histological examination of resected bronchus showed chronic bronchiectasis and electronmicroscopically complete lack of both inner and outer dynein arms. Hospital course was uneventful and symptoms were much improved.

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Kartagener's Syndrome: A Report of 2 Cases (Review of the literature and report of two cases in same family) (남매에서 발생한 Kartagener씨 증후군 - 2례 보고 -)

  • 정수상
    • Journal of Chest Surgery
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    • v.15 no.1
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    • pp.67-72
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    • 1982
  • Kartagener`s syndrome is a clinical entity composed of situs inversus, bronchiectasis and chronic sinusitis. This syndrome is rare and is usually detected in childhood. Whether the bronchiectasis in Kartagener`s syndrome is congenital or acquired is still controversial. Some familial cases of this syndrome have been reported, and in these cases the genetic pattern is compatible with an autosomal recessive inheritance with some degree of pleotropism. The authors experienced having 2 cases of Kartagener`s syndrome among 6 siblings in a family. The case No. 1 have had bilateral Caldwell Luc operation, posterior ethmoidectomy, left upper Iobec-tomy with excellent result. The case No. 2 denied to have surgical management.

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Four Cases of Kartagener's Syndrome (Kartagener 증후군 4예)

  • Lee, Yong-Chul;Song, Hang-Yong;Lim, Suk-Tae;Kim, Hyung-Chung;Lee, Heung-Bum;Lee, Young-Seung;Rhee, Yang-Keun;Chung, Jae-Man
    • Tuberculosis and Respiratory Diseases
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    • v.41 no.6
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    • pp.663-669
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    • 1994
  • Kartagener's sydrome is an autosomaly inherited recessive condition characterized by situs inversus, bronchiectasis, and chronic sinusitis. And recently it was recognized as a subclass of dyskinetic cilia syndrome which caused by a defect in mucociliary transport owing to immotile or dyskinetic beating of cilia. Electron microsopy of cilia from sperm tails, nasal and bronchial epithelium of patients reveals the partial or complete absence of dynein arms. Our four patients were diagnosed as a Kartagener's syndrome by classic triad. We carried out electron microscopy of cilia of the nasal mucosa. And many other tests were done. One patient had squamous cell carcinoma of the lung, and another one patient revealed features of adult respiratory distress syndrome at admission. All patients improved with conservative therapy such as physiotherapy, bronchodilater, antibiotics except one patient who mechanical ventilation was required. A brief review of literature was made.

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Mycobacterium abscessus Lung Disease in a Patient with Kartagener Syndrome

  • Kim, Jung Hoon;Song, Won Jun;Jun, Ji Eun;Ryu, Duck Hyun;Lee, Ji Eun;Jeong, Ho Jung;Jeong, Suk Hyeon;Kang, Hyung Koo;Kim, Jung Soo;Lee, Hyun;Chon, Hae Ri;Jeon, Kyeongman;Kim, Dohun;Kim, Jhingook;Koh, Won-Jung
    • Tuberculosis and Respiratory Diseases
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    • v.77 no.3
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    • pp.136-140
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    • 2014
  • Primary ciliary dyskinesia (PCD) is characterized by the congenital impairment of mucociliary clearance. When accompanied by situs inversus, chronic sinusitis and bronchiectasis, PCD is known as Kartagener syndrome. The main consequence of impaired ciliary function is a reduced mucus clearance from the lungs, and susceptibility to chronic respiratory infections due to opportunistic pathogens, including nontuberculous mycobacteria (NTM). There has been no report of NTM lung disease combined with Kartagener syndrome in Korea. Here, we report an adult patient with Kartagener syndrome complicated with Mycobacterium abscessus lung disease. A 37-year-old female presented to our hospital with chronic cough and sputum. She was ultimately diagnosed with M. abscessus lung disease and Kartagener syndrome. M. abscessus was repeatedly isolated from sputum specimens collected from the patient, despite prolonged antibiotic treatment. The patient's condition improved and negative sputum culture conversion was achieved after sequential bilateral pulmonary resection.

A Case Kartagener's Syndrome with Various Ultrastructural Defects (다양한 형태의 섬모 미세구조결함을 보인 Kartagener 증후군 1예)

  • Lee, Sung-Ho;Park, Jung-Ho;Jang, Ho-Sik;Kim, Hyun-Su;Kang, Kyeong-Woo;Kim, Ho-Chul;Kwon, Kun-Young
    • Tuberculosis and Respiratory Diseases
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    • v.53 no.4
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    • pp.457-462
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    • 2002
  • Karagener's syndrome is an inherited condition characterized by triad of chronic paranasal sinusitis, situs inversus, and bronchiectasis, Since 1976, Afzelius found a lack of dynein arm in immotile spermatozoa by electron microscopy, numerous recent studies have focused on the ultrastructural defect in the cilia and reported that the variety type of ultrastructural defect in immotile cilia syndrome. We report a female patient who had the Katagener's triad with rare multiple ultrastructural defect of cilia in one patient. The electron microscopic examination showed partial dynein arm defect, loss of radial spoke, microtubular transposition, and giant cilia.

Morphological Adaptation after Bilateral Lung Transplantation in a Patient with Kartagener Syndrome: A Case Report

  • Kim, Chang Hun;Son, Bong Soo;Son, Joohyung;Kim, Do Hyung
    • Journal of Chest Surgery
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    • v.55 no.1
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    • pp.77-80
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    • 2022
  • Kartagener syndrome (KS) is a rare disease with an incidence of 1 in 20,000 to 30,000 births. There is no cure for KS, and conservative medical treatments are used to relieve symptoms and prevent disease progression. Lung transplantation (LT) is the only treatment option for end-stage KS. Since patients with KS have anatomical abnormalities such as situs inversus totalis, which often require surgery to correct, most reports are related to surgical techniques. Reports about morphological adaptations and changes in transplanted lung structure after LT in patients with KS are rare. We performed LT in a patient with KS and observed morphological adaptation of the lungs for 6 months on chest computed tomography using a quantitative evaluation tool (Chest Image Platform; Harvard University Disability Resources, Cambridge, MA, USA).

Overview of respiratory diseases in terms of insurance medicine (주요 호흡기질환의 보험의학적 이해)

  • Lee, Sin-Hyung
    • The Journal of the Korean life insurance medical association
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    • v.30 no.2
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    • pp.20-27
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    • 2011
  • Lung diseases are common in general population and they are also important in insurance medicine. According to current textbook of insurance medicine, there are references of western research which performed at 1980s and early 2000. It's necessary to update the reference. In this article, several respiratory disorders such as Kartagener's syndrome, bronchial asthma, drug-resistant tuberculosis, etc are reviewed in terms of insurance medicine. Reference articles were used in Korean study, if possible. I hope this article being a role of helping many insurance doctors and underwriters in Korea.

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