Kartagener’s syndrome: A case report

Taušan, Đorđe; Ristić, Anđelka; Zvezdin, Biljana

doi:10.2298/vsp141020072t

Cited by 3 publications

(2 citation statements)

References 10 publications

(9 reference statements)

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“…Primary ciliary dyskinesia (PCD) is a group of autosomal recessive disorders, and the subgroup that presents with the triad of sinusitis, bronchiectasis, and dextrocardia is called Kartagener's syndrome (KS). 1 Described by Kartagener in 1933, 2 this syndrome has an incidence of1-2/30,000 birth 3 and is seen in 40% to 50% of patients with PCD. 4 The ciliary dyskinesia and ineffective mucociliary clearance affect many tissues and organs such as the nasal mucosa and paranasal sinuses.…”

Section: Introductionmentioning

confidence: 99%

<p>Anesthesia During Functional Endoscopic Sinus Surgery for Kartagener’s Syndrome: A Case Report and Literature Review</p>

El-Radaideh¹,

Al‐Qudah²,

Alali³

et al. 2020

IJGM

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Introduction: Kartagener's syndrome (KS) is a ciliopathic, autosomal recessive disorder characterized by the triad of situs inversus, chronic sinusitis, and bronchiectasis. The abnormal ciliary structure and function lead to variable clinical manifestations, including dextrocardia, pneumonia, bronchitis, chronic rhinosinusitis, otitis media, reduced fertility in women, and infertility in men. This article reports our experience on general anesthesia with endotracheal intubation during functional endoscopic sinus surgery (FESS) in a patient with KS. Case Presentation: A 44-year-old man was admitted to our hospital with chronic nasal obstruction, postnasal drip, chronic sinusitis, and chronic non-productive cough for FESS. The patient's heart was on the right side of his chest. A chest roentgenogram and a high-resolution chest and abdomen computed tomography (CT) scan identified dextrocardia, situs inversus, and chronic bronchitis and bronchiectasis involving both lung bases. CT sinuses showed mucosal thickening of bilateral maxillary and ethmoid and sphenoid sinuses. The patient was prescribed oral medications and nasal spray for crepitations and wheezes heard over bilateral lung fields. Intensive chest physiotherapy and supportive care prior to surgery were provided to prevent worsening of lung function. FESS with bilateral frontal polypectomy was performed. All hemodynamic parameters were stable. The emergence from anesthesia was smooth. After~20 minutes in the post-anesthesia care unit, the patient was fully awake and pain-free. He was then transferred to the surgical intensive care unit and subsequently to the ward. The postoperative period was uneventful. The patient felt subjectively "very well" and was discharged from the hospital on the 2nd postoperative day. Conclusion: Anesthesiologists must be aware of cardiopulmonary inversion that could challenge the management of patients with KS. To avoid respiratory depression caused by long-acting systemic opioids, we suggest using short-acting opioids during general anesthesia and for postoperative pain relief.

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Section: Introductionmentioning

confidence: 99%

<p>Anesthesia During Functional Endoscopic Sinus Surgery for Kartagener’s Syndrome: A Case Report and Literature Review</p>

El-Radaideh¹,

Al‐Qudah²,

Alali³

et al. 2020

IJGM

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“…The main treatment goals are to prevent the progression of the disease, preserve pulmonary function, and improve quality of life. Standard treatment for sinopulmonary infections in KS includes mucolytics, bronchodilators, antibiotics, and chest physiotherapy 17. In patients with frequent exacerbations of bronchiectasis (≥3 times/year), a long-term low-dose prophylactic antibiotic is required.…”

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confidence: 99%

Kartagener Syndrome: A Case Report

Willim

Pebriadi

Munthe³

et al. 2022

AMCR

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Kartagener syndrome (KS) is a rare autosomal recessive disorder characterized by a clinical triad of situs inversus, chronic sinusitis, and bronchiectasis. Impaired ciliary motility due to abnormal ciliary structure or function is the main pathophysiological problem in KS. A 38-year-old woman presented to our outpatient clinic with a productive cough, fever, and shortness of breath for 1 month. She has had recurrent episodes of respiratory tract infections since childhood. Clinical investigations revealed situs inversus, sinusitis, and bronchiectasis. She was diagnosed with KS and treated with antibiotics, mucolytics, bronchodilators, and chest physiotherapy. Patients with KS present with chronic recurrent respiratory tract infections due to ineffective mucociliary clearance. Early diagnosis is important to improve prognosis. The main goals in the management of KS are to prevent the progression of the disease, preserve pulmonary function, and improve quality of life.

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The impact of primary ciliary dyskinesia on female and male fertility: a narrative review

Newman

Chopra

Dossett

et al. 2023

Human Reproduction Update

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BACKGROUND Primary ciliary dyskinesia (PCD) is a genetic condition affecting the structure and function of sperm flagellum and motile cilia including those in the male and female reproductive tracts. Infertility is a commonly reported feature of PCD, but there is uncertainty as to how best to counsel patients on their fertility prognosis. OBJECTIVE AND RATIONALE This review aimed to summarize the prevalence of subfertility, possible underlying mechanisms, and the success of ART in men and women with PCD. The efficacy of ART in this patient group is relatively unknown and, hence, the management of infertility in PCD patients remains a challenge. There are no previous published or registered systematic reviews of fertility outcomes in PCD. SEARCH METHODS Systematic literature searches were performed in Medline, Embase, Cochrane Library, and PubMed electronic databases to identify publications between 1964 and 2022 reporting fertility outcomes in men and women with PCD. Publications were excluded if they reported only animal studies, where gender was not specified or where subjects had a medical co-morbidity also known to impact fertility. Quality of evidence was assessed by critical appraisal and application of an appraisal tool for cross-sectional studies. The primary outcomes were natural conception in men and women with PCD, and conception following ART in men and women with PCD. OUTCOMES A total of 1565 publications were identified, and 108 publications were included after screening by two independent researchers. The quality of available evidence was low. The exact prevalence of subfertility in PCD is unclear but appears to be higher in men (up to 83% affected) compared to women (up to 61% affected). Variation in the prevalence of subfertility was observed between geographic populations which may be explained by differences in underlying genotype and cilia function. Limited evidence suggests subfertility in affected individuals is likely caused by abnormal cilia motion in the fallopian tubes, endometrium and efferent ductules, and dysmotile sperm. Some men and women with PCD benefited from ART, which suggests its use should be considered in the management of subfertility in this patient group. Further epidemiological and controlled studies are needed to determine the predictors of fertility and optimal management in this patient group. WIDER IMPLICATIONS It is important that patients with PCD receive evidence-based counselling about the potential impact of their condition on their fertility prognosis and what management options may be available to them if affected. Understanding the pathophysiology and optimal management of subfertility in PCD will increase our understanding of the role of cilia and the impact of wider secondary ciliopathies on reproduction.

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Kartagener’s syndrome: A case report

Abstract: Treatment for patients with this syndrome has not been established yet, but it is important to control chronic lung infections and prevent declining of lung function.

Cited by 3 publications

References 10 publications

<p>Anesthesia During Functional Endoscopic Sinus Surgery for Kartagener’s Syndrome: A Case Report and Literature Review</p>

<p>Anesthesia During Functional Endoscopic Sinus Surgery for Kartagener’s Syndrome: A Case Report and Literature Review</p>

Kartagener Syndrome: A Case Report

The impact of primary ciliary dyskinesia on female and male fertility: a narrative review

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