Una regresión poco común de los síntomas de un síndrome de Kartagener

Serapinas, Danielius; Staikūnienė, Jūratė; Barkauskienė, Diana; Jackutė, Jurgita; Sakalauskas, Raimundas

doi:10.1016/j.arbres.2012.02.021

Cited by 4 publications

(3 citation statements)

References 10 publications

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“…Además, la ecografía abdominal ayuda a localizar la posición de las vísceras abdominales y detectar hernia diafragmática, asplenia o poliesplenia, alteraciones renales (como agenesia renal o riñón en herradura) o de la vía biliar. 5,6 A nivel respiratorio puede ocurrir sinusitis, neumonías de repetición, o el desarrollo de bronquiectasias producidas por la ausencia de transporte mucociliar que provocará estasis de las secreciones al estar la estructura ciliar alterada dificultando su aclaramiento, ocasionando el síndrome de Kartagener, 7 como se sospechó en nuestro paciente. Las alteraciones del cilio, conocidas como DCP o síndrome del cilio inmóvil, están asociadas hasta en 50% de los casos con el SIT, y se postula que puede haber un vínculo que explique esta relación entre el cilio móvil y el eje de izquierda a derecha, que estaría causado por un flujo anormal generado por los monocilios móviles en el nodo embrionario, lo cual es necesario para establecer la lateralidad de estos defectos.…”

Section: Discussionunclassified

Neumonías de repetición en escolar con situs inversus totalis y sospecha de discinesia ciliar primaria

Martínez-Sebastián¹

2021

Revista Mexicana De Pediatría

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Section: Discussionunclassified

Neumonías de repetición en escolar con situs inversus totalis y sospecha de discinesia ciliar primaria

Martínez-Sebastián¹

2021

Revista Mexicana De Pediatría

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“…Impaired ciliary motility could cause perioperative respiratory difficulty in patients with KS. 10 Serapinas et al 11 recommend a combination of physiotherapy and physical exercise to enhance respiratory tract clearance, along with the aggressive treatment of upper and lower respiratory tract infections. We provided intensive physiotherapy and supportive pulmonary care prior to surgery for our patient, along with the antibiotic levofloxacin for preventing the progressing of the respiratory tract infection.…”

Section: Discussionmentioning

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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“…Of the 372 genes identified,164 were known, strongly suggesting that the remaining 208 may be new cilia-related genes, which provides a number of candidates for investigation in PCD/KS. However, it is likely that not all mutations in these genes will cause abnormal ciliary movement, which indirectly reflects the intrinsically complex link between genetic variation and morbid phenotype 666768…”

Section: Pathophysiology and The Role Of Genetic Factorsmentioning

confidence: 99%

Management of primary ciliary dyskinesia/Kartagener′s syndrome in infertile male patients and current progress in defining the underlying genetic mechanism

Sha

Ding

2014

Asian J Androl

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Kartagener's syndrome (KS) is an autosomal recessive genetic disease accounting for approximately 50% of the cases of primary ciliary dyskinesia (PCD). As it is accompanied by many complications, PCD/KS severely affects the patient's quality of life. Therapeutic approaches for PCD/KS aim to enhance prevention, facilitate rapid definitive diagnosis, avoid misdiagnosis, maintain active treatment, control infection and postpone the development of lesions. In male patients, sperm flagella may show impairment in or complete absence of the ability to swing, which ultimately results in male infertility. Assisted reproductive technology will certainly benefit such patients. For PCD/KS patients with completely immotile sperm, intracytoplasmic sperm injection may be very important and even indispensable. Considering the number of PCD/KS susceptibility genes and mutations that are being identified, more extensive genetic screening is indispensable in patients with these diseases. Moreover, further studies into the potential molecular mechanisms of these diseases are required. In this review, we summarize the available information on various aspects of this disease in order to delineate the therapeutic objectives more clearly, and clarify the efficacy of assisted reproductive technology as a means of treatment for patients with PCD/KS-associated infertility.

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Una regresión poco común de los síntomas de un síndrome de Kartagener

Cited by 4 publications

References 10 publications

Neumonías de repetición en escolar con situs inversus totalis y sospecha de discinesia ciliar primaria

Neumonías de repetición en escolar con situs inversus totalis y sospecha de discinesia ciliar primaria

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

Management of primary ciliary dyskinesia/Kartagener′s syndrome in infertile male patients and current progress in defining the underlying genetic mechanism

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