Cross-sectional evaluation of the saccharin transit time test for primary ciliary dyskinesia: did we discard this tool too soon?

ABSTRACT BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare and heterogeneous disease that is difficult to diagnose and requires complex and expensive diagnostic tools. The saccharin transit time test is a simple and inexpensive tool that may assist in screening patients with PCD. OBJECTIVES: This study aimed to compare changes in the electron microscopy findings with clinical variables and saccharin tests in individuals diagnosed with clinical PCD (cPCD) and a control group. DESIGN AND SETTING: An observational cross-sectional study was conducted in an otorhinolaryngology outpatient clinic from August 2012 to April 2021. METHOD: Patients with cPCD underwent clinical screening questionnaires, nasal endoscopy, the saccharin transit time test, and nasal biopsy for transmission electron microscopy. RESULTS: Thirty-four patients with cPCD were evaluated. The most prevalent clinical comorbidities in the cPCD group were recurrent pneumonia, bronchiectasis, and chronic rhinosinusitis. Electron microscopy confirmed the clinical diagnosis of PCD in 16 of the 34 (47.1%) patients. CONCLUSION: The saccharin test could assist in screening patients with PCD due to its association with clinical alterations related to PCD.

Anesthetic considerations during surgical intervention in Woake's syndrome: a case report / 대한마취과학회지

BACKGROUND: Woake's syndrome (WS) is a recurrent nasal polyposis, accompanied by broadening of the nose, frontal sinus aplasia, dyscrinia, and bronchiectasis. There has been no previous report on anesthetic management in patients with WS. CASE: We describe a case involving a 13-year-old male patient with WS who was scheduled for septorhinoplasty for necrotic ethmoiditis. Anesthesia was induced and maintained with propofol and remifentanil using a target-controlled infusion device. The anesthetic considerations of this rare syndrome and the advantages of an intravenous infusion method over local and volatile anesthesia for these patients are discussed. We report on caveats, such as pulmonary dysfunction during the anesthetic management, and nasal structural problems encountered in WS patients. CONCLUSIONS: Given that conventional inhaled anesthesia reduces ciliary movement and that local anesthesia with sedative has several disadvantages, perioperative control and precautions against respiratory infections by using antibiotics, and preventing cilio-depressant actions, are important for anesthetic management.

Total intravenous anesthesia for Kartagener's syndrome: A case report

Kartagener's syndrome (KGS) is an autosomal recessive disorder which possible to link the occurrence of abnormal ciliary movement and abnormal position of the body organs. Considering the fact that airway ciliary function plays an important role in the primary pulmonary defense mechanism, prevent the ciliodepressant actions are also important for anesthetic management. We described successful anesthetic management of a 44-year-old male scheduled for endoscopic sinus surgery who had a history of frequent epistaxis, anosmia and situs inversus totalis. Anesthesia was induced and maintained with propofol and remifentanil using a target controlled infusion device.