Saccharin test is a method for estimating mucociliary clearance in the upper respiratory passage. Its application in a clinically diagnosed patient with Kartagener's syndrome is reported

Deivanayagam, R

Mukhtar Admed, P

Rajaram, K

Ramu, S

Swaminathan, T

NIRT Institutional Repository

33SACCHARIN TEST OF MUCOCILIARY CLEARANCE INKARTAGENER’S SYNDROMERAJARAM, K., RAMU, S, PRABHAKAR, R., DEIVANAYAGAM, P., MUKHTAR AHAMEDAND SWAMINATHAN, T.,Tuberculosis Research Centre project at Madurai and Department of Radiologyand Otorhinolaryngology, Government Rajaji Hospital, Madurai 625 020.reported.IntroductionPrimary ciliary dyskinesia, also knownas Immotile cilia syndrome is now knownto be a syndrome with many manifestationswith the basic defect of d&ordered ciliarymotility. Kartaaener’s syndrome comprisesabsent or inflamed frontal sinuses, situsinversus with dextrocardia and bronchiecta-sis and is now considered a manifestationof immotile cilia syndrome. A simplebedside method of diagnosing reducedmucociliary clearance in these disordershas been reported. We report its applicationin a patient clinically and radiologicallydiaqnosed as Kartagener’s syndrome.Case ReportA 45 year old housewife presented tothe chest clinic with nasal obstruction,running nose and cough with expectorationoff and on for nearly 30 years. She hadexperienced intermittent fever for thepast year. She had undergone a rightlower lobectomy in 1970 for “recurrentcouqh with copious expectoration”. Fulldetails were not available. She has hadno past history of measles or whoopingcough and denied any food or foreignbody aspiration. On examination she wasfound to be pale, thin and without digitalclubbing. Her trachea was shifted to rightside and she exhibited fine to mediumbibasilar crepitations which persistedafter coughing. Heart sounds were betterheard on the right chest and the liverdullness was absent on the right side.The central nervous system appearedclinically normal. On nasal examination,septal deflectjon to right with bilateralhypertrophied turbinates were seen. Therewas mucopus on the nasal floor on theright side. The cords appeared normalon indirect laryngoscopy. Roth tympanicmembranes were retracted. Urine analysisrevealed no casts, no albumin and nosugar. The peripheral blood count and ery-throcyte sedimentation was normal. Sheexhibited a moderate iron deficiency ana-ernia. Acid fast bacilli were not seenon sputum smear (concentration method)on three days. Mantoux (with PPD) revealed0 mm induration at 48 hours.Skiagram paranasal sinuses revealedbilateral maxillary haziness (with mucosaof five mm +) and absence of frontalsinuses. The skiagram chest (PA view)revealed dextrocardia, tramlining andlinear or irregular opacities left lowerzone and fundus gas beneath right dome(Fig I). Bronchogram revealed predominantlytubular bronchiectasis of the left lowerlobe.34Fig 1. Skiagram chest PA view showing evidenceof dextro cardia, fundus shadow on theright side, and bronchiectatic changes leftlower zone.Saccharin TestA small grain of saccharin (approximately1/10th of a commercially available tabletvisually measured) is placed (with thehelp of a pair of forceps) two centimetresfrom the free edge of ala nasi on theinferior turbinate. The patient sits upduring the entire period of testing. Thepatient is asked to indicate to the investi-gator, as soon as he/she appreciates thesweet taste of saccharin. Most normalsreport the taste within thirty minutes.The patient under report took one hourand fifty two minutes.DiscussionAufzelius reported abnormal respiratorymucosal ciliary activity in associationwith Kartagener’s syndrome. He and histeam have reported extensively. on abnormalcilia in several syndromes 1, 2, 3, 4Mucociliary clearance is impaired inany disorder affecting the ciliary structureand function and this leads to recurrentor chronic respiratory infections. TheLUNG INDIA (1987) V, No.1; (P.34)schematic representation of the electronmicroscopic appearance of a cross sectionof a cilium is shown in Fig.2. Most reportspertain to abnormality or absence ofthe dynein arms. Functional ciliary dyskin-esia has also been reported: Other reportedabnormalities include absence of connectingspokes, deletion of control tubules, missingtubules, extra-tubules, compound ciliaand intracytoplasmic cilia. These areall considered to be due to mutation.Fig 2. Diagram of ultra structure of respiratorycilium.Even when the dynein arms are absent,these cilia exhibit some movement - theyare not totally immotile. This has ledto the use of the term - primary ciliarydyskinesia, which is gaining wide acceptance.Ciliary motility has been, measured byphotometry 5 . Stanley et al studied 16patients with primary ciliary dyskinesiaand found that all had saccharin timeof more than 60 minutes 14 of the 16patients showed sinus mucosa of morethan 5 mm thickness.The prevalence of primary ciliary dyskine-sia is estimated to be between 1 in 15,000Rajaram et al: Saccharin test in Kartagenar’s .... 35to 1 in 30,000 live births (Rott6)Saccharin test for mucociliary clearanceof the upper respiratory tract may beused as a screening test for primary ciliarydyskinesia syndrome of which Kartagener’ssyndrome is a subset. Electron microscopyfor ultrastructural defects remains a researchtool and is not available in most partsof the country.REFERENCES1. Stanley, P., Macwilliam, L., Greenstrone, M., Mackay,I., and Cole, P., (1984): Efficacy of a saccha-rin test of screening to detect an abnormalmucociliary clearance. Brit. J. Dis. Chest, 78,62-65.2. Aufzelius, B.A., (1976): A human syndrome causedby immotile cilia. Science 193, 317-319.3. Aufzelius B.A. (1979): The Immotile Cilia Synd-rome and other ciliary diseases. Inter. Rev.Path. 19, 1-43.4. Aufzelius, B.A. (1981): ‘Immotile Cilia’ Syndrome andciliary abnormalities induced by infection andinjury. Am. Rev. Res. Dis. 124, 107-109.5. Greenstone. M., Dewar, A., and Cole, P. (1983)Ciliary Dyskinesia with normal ultrastructure.Thorax. 38, 875-876.6. Rott, H.D. (1979): Kartagener’s Syndrome andthe Syndrome of lmmotile Cilia, Human Genetics.46, 249-251.Correspondence/request for reprints to: Dr. K. Rajaram,ICMR/TRC Madurai TB Project, Govt. Rajaji Hospital,Madurai, Tamil Nadu.

Saccharin test of mucociliary clearance in Kartagener's syndrome

http://eprints.nirt.res.in/217/1/198718.pdf

Saccharin test of mucociliary clearance in Kartagener's syndrome

Abstract

Similar works

Full text

Available Versions

NIRT Institutional Repository