scholarly journals Taste and smell function in Wolfram syndrome

2019 ◽  
Author(s):  
Raul Andres Alfaro ◽  
Tasha Doty ◽  
Anagha Narayanan ◽  
Heather Lugar ◽  
Tamara Hershey ◽  
...  

Abstract BACKGROUND: Wolfram syndrome is a rare genetic disease characterized by insulin-dependent diabetes, optic nerve atrophy, sensorineural hearing loss and neurodegeneration. Although olfactory dysfunction, a classical clinical marker of neurodegenerative processes, has been reported in Wolfram syndrome, its use as a clinical marker in Wolfram is limited due to data scarcity. In addition, it is unknown whether Wolfram syndrome affects the sense of taste.METHODS: Smell and taste perception were assessed in participants with Wolfram syndrome (n=40) who were 15.1 ± 6.0 years of age (range: 5.1- 28.7 years) and two sex- and age-matched control groups: one group with type 1 diabetes mellitus (T1D; n=25) and a healthy control group (HC; n=29). Smell sensitivity was assessed by measuring n-butanol detection thresholds and smell identification by using the University of Pennsylvania Smell Identification Test (UPSIT). Taste function was assessed using NIH Toolbox, which includes the assessment of sucrose (sweet) taste preference, and perceived intensity of sucrose, sodium chloride (salty), and quinine hydrochloride (bitter) both in the tip of the tongue (regional test) and the whole mouth. RESULTS: Smell sensitivity was not significantly different among groups; however, smell identification was impaired in Wolfram syndrome, as reflected by significantly lower UPSIT scores in Wolfram syndrome compared to HC and T1D (P<0.001). Compared to participants in the control groups, participants with Wolfram syndrome had a blunted perception of sweetness and saltiness when taste stimuli were applied regionally (P<0.05), but differences in perceived intensity were no longer significant among groups when taste stimuli were tasted with the whole mouth. Groups preferred similar sucrose concentrations. CONCLUSION: Wolfram syndrome was associated with olfactory dysfunction. However, the olfactory dysfunction was qualitative (related to smell identification) and not secondary to olfactory insensitivity or diabetes, suggesting is arising from dysfunction in central olfactory brain regions. In contrast to olfaction, and despite decreased perception of taste intensity in the anterior tongue, the sense of taste was overall well-conserved in individuals with Wolfram syndrome. Future longitudinal studies of taste and smell perception in Wolfram syndrome will be important to determine the use of the chemical senses as clinical markers of disease progression.

2020 ◽  
Author(s):  
Raul Andres Alfaro ◽  
Tasha Doty ◽  
Anagha Narayanan ◽  
Heather Lugar ◽  
Tamara Hershey ◽  
...  

Abstract BACKGROUND : Wolfram syndrome is a rare genetic disease characterized by insulin-dependent diabetes, optic nerve atrophy, sensorineural hearing loss and neurodegeneration. Although olfactory dysfunction, a classical clinical marker of neurodegenerative processes, has been reported in Wolfram syndrome, its use as a clinical marker in Wolfram is limited due to data scarcity. In addition, it is unknown whether Wolfram syndrome affects the sense of taste. METHODS: Smell and taste perception were assessed in participants with Wolfram syndrome (n=40) who were 15.1 ± 6.0 years of age (range: 5.1- 28.7 years) and two sex- and age-matched control groups: one group with type 1 diabetes mellitus (T1D; n=25) and a healthy control group (HC; n=29). Smell sensitivity was assessed by measuring n-butanol detection thresholds and smell identification by using the University of Pennsylvania Smell Identification Test (UPSIT). Taste function was assessed using NIH Toolbox, which includes the assessment of sucrose (sweet) taste preference, and perceived intensity of sucrose, sodium chloride (salty), and quinine hydrochloride (bitter) both in the tip of the tongue (regional test) and the whole mouth. RESULTS: Smell sensitivity was not significantly different among groups; however, smell identification was impaired in Wolfram syndrome, as reflected by significantly lower UPSIT scores in Wolfram syndrome compared to HC and T1D (P<0.001). Compared to participants in the control groups, participants with Wolfram syndrome had a blunted perception of sweetness and saltiness when taste stimuli were applied regionally (P<0.05), but differences in perceived intensity were no longer significant among groups when taste stimuli were tasted with the whole mouth. Groups preferred similar sucrose concentrations. CONCLUSION : Wolfram syndrome was associated with olfactory dysfunction. However, the olfactory dysfunction was qualitative (related to smell identification) and not secondary to olfactory insensitivity or diabetes, suggesting is arising from dysfunction in central olfactory brain regions. In contrast to olfaction, and despite decreased perception of taste intensity in the anterior tongue, the sense of taste was overall well-conserved in individuals with Wolfram syndrome. Future longitudinal studies of taste and smell perception in Wolfram syndrome will be important to determine the use of the chemical senses as clinical markers of disease progression.


2019 ◽  
Vol 98 (8) ◽  
pp. NP125-NP130
Author(s):  
Serhan Derin ◽  
Selvet Erdogan ◽  
Murat Sahan ◽  
Mehmet Fatih Azik ◽  
Hatice Derin ◽  
...  

Ocular and ophthalmological adverse effects may be seen in β-thalassemia major (BTM) patients treated with regular blood transfusions and iron-chelating agents. We hypothesized that olfactory dysfunction may be present in this population. In this study, we aimed to investigate olfactory dysfunction in patients with BTM and determine the etiological factors. A total of 43 patients with BTM were included in the study. Forty-three patients without any nasal complaints, history of facial trauma, or nasal surgery were included as the controls. All participants had nasal endoscopy. The iron-chelating agents used, their duration of use, as well as hemoglobin and ferritin levels of the BTM patients were recorded. Sniffin’ Sticks test (SST) was used to assess olfactory functions, and BTM and control groups were compared for the results. The correlations of SST scores with the other study parameters were analyzed. Eight (18.6%) of 43 patients in the BTM group had hyposmia while none of the patients in the control group had hyposmia ( P < .001). Older age, low-hemoglobin level, and longer use of deferoxamine were found to be correlated with olfactory dysfunction. Olfactory dysfunction may be seen in BTM patients treated with iron-chelating agents. The results of this study suggest that screening for olfactory function may be needed in routine follow-up of BTM patients.


Nutrients ◽  
2019 ◽  
Vol 11 (1) ◽  
pp. 155 ◽  
Author(s):  
Emma Feeney ◽  
Laura Leacy ◽  
Mark O’Kelly ◽  
Niamh Leacy ◽  
Abbie Phelan ◽  
...  

Taste is influenced by several factors. However, whether habitual exercise level is associated with differences in taste perception has received little investigation. The aim of this study was to determine if habitual exercise is associated with differences in taste perception in men. Active (n = 16) and inactive (n = 14) males, between ages 18–55, underwent two days of sensory testing, using prototypical taste stimuli of high and low concentrations for sweet, salt, bitter, sour, umami, and carbohydrate (maltodextrin). Mean perceived intensity and hedonic ratings were recorded. Eating behaviour was assessed by the three factor eating questionnaire and food intake by EPIC food frequency questionnaire (FFQ). There were moderate to large differences between the two groups in perceived intensity for sweet taste at the high concentration and umami taste at both high and low concentrations, with active males recording a higher perceived intensity (p < 0.05 for all). The active group also recorded a greater dislike for umami low and carbohydrate low concentration (p < 0.01). Salt, bitter and sour perception did not significantly differ between the two groups. FFQ analysis showed no difference in % energy from macronutrients between the groups. Eating behaviour traits correlated with sweet taste intensity and umami taste liking, independent of activity status. Results indicated that sweet and umami taste perception differ in active compared to inactive males. Habitual exercise level should be considered in taste perception research and in product development. Whether differences in taste perception could be one factor influencing food intake and thus energy balance with habitual exercise warrants further investigation.


2009 ◽  
Vol 111 (5) ◽  
pp. 958-962 ◽  
Author(s):  
Gemma Escartin Martin ◽  
Carme Junqué ◽  
Montserrat Juncadella ◽  
Andreu Gabarrós ◽  
Maria Angels de Miquel ◽  
...  

Object Olfactory dysfunction has an important impact on quality of life. In patients with subarachnoid hemorrhage (SAH), anosmia has mainly been reported after surgery for aneurysms of the anterior communicating artery (ACoA). The authors studied whether and how frequently patients with ACoA aneurysms present with smell identification deficits in 2 treatment groups (endovascular and surgical treatment). Methods A prospective study was conducted of patients with SAH caused by ruptured ACoAs and who had a Glasgow Outcome Scale score of 1 or 2, in comparison with a control group matched by age and sex. Olfactory function was assessed using the University of Pennsylvania Smell Identification Test (UPSIT). Results A total of 39 patients were enrolled. A marked olfactory impairment was observed in patients with ruptured ACoAs compared with the control group (p < 0.001). Seventeen patients with ruptured ACoAs (44%) compared with 1 patient in the control group (3%) showed a smell identification deficit according to performance on the UPSIT (p < 0.001). Both groups that underwent treatment presented with olfactory impairment. Ten (59%) of 17 patients who underwent aneurysmal clip placement versus 6 (28.5%) of 21 patients who underwent coil embolization scored below the 25th percentile on the UPSIT, and surgical patients also performed worse than endovascular patients (p = 0.048). The authors observed a worse performance on the olfactory test in patients subjected to endovascular coil embolization when cerebral vasospasm (p = 0.037) or frontal cerebral lesions (p = 0.009) were present. This difference was not observed in patients who underwent surgery. Conclusions Olfactory disorders after SAH caused by rupture of the ACoA are very frequent and were present in both treatment groups. Cerebral vasospasm and frontal lobe lesions are related to worse performance on an olfactory test in patients undergoing endovascular coil embolization.


2010 ◽  
Vol 80 (1) ◽  
pp. 65-73 ◽  
Author(s):  
Pei-Min Chao ◽  
Wan-Hsuan Chen ◽  
Chun-Huei Liao ◽  
Huey-Mei Shaw

Conjugated linoleic acid (CLA) is a collective term for the positional and geometric isomers of a conjugated diene of linoleic acid (C18:2, n-6). The aims of the present study were to evaluate whether levels of hepatic α-tocopherol, α-tocopherol transfer protein (α-TTP), and antioxidant enzymes in mice were affected by a CLA-supplemented diet. C57BL/6 J mice were divided into the CLA and control groups, which were fed, respectively, a 5 % fat diet with or without 1 g/100 g of CLA (1:1 mixture of cis-9, trans-11 and trans-10, cis-12) for four weeks. α-Tocopherol levels in plasma and liver were significantly higher in the CLA group than in the control group. Liver α-TTP levels were also significantly increased in the CLA group, the α-TTP/β-actin ratio being 2.5-fold higher than that in control mice (p<0.01). Thiobarbituric acid-reactive substances were significantly decreased in the CLA group (p<0.01). There were no significant differences between the two groups in levels of three antioxidant enzymes (superoxide dismutase, glutathione peroxidase, and catalase). The accumulation of liver α-tocopherol seen with the CLA diet can be attributed to the antioxidant potential of CLA and the ability of α-TTP induction. The lack of changes in antioxidant enzyme protein levels and the reduced lipid peroxidation in the liver of CLA mice are due to α-tocopherol accumulation.


1993 ◽  
Vol 30 (2) ◽  
pp. 227-230 ◽  
Author(s):  
Andrew Mccance ◽  
David Roberts-Harry ◽  
Martyn Sherriff ◽  
Michael Mars ◽  
William J.B. Houston

The study models of a group of adult Sri Lankan patients with clefts of the secondary palate were investigated. Tooth-size and arch-dimension comparisons were made with a comparable control group. Significant differences were found between the cleft and control groups in tooth sizes, chord lengths, and arch widths. The cleft group dimensions were generally smaller than those of the control group. Overjets were larger in the cleft group.


Author(s):  
Hasanul Arifin Zul And Masitowarni Siregar

This thesis is focused on the investigation of the effect of applying animal cartoon pictures on students’ achievement in writing narrative text. This study aims to find whether applying animal cartoon pictures significantly affect the students’ writing achievement or not. The data in this study were obtained by administering a written test. The population was the 2015/2016 first year (grade XI) of SMA Swasta Nusantara Lubuk Pakam and 66 students were taken as the sample by using random sampling. The sample was divided into two groups, experimental and control groups. The experimental group was taught by applying animal cartoon pictures while the control group without animal cartoon pictures (x = lecturing). The data were taken the scores from the pre-test and post-test to both experimental and control groups. These data were analyzed by using t-test. The result of computing the t-test obviously showed that t-observed is higher than t-table (5.21 >1,67) with the degree of freedom 64 (df =N-2) at the level significance 0,05 one tail test. It showed that the application of animal cartoon pictures significantly affected the students of SMA Swasta Nusantara Lubuk Pakam achievement in writing narrative text.


Author(s):  
Meryanti Napitupulu And Anni Holila Pulungan

This study was conducted as an attempt to discover the effect of applying Demonstration Method on students’ achievement in speaking skill. It was an experimental research. The subject was students of Grade XII, Vocational High School (Sekolah Menengah Kejuruan: SMK), which consisted of 79 students. The research was divided into two groups: experimental and control groups. The instrument used to collect the data was speaking test. To obtain the reliability of the test, the writer applied Kuder Richardson 21 formula. The result of the reliability was 0.7, and it was found that the test was reliable. The data were analyzed by using t-test formula. The analysis showed that the scores of the students in the experimental group were significantly higher than the scores of the students in the control group at the level of significant m = 0.05 with the degree of freedom (df) 77, t-observed value 8.9 > t-table value 1.99. The findings indicate that using Demonstration Method significantly affected the students’ achievement in speaking skill. So, English teachers are suggested to use Demonstration Method in order to improve students’ achievement in speaking skill.


2016 ◽  
Vol 1 (1) ◽  
pp. 22
Author(s):  
Nazli Zainuddin ◽  
Nurul Azira Mohd Shah ◽  
Rosdan Salim

Introduction: The role of virgin coconut oil in the treatment of allergic rhinitis is controversial. Thus, the aim of the present study is to determine the effects of virgin coconut oil ingestion, in addition to standard medications, on allergic rhinitis. We also studied the side effects of consumption of virgin coconut oil. Methods: Fifty two subjects were equally divided into test and control groups. All subjects received a daily dose of 10mg of loratadine for 28 days. The test group was given 10ml of virgin coconut oil three times a day in addition to loratadine. The symptoms of allergic rhinitis were scored at the beginning and end of the study. Results:, the symptom score were divided into nasal and non-nasal symptom scores. Sneezing score showed a significant difference, however the score was more in control group than test group, indicating that improvement in symptom was more in control group. The rest of the nasal symptom and non-nasal symptom score showed no significant difference between test and control groups. Approximately 58% of the test subjects developed side effects from consumption of virgin coconut oil, mainly gastrointestinal side effects. Conclusion: In the present study, ingestion of virgin coconut oil does not improve the overall and individual symptoms of allergic rhinitis, furthermore it has side effects.


1970 ◽  
Vol 2 (3) ◽  
Author(s):  
Ermiati E ◽  
Imas Rafiyah ◽  
Devi Kusnanti

Anemia merupakan salah satu penyebab terjadinya perdarahan dan bisa mengakibatkan kematian pada ibu hamil. Suplementasi besi merupakan program pemerintah dalam mengatasi anemia pada wanita hamil dan beberapa wanita hamil masih ada yang belum patuh dalam mengkonsumsi tablet besi. Oleh karena itu, diperlukan sebuah metode yang bisa meningkatkan kepatuhan wanita hamil dalam mengonsumsi tablet besi yaitu dengan short message service (SMS) reminder. Penelitian ini bertujuan untuk mengidentifikasi efektivitas SMS reminderterhadap kepatuhan konsumsi tablet besi pada ibu hamil di (unit pelayanan terpadu) UPT Puskesmas Cibuntu Kota Bandung. Rancangan penelitian quasi experimentaldengan desain posttest-only with control group designdengan teknik pengambilan sampel yang digunakan adalah purposive sampling. Penelitian ini dilaksanakan dari bulan April–Mei tahun 2014 di Puskesmas X Kota Bandung. Sampel berjumlah 40 orang wanita hamil yang tidak patuh dalam mengonsumsi tablet besi yang dibagi menjadi 20 kelompok intervensi dan 20 kelompok kontrol. Data diperoleh melalui kuesioner dalam bentuk self report.Analisis data yang digunakan adalah analisis deskriptif dan uji chi square. Hasil penelitian menunjukkan sebagian besar kelompok intervensi 17 orang (85%) patuh dan sebagian besar kelompok kontrol 16 orang (80%) tidak patuh. Hasil analisis dengan menggunakan chi squaredidapatkan p(0.000) dengan taraf signifikan <0.05 sehingga dapat disimpulkan bahwa SMS reminder efektif terhadap kepatuhan konsumsi tablet besi pada wanita hamil dan bisa dijadikan bahan pertimbangan bagi pihak Puskesmas untuk menjalin kerjasama dengan pihak provideratau mengajukan dana ke Dinas Kesehatan untuk mengaktifkan SMS reminderdi Puskesmas.Kata kunci: Kepatuhan, SMS reminder,tablet besi, wanita hamil AbstractAnemia is one of the leading causes of hemorrhage during pregnancy, and it can cause death to expectant mothers. Iron supplementation is a government program to overcome anemia among expectant mothers, and some of the expectant mothers still do not obediently consume iron tablets. Therefore, to increase the obedience of the expectant mothers on consuming the iron tablets, SMS reminder is the appropriate method needed. This research aims to identify the effectiveness of SMS reminder to the obedience of iron tablets consumption among expectant mothers at UPT Puskesmas Cibuntu , Bandung. The research employed quasi-experimental design with posttest-only control group design using purposive sampling technique. The research was held on April–May 2014 at UPT Puskesmas Cibuntu, Bandung. Forty expectant mothers consuming iron tablets disobediently divided into 20 intervention groups and 20 control groups were taken as the samples. Questionnaire in form of self report was used to obtain data. In addition, descriptive analysis and chi square test were applied to analyze the data. The result of the research showed that 17 persons (85%) of intervention groups were mostly obedient, and 16 persons (80%) of control groups were mostly disobedient. From the analysis using chi square, it was found the difference of the obedience of iron tablets consumption after SMS reminder (p=0.00) had been delivered with significant level <0.05. Based on the result of this research, SMS reminder had an effective impact toward the obedience of iron tablets consumption among the expectant mothers. The result will be a good consideration for Puskesmas to cooperate with provider or proposed some funds to Dinas Kesehatan to activate SMS reminder.Key words:Expectant mothers, obedience, SMS reminder, iron tablets


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