Mitochondrial encephalomyopathy involves ophthalmology otorhinolaryngology neurology and their clinical features

xlomafota13 shared this article with you from Inoreader Mitochondrial encephalomyopathy involves ophthalmology otorhinolaryngology neurology and their clinical features Via cortical auditory Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2021 Jun;35(6):529-534. doi: 10.13201/j.issn.2096-7993.2021.06.010. ABSTRACT Objective:This study aimed to provide better understanding of the otolaryngologic features, combined with ophthalmologic and neurologic characteristics in mitochondrial encephalomyopathy（MEM）, and to help ENT and auditory practitioner making correct diagnosis as well. Methods:Twenty-eight patients with MEM were enrolled between September 2001 and January 2020. Information about family histories and clinical symptoms was retrospectively analyzed. All patients underwent otorhinolaryngological, ophthalmological and neurological examinations, including: pure-tone audiometry, acoustic immittance（AI）, distortion-product otoacoustic emissions（DPOAE）, auditory brainstem response（ABR）, cochlear micropotential（CM）, speech discrimination score（SDS）, electroneurography（ENoG）, computed tomog raphy（CT） of the temporal bone and cranial magnetic resonance weighted imaging scan（MRI）, muscle biopsy and mtDNA gene testing. Results:ENT subjective manifestations were present in 15 cases （53.6%） with sensorineural hearing loss（SNHL）, 4（14.3%） with tinnitus, 4（14.3%） with facial weakness, 3（10.7%） with dysphagia, 1（3.6%） with auditory agnosia. Ophthalmological and neurological symptoms included ptosis in 16 cases （57.1%）, exercise intolerance in 16（57.1%）, optic atrophy in 15（53.6%）, muscular atrophy in 6（21.4%）, and stroke-like episodes in 5（17.9%）. The results of objective examinations were as follows: DPOAE were not elicited in 18（64.3%） cases, ABR abnormalities in 18（64.3%） cases, hearing threshold shift in 15（53.6%） cases, AI normal and CM was not detected in all cases, SDS decreased in 6（21.4%） cases, facial ENoG abnormalities in 4（14.3%） cases, laryngeal ENoG abnormalities in 3（10.7%） cases, EMG abnormalities in 6（21.4%） cases, and ECG abnormalities in 8（28.6%） cases. Temporal CT were normal, but cranial MRI abnormalities were found in 19 cases（67.9%）, including central nerve demyelination, white matter hyperintensities, generalized cerebellar and cerebral atrophy, multiple cortical/subcortical infarct-like lesions, basal ganglia calcification. Conclusion:Multisystemic syndromes in MEM can present as a variety of otolaryngological, ophthalmological and neurological abnormalities, such as ptosis, audio-visual disturbance, exercise intolerance and stroke-like episodes etc. SNHL, tinnitus, auditory agnosia, facial weakness and dysphagia were ENT specific manifestations. SNHL in MEM is bilateral symmetrical progressive or of sudden onset since teenage. mtDNA testing may be helpful for adolescent patient whose SNHL was associated with neuromuscular symptoms. Muscle biopsy should be considered when middle-aged patients developed facial weakness and dysphag ia. DPOAE and ABR are the optimal objective audiometric tests to monitor the progression of MEM associated with SNHL. PMID:34304513 | DOI:10.13201/j.issn.2096-7993.2021.06.010 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

A preliminary study on the negative wave mismatch in patients with unilateral total sudden deafness

xlomafota13 shared this article with you from Inoreader A preliminary study on the negative wave mismatch in patients with unilateral total sudden deafness Via cortical auditory Lin Chung Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2021 May;35(5):391-394. doi: 10.13201/j.issn.2096-7993.2021.05.002. ABSTRACT Objective:To observe the characteristics of different negativity negativity （MMN） in patients with unilateral sudden deafness, and compare them with normal MMN, in order to provide theoretical reference for discussing the pathogenesis of unilateral sudden deafness and their relationship with the auditory centers, and to provide theoretical basis for the treatment of sudden deafness in the future. Methods:Twenty-six cases of unilateral total sudden deafness were recruited as experimental group, 25 cases of normal healthy people as control group, the MMN inspections was performed respectively, the two groups using classical mode of oddball, standard and deviation stimulate with 1000 Hz and 2000 Hz short pure tone test MMN respectively, to observe the MMN latency and amplitude characteristics, and compare the latent period and amplitude difference between the two groups. Results:Among the 51 subjects, only 1 patient with unilateral total sudden deafness did not elicit MMN waveform, while the rest were all induced. The average incubation period of MMN in the experimental group was （162.03±38.64） ms, the average amplitude was （2.83±1.14）μV, and the mean age was （48.64±10.27） y. While the average incubation period of MMN in the control group was （197.52±27.43） ms, the average amplitude was （2.58±1.07）μV, and the mean age was （45.00±8.20） y. The MMN latency was significantly different between the two groups （P<0.01）. There was no statistical difference in amplitude between the two groups （P>0.05）. There was no statistical difference in age between the two groups （P>0.05）. Conclusion:The latency period of MMN of unilateral total sudden deafness is shorter than that of the control group. It suggests th at the auditory center function of patients with acute sudden deafness has changed, and we speculate that the auditory center of patients with acute sudden deafness may have corresponding emergency changes, so as to make its function have adaptive changes, which will provide further reference for the discussion of the pathophysiological mechanism and treatment of sudden deafness in the future.We speculated that acute unilateral auditory deprivation caused by unilateral total deafness sudden deafness has an impact on cerebral cortical auditory function, which provides further reference for the discussion of pathophysiological mechanism and treatment plan of sudden deafness in the future. PMID:34304460 | DOI:10.13201/j.issn.2096-7993.2021.05.002 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Anatomical implication of less occurrence of inferior oblique muscle entrapment in orbital floor trapdoor fracture

xlomafota13 shared this article with you from Inoreader Anatomical implication of less occurrence of inferior oblique muscle entrapment in orbital floor trapdoor fracture Via radiol anat Surg Radiol Anat. 2021 Jul 27. doi: 10.1007/s00276-021-02808-4. Online ahead of print. ABSTRACT PURPOSE: To examine the anatomy of the inferior oblique (IO) muscle and its surrounding structures to clarify why IO muscle entrapment develops less in orbital floor trapdoor fractures. METHODS: Computed tomographic (CT) images on the unaffected sides were obtained from 64 patients with unilateral orbital fractures. On coronal planes, presence or absence of an infraorbital g roove below the IO muscle was confirmed. At the level of the medial margin of the infraorbital groove/canal, the distance from the orbital floor to the IO muscle (IO-floor distance), the thickness of the orbital floor, and the shortest distance from the inferior rectus (IR) muscle to the orbital floor (shortest IR-floor distance) were measured. On quasi-sagittal planes, the distances from the inferior orbital rim to the inferior margin of the IO muscle (IO-rim distance) and the most anterior point of the infraorbital groove (groove-rim distance) were measured. RESULTS: The infraorbital groove was found below the IO muscle in eight patients (12.5%), and the IO-rim and IO-floor distances were significantly longer than the groove-rim and shortest IR-floor distances, respectively (p < 0.001). The orbital floor below the IO muscle was significantly thicker than that below the IR muscle (p < 0.001). CONCLUSION: Although the medial margin of the infraorbital groove is the most common fracture site, the IO muscle was not located above the groove in most cases. A longer IO-floor distance and thicker orbital floor below the IO muscle may also contribute to less occurrence of IO muscle entrapment in orbital floor trapdoor fractures. PMID:34313811 | DOI:10.1007/s00276-021-02808-4 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Radiosynthesis and in silico bioevaluation of 131 I-Sulfasalazine as a highly selective radiotracer for imaging of ulcerative colitis

xlomafota13 shared this article with you from Inoreader Radiosynthesis and in silico bioevaluation of <sup>131</sup> I-Sulfasalazine as a highly selective radiotracer for imaging of ulcerative colitis Via radioactive iodine Chem Biol Drug Des. 2021 Jul 27. doi: 10.1111/cbdd.13929. Online ahead of print. ABSTRACT This study demonstrated the tracking of ulcerative colitis, which is considered a stressful immune disease. Although there are many ways to test for this disease including dependence on gases, dyes, and painful anal endoscopy, these treatment modalities have many disadvantages. Hence, it is the utmost need of time to discover new methods to detect this chronic immune disease and to avoid the d efects of traditional methodologies. Sulfasalazine (SSD) was labeled with iodine-131 (Half-life: 8 days, Energy: 971 keV) under optimum reaction conditions including the amount of reducing agent, pH factor, Chloramine-T (Ch-T) amount, and incubation period. Characterization was performed using 1 H/ 13 C-NMR, ESI-MS, and HPLC (UV/ Radio) techniques. The biodistribution study was performed in normal and ulcerative mice models as well as in silico molecular docking study was performed to evaluate the possible mechanism of action to target peroxisome proliferator-activated receptor gamma (PPARγ). The high radiolabeling yield of [131 I]-sulfasalazine ([131 I]-SSD) was achieved ≥ 90% through the direct labeling method with radioactive iodine-131 in the presence of chloramine-T (100 μg). The radiotracer [131 I]-SSD was observed to be stable in normal saline and freshly eluted serum up to 12 h at ambient temperature (37°C ± 2°C). The radiotracer [131 I]-SSD showed the highest uptake in the targeted organ (i.e. ulcerative colon) which was observed to be ≥ 75% injected dose per gram (% ID/g) organ for 24 h post-injection (p.i). Furthermore, in silico data collected from molecular modeling analysis of SSD and [131 I]-SSD with antimicrobial protein (PDB code: 3KEG) and peroxisome proliferator-activated receptor-gamma (PPARγ) (PDB code: 4XTA) showed azoreductase activity and high binding potential for PPAR-γ site, respectively. The results of biological studies obtained in this study enlighten the usefulness of radiotracer [131 I]-SSD as a potential imaging agent for ulcerative colitis. PMID:34314572 | DOI:10.1111/cbdd.13929 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Prevalence of Hashimoto Thyroiditis in Adults With Papillary Thyroid Cancer and Its Association With Cancer Recurrence and Outcomes

xlomafota13 shared this article with you from Inoreader Prevalence of Hashimoto Thyroiditis in Adults With Papillary Thyroid Cancer and Its Association With Cancer Recurrence and Outcomes Via radioactive iodine JAMA Netw Open. 2021 Jul 1;4(7):e2118526. doi: 10.1001/jamanetworkopen.2021.18526. ABSTRACT IMPORTANCE: Hashimoto thyroiditis (HT) has been suggested to be associated with papillary thyroid cancer (PTC) development. However, its association with PTC progression remains unclear. OBJECTIVE: To examine the association between HT and PTC presentation and outcomes. DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study included a review of patients aged 18 to 75 years who had pathologically confirmed PTC treated at a single center in China from January 1, 2001, to December 31, 2014. Data analysis was performed from November 1 to December 31, 2020. EXPOSURES: Coexistent HT was defined according to evaluation of postoperative paraffin sections. MAIN OUTCOMES AND MEASURES: The primary outcome was the association of HT with PTC-related mortality, assessed using Cox proportional hazards regression models. The secondary outcome was the association of HT with aggressive characteristics and structural recurrence of PTC, assessed using logistic regression and Cox proportional hazards regression with and without adjustment for related factors. RESULTS: Of 9210 patients with PTC (mean [SD] age, 43.6 [12.0] years; 6872 [75%] women) included in the analysis, 1751 (19%) had HT. In the logistic regression model, HT was negatively associated with frequencies of primary tumor size of 4 cm or greater (adjusted odds ratio [aOR], 0.20; 95% CI , 0.12-0.33; P < .001), gross extrathyroidal extension (aOR, 0.44; 95% CI, 0.36-0.54; P < .001), extranodal extension (aOR, 0.66; 95% CI, 0.55-0.80; P < .001), and distant metastasis (aOR, 0.17; 95% CI, 0.04-0.71; P = .02). After a median follow-up of 85 months (range, 12-144 months), 131 PTC-related deaths were identified in the cohort; 2 patients who died had HT. Patients with HT had significantly superior outcomes compared with patients without HT in terms of unadjusted 10-year disease-specific survival (99.9% vs 96.6%; log-rank P < .001) and recurrence-free survival (92.0% vs 87.6%; log-rank P = .001). After adjusting for sex, age, primary tumor size, extrathyroidal extension, lymph node metastasis, distant metastasis, extent of surgery, and radioactive iodine ablation, HT was associated with decreased PTC-related mortality (hazard ratio [HR], 0.19; 95% CI, 0.05-0.76; P = .02). Stratified analysis showed that HT was associated with less frequent structural recurrence in patients with extrathyroidal extension (HR, 0.52; 95% CI, 0.38-0.71; P < .001; P = .002 for interaction) or after total thyroidectomy (HR, 0.50; 95% CI, 0.35-0.69; P < .001; P = .009 for interaction). CONCLUSIONS AND RELEVANCE: In this cohort study, patients with coexistent HT had less aggressive characteristics at presentation and better outcomes of PTC than did patients without HT. The findings suggest that autoimmune thyroiditis has a protective role in association with thyroid cancer. PMID:34313737 | DOI:10.1001/jamanetworkopen.2021.18526 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Designable Assembly of Aluminum Molecular Rings for Sequential Confinement of Iodine Molecules

xlomafota13 shared this article with you from Inoreader Designable Assembly of Aluminum Molecular Rings for Sequential Confinement of Iodine Molecules Via radioactive iodine Angew Chem Int Ed Engl. 2021 Jul 27. doi: 10.1002/anie.202107227. Online ahead of print. ABSTRACT Although numerous adsorbent materials have been reported for radioactive iodine capture, demands for the development of new absorbents that are economically viable and featured with reliable synthetic protocols still exist. Herein, in this paper, a coordination-driven self-assembly strategy towards adsorbents for sequential confinement of iodine molecules is reported. It should be noti ced that these adsorbents are versatile heterometallic frameworks constructed from aluminum molecular rings of varying size, flexible copper ions and conjugated carboxylate ligands. Additionally, these materials can quickly remove iodine from cyclohexane solutions with a high removal rate (98.8%) and considerable loading capacity (555.06 mg/g). Heterometallic frameworks provided distinct pore sizes and binding sites toward iodine molecules and realize crystallographic visualization concerning sequential confinement of iodine molecules. This work not only sets up a bridge between molecular rings and infinite porous networks but also reveals molecular details for the underlying host-guest binding interactions at crystallographic resolution. PMID:34314080 | DOI:10.1002/anie.202107227 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Ectopic opening of the common bile duct into the duodenal bulb with recurrent choledocholithiasis: A case report

paythelady.612 shared this article with you from Inoreader Ectopic opening of the common bile duct into the duodenal bulb with recurrent choledocholithiasis: A case report Μέσω pubmed: "world j clin cases"... World J Clin Cases. 2021 Jul 6;9(19):5332-5338. doi: 10.12998/wjcc.v9.i19.5332. ABSTRACT BACKGROUND: Ectopic opening of the common bile duct is a condition with low incidence. Patients with an ectopic common bile duct opening have a high incidence of common bile duct stones and acute cholangitis. Patients with atypical symptoms and imaging findings are easily misdiagnosed; moreover, it is difficult to retrieve stones by endoscopic retrograde cholangiopancreatography, and common bile duct stones are prone to postsurgical recurrence. CASE SUMMARY: A 45-year-old male patient presented with "intermittent upper abdominal pain and elevated liver enzymes for 1 wk". Transabdominal ultrasound indicated dilation of the common bile duct and the presence of stones. Magnetic resonance imaging showed that the common bile duct was dilated with stones and that its opening was ectopic. Endoscopic retrograde cholangiopancreatography revealed an ab normal opening of the common bile duct into the duodenal bulb and the presence of common bile duct stones. Laparoscopic extrahepatic choledochectomy and hepatoenteric anastomosis were performed. After surgery, the patient recovered well and was discharged. The patient has been followed up for 2 years since the operation. He has not experienced stone recurrence, and his liver function and quality of life are good. CONCLUSION: Improved understanding of ectopic opening of the common bile duct is needed for clinicians to provide patients with appropriate treatment. PMID:34307586 | PMC:PMC8283612 | DOI:10.12998/wjcc.v9.i19.5332 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Hemoglobin Fukuoka caused unexpected hemoglobin A1c results: A case report

paythelady.612 shared this article with you from Inoreader Hemoglobin Fukuoka caused unexpected hemoglobin A<sub>1c</sub> results: A case report Μέσω pubmed: "world j clin cases"... World J Clin Cases. 2021 Jul 16;9(20):5568-5574. doi: 10.12998/wjcc.v9.i20.5568. ABSTRACT BACKGROUND: Glycated hemoglobin (Hb) (HbA1c) is an indicator that is used to diagnose and monitor the treatment of diabetes. Many factors can affect the detection of HbA1c. One of the most important of these factors is the Hb variant. Here, we report a rare Hb variant and evaluate its effect on HbA1c. CASE SUMMARY: A 35-year-old man was suspected of harboring an Hb variant following the measurement of HbA1c with the Variant II Turbo 2.0 Hb detection system during a routine examination. Subsequently, we used the Arkray HA-8160 and ARCHITECT c4000 system to reanalyze HbA1c. Finally, the Hb variant was detected with a Capillary2FP analyzer that operates on the principle of capillary electrophoresis. We also used gene sequencing to investigate the mutation site. The value of HbA1c detected with the Variant II Turbo 2.0 system was 52.7%. However, the Arkray HA-8160 system did not display a result while the ARCHITECT c16000 system showed a result of 5.4%. The Capillary2FP analyzer did not reveal any abnormal Hb zones. However, gene sequencing identified the presence of a mutation in the Hb β2 chain [CD2(CAC>TAC), His>Tyr, HBB: c.7C>T]; the genotype was Hb Fukuoka. CONCLUSION: Hb variants could cause abnormal HbA1c results. For patients with Hb variants, different methods should be used to detect HbA1c. PMID:34307611 | PMC:PMC8281434 | DOI:10.12998/wjcc.v9.i20.5568 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Brugada syndrome associated with out-of-hospital cardiac arrest: A case report

paythelady.612 shared this article with you from Inoreader Brugada syndrome associated with out-of-hospital cardiac arrest: A case report Μέσω pubmed: "world j clin cases"... World J Clin Cases. 2021 Jul 6;9(19):5345-5351. doi: 10.12998/wjcc.v9.i19.5345. ABSTRACT BACKGROUND: Brugada syndrome (BrS) is an inherited disease characterized by an electrocardiogram (ECG) with a coved-type ST-segment elevation in the right precordial leads (V1-V3), which predisposes to sudden cardiac death (SCD) due to polymorphic ventricular tachycardia or ventricular fibrillation in the absence of structural heart disease. We report the case of a 29-year-old man with out-of-hospital cardiac arrest. BrS is associated with a high incidence of SCD in adults, and increasing the awareness of BrS and prompt recognition of the Brugada ECG pattern can be lifesaving. CASE SUMMARY: A 29-year-old man suffered from out-of-hospital cardiac arrest, and after defibrillation, his ECG demonstrated a coved-type elevated ST segment in V1 and V2. These findings were compatible with type 1 Brugada pattern, and ECG of his brother showed a type 2 Brugada pattern. The diagnosis was BrS, NYHF IV, multiple organ dysfunction syndrome, sepsis, and hypoxic ischemic encephalopathy. The patient had no arrhythmia episodes after discharge throughout a follow-up period of 36 mo. CONCLUSION: Increasing awareness of BrS and prompt recognition of the Brugada ECG pattern can be lifesaving. PMID:34307588 | PMC:PMC8283591 | DOI:10.12998/wjcc.v9.i19.5345 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Giant androgen-producing adrenocortical carcinoma with atrial flutter: A case report and review of the literature

paythelady.612 shared this article with you from Inoreader Giant androgen-producing adrenocortical carcinoma with atrial flutter: A case report and review of the literature Μέσω pubmed: "world j clin cases"... World J Clin Cases. 2021 Jul 16;9(20):5575-5587. doi: 10.12998/wjcc.v9.i20.5575. ABSTRACT BACKGROUND: Adrenocortical carcinoma (ACC), the second most aggressive malignant tumor, lacks epidemiological data worldwide; therefore, every new case can improve the understanding of the pathology and treatment of this malignancy. CASE SUMMARY: We present the case of a 66-year-old Caucasian woman with a giant androgen-producing ACC (21 cm × 17 cm × 12 cm; 2100 g), without metastases, which unusually presented with an acute onset of atrial flutter and congestive heart failure. The cardiac complications observed in our case support the hypothesis that androgen excess in women is a cardiovascular risk factor. Androgen excess in women can be a rare cause of reversible dilated cardiomyopathy, therefore a comprehensive approach to the patient is essential to improve the recognition of androgen-secreting ACC. The atrial flutter was remitted aft er initiation of drug treatment during admission. The severe heart failure was totally remitted at 6 mo after radical open surgery to remove the giant ACC. CONCLUSION: Radical open surgery to remove a giant androgen-producing ACC was the first-line treatment to cure the excess of androgen, which determined the total remission of cardiac complications at 6 mo after surgery in the women of this case report. PMID:34307612 | PMC:PMC8281402 | DOI:10.12998/wjcc.v9.i20.5575 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.