Τρίτη 2 Μαρτίου 2021

Venetoclax in combination with chidamide and dexamethasone in relapsed/refractory primary plasma cell leukemia without t(11;14): A case report

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World J Clin Cases. 2021 Feb 16;9(5):1175-1183. doi: 10.12998/wjcc.v9.i5.1175.

ABSTRACT

BACKGROUND: Conventional therapies for primary plasma cell leukemia (pPCL) are usually ineffective, with a short remission time with the use of multiple myeloma medications, showing aggressiveness of pPCL. B-cell lymphoma-2 inhibitor venetoclax is usually used for relapsed/refractory multiple myeloma (RRMM) with t(11;14). There are very few studies published on the use of venetoclax in pPCL without t(11;14). Similarly, histone deacetylase inhibitors are considered effective for the treatment of RRMM, but there are no reports on their use in pPCL.

CASE SUMMARY: A 57-year-old woman with severe anemia, thrombocytopenia, multiple bone destruction, impaired renal function, and 42.7% of peripheral plasma cells is reported. After multiple chemotherapy regimens and chimeric antigen receptor T-cell treatment, the disease progressed again. The patient h ad very good partial response and was maintained for a long time on venetoclax in combination with chidamide and dexamethasone therapy.

CONCLUSION: The success of venetoclax-chidamide-dexamethasone combination therapy in achieving a very good partial response suggested that it can be used for refractory/relapsed pPCL patients who have been exhausted with the use of various drug combinations and had poor survival outcomes.

PMID:33644182 | PMC: PMC7896656 | DOI:10.12998/wjcc.v9.i5.1175

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Polidocanol sclerotherapy for multiple gastrointestinal hemangiomas: A case report

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World J Clin Cases. 2021 Feb 26;9(6):1483-1489. doi: 10.12998/wjcc.v9.i6.1483.

ABSTRACT

BACKGROUND: Gastrointestinal (GI) hemangioma has a low incidence among systemic hemangiomas, and some GI hemangiomas occur in the intestine, stomach, and esophagus. Polidocanol has been increasingly used in sclerotherapy. However, this paper reports that minimally invasive treatment of multiple hemangiomas with large diameters can achieve satisfactory results by multipoint injection.

CASE SUMMARY: A 46-year-old female patient was hospitalized in another hospital for cough. We accidentally found thickening of the lower esophagus by chest computed tomography. The patient was eventually diagnosed with multiple GI hemangiomas and underwent a series of examinations including esophagogastroduodenoscopy (EGD), endoscopic ultrasound, and magnetic resonance imaging. We calculated the dose of polidocanol according to the volumes of the hemangiomas, fixe d the target vein with the help of a transparent cap, and then administered polidocanol via multipoint injection into the hemangiomas under endoscopic guidance. EGD and endoscopic ultrasound showed that the hemangiomas disappeared. The color of the esophageal mucosa returned to normal 1 mo after sclerotherapy.

CONCLUSION: Sclerotherapy may be a safe and effective method for treating multiple hemangiomas of the alimentary canal.

PMID:33644219 | PMC:PMC7896683 | DOI:10.12998/wjcc.v9.i6.1483

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Imaging characteristics of a rare case of monostotic fibrous dysplasia of the sacrum: A case report

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World J Clin Cases. 2021 Feb 16;9(5):1111-1118. doi: 10.12998/wjcc.v9.i5.1111.

ABSTRACT

BACKGROUND: Fibrous dysplasia (FD) is a common benign intramedullary fibro-osseous lesion. Involvement of the spine is rare, with the literature including only case reports, and cases of monostotic FD (MFD) in the sacrum are extremely rare. A correct preoperative diagnosis of spinal MFD is important for clinicians to select proper treatment.

CASE SUMMARY: We retrospectively assessed a case report of MFD in the sacrum. This patient was examined by computed tomography (CT) and magnetic resonance imaging (MRI), and the diagnosis was confirmed by pathology. A review of the literature was performed to analyze the imaging characteristics and differential diagnoses of spinal MFD. For our patient, the CT scan showed the lesion to be expansile, with ground glass opacity and a sclerotic rim. On MRI, the lesion showed iso-low signal intensity on T1WI and iso-high signal intensity on T2WI. A low signal rim was found on T1WI and T2WI. Our patient was treated by posterior focal excision, decompression, bone grafting, fusion and pedicle screw fixation. A satisfactory result was achieved, with pain disappearance. No complications had occurred at the 1-year follow up.

CONCLUSION: MFD is an expansile osteolytic change. Ground glass opacity and a sclerotic margin are obvious characteristics. The lesion often involves the vertebral body and posterior element. Knowledge of these imaging characteristics of spinal FD could be helpful for diagnosis and prevent unnecessary procedures.

PMID:33644174 | PMC:PMC7896654 | DOI:10.12998/wjcc.v9.i5.1111

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Bilateral musculocutaneous neuropathy: A case report

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World J Clin Cases. 2021 Feb 16;9(5):1237-1246. doi: 10.12998/wjcc.v9.i5.1237.

ABSTRACT

BACKGROUND: Isolated musculocutaneous nerve injury is a rare condition. Herein, we report the first case of bilateral musculocutaneous neuropathy after vigorous stretching of both upper extremities with normal results of sensory nerve action potential. Clinicians should be aware of this rare condition that can appear bilaterally. In addition, the interpretation of the aberrant electrodiagnostic study results of this case was discussed.

CASE SUMMARY: A 29-year-old male complaining of bilateral forearm tingling and upper extremity weakness visited the outpatient clinic. The symptoms began 6 mo prior, and he visited another hospital before visiting our department. The diagnosis was not made even after cervical spine magnetic resonance imaging, electrodiagnostic study, brain magnetic resonance imaging, and arteriography were conducted. The patient performed unique exercises that stretched the pectoralis minor and coracobrachialis muscles. On the follow-up electrodiagnostic study, abnormal spontaneous activities in the bilateral biceps and brachialis muscles were observed. The patient was diagnosed with bilateral musculocutaneous neuropathy. Steroid pulse therapy was administered for approximately 6 wk. After treatment, his muscle strength returned to the predisease condition.

CONCLUSION: Clinicians should be aware of this condition, have adequate understanding of anatomy, and advise to correct inappropriate exercises.

PMID:33644190 | PMC:PMC7896669 | DOI:10.12998/wjcc.v9.i5.1237

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Δευτέρα 1 Μαρτίου 2021

Defective expression of the peroxisome regulators PPARα receptors and lysogenesis with increased cellular senescence in the venous wall of chronic venous disorder

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Histol Histopathol. 2021 Mar 1:18322. doi: 10.14670/HH-18-322. Online ahead of print.

ABSTRACT

The pathogenesis of chronic venous disorder (CVeD) remains partially understood. A marked wall remodeling has been shown with potential accelerated tissue senescence. We have investigated the expression of peroxisome proliferator-activated receptor (PPAR) isoforms transcription factor EB (TFEB) as regulatory molecules of cellular homeostasis and makers of peroxisomal and lysosomal biogenesis. We have also quantified p16 expression as a cellular senescence marker. In specimens of maior safena vein from 35 CVeD and 27 healthy venous controls (HV), we studied the expression of PPAR-α, PPAR-β/δ, PPAR-γ, TFEB and p16 by RT-qPCR and immunohistochemical techniques. We have demonstrated a reduced gene and protein expression of the PPAR-α and PPAR-β/δ isoform as well as that of TFEB in the venous wall of CVeD patients, suggesting an altered per oxisomal and lysosomal biogenesis associated with an increased cellular senescence shown by increased p16 expression.

PMID:33645625 | DOI:10.14670/HH-18-322

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Revision of cartilage tympanoplasty with endoscopic butterfly inlay myringoplasty

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Eur Arch Otorhinolaryngol. 2021 Mar 1. doi: 10.1007/s00405-021-06669-w. Online ahead of print.

ABSTRACT

PURPOSE: While the use of cartilage graft for revision tympanoplasty is quite common, the data on the revision of cartilage tympanoplasty are limited. Our study aims to present the results of our patients who underwent endoscopic butterfly inlay myringoplasty for the revision of cartilage tympanoplasty.

METHODS: Demographic data, ear sides, perforation locations, graft success rates and hearing results of the patients who underwent endoscopic butterfly inlay myringoplasty for revision of cartilage tympanoplasty between Sep 2017 and Jan 2020 were evaluated.

RESULTS: A total of 17 patients with 7 (41%) females and 10 (59%) males were included in our study. Eight of the ears (47%) were right and nine (53%) were left. Perforation locations included ten (59%) anterior, six (35%) inferior, and one (6%) posterior. The graft succ ess rate of our study was 100%. No changes were observed in post-operative air conduction, bone conduction and air-bone gap values compared to the pre-operative period (p > 0.05).

CONCLUSIONS: Endoscopic butterfly inlay myringoplasty for the revision of cartilage tympanoplasty is considered to be a safe surgical technique with satisfactory anatomical and hearing results.

PMID:33646345 | DOI:10.1007/s00405-021-06669-w

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Survival outcomes of patients with subglottic squamous cell carcinoma : a study of the National Cancer Database

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Eur Arch Otorhinolaryngol. 2021 Mar 1. doi: 10.1007/s00405-021-06712-w. Online ahead of print.

ABSTRACT

BACKGROUND: Subglottic squamous cell carcinoma (SCC) represents less than 5% of all laryngeal cancers. Our objective was to better characterize survival using the National Cancer Database (NCDB) registry from 2004 to 2015.

RESULTS: 403 patients met inclusion criteria. 63.8% presented with advanced-stage disease. Treatment regimens were as follows: 15.9% underwent surgery alone, 16.9% underwent surgery followed by adjuvant therapy, and 67.2% underwent primary chemo/radiation (C/RT). Five-year overall survival (OS) was 58.6% for Stage I and II patients, 49.1% for Stage III, and 36.3% for stage IV. Adjusted OS for all-stage patients was worse with C/RT compared to upfront surgery (40.6% vs. 58.4%; HR 1.83 [95%CI 1.29-2.61] p < 0.001) and adjusted OS for stage 4 disease was significantly worse with C/RT compared to surgery (26.0 % vs. 45.2%, HR 1.79 [95%CI 1.17-2.73] p = 0.007).

CONCLUSION: Majority of patients were treated with primary C/RT. Adjusted survival favors upfront surgery versus C/RT, especially in patients with Stage IV disease.

PMID:33646344 | DOI:10.1007/s00405-021-06712-w

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Laser treatment of specific scar characteristics in hypertrophic scars and keloid: A systematic review

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J Plast Reconstr Aesthet Surg. 2021 Jan;74(1):48-64. doi: 10.1016/j.bjps.2020.08.108. Epub 2020 Sep 20.

ABSTRACT

BACKGROUND: Hypertrophic scarring and keloid can cause significant emotional and physical discomfort. Cosmetic appearance, functional limitations, pain and pruritus form a degree of impairment. While the etiology is not fully known, there is a wide array of treatment options, which include excision, radiation, cryotherapy, silicone gel sheeting, and intralesional injections. A relatively new modality is laser therapy. While results are promising, the number of different laser systems is substantial. This review evaluates the available evidence regarding outcomes on specific objective characteristics (i.e., erythema, pigmentation, height, and pliability) of the different laser systems.

METHODS: A systematic literature review was performed using MEDLINE, Cochrane Library, and EMBASE. Data on scar characteristics were ext racted from scar scales Vancouver Scar Scale (VSS) and Patient and Observer Scar Assessment Scale (POSAS), and from objective measurement tools.

RESULTS: Heterogeneity was seen in a lot of aspects: maturity of scar, origin of scar, follow-up, and number of treatments. The fractional ablative lasers CO2 10,600 nm and Er:YAG 2940 nm were found to produce the best results regarding erythema, height, and pliability, while the flash lamp-pumped pulsed dye laser (PDL) 585 nm scored slightly below that.

CONCLUSIONS: Laser systems, and specifically the fractional ablative lasers CO2 and Er:YAG, improved various characteristics of excessive scarring. An overview of preferred laser modality per scar characteristic is presented. Accounting for the methodological quality and the level of evidence of the data, future research in the form of randomized trials with comparable standardized scar scales is needed to confirm these results.

PMID:33645505 | DOI:10.1016/j.bjps.2020.08.108

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Voice Disorders Detection Through Multiband Cepstral Features of Sustained Vowel

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This study aims to detect voice disorders related to vocal fold nodule, Reinke's edema and neurological pathologies through multiband cepstral features of the sustained vowel /a/. Detection is performed between pairs of study groups and multiband analysis is accomplished using the wavelet transform. For each pair of groups, a parameters selection is carried out. Time series of the selected parameters are used as input for four classifiers with leave-one-out cross validation. Classification accuracies of 100% are achieved for all pairs including the control group, surpassing the state-of-art methods based on cepstral features, while accuracies higher than 88.50% are obtained for the pathological pairs.
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The German Restructured Vocal Fatigue Index and Characteristics of Dysphonic and Vocally Healthy Populations

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The Vocal Fatigue Index (VFI), a 19-item psychometric self-report questionnaire, enables individuals with vocal fatigue (VF) to be identified and their complaints to be characterized. The purpose of this study was to improve the German-language version (VFI-G) and to evaluate further vocal fatigue-related characteristics of dysphonic and control populations.
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Outcomes of tympanoplasty in a low resource setting: Our experience

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Abstract

Tympanoplasty outcomes are well established in high income nations, but less reported in low‐middle income countries (LMICs). LMICs see a higher prevalence of chronic suppurative otitis media, with anecdotal evidence of greater disease severity at presentation. Here we find that tympanoplasty performed by local surgeons in the LMIC of Cambodia has 85.8% successful closure, which is comparable to other settings. For audiological outcomes, there was a mean reduction in the air‐bone gap of 15.8dB, and improvement in the air‐conduction average in 85.5% of participants. We find no evidence that tympanoplasty outcomes in LMICs should differ from those in other settings.

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