Dupilumab for STAT3-Hyper-IgE Syndrome With Refractory Intestinal Complication

xlomafota13 shared this article with you from Inoreader Dupilumab for STAT3-Hyper-IgE Syndrome With Refractory Intestinal Complication Via Traditional dysphagia therapy Pediatrics. 2021 Aug 20:e2021050351. doi: 10.1542/peds.2021-050351. Online ahead of print. ABSTRACT STAT3 hyper-immunoglobulin E syndrome (STAT3-HIES) is a rare primary immunodeficiency syndrome characterized by elevated serum immunoglobulin E levels, eczema, recurrent skin and respiratory tract infections, and several gastrointestinal (GI) problems. GI manifestations, such as gastroesophageal reflux disease, dysphagia, abdominal pain, gut dysmotility, bowel perforation, eosinophilic esophagitis, and diarrhea, have been reported in 60% of patients. Until now, there was no efficient treatment that could effectively manage all aspects of the syndrome. In this report, we present the case of a 21-year-old man who suffered from undetectable pathogenic refractory diarrhea that persisted >21 days despite aggressive antibiotic and steroid treatment since he was 2 years old. STAT3 Int10(-2)A > G splicing mutation-caused STAT 3-HIES was diagnosed by next-generation sequencing. The patient had suffered recurrent intestinal and colon perforations since he was 10 years old. He had received multiple surgeries and continuous systemic intravenous immunoglobulin therapy to manage his GI symptoms. However, refractory diarrhea occurring >5 to 6 times per day with severe eczematous dermatitis and frequent abscess formation remained threats to his life. Dupilumab 300 mg every 3 weeks was prescribed to control his skin problems, but the patient's diarrhea also completely subsided. As such, it appears that dupilumab may not only effectively treat the skin inflammation but also the GI manifestation-related inflammation of STAT3-HIES refractory to traditional immunomodulators. PMID:34417287 | DOI:10.1542/peds.2021-050351 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

'Treatment of En Coup de Sabre Deformity with Fat Grafting and Demineralized Bone Matrix: A Case Series'

xlomafota13 shared this article with you from Inoreader "Treatment of En Coup de Sabre Deformity with Fat Grafting and Demineralized Bone Matrix: A Case Series" Via Journal of plastic, reconstructive & aesthetic surgery : JPRAS J Plast Reconstr Aesthet Surg. 2021 Jun 7:S1748-6815(21)00290-4. doi: 10.1016/j.bjps.2021.05.038. Online ahead of print. ABSTRACT En coup de sabre deformity (ECDS) is a form of localized scleroderma in the frontoparietal region caused by progressive subcutaneous tissue atrophy and bony defect. Although ECDS involves two layers, skin/subcutaneous tissue and bone, the existing literature mainly focuses only on treating the skin/subcutaneous tissue layer. In this case series, we aimed to propose a novel approach that includes the combined use of fat grafting and demineralized bone matrix (DBM). Four patients with ECDS deformity, operated between February 2016 and October 2018, were retrospectively evaluated. All the patients were treated with the novel approach. Patients were evaluated with localized scleroderma scale and computed tomography (CT) scan in the preoperative period and at the annual follow-up. We observed remarkable improveme nt in the localized scleroderma scale including appearance, palpation, and size scores in all patients at the annual follow-up. CT scans at the annual follow-up revealed new callus formation at the bony defect area in all patients. Reinforcing fat grafting with DBM could promote healing of the bony and skin/subcutaneous tissue defects associated with ECDS. PMID:34417126 | DOI:10.1016/j.bjps.2021.05.038 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Dural sac shrinkage signs on magnetic resonance imaging at the thoracic level in spontaneous intracranial hypotension-its clinical significance

xlomafota13 shared this article with you from Inoreader Dural sac shrinkage signs on magnetic resonance imaging at the thoracic level in spontaneous intracranial hypotension-its clinical significance Via leak of cerebrospinal fluid Acta Neurochir (Wien). 2021 Aug 20. doi: 10.1007/s00701-021-04933-w. Online ahead of print. ABSTRACT BACKGROUND: Spontaneous intracranial hypotension (SIH) is secondary to a cerebrospinal fluid leak at the spinal level without obvious causative events. Several signs on brain and cervical spine magnetic resonance (MR) imaging (MRI) have been associated with SIH but can be equivocal or negative. This retrospective study sought to identify characteristic SIH signs on thoracic spinal MRI. METHODS: Cranial and spinal MR images of 27 consecutive patients with classic SIH symptoms, who eventually received epidural autologous blood patches (EBPs), were analyzed. RESULTS: The most prevalent findings on T2-weighted MRI at the thoracic level were anterior shift of the spinal cord (96.3%) and dorsal dura mater (81.5%), probably caused by dural sac shrinkage. These dural sac shrinkage signs (DSSS) were frequently accompanied by cerebrospinal f luid collection in the posterior epidural space (77.8%) and a prominent epidural venous plexus (77.8%). These findings disappeared in all six patients who underwent post-EBP spinal MRI. Dural enhancement and brain sagging were minimum or absent on the cranial MR images of seven patients, although DSSS were obvious in these seven patients. For 23 patients with SIH and 28 healthy volunteers, a diagnostic test using thoracic MRI was performed by 13 experts to validate the usefulness of DSSS. The median sensitivity, specificity, positive-predictive value, negative-predictive value, and accuracy of the DSSS were high (range, 0.913-0.931). CONCLUSIONS: Detection of DSSS on thoracic MRI facilitates an SIH diagnosis without the use of invasive imaging modalities. The DSSS were positive even in patients in whom classic cranial MRI signs for SIH were equivocal or minimal. PMID:34415442 | DOI:10.1007/s00701-021-04933-w View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Cricothyroid Muscle Dysfunction Affects Aerodynamic Performance in Patients with Unilateral Vocal Fold Paralysis

xlomafota13 shared this article with you from Inoreader Cricothyroid Muscle Dysfunction Affects Aerodynamic Performance in Patients with Unilateral Vocal Fold Paralysis Via Voice Publication date: Available online 20 August 2021 Source: Journal of Voice Author(s): Kuo-Cheng Liu, Yi-An Lu, Li-Ang Lee, Hsueh-Yu Li, Alice MK Wong, Yu-Cheng Pei, Tuan-Jen Fang View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

[18F]FDG-PET/CT in prone compared to supine position for optimal axillary staging and treatment in clinically node-positive breast cancer patients with neoadjuvant systemic therapy

xlomafota13 shared this article with you from Inoreader [<sup>18</sup>F]FDG-PET/CT in prone compared to supine position for optimal axillary staging and treatment in clinically node-positive breast cancer patients with neoadjuvant systemic therapy Via radioactive iodine EJNMMI Res. 2021 Aug 21;11(1):78. doi: 10.1186/s13550-021-00824-4. ABSTRACT PURPOSE: Axillary staging before neoadjuvant systemic therapy in clinically node-positive breast cancer patients with tailored axillary treatment according to the Marking Axillary lymph nodes with radioactive iodine seeds (MARI)-protocol, a protocol developed at the Netherlands Cancer Institute, is performed with [18F] fluorodeoxyglucose (FDG) positron emission tomography and computed tomography (PET/CT). We aimed to assess the value of FDG-PET/CT in prone compared to standard supine position for axillary staging. METHODS: We selected patients with FDG-PET/CT in supine and prone position who underwent the MARI-protocol. One hour after administration of 3.5 MBq/kg, [18F]FDG-PET was performed with a low-dose prone position CT-thorax followed by a supine whole-body scan. Scans were separately reviewed by two nuclear medicine physicians and categorized by number of FDG-positive axillary lymph nodes (ALNs; cALN<4 or cALN≥4). Main outcome was axillary up- or downstaging. RESULTS: Of 153 patients included, 24 (16%) patients were up- or downstaged at evaluation of prone images: One observer upstaged 14 patients, downstaged 3 patients and reported a higher number of ALNs (3.6 vs. 3.2, p < 0.001), while staging (4 up- and 5 downstaged) and number of ALNs (2.8 vs. 2.8) did not differ for the other. Observers agreed on up- or downstaging in only 1 (1%) pa tient. Irrespective of supine or prone position scanning, observers agreed on axillary staging in 124 (81%) patients and disagreed in 5 (3%). Interobserver agreement was lower with prone assessments (86%, K = 0.67) than supine (92%, K = 0.80). CONCLUSIONS: Axillary staging with FDG-PET/CT in prone compared to supine position did not result in concordant up- or downstaging. Therefore, FDG-PET/CT in supine position only can be considered sufficient for axillary staging. PMID:34417932 | DOI:10.1186/s13550-021-00824-4 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Organ Preservation Strategies in Laryngeal Chondrosarcoma

xlomafota13 shared this article with you from Inoreader Organ Preservation Strategies in Laryngeal Chondrosarcoma Via The Laryngoscope Objectives/Hypothesis Laryngeal chondrosarcoma (LC) is a rare, slowly growing malignancy. The preferred treatment is laryngeal preservation surgery (LPS). Some patients may require multiple interventions or total laryngectomy (TL). We investigated risk factors for retreatment and TL, and assessed the impact of LPS on oncological and functional outcomes. Study Design Case series Methods We searched our institution database for LC. Tumor grading, localization, and margin status were tested as predictors of recurrence and organ preservation. Results We included 21 patients (seven females, mean age 58 ± 12 years). LPS was applied in 20 (95.2%) of them as a primary procedure. Six patients were treated by transoral approach and 14 received "open-neck" LPS. Fifteen (71.4%) were operated only once, while six patients underwent a total of 15 adjunctive procedures. Additional operations were always performed for recurrence of tumors localized within the cricoid plate. The histological grading was G1 in 81% and G2 in 19%. However, two patients with a primary G1 LC showed a G2 recurrence. Reoperations for recurrence were more frequent among patients with G2 in respect to G1 histology (83% vs. 7%, P < .001). Fifty percent of G2 LC and 8% of G1 underwent TL (P < .05). Margin status had no influence on recurrence rate. Conclusions Patients with G2 LC have more recurrences requiring surgery and a higher incidence of TL. Cricoid plate localization is relevant for organ preservation. Margin status signals possible disease persistence, without influencing the need for future surgeries. Need for reoperation entails a risk of not being able to maintain organ functionality. Level of Evidence 4 Laryngoscope, 2021 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Influence of high tissue-absorbed dose on anti-thyroid antibodies in radioiodine therapy of Graves' disease patients

xlomafota13 shared this article with you from Inoreader Influence of high tissue-absorbed dose on anti-thyroid antibodies in radioiodine therapy of Graves' disease patients Via radioactive iodine Adv Clin Exp Med. 2021 Aug 19. doi: 10.17219/acem/137779. Online ahead of print. ABSTRACT BACKGROUND: The results of radioactive iodine (RAI) treatment for Graves' disease (GD) are related to the choice of diagnostic and dosimetry protocols, the steroid protection used, and the subsequent 131I dose. The effect of a high tissue-absorbed dose on the level of anti-thyroid antibodies (ATA) has been rarely considered. OBJECTIVES: To estimate the effect of the first RAI therapy wit h a dose of 250 Gy on anti-thyreoperoxidase (anti-TPO) and anti-thyroid-stimulating hormone (TSH) receptor thyrotropin receptor antibody - TRAb levels in GD patients. MATERIAL AND METHODS: The analysis encompassed 46 consecutive patients with clinical presentation of GD. We examined the serum levels of TSH, free thyroxine (FT4), anti-TPO, TRAb, thyroid volume (ThV), 131I effective half-life (EHL), introduction of steroid protection, levothyroxine dose used in thyroid replacement therapy - TRT, and effectiveness of treatment. RESULTS: As a result of RAI treatment, hypothyroidism was found in 35 patients (76.1%), euthyroidism in 7 patients (15.2%) and hyperthyroidism in 4 patients (8.7%). After RAI, we observed ThV reduction and increased anti-TPO (p = 0.001 and p = 0.001, respectively). It was found that a shorter EHL correlated with a higher baseline TRAb concentration and lower final anti-TPO serum concentration (p = 0.03 and p = 0.01, respectively). Lower final TRAb wa s found in patients with steroid protection (p = 0.049). Intergroup comparison of patients without steroid protection showed significantly higher final anti-TPO concentation (p = 0.02). Intergroup comparison of patients with TRT revealed significantly higher final anti-TPO concentration (p = 0.04). CONCLUSIONS: The application of a high absorbed dose of 250 Gy in GD resulted in high efficacy of RAI therapy at 1-year follow-up. An increased ATA level and its relationships with EHL and ThV reduction were observed at 1-year follow-up. There is a possible relationship between steroid protection and anti-TPO concentration. PMID:34418335 | DOI:10.17219/acem/137779 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Development and validation of an improved classification and risk stratification system for carotid body tumors: A multinational collaborative cohort study

xlomafota13 shared this article with you from Inoreader Development and validation of an improved classification and risk stratification system for carotid body tumors: A multinational collaborative cohort study Via Head & Neck Abstract Background This study aims to develop and validate a new classification system that better predicts combined risk of neurological and neurovascular complications following CBT surgery, crucial for treatment decision-making. Methods Multinational retrospective cohort study with 199 consecutive cases. A cohort of 132 CBT cases was used to develop the new classification. To undertake external validation, assessment was made between the actual complication rate and predicted risk by the model on an independent cohort (n = 67). Results Univariate analyses showed statistically significant associations between developing a complication and the following factors: craniocaudal dimension, volume, Shamblin classification, and Mehanna types. In the multivariate prognostic model, only Mehanna type remained as a significant risk predictor. The risk of developing complications increases with increasing Mehanna type. Conclusions We have developed and then validated a new classification and risk stratification system for CBTs, which demonstrated better prognostic power for the risk of developing neurovascular complications after surgery. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Human orbital muscle in adult cadavers and near-term fetuses: its bony attachments and individual variation identified by immunohistochemistry

xlomafota13 shared this article with you from Inoreader Human orbital muscle in adult cadavers and near-term fetuses: its bony attachments and individual variation identified by immunohistochemistry Via radiol anat Surg Radiol Anat. 2021 Aug 21. doi: 10.1007/s00276-021-02819-1. Online ahead of print. ABSTRACT PURPOSE: To compare fetal and adult morphologies of the orbital muscle (OM) and to describe the detailed topographical anatomy in adults. METHODS: Using unilateral orbits from 15 near-term fetuses and 21 elderly cadavers, semiserial horizontal or sagittal paraffin sections were prepared at intervals of 20-100 µm. In addition to routine histology, we performed immunohistoche mistry for smooth muscle actin. RESULTS: At near term, the OM consistently extended widely from the zygomatic bone or the greater wing of the sphenoid to the maxilla or ethmoid. Thus, it was a large sheet covering the future inferior orbital fissure. In contrast, the adult OM was a thin and small muscle bundle connecting (1) the greater wing of the sphenoid to the maxilla (11/19 cadavers), (2) the lesser wing of the sphenoid to the maxilla (5/19) or the greater wing (3/19). The small OM was likely to be restricted within the greater wing (5/19 cadavers) or the maxilla (3/19). Two of these five types of OM coexisted in eight orbits. OM attachment to the lesser wing was not seen in fetuses, whereas ethmoid attachment was absent in adults. CONCLUSIONS: The lesser wing attachment of the OM seemed to establish after birth. A growing common origin of the three recti was likely involved in "stealing" the near-term OM attachment from the ethmoid. The strong immunoreactivity of r emnant-like OM in the elderly suggests that OM contraction is still likely to occur against the increased flow through a thin vein. However, the contraction might have no clinical significance. PMID:34417852 | DOI:10.1007/s00276-021-02819-1 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Expected Costs of Primary Dental Treatments and Endoscopic Sinus Surgery for Odontogenic Sinusitis

xlomafota13 shared this article with you from Inoreader Expected Costs of Primary Dental Treatments and Endoscopic Sinus Surgery for Odontogenic Sinusitis Via The Laryngoscope Objectives Treatment of odontogenic sinusitis (ODS) due to apical periodontitis (AP) is highly successful when both dental treatment and endoscopic sinus surgery (ESS) are performed. Variation exists in the literature with regard to types and timing of dental treatments and ESS when managing ODS. This study modeled expected costs of different primary dental and sinus surgical treatment pathways for ODS due to AP. Study Design Decision-tree economic model. Methods Decision-tree models were created based on cost and treatment success probabilities. Using Medicare and consumer online databases, cost data were obtained for the following dental and sinus surgical treatments across the United States: root canal therapy (RCTx), revision RCTx, apicoectomy, extraction, dental implant, bone graft, and ESS (maxillary, ± anterior ethmoid, ± frontal). A literature review was performed to determine probabilities of dental and sinus disease resolution after different dental treatments. Expected costs were determined for primary dental extraction, and ESS pathways, and sensitivity analyses were performed. Results Expected costs for the three different primary treatment pathways when dental care was in-network and all diseased sinuses opened during ESS were as follows: dental extraction ($4,753.83), RCTx ($4,677.34), and ESS ($7,319.85). Conclusions ODS due to AP can be successfully treated with primary dental treatments, but ESS is still frequently required. Expected costs of primary dental extraction and RCTx were roughly equal. Primary ESS had a higher expected cost, but may still be preferred in patients with prominent sinonasal symptoms. Patients' insurance coverage may also impact decision-making. Level of Evidence N/A Laryngoscope, 2021 View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.

Hidradenitis suppurativa: a folliculotropic disease of innate immune barrier dysfunction?

xlomafota13 shared this article with you from Inoreader Hidradenitis suppurativa: a folliculotropic disease of innate immune barrier dysfunction? Via Experimental Dermatology Abstract The innate immune system of human skin consists of a multi-layered barrier consisting of cells and soluble effector molecules charged with maintaining homeostasis and responding to insults and infections. It has become increasingly clear that these barrier layers become compromised in skin diseases, especially in disorders of an (auto)inflammatory nature. In the case of hidradenitis suppurativa, great strides have been made in recent years in characterising the underlying breakdown in homeostatic innate immunity, including an increasing understanding of the central role of the hair follicle in this process. This breakdown appears to occur at multiple levels: the pilosebaceous unit, associated epithelium, the cutaneous microbiome, alteration of immune cell function, and local molecular events such as complement activation. This review seeks to summarize, contextualize and analyse critically our current understanding of how these innate immune barriers become dysregulated in the ear ly stage(s) of hidradenitis suppurativa, and to speculate on where potential hidradenitis suppurativa research could be most fruitful. View on the web Inoreader. Take back control of your news feed. Follow us on Twitter and Facebook.