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.

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.