Evaluation of preterm infants having bronchopulmonary dysplasia with echocardiography and serum biomarkers.

BACKGROUND AND OBJECTIVES: Pulmonary hypertension is frequent in infants with bronchopulmonary dysplasia. Echocardiography is easy to perform, non-invasive, and recommended by guidelines even though solely it is not enough. Catheterisation is gold standard but invasive, expensive, and not cost effective. Therefore, we aimed to assess to find out the role of biomarkers besides echocardiography in the diagnosis of pulmonary hypertension in preterm with bronchopulmonary dysplasia. METHODS: This study is done during the time period January 2016-2017. The diagnosis of pulmonary hypertension was assessed by echocardiography at 36 weeks later repeated at 3rd and 6th months. We also repeated biomarkers at 3rd and 6th months. The infants born ≤ 28 weeks in Erciyes University hospital who were diagnosed bronchopulmonary dysplasia were included. Infants with genetic syndromes, structural lung, and CHDs were excluded. Patients without bronchopulmonary dysplasia but having pulmonary hypertension due to other reasons and patients having echocardiograms without adequate images were excluded. RESULTS: At initial, 21/59 patients had bronchopulmonary dysplasia-pulmonary hypertension (Group 1), 21/59 had no bronchopulmonary dysplasia-pulmonary hypertension (Group 2), and 17/59 had bronchopulmonary dysplasia without pulmonary hypertension (Group 3). Systolic pulmonary artery pressure and pulmonary vascular resistance were found high in Group 1 (36 mmHg; p <0.001, 1.25 Woods Unit; p < 0.0017, respectively). Tricuspid annular plane systolic excursion values of Group 1 were low. Median serum kallistatin levels of Group 1 were lower than the other groups (230.5 (114.5-300.5) µg/ml; p < 0.005). During the study period, pulmonary hypertension of 14/21 bronchopulmonary dysplasia-pulmonary hypertension resolved, six patients in Group 3 developed pulmonary hypertension. However, there was no difference in the biomarkers of these six patients. CONCLUSION: In the diagnosis and the follow-up of pulmonary hypertension in bronchopulmonary dysplasia patients, besides echocardiography kallistatin, gelsolin, NT-probrain natriuretic peptide, homocysteine, and cystatin-C levels can be used. Further studies were required with large sample sizes.

Quality of life after radio frequency ablation turbinate reduction (RFATR) among patients with rhinitis medicamentosa & withdrawal from decongestant topical spray abuse.

OBJECTIVE: Radiofrequency ablation (RFA) reliefs nasal obstruction and improves quality of life (QoL) in patients suffering from inferior turbinate hypertrophy (ITH). A substantial benefit was noted among patients suffering from Rhinitis Medicamentosa (RM), enabling ending decongestant spray abuse. Our aim was to establish the benefit from RFA with respect to QoL in patients suffering from ITH, due to the presence of RM. STUDY DESIGN: Prospective cohort study. METHODS: Prospective Cohort study, including patients suffering from ITH undergoing RFA between 9.2017 and 9.2019 in Tel Aviv Medical Center. The cohort was divided to RM and non-RM (including allergic, non-allergic) patients. The differences between the groups were compared before and after RFA, and included patients' complaints, clinical findings and QoL questionnaires (SNOT-22 & NOSE). In the RM group, the ability to wean from decongestants was also described. RESULTS: Our data demonstrated subjective QoL improvement following RFA (88.9 %, N = 90). All RM patients withdrawaled from nasal decongestant spray. NOSE questionnaire demonstrated a significant improvement in QoL after RFA in the RM group (PV = 0.025). SNOT-22 did not demonstrate significant difference in QoL between RM and the reference group (PV = 0.1). Rates of MCID>8.3 were high, without significant difference between the groups (PV = 0.2). CONCLUSION: RFA demonstrated effectiveness in achieving of withdrawal from decongestant spray in RM patients and may be a possible definitive treatment option for this group. The nasal obstruction component in SNOT-22 questionnaire & NOSE questionnaire showed improved QoL in comparison to controls. High QoL after RFA was established in our entire cohort.

Adaptation and Validation of the Hebrew Version of the Nasal Obstruction Symptom Evaluation (NOSE) Scale.

BACKGROUND: Nasal obstruction is one of the most common complaints in the practice of rhinology. OBJECTIVES: To adapt the Nasal Obstruction Scale Evaluation (NOSE) questionnaire to Hebrew (H-NOSE) and to assess its sensitivity and specificity. METHODS: Candidates for surgical intervention due to isolated nasal obstruction and healthy volunteers (controls) were included in the validation. The English NOSE questionnaire was translated into Hebrew and re-translated for translation validity. Patients completed the H-NOSE questionnaire before and after surgery for nasal obstruction. The same questionnaire was completed by the controls. Test-retest reliability was performed within 2 weeks. Psychometric properties (reliability, reproducibility, validity, and responsiveness) were assessed by a test-retest procedure, internal consistency, correlation to the Hebrew Sino-Nasal Outcome Tool 22 (He-SNOT-22), and response sensitivity. RESULTS: In total, 179 patients with nasal obstruction and 74 controls completed the questionnaire. Cronbach's alpha score was 0.93 for internal consistency. The receiver operating characteristic curve demonstrated high sensitivity and specificity (< 90%) and area under the curve was 0.97. We found no significant difference in test-retest reliability. The difference between the pre- and postoperative questionnaire scores was highly significant (13.9 ± 4.0 vs. 3.2 ± 4.1, respectively, P < 0.001). CONCLUSIONS: The H-NOSE questionnaire demonstrated reliable internal consistency, sensitivity, specificity, and reliability. The Hebrew version differentiated between patients and heathy controls and was easy to administer. This instrument is useful for Hebrew speaking patients who undergo surgery for nasal obstruction.

Bell's palsy and pregnancy: Incidence, comorbidities and complications. A meta-analysis and systematic review of the literature.

OBJECTIVES: Data on the association between Bell's palsy (BP) and pregnancy is scarce and there is an ongoing debate regarding the association of BP and pregnancy. MAIN OUTCOME MEASURES: We aimed to investigate the prevalence of BP among pregnant patients and determine the frequency of pregnant women in BP cohorts and vice versa, assess which term of the pregnancy and peripartum bears a higher risk for BP occurrence, and determine the prevalence of maternal comorbidities associated with BP during pregnancy. DESIGN: Meta analysis. SETTINGS: Screening standard articles and extracting data from Ovid MEDLINE (1960-2021), Embase (1960-2021), and Web of Science (1960-2021). All study types were included except for case reports. MEASURES: Data were pooled by means of both fixed and random-effects models. RESULTS: The search strategy identified 147 records. Twenty-five of the studies that met our inclusion criteria described 809 pregnant patients with BP in a total of 11,813 BP patients and they were included in the meta-analysis. The incidence of BP among the pregnant patients was 0.05%; The incidence of pregnant patients among all BP patients was 6.62%. Most of the BP occurrences were during the third trimester (68.82%). The pooled incidence of gestational diabetes mellitus; hypertension; pre-eclampsia/eclampsia and fetal complications among the pregnant patients with BP was 6.3%, 13.97%, 9.54%, and 6.74%, respectively. CONCLUSIONS: This meta-analysis revealed a low incidence of BP during pregnancy. A Higher proportion occurred during the third trimester. The association of BP and pregnancy warrants further exploration.

Histopathological Characteristics of N1b Papillary Thyroid Carcinoma are Associated with Risk of Recurrence.

BACKGROUND: The presence of cervical lymph node (LN) metastasis at the initial presentation of papillary thyroid carcinoma (PTC) constitutes an independent risk factor for disease recurrence, increases the risk for mortality, and impacts overall outcome. The 2016 American Joint Committee on Cancer raised the age cutoff for PTC staging from 45 to 55 years for better prediction of overall survival. Age > 55 years is considered a significant risk factor for a more aggressive and advanced disease with worse outcomes. We identified histopathological factors for disease recurrence in PTC patients younger and older than 55 years of age. METHODS: Data on all patients who underwent thyroid surgery due to PTC between 2006 and 2018 in the Tel Aviv Sourasky Medical Center were retrieved for this retrospective cohort study. Patients with lymph node (LN) metastases were further investigated for preoperative presentation, pathological characteristics, and recurrence. A multivariate analysis was used to detect predictors for recurrence and patient outcome for each age-group. RESULTS: Twenty-two of the 183 patients (12%) with PTC who met the inclusion criteria and had sufficient follow-up period sustained recurrence. The predictors of recurrence varied between the two age-groups. The size of thyroid lesions (p = 0.003) was identified as a risk factor in the older group, while the number of metastatic cervical LNs (p = 0.001) and the ratio of metastatic-to-total dissected cervical LNs (p = 0.027) were the main predictors of recurrence for the younger group. CONCLUSION: The histopathological factors predictive for disease recurrence differed among PTC patients younger and older than 55 years of age.

Laryngectomy-free survival after salvage partial laryngectomy: a systematic review and meta-analysis.

PURPOSE: Radiotherapy (RT) is widely used for early glottic cancer. Patients failing this treatment are referred to surgical management of their disease. Salvage partial laryngectomy (SPL) has the advantage of preserving laryngeal function with total laryngectomy (TL) remaining as a last resort. The purpose of this study was to determine the efficacy of SPL in preventing total laryngectomy, following failed RT, for early glottic cancer. METHODS: A meta-analysis of all published English literature was performed. All publications that included patients undergoing SPL were reviewed according to the preferred reporting items for systematic reviews and meta-analyses (PRISMA) reporting guidelines. The search strategy identified 154 relevant articles. A total of 15 retrospective studies that included 323 suitable patients were subsequently analyzed in this meta-analysis. The main outcome measure was the rate of laryngectomy-free survival (LFS) following SPL. The indications for salvage TL (i.e., disease recurrence and poorly functional larynxes) as well as subgroup analyses for open and trans-oral SPLs were also calculated. RESULTS: The overall rate of LFS following SPL was 81.2% (fixed effects model range: 75.7-86.8%). Salvage TL following SPL were performed in 96.8% due to disease recurrence and in 3.2% to poorly functional larynxes. A subgroup analysis showed a 90.4% LFS after open SPL and 78.6% following trans-oral SPL. CONCLUSION: A high rate of successful salvage partial laryngectomies, regardless of surgical technique, is to be anticipated in well-selected patients after RT failure.

Prophylactic central neck dissection has no advantage in patients with metastatic papillary thyroid cancer to the lateral neck.

BACKGROUND: Papillary thyroid cancer (PTC) usually metastasizes via lymphatic channels in a sequential fashion, first to the central compartment, followed by the lateral neck. PTC patients diagnosed with lateral neck disease (N1b) without proof for central involvement traditionally undergo prophylactic central neck dissection (pCND). However, substantial evidence on outcomes to support this approach is lacking. MATERIALS AND METHODS: We conducted a dual center retrospective study to compare the rate of central neck recurrence between N1b PTC patients undergoing pCND and those spared pCND. All patients diagnosed with N1b PTC who underwent total thyroidectomy and lateral neck dissections with or without pCND between January 1998 and December 2015 were included in this study. The rates of central neck recurrences were compared between the groups. RESULTS: The 111 patients who met the inclusion criteria were 44 females (39.6%) and 67 males (60.4%), with a mean age of 50.2 ± 17.7 years, and a mean follow-up of 10.2 ± 5.3 years. Sixty patients (54.1%) underwent a pCND and 51 patients (45.9%) did not (non-pCND). During follow-up, 18 patients (16.2%) had level VI recurrences, 13 in the pCND group and 5 in the non-pCND group. Cox-regression models with propensity scoring did not reveal any inclination or an advantage for performing pCND. CONCLUSION: The present study demonstrated no advantage in performing pCND to prevent central neck recurrence among PTC patients with lateral neck involvement only. These findings question the need for pCND in patients without clinical evidence of central neck disease.

Meta-analysis of time to extrusion of tympanostomy tubes by tympanic membrane quadrant.

OBJECTIVES: To investigate the published time to extrusion of tympanostomy tubes inserted in the various quadrants of the tympanic membrane. METHODS: Publications were selected by a search with 'PubMed', 'Embase' and 'Web of Science'. A meta-analysis of time to extrusion as a function of tympanic membrane quadrant intubation was performed. DESIGN, SETTING, PARTICIPANT-NOT RELEVANT MAIN OUTCOME MEASURED: Extrusion rate of tympanostomy tubes inserted in the various quadrants of the tympanic membrane. RESULTS: Eleven studies describing 2232 tympanostomy tubes were enrolled into the quantitative meta-analysis. The extrusion rate was evaluated at 3-month intervals up to 24 months post-intubation, and it did not differ significantly at any of the time points tested for the superior and inferior anterior quadrants. The cumulative extrusion rates were as follows: 11 and 9%, 32 and 23%, 59 and 36%, 80 and 67%, 87 and 70%, 88 and 82%, and 96 and 89% in the superior and anterior quadrants, respectively, at 3, 6, 9, 15, 18, 21 and 24 months post-intubation, respectively. CONCLUSION: Time to extrusion of tympanostomy tubes are similar for all 3 tympanic membrane quadrants. The anterior superior quadrant intubation has no superiority in terms of intubation time.

Long-term all-cause mortality and its association with cardiovascular risk factors in thyroid cancer survivors: an Israeli population-based study.

BACKGROUND: The global incidence of thyroid cancer (TC) has risen considerably during the last three decades, while prognosis is generally favorable. We assessed the long-term all-cause mortality in TC survivors compared to the general population, and its association with cardiovascular risk factors. METHODS: Individuals diagnosed with TC during 2001-2014 (TC group) and age- and sex-matched individuals from the same Israeli healthcare system without thyroid disease or a cancer history (non-TC group) were compared. Cox regression hazard ratios (HRs) and 95% confidence intervals (95%CIs) for all-cause mortality were calculated by exposure status. RESULTS: During a 15-year follow-up (median 8 years), 577 TC survivors out of 5677 (10.2%) TC patients and 1235 individuals out of 23,962 (5.2%) non-TC patients died. The TC survivors had an increased risk of all-cause mortality (HR = 1.89, 95%CI 1.71-2.10), after adjusting for cardiovascular risk factors already present at follow-up initiation. This increased risk was most pronounced in the 55- to 64-year-old age group (HR = 1.49, 95%CI 1.33-1.67). The TC survivors who died by study closure had more hypertension (14.6% vs. 10.3%, P = 0.002), more dyslipidemia (11.4% vs. 7.2%, P <  0.001), and more cardiovascular disease (33.6% vs. 22.3%, P = 0.05) compared to those who died in the non-TC group. CONCLUSIONS: This large cohort study showed higher all-cause mortality with a higher prevalence of hypertension, dyslipidemia, and cardiovascular disease among TC survivors compared to matched non-TC individuals. Primary and secondary prevention of cardiovascular risk factors in TC survivors is mandatory.

Low rates of airway intervention in adult supraglottitis: A case series and meta-analysis.

PURPOSE: Acute supraglottitis (SG) can potentially lead to rapid airway obstruction. The last few decades have witnessed a shift towards a more conservative approach in airway management of adult SG. This study aims to evaluate this watchful approach based on a large case series combined with a high-level meta-analysis of all reports in the English literature. METHODS: Retrospective case series and meta-analysis. The medical records of all adult patients diagnosed as having SG who were hospitalized in a large-volume tertiary referral center between January 2007 and December 2018 were reviewed. A meta-analysis was conducted on all English literature published between 1990 and 2018. RESULTS: A total of 233 patients (median age 49.1 years, 132 males), were admitted due to acute SG during the study period. No airway intervention was required in 228 patients (97.9%). Five patients (2.1%) required preventive intubation, and two of them (0.9%) were later surgically converted to a tracheotomy. Patients who required airway intervention had higher rates of diabetes (P = .001), cardiovascular diseases (P = .036) and other comorbidities (P = .022). There was no mortality. The meta-analysis revealed that the overall intubation rates random effects model was 8.8% [95% confidence interval (CI) 4.6%-14.0%] and that the tracheotomy random effects model was 2.2% (95% CI; 0.5%-4.8%). The overall mortality rate was 0.89%. CONCLUSIONS: This study provides evidence of low rates of surgical airway intervention in patients diagnosed with SG worldwide. A conservative approach in adult SG is safe and should be advocated. LEVEL OF EVIDENCE: 2.

Correction to: Total thyroidectomy with therapeutic level II-IV neck dissection for papillary thyroid carcinoma: level VI recurrence patterns.

The original article can be found online.

Total thyroidectomy with therapeutic level II-IV neck dissection for papillary thyroid carcinoma: level VI recurrence patterns.

OBJECTIVE: Lateral and central compartments cervical lymph nodes metastases are common among patients with papillary thyroid carcinoma (PTC). Elective level VI neck dissection during thyroidectomy and lateral neck dissection (LND) for the treatment of PTC with lateral compartment lymph node metastases is controversial because of the uncertain benefit in clinical outcomes and increased risks of surgical morbidity. We aimed to determine the potential benefit of elective level VI neck dissection in patients with cN1 papillary thyroid carcinoma (PTC) by investigating the rate and pattern of locoregional recurrence in PTC patients who underwent total thyroidectomy and therapeutic lateral node dissection (LND; levels II-IV) without elective level VI dissection. METHODS: A retrospective cohort study. Data on demographics, clinical presentation and workup, intraoperative and pathological report, postoperative course, adjuvant therapy, recurrence patterns, and overall survival were retrieved from the medical charts of patients who underwent thyroid surgery in our hospital between January 2006 and December 2017. RESULTS: A total of 1415 thyroidectomies were performed during the study period, of which 802 (56.67%) were for PTC. Of those PTC patients, 228 (28.42%) also underwent LND (levels II-VI) during the same thyroidectomy procedure. Thirty-four (14.91%) of those 228 patients, underwent total thyroidectomy with therapeutic lateral ND II-IV without elective level VI ND. During the follow-up period, five (14.7%) of the latter cohort were diagnosed with recurrence in central neck (level VI) lymph nodes, and four of them (11.7%) were diagnosed with ipsilateral recurrence at level VI. CONCLUSION: Our results revealed 11.7% rate of clinically significant recurrent disease in ipsilateral level VI which, in our opinion, does not justify routine prophylactic level VI ND dissection when the ipsilateral lateral neck is operated for metastases.

Acute supraglottic laryngitis complicated by vocal fold immobility: prognosis and management.

PURPOSE: Acute supraglottic laryngitis (ASL) is manifested by supraglottic inflammation that has the potential for rapid and fatal airway obstruction. Complete/incomplete vocal fold immobility (VFIm) in the setting of ASL may contribute to airway obstruction. The rate of VFIm complicating ASL is not known, and it is not clear whether its occurrence alters the course and the management of ASL, particularly the need to secure the airway (by endotracheal intubation/tracheostomy). This study seeks to describe the natural history of VFIm associated with ASL (ASLIm) and to determine the added effect of VFIm on ASL severity, management, and the need for intervention to secure the airway. METHODS: This is a retrospective cohort study in a tertiary referral center. The medical records of all patients hospitalized due to ASL between January 2007 and December 2016 were reviewed. RESULTS: Of the 214 patients admitted due to ASL, VFIm was identified in 9 (4.2%). The VFIms resolved within 1-90 days in all 8 patients with available follow-up of 1-3 months. One patient required endotracheal intubation. The 9 ASLIm patients had significantly higher rates of hoarseness and a history of diabetes mellitus. There was no group difference in the need to secure the airway. CONCLUSIONS: VFIm is an apparently uncommon finding among patients with ASL. It appears to be usually transient, short-lasting, and full recovery can be expected. VFIm did not alter the ASL course, nor did it put our patients at increased risk for the need for intervention to secure the airway. LEVEL OF EVIDENCE: 4.

Structured, Protocol-Based Pulse-Oximetry Measurement Improves the Evaluation of Hypoxemic Patients at Hospital Admission.

BACKGROUND: Accurate pulse oximetry reading at hospital admission is of utmost importance, mainly for patients presenting with hypoxemia. Nevertheless, there is no accepted or evidence-based protocol for such structured measuring. OBJECTIVES: To devise and assess a structured protocol intended to increase the accuracy of pulse oximetry measurement at hospital admission. METHODS: The authors performed a prospective comparison of protocol-based pulse-oximetry measurement with non-protocol based readings in consecutive patients at hospital admission. They also calculated the relative percentage of improvement for each patient (before and after protocol implementation) as a fraction of the change in peripheral capillary oxygen saturation (SpO2) from 100%. RESULTS: A total of 460 patients were recruited during a 6 month period. Implementation of a structured measurement protocol significantly changed saturation values. The SpO2 values of 24.7% of all study participants increased after protocol implementation (ranging from 1% to 21% increase in SpO2 values). Among hypoxemic patients (initial SpO2 < 90%), protocol implementation had a greater impact on final SpO2 measurements, increasing their median SpO2 readings by 4% (3-8% interquartile range; P < 0.05). Among this study population, 50% of the cohort improved by 17% of their overall potential and 25% improved by 50% of their overall improvement potential. As for patients presenting with hypoxemia, the median improvement was 31% of their overall SpO2 potential. CONCLUSIONS: Structured, protocol based pulse-oximetry may improve measurement accuracy and reliability. The authors suggest that implementation of such protocols may improve the management of hypoxemic patients.

Evaluation of Cardiovascular Changes in Children with BAVs.

The objectives of this study were to investigate left ventricular (LV) function, aortic dilation, and atherosclerosis in children with mildly deteriorated isolated bicuspid aortic valve (BAV) function using echocardiographic studies and biochemical markers of atherosclerosis and to correlate results with normal children. Biochemical analyses indicating cardiovascular risk of atherosclerosis and vascular changes in the aorta in relation to BAV were performed in 41 children aged 5-15 years old with isolated BAV and in 25 children with tricuspid aortic valves. Evaluations of aortic valve structures and functions; examinations of the LV M-mode and ascending aorta Doppler; and measurements of the LV Tei index (MPI), propagation velocity, ascending aorta at four levels, and carotid intima-media thickness (CIMT) were performed. There were no statistically significant differences in CIMTs, plasma matrix metalloproteinase-9, tissue metalloproteinase inhibitor-1 levels, or other biochemical parameters indicating cardiovascular risk or atherosclerosis between study and control groups. Deterioration of LV function, which could not be seen with M-mode echocardiography, was evident by MPI. MPI values in the study versus control groups were 0.46 ± 0.080 versus 0.40 ± 0.086 (p < 0.05). Diameters of the aorta in the study and control groups were 19.7 ± 4.7 and 17.2 ± 2.8 mm (p < 0.05) at the sinotubular junction level and 20.6 (14.4-40.5) and 18.3 (12.4-24) mm at the ascending aorta level (p < 0.05). Increased aortic valve insufficiency was related to increased aortic diameter. No sign of atherosclerosis was detected in children with BAV. Deterioration of LV function was seen using MPI, and aortic dilation was related to the severity of aortic valve insufficiency.

Head and Neck Cutaneous Squamous Cell Carcinoma Clinicopathological Risk Factors according to Age and Gender: A Population-based Study.

BACKGROUND: Clinicopathological risk factors for cutaneous squamous cell carcinoma of the head and neck (CSCCHN) are associated with local recurrence and metastasis. OBJECTIVES: To compare the incidence and risk factors of CSCCHN by age and gender in order to help refine the clinical evaluation and treatment process. METHODS: Clinical and pathological data of all patients diagnosed with CSCCHN during 2009-2011 were obtained from a central pathology laboratory in Israel. Estimated incidence rate calculation was standardized to the 2010 Israeli population. Independent risk factors for poorly differentiated CSCCHN were analyzed using logistic regression. RESULTS: CSCCHN was diagnosed in 621 patients. Mean age was 75.2 years; mean tumor horizontal diameter was 11.1 ± 6.8 mm. The overall estimated incidence rate in males was higher than in females (106.2 vs. 54.3 per 1,000,000, P 0.001). Twenty cases (3.2%) had poorly differentiated CSCCHN. Scalp and ear anatomic locations were observed more often in males than in females (22.1% vs. 6.1% and 20.3% vs. 3.3%, respectively, P < 0.001). Per 1 mm increment, tumor horizontal diameter increased the risk for poorly differentiated CSCCHN by 6.7% (95% CI 1.3-12.4%, P = 0.014). CONCLUSIONS: CSCCHN clinicopathological risk factors are not distributed evenly among different age and gender groups.

Vitamin D Antibodies in Systemic Sclerosis Patients: Findings and Clinical Correlations.

BACKGROUND: Vitamin D is a pivotal factor in calcium homeostasis and exerts immunomodulatory effects. Hypovitamin D has been demonstrated in systemic sclerosis (SSc) patients and may be related to more severe disease of longer duration and with extensive skin involvement. OBJECTIVES: To seek anti-vitamin D antibodies in SSc patients, as found by previous research in patients with systemic lupus erythematosus (SLE). METHODS: The study included 54 SSc patients and 41 volunteers. Immunoglobulin (Ig) G and IgM autoantibody levels against 25(OH)D and 1,25(OH)D were obtained from patients and controls and were compared. SSc patients were assessed for autoantibody profile and disease severity. RESULTS: Vitamin D antibodies were present in 87% of SSc patients and 42% of controls. Higher levels of anti-25(OH)D IgM antibodies were detected in SSc patients compared to controls (0.48 ± 0.22 vs. 0.29 ± 0.29, respectively, P = 0.002); however, IgG levels were lower in the SSc patients. No such discriminative. effect was found regarding anti-1,25(OH)D antibodies between SSc and controls. No correlation was found between,vitamin D antibodies and other autoantibodies, disease severity, or target organ damage. CONCLUSIONS: To the best of our knowledge; this is the first study of these novel, anti-vitamin D antibodies in SSc patients and the first time a correlation between IgM 25(OH) vitamin D antibodies and scleroderma has been identified. Further research on the pathophysiological significance and therapeutic potential of vitamin D is required.

Early onset marfan syndrome: Atypical clinical presentation of two cases.

Early onset Marfan Syndrome (eoMFS) is a rare, severe form of Marfan Syndrome (MFS). The disease has a poor prognosis and most patients present with resistance to heart failure treatment during the newborn period. This report presents two cases of eoMFS with similar clinical features diagnosed in the newborn period and who died at an early age due to the complications related to the involvement of the cardiovascular system.

Direct Transvestibular Feminization Thyroid Laryngochondroplasty.

BACKGROUND: Feminization laryngochondroplasty (FLC) methods have evolved from using a midcervical incision to a submental, less visible incision. The scar may be unacceptable to the patient because it signals gender reassignment surgery. An endoscopic transoral approach to FLC inspired by transoral endoscopic thyroidectomy was recently suggested to avoid the neck scar; however, it requires special equipment and has a long learning curve. A vestibular incision is used to approach the chin in lower-third facial feminization surgery. The authors propose that this incision may be extended to the thyroid cartilage in performing direct FLCs. The authors describe their experience with a novel, minimally invasive, direct transvestibular use of the chin-reshaping incision. METHODS: The medical records of all patients who underwent direct transvestibular FLC (DTV-FLC) from December of 2019 to September of 2021 were retrieved and reviewed for this retrospective cohort study. Data on the operative, postoperative, and follow-up courses, complications, and functional and cosmetic results were retrieved. RESULTS: Nine transgender women were included. Seven DTV-FLCs were performed during lower-third facial feminization surgery, and two were isolated DTV-FLCs. One was a revision DTV-FLC. Transient minor complications were encountered and resolved by the postoperative visit at 1 to 2 months. Vocal fold function and voice quality remained intact. Eight available patients were satisfied with the surgical results. A blinded assessment by eight plastic surgeons determined that seven procedures were successful. CONCLUSION: The novel DTV-FTLC approach either in isolation or as part of lower-third facial feminization surgery facilitated scar-free FLC with satisfactory cosmetic and functional results. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Closure of transcatheter ventricular septal defect using LifetechTM Konar-MF Occluder in children weighing less than 10 kilograms: mid-term results, a tertiary single center experience.

OBJECTIVE: Transcatheter closure of medium and large ventricular septal defects (VSDs) in young children is limited due to the use of over-sized devices that can cause hemodynamic instability and arrhythmia. In this study, we aimed to retrospectively evaluate the safety and efficacy of the device in the mid-term in children weighing less than 10 kg whose transcatheter VSD was closed only with the Konar-MFO device. PATIENTS AND METHODS: Among 70 children whose transcatheter VSD was closed between January 2018 and January 2023, 23 patients weighing less than 10 kg were included in the study. Retrospectively, the medical records of all patients were reviewed. RESULTS: The mean age of the patients was 7.3 (4.5-26) months. 17 of the patients were females, 6 of them were males, F/M: 2.83. The average weight was 6.1 (3.7-9.9) kg. The mean the pulmonary blood flow/ systemic blood flow (Qp/Qs) was 3.3 (1.7-5.5). The mean defect diameter was 7.8 mm (5.7-11) for the left ventricle (LV) side, and 5.7 mm (3-9.3) for the right ventricle (RV) side. Based on the utilized device dimensions, the measurements on the LV side were recorded as 8.6 mm (range 6-12), while those on the RV side were recorded as 6.6 mm (range 4-10). Antegrade technique was applied to 15 (65.2%) patients and retrograde technique was applied to 8 (34.8%) patients in the closure procedure. The procedure success rate was 100%. The incidence of death, device embolization, hemolysis, or infective endocarditis was zero. CONCLUSIONS: Perimembranous and muscular VSDs in children under 10 kg can be successfully closed under the management of an experienced operator with the Lifetech Konar-MFO device. This is the first study in the literature to evaluate the efficacy and safety of the device in children under 10 kg in whom only Konar-MFO VSD occluder device is used for transcatheter VSD closure.