Outcomes of coblation tonsillectomy versus bipolar electrocautery tonsillectomy in pediatric population

Background: Bipolar electrocautery tonsillectomy has been the preferred technique for many otolaryngologists, yet coblation tonsillectomy is gaining popularity in the current practice. This study aims at comparing both techniques in terms of pain, bleeding, and healing. Result(s): A total of 120 patients were randomly divided into two equal groups. Overall mean pain score associated with coblation tonsillectomy was statistically less than that caused by bipolar electrocautery throughout the follow-up period (p < 0.001). The difference in pain duration was statistically longer for the bipolar group. The incidence of postoperative hemorrhage-both reactionary and secondary-was statistically higher in the bipolar group. Coblation tonsillectomy showed statistically shorter duration of healing (p < 0.001). Conclusion(s): Coblation tonsillectomy is associated with less pain severity and shorter pain duration, fewer bleeding incidents, and more prompt healing.Copyright © 2022, The Author(s).

Monitored Anesthesia Care in Uterine Artery Embolization for Leiomyomas and Adenomyosis

Background: Patients undergoing an interventional radiology procedure report some degree of anxiety. Therefore, procedure-related anxiety needs to be managed. The aim of our study was to investigate patient satisfaction with monitored anesthesia care (MAC) for uterine artery embolization (UAE)-related procedural anxiety in symptomatic uterine fibroids or adenomyosis. Method(s): Between May 2021 and June 2022, 36 patients with symptomatic fibroids or adenomyosis underwent UAE with MAC. Follow-up evaluations consisted of clinical symptoms, degree of satisfaction with MAC in UAE, and complications. Result(s): MAC in UAE was successfully performed in all patients. UAE significantly reduced patients' complaints such as bleeding and pain: the scores for bleeding and pain were significantly reduced after 3 months of UAE compared with those before UAE, indicating the effectiveness of UAE. The mean score of satisfaction with MAC in UAE was 4.3 points, meaning that 94.4% of women were satisfied or very satisfied. No major complications were observed. Conclusion(s): MAC in UAE for symptomatic uterine fibroids or adenomyosis can be emotionally effective and safe for patients who are anxious about the procedure.Copyright © 2023 The Author(s).

Diagnosis of Nasal Breathing Using a Rhinomanometer in Patients with Osa Prescribed Conservative Treatment with Intraoral Devices

In recent years, diagnostics in the field of medicine has developed at an extremely rapid pace, thanks to the use and improvement of new medical devices and devices. The problem of timely and adequate diagnosis and treatment of the syndrome of obstructive sleep apnea (OSA) and snoring is particularly relevant world-wide. The disease has been proven to worsen the patients' quality of life, and may even threaten it. Obstructive sleep apnea and snoring syndrome (OSAS) is a widespread disease of social importance in which there is a reduction or cessation of airf low through the nose/mouth during sleep due to upper airway collapse. Obstructive sleep apnea affects the cardiovascular, endocrine, neurocognitive and other systems of the body. There are symptoms of loud snoring, choking, hypoxemia, and micro-awakenings, leading to sleep frag-mentation, daytime fatigue, and sleepiness. The latter greatly worsens the quality of life of patients. There are real risks to the life and health of the patient and others, given the possibility of falling asleep at the wheel in drivers with sleep apnea and participation in traffic accidents. To diagnose the syndrome, a poly-somnographic study is performed, which is still the gold standard. For a better diagnosis, it is recommend-ed to combine it with rhinomanometry. Treatment of OSA includes control of risk factors and removal of obstructive factors that make breathing difficult. Severe OSA syndrome is treated with continuous positive pressure ventilation (CPAP) during sleep, possibly in combination with intraoral devices. Rhinomanome-try can also be used to monitor the effectiveness of CPAP therapy in severe forms of the syndrome by deter-mining tissue resistance. The impact on patients with a milder form of OSA treated with intraoral devices is also monitored. The method can also be used in patients with allergic rhinitis, sinusitis of rhinogenic and other origin, and patients with orthodontic deformities. The correct choice of intraoral appliances for conservative treatment of OSA and timely diagnosis are key to successful treatment.Copyright © 2022, Bulgarian-American Center. All rights reserved.

Subclinical myocardial dysfunction in patients with persistent dyspnea one year after COVID-19

Introduction: Coronavirus disease 2019 (COVID-19) rapidly spread across the globe,evolving into a global pandemic,with a crucial impact on healthcare systems. Several short-term follow-up studies emphasized the persistence of symptoms, referred as long COVID, in a significant number of discharged patients even without history of cardiopulmonary diseases, with dyspnea being one of the most frequent complaint [1-3]. Even though those reports on recovered COVID-19 patients did not describe major left ventricle (LV) function abnormalities, subtle cardiac changes may be present. Purpose(s): We aimed to investigate the presence of subclinical cardiac dysfunction, assessed by transthoracic echocardiography (TTE) in recovered COVID-19 patients, without previous cardiopulmonary disease at 1 year follow-up. Method(s): 310 COVID-19 consecutive hospitalized patients were prospectively included between March and April 2020. 66 patients out of 251 recovered patients had no previous history of coronary artery disease, arrhythmia, arterial hypertension, valvular heart disease, asthma, chronic obstructive pulmonary disease and obstructive sleep apnea, respectively and were included in the final analysis (Figure 1). The follow-up consisted in 2 parts, a 6-months visit including clinical and physical examination, chest computed tomography and spirometry and a 12-months visit including clinical and physical examination, spirometry and TTE. Result(s): 66 patients (mean age 51.39+/-11.15 years, 45 (68.2%) males) were included in the final analysis. 23 (34.8%) patients reported dyspnea at 1 year. TTE parameters were in the normal range, with a mean LV ejection fraction of 56.9+/-4.6%, mean global longitudinal strain (GLS) of -20.9+/-2.3%, global constructive work (GCW) of 2381.4+/-463.6 mmHg% and global work index (GWI) of 2132.5+/-419.2 mmHg%. Type 1 diastolic dysfunction was observed in 11 (16.7%) patients. One (1.5%) patient had type 2 diastolic dysfunction. A normal respiratory pattern was reported in 31 (47%) patients at 6 months spirometry, while 19 (28.8%) patients had a restriction pattern. No significant differences regarding clinical, laboratory or imaging findings at baseline were found between groups. The following TTE parameters were significantly different in patients with and without dyspnea at 1 year: GLS (-19.97+/-2.14 vs. -21.38+/-2.37, p=0.039), GCW (2183.72+/-487.93 vs. 2483.14+/-422.42, p=0.024) and GWI (1960.06+/-396.21 vs. 2221.17+/-407.99, p=0.030). Multivariable logistic regression showed that GCW and GWI were inversely and independently associated with persistent dyspnea, one year after COVID-19 (p=0.035, OR 0.998, 95% CI 0.997-1.000;p=0.040, OR 0.998, 95% CI 0.997-1.000) (Table 1). Conclusion(s): Persistent dyspnea one year after COVID-19 was present in more than a third of patients without known cardiovascular or pulmonary diseases. GCW and GWI were the only echocardiographic parameters independently associated with symptoms, suggesting a decrease in myocardial performance in this population and subclinical cardiac dysfunction.

Avascular necrosis in pediatric mandibular distraction - Report of a rare complication with a review on the pathogenic mechanism and management strategies

Avascular osseo-necrosis is one of the rare complications that has never been reported in pediatric mandibular distraction. However extensive periosteal stripping, developing tooth buds and aberrations related to the inferior alveolar canal in malformed syndromic mandible may lead to compromised vascularity to the osteotomised segments leading to avascular necrosis after monofocal mandibular distraction. The aim of the current case report is to describe this rare complication after pediatric MMDO and discuss in detail the possible etio-pathologic mechanisms and provide an insight for the management strategies.Copyright © 2021 The Authors

OSA patients' opinions concerning their disease and treatment during the pandemic

The COVID-19 pandemic has changed the operation of healthcare systems worldwide reducing hospital based services, and increasing telemedicine solutions for the diagnosis and treatment of obstructive sleep apnoea (OSA). Aim(s): to evaluate the opinion of OSA patients concerning their disease during the COVID pandemic,their treatment and follow up. Method(s): A telephone questionnaire based survey was conducted on OSA patients treated with CPAP including basic information (age, gender, marriage, education level, etc.), opinion towards the changes of medical practice during the pandemic, history of OSA, Epworth Sleepiness Scale (ESS), Athens Insomnia Scale (AIS), DAR-5 (anger), PHQ-4(depression and anxiety), Heaviness of Smoking Index (HSI) and Loneliness scale (LS). Adherence on CPAP was assessed objectively by telemonitoring. Result(s): 100 OSA patients 54.3+/-12.5 years (72% males) were evaluated presenting with ESS: 4.3+/-2.95, AIS: 3.14+/-3.3, PHQ-4: 2.29+/-1.72, DAR-5: 6.7+/-1.95 and HSI 3.5+/-.1.7. Mean CPAP use was 5.8+/-1.65 with 2.9+/-2.5years OSA history (96% used CPAP the same as before the pandemic). 41% considered themselves as high risk group for severe disease due to COVID because they suffered from OSA, and 25% believed CPAP could be protective from COVID. Patients infected with COVID (31%) did not change their CPAP use. 30% believed they received worse healthcare facilities than before and 16% presented worse sleep quality. Conclusion(s): During the pandemic, OSA patients continued to use their CPAP as before;however they believed that they should have better healthcare facilities. They considered themselves as a high risk group for COVID infection.

Predictive factors for Long COVID in adults previously hospitalized for COVID-19

Introduction: Long COVID was defined by the persistence or development of symptoms beyond 4 weeks from the onset of the disease. The aim of our study is to describe the prevalence of Long COVID in survivors after discharge and to determine predictive factors for Long COVID. Method(s): It was a retrospective study of hospitalized patients with COVID-19 in pneumology department D of Abderahman Mami hospital. We divided patients in 2 groups: G1: 94 patients with Long COVID and G2: 43 asymptomatic patients after discharge. Result(s): The prevalence of Long COVID was 68%. Female sex was associated with persistent symptom (53.2% vs 30.2%, p=0.01). The risk of persistent symptoms was higher in patients with moderate or severe obesity (51.6% vs 33%;p=0.04). Comorbidities were more frequent in G1 (73% G1 vs 65%, p=0.3). Asthma and Obstructive Sleep Apnea were associated with persistent symptoms (73% vs 65%, p=0.03 and 8% vs 0%, p=0.04, respectively). Patients in G1 had a higher frequency of fatigue than those in G2 (80% vs 58%, p=0.005). Most cases were mild, but 17% of patients required ventilatory support. Long COVID showed a strong correlation with Length of stay (r2= 0.18, p=0.03). The most common persistent symptoms reported were: fatigue (75.8%), shortness of breath (70.8%) and join pain (38.5%). Conclusion(s): We have observed that long COVID affected especially women and patients with respiratory comorbidities and those requiring prolonged hospital stay.

Kidney Limited Complement-Mediated Thrombotic Microangiopathy- a Challenging Case

Introduction: Complement-mediated thrombotic microangiopathy (CM-TMA) is a rare disease characterized by microangiopathic hemolytic anemia (MAHA), thrombocytopenia and organ injury. The absence of hemolysis and thrombocytopenia is rare. We present a case of kidney limited CM-TMA successfully treated with eculizumab. Method(s): A 36 year-old man with poorly controlled hypertension, obesity, CKD (baseline creatinine (sCr) 2,6mg/dL, albuminuria 150mg/g), hyperlipidemia, obstructive sleep apnea, hyperuricemia, SARS-CoV-2 infection 3 months earlier, and family history of CKD of unknown etiology (father started kidney replacement therapy (KRT) at young age) presented to the ER with high blood pressure and right hemiplegy. Head CT scan showed left thalamo-capsular hemorrhage. Oftalmologic exam was normal. Laboratory findings were: hemoglobin (Hb) 12.5g/dL, elevated white cell count (17.900/uL), platelet count 214.000/uL, sCr 4.3mg/dL, lactate dehydrogenase (LDH) 303U/L. Urine dipstick revealed protein+ and Hb++. Chest X-ray showed signs of pneumonia. The patient was admitted in ICU and mechanically ventilated. After 3 weeks, renal function recovered to its baseline (sCr 1.5mg/dL, no proteinuria) without KRT, and the patient was transferred to the medical ward. Several infectious complications prolonged hospital stay. After 3 months, a new mild SARS-CoV-2 infection was detected. At this time: Hb 9.9g/dL, platelets 220.000/uL, sCr 2.2mg/dL. Six days later the patient showed Hb 9.5 g/dL, without reticulocytosis, platelets 195.000/uL, sCr 6.3mg/dL, LDH 348U/L, normal haptoglobin, no schizocytes on blood smear. After 3 days, the patient was anuric and sCr increased to 10mg/dL, prompting KRT. Kidney ultrasound showed no abnormalities. Autoimmunity study was negative, normal C3/C4, no monoclonal gammopathy, and negative viral serologies. Kidney biopsy (KB) was performed as the etiology of AKI remained unclear. Light microscopy revealed thickned glomerular capillary walls with subendothelial expansion forming double contouring, arteriolar intimal expansion and fibrin thrombi occluding the vascular lumina. Scarse C3 deposition was observed in capillary walls. Since the morphological features were consistent with TMA, secondary causes were excluded and primary causes also investigated: ADAMTS13 activity, complement factor B and I were within normal range, slight decrease of factor H with normal anti factor H antibody. The molecular studies of complement genes were performed by NGS-based gene panel revealing a rare heterozygous missense mutation on gene CFB, c.1189G>A (p.Asp397Asn), described as a genetic risk factor of CM-TMA in the presence of a trigger. Result(s): Treatment with eculizumab was started and the patient showed signs of kidney recovery allowing KRT suspension 1 month later (sCr 5.53mg/dL). Of note, the patient never presented MAHA or thrombocytopenia. After 5 months, renal function improved to sCr 3.9mg/dL. Conclusion(s): We report a case of CM-TMA with isolated kidney injury without laboratory hallmarks of TMA. Patients usually require a secondary trigger for the disease to manifest, and in this case SARS-CoV-2 infection may have been the causative agent. A mutation in gene CFB may have predisposed the patient to the outcome. KB was crucial for diagnosis and prompted the treatment with eculizumab with partial recovery without the need for chronic KRT. No conflict of interestCopyright © 2023

MoCA-T as a remote assessment tool to determine cognitive impairment in patients with moderate to severe OSA

The COVID-19 pandemic has led to the emergence of technological tools that allow remote assessment on patients. The Montreal Cognitive Assessment Test Telephone Version (MoCA-T) is one of them. Considered as a telemedicine resource that allows remote cognitive screening in pandemic era and in the population with difficulties in accessing health centers. Mild cognitive impairment (MCI) is a common finding in patients with Obstructive Sleep Apnea Hypopnea Syndrome (OSAHS). However, the application of telephone tests for cognitive screening has not been studied. To determine the frequency of cognitive deterioration through the application of MoCA-T as a remote screening test in patients with moderate and severe OSAHS is the main objective of this work. To do this, the MoCA-T test was applied to 104 patients with polysomnographic diagnostic capabilities for moderate and severe OSAHS between ages 18 and 65, excluding patients with comorbidities that affect cognitive ones. Abnormal MoCA-T results were obtained in 43% of patients, with the cognitive domains of memory and attention being the most affected. Finally, abnormal MoCA-T results correlated with self-perception of memory difficulties, being more frequently altered in those who manifest cognitive complaints. The MoCA-T test could be a brief, validated and feasible technological tool for cognitive screening of patients with OSAHS in times of pandemic and in patients with care barriers.Copyright © 2023 Sociedad Neurologica Argentina

Diagnostic utility of the modified Berlin Questionnaire vs Berlin Questionnaire for the diagnosis of obstructive sleep apnoea in adults with a history of COVID-19 infection

Background: We have recently developed a modified Berlin Questionnaire (mBQ) based on three subcategories, ignoring obesity and hypertension, and showed that COVID-19 patients with high-risk obstructive sleep apnoea (OSA) had worse prognosis compared to those with low-risk OSA. Aims and Objective: We aimed to validate the mBQ and compare the results with the BQ in the same cohort. Method(s): Out of 320 subjects from the initial study, 70 completed the BQ and underwent an overnight polysomnography (PSG). The mBQ included 3 subcategories;snoring intensity/frequency, witnessed apneas, and morning/daytime tiredness. The patients were classified as high-risk OSA when they were positive on at least 2 subcategories. The apnoea-hypopnoea-index threshold on the PSG was set as 15 events/h. Result(s): In all, 27 patients (39%) were categorized as having OSA based on the PSG;the true positive rate was 85.2% for the mBQ, and 70.4% for the BQ. For the 43 cases without OSA on the PSG, the true negative rate was 90.7% for the mBQ, and 67.4% for the BQ. The mBQ had a sensitivity of 85.2%, a specificity of 90.7%, and the area under the curve was 0.87 (95% CI 0.78 - 0.97). Corresponding values were 60.6%, 78.4%, and 0.72 (95% CI 0.60 - 0.84) for the BQ, respectively. Conclusion(s): The mBQ had a better sensitivity and specificity compared to the BQ for OSA diagnosis in adults with a history of COVID-19 infection. The mBQ can be used in clinical management of cases with high-risk OSA in hospitals with long waiting lists for PSG, and to eliminate unnecessary PSG investigations for adults with low-risk OSA. Clinical trial registration: NCT04363333.

Evolution of asthmatic patients at 6 and 12 months after acute COVID-19 recovery

Nowadays, beyond the acute phase of COVID-19, there is a growing interest in the clinical manifestations that appear after the recovery of this phase, giving the potential repercussions that this could have in the recovered population. Asthma is a prevalent disease, and it is possible that its pathogenic basis could affect the postCOVID-19 course. Objective(s): evaluate the evolution of asthmatic patients at 6 and 12 months after the recovery of COVID-19. Method(s): Data was collected through manual revision of electronic clinical histories. There were included as demographic features the age and sex, and comorbidities such as smoking habit, COPD, obesity (BMI > 30 Kg/m2), obstructive sleep apnea, gastroesophagic reflux, rinosinusitis, nasal poliposis, anxiety and depression. Patients were classified according to whether they had T2 asthma or not. There were considered previous pneumonia, need of conventional hospitalization or ICU. Symptoms (chest pain, cough, sputum production and dyspnoea) and the development of further complications were also recorded. Result(s): After analyzing the variables at 6 months after recovery, we found statistical significance in the presence of chest pain in patients with previous COVID-19 pneumonia (p=0.009). In the same period, lower eosinophil blood levels were found in patients that had dyspnoea (p=0.043). Additionally, there was a significant association between smoking habit and pulmonary embolism at 12 months after recovery (p=0.025). Conclusion(s): Developing COVID-19 associated pneumonia in asthmatic patients is related to the presence of chest pain at 6 months after recovery. Also, the smoking habit seems to be associated with thromboembolic disease in postCOVD-19 period.

Consequences of anastomotic leaks after minimally invasive esophagectomy: A single-center experience

Background: Anastomotic leak (AL) after minimally invasive esophagectomy (MIE) is a well-described source of morbidity for patients undergoing surgical treatment of esophageal neoplasm. With improved early recognition and endoscopic management techniques, the long-term impact remains unclear. Method(s): A retrospective review was conducted of patients who underwent MIE for esophageal neoplasm between January 2015 and June 2021 at a single institution. Cohorts were stratified by development of AL and subsequent management. Baseline demographics, perioperative data, and post-operative outcomes were examined. Result(s): During this period, 172 MIEs were performed, with 35 of 172 (20.3%) complicated by an AL. Perioperative factors independently associated with AL were post-operative blood transfusion (leak rate 52.9% versus 16.8%;p = 0.0017), incompleteness of anastomotic rings (75.0% vs 19.1%;p = 0.027), and receiving neoadjuvant therapy (18.5% vs 30.8%;p < 0.0001). Inferior short-term outcomes associated with AL included number of esophageal dilations in the first post-operative year (1.40 vs 0.46, p = 0.0397), discharge disposition to a location other than home (22.9% vs 8.8%, p = 0.012), length of hospital stay (17.7 days vs 9.6 days;p = 0.002), and time until jejunostomy tube removal (134 days vs 79 days;p = 0.0023). There was no significant difference in overall survival between patients with or without an AL at 1 year (79% vs 83%) or 5 years (50% vs 47%) (overall log rank p = 0.758). Conclusion(s): In this large single-center series of MIEs, AL was associated with inferior short-term outcomes including hospital length of stay, discharge disposition other than to home, and need for additional endoscopic procedures, without an accompanying impact on 1-year or 5-year survival. Key message: In this large, single-center series of minimally invasive esophagectomies, anastomotic leak was associated with worse short-term outcomes including hospital length of stay, discharge disposition other than to home, and need for additional endoscopic procedures, but was not associated with worse long-term survival. The significant association between neoadjuvant therapy and decreased leak rates is difficult to interpret, given the potential for confounding factors, thus careful attention to modifiable pre- and peri-operative patient factors associated with anastomotic leak is warranted.Copyright © 2022 The Authors

Is sleep impaired in post-COVID syndrome vs. healthy controls? Preliminary data

Background: People with post-COVID syndrome (PCS) suffer from persisting symptoms, e.g. self-reported sleep disorders (31%). However, sleep has not yet been objectively measured by polysomnography (PSG) in PCS. Objective(s): To examine differences in sleep characteristics between PCS and healthy controls (HC). Method(s): People with PCS and HC were included in this prospective trial. All subjects performed baseline characteristics, 1-minute sit-to-stand test (STST), subjective impairments of sleep and a single night sleep assessment in a sleep lab via PSG and Whoop strap (digital health coach). Post-COVID functional scale (PCFS) and Ordinal Scale for Clinical Improvement (OSCI) were assessed only in PCS. Result(s): To date, 20 PCS patients (49+/-11y, FVC 87+/-12%pred., DLCO: 81+/-19%pred., PCFS: 2.8+/-0.9, OSCI: 2.7+/-1.3pts) and 3 HC (44+/-9y, FVC 92+/-3%pred., DLCO 105+/-25%pred.) completed the trial. Only PCS patients reported an impaired sleep ("Is your sleep impaired since COVID?" [yes: 89%]) mainly due to insomnia in the middle of the night (61%). Total sleep time and the distribution of sleep stages (light, deep, REM) were comparable between PCS and HC. The REM latency trended to be longer in PCS vs. HC (114+/-51 vs. 52+/-17 min.). Apnea-hypopnea index (AHI) trended to be higher in PCS (8.9+/-8.5 vs. 0.9+/-1.2 events/h), 55.6% of PCS reported an AHI>5/h. The individual quality of recovery after the study night was classified to be "adequate" (PCS: 56+/-21%, HC: 52+/-15%). Conclusion(s): In PCS, sleep seems to be subjectively and objectively impaired compared to HC. A significant number of PCS patients (44%) was diagnosed with obstructive sleep apnea. Hence, measuring sleep might be an important diagnostic tool in the management of PCS.

Long COVID: Rapid Evidence Review

Postacute sequelae of COVID-19, also known as long COVID, affects approximately 10% to 30% of the hundreds of millions of people who have had acute COVID-19. The Centers for Disease Control and Prevention defines long COVID as the presence of new, returning, or ongoing symptoms associated with acute COVID-19 that persist beyond 28 days. The diagnosis of long COVID can be based on a previous clinical diagnosis of COVID-19 and does not require a prior positive polymerase chain reaction or antigen test result to confirm infection. Patients with long COVID report a broad range of symptoms, including abdominal pain, anosmia, chest pain, cognitive impairment (brain fog), dizziness, dyspnea, fatigue, headache, insomnia, mood changes, palpitations, paresthesias, and postexertional malaise. The presentation is variable, and symptoms can fluctuate or persist and relapse and remit. The diagnostic approach is to differentiate long COVID from acute sequelae of COVID-19, previous comorbidities, unmasking of preexisting health conditions, reinfections, new acute concerns, and complications of prolonged illness, hospitalization, or isolation. Many presenting symptoms of long COVID are commonly seen in a primary care practice, and management can be improved by using established treatment paradigms and supportive care. Although several medications have been suggested for the treatment of fatigue related to long COVID, the evidence for their use is currently lacking. Holistic treatment strategies for long COVID include discussion of pacing and energy conservation;individualized, symptom-guided, phased return to activity programs;maintaining adequate hydration and a healthy diet;and treatment of underlying medical conditions.Copyright © 2022 American Academy of Family Physicians.

Prevalence of sleep-related breathing disorders in longCOVID

Background: Following a SARS-CoV-2 infection, symptoms such as fatigue, shortness of breath, or thoracic pain may persist for months after the illness (long-COVID). We investigated the prevalence of sleep-related breathing disorders (SRBD) in long-COVID. Patients and Methods: Long-COVID patients who underwent inpatient rehabilitation and received diagnostic polysomnography or polygraphy were included in this retrospective data analysis. Result(s): 70 patients (33 women) were included, 4 with pre-existing obstructive sleep apnea (OSA). Among the remaining 66 patients, 22 (33%) had SRBD (4 with central sleep apnea and 18 with OSA). Thus, overall SRBD prevalence was 26/70 (37%). Patients with SRBD were significantly older and showed a higher proportion of men. In addition, SRBD patients were more likely to require oxygen therapy, noninvasive ventilation, or invasive ventilation as part of their COVID-19-related hospitalisation prior to rehabilitation. View inline Conclusion(s): In Long-COVID, SRBD prevalence in our sample is 33% and 37%, respectively, which is higher than average. The diagnostic portfolio in Long-COVID should therefore include screening for the presence of SRBD. SRBD patients show a more intensive need for therapy in the context of their COVID-19-associated hospitalization, although this may be attributed to older age.

Single-centre prospective clinical and procedural analysis identifying causes of delay to haematopoietic stem cell transplant in MPS I (Hurler syndrome) patients

The international standard treatment for mucopolysaccharidosis type I - Hurler syndrome (MPS1H) is haemopoietic stem cell transplant (HSCT) preceded by intravenous enzyme replacement therapy (ERT), with HSCT ideally undertaken before 18 months age to achieve best outcome. The invasive nature and high risk of morbidity and mortality associated with HSCT, in addition to a complex patient cohort, demands an extensive pre-transplant work-up to minimise risks where avoidable. This is achieved by collaboration between transplant and specialist paediatric LD-metabolic services. Transplant may be delayed due to clinical complications pre-transplant, but non-clinical disruptions have also been encountered in practice causing delays from time of diagnosis to transplantation. This work aimed to identify clinical complications and non-clinical disruptions in this process, and to identify areas of improvement for clinical practice, ultimately to achieve timely intervention and optimise clinical outcomes. A single-centre prospective clinical and procedural analysis of 7 MPS1H patients undergoing HSCT between April 2020 - January 2021 was completed. Age at diagnosis (median(range)) was 10 (1.5–25) months. Time from diagnosis to starting ERT (median(range)) was 10 (3–26) days. Time from diagnosis to transplant (median(range)) was 158 (101–189) days, with age at transplant 14 (6.5–30) months. Multiple reasons causing delay were identified. Clinical factors included presence of dilated cardiomyopathy, requirement for adenotonsillectomy to treat obstructive sleep apnoea, Covid-19 infection, vascular device infection, and acute neurosurgical issues including hydrocephalus requiring ventriculoperitoneal shunt and cervical spine stenosis requiring decompression. Non-clinical factors identified included late cancellation of required investigations, missed clinic appointments, and issues with accessing HSCT donors due to UK/European political situation and Covid-19 restrictions. Clear communication between teams was found to be a key identifying factor in ensuring timely completion of the pre-HSCT.

Cancer, Covid and Control of Ra - a Toxic Combination?

Case report - Introduction: This case highlights the dilemma of keeping rheumatoid arthritis disease under control in active cancer cases and establishing a consistent multidisciplinary dialogue during a pandemic and staffing crises. During chemotherapy and active cancer treatment, disease-modifying therapies (conventional and biologic) are often stopped. In some cases, the potential benefits versus risks of restarting usual therapies have to be balanced against risks of suppressing disease activity with highdose steroids. Risks of infection (common and atypical) need to be considered. Case report - Case description: A is a 67-year-old female nonsmoker diagnosed with seropositive rheumatoid arthritis (RF, anti - CCP positive) in 2008. Other conditions include type 2 diabetes, atrial fibrillation (on warfarin), hypothyroidism and obstructive sleep apnoea. Due to active disease, despite triple therapy (methotrexate, sulphasalazine and hydroxychloroquine), anti-TNF therapy (etanercept) commenced in 2009 with primary non-response. However, she responded well to B-cell therapy (rituximab) in conjunction with oral methotrexate (25mg weekly) receiving annual infusions from 2010 to 2016. In 2017, an elective sleeve gastrectomy procedure for high BMI was abandoned after peritoneal deposits of concern were noted. Histology and CT imaging were consistent with a primary peritoneal malignancy (Stage 3c low-grade serous adenocarcinoma). Treatment involved debulking surgery (total abdominal hysterectomy, bilateral salpinoophorectomy, omentectomy) and tamoxifen. Treatment for rheumatoid arthritis stalled during this period but as frequent steroids were required for active joint inflammation, in agreement with the oncologists, she had a rituximab cycle in 2018. Unfortunately, in 2019 she had signs of cancer progression (elevated tumour markers, CT imaging) and has subsequently started carboplatin chemotherapy. She has been unable to continue methotrexate or rituximab pending completion of the chemotherapy cycles (ongoing). However, her arthritis is now uncontrolled without increased steroids. Due to recurrent flares, her maintenance dose has been increased from 5mg to 7.5-10mg prednisolone daily until we can establish if it is safe and appropriate to recommence her usual arthritis regime. Even without disease-modifying therapy like methotrexate and rituximab, risk of infection (including atypical ones) is still significant with the combination of chemotherapy and steroids. Risk of progressive joint damage and adverse quality of life with active arthritis also needs to be considered. Staffing crises, exacerbated by COVID pandemic issues, have added to complexity of decision making and coordination of regular multidisciplinary discussions regarding treatment. Case report - Discussion: Cancer is a known association in rheumatoid arthritis patients with a twofold higher risk of lymphoma compared to the general population. Whether condition or treatment affects risk remains unclear as immune dysregulation is relevant in both autoimmunity and cancer. Paraneoplastic, recent onset arthritis, chemotherapy- or immunotherapy-induced arthralgia/arthritis are also well documented. This case had a seropositive rheumatoid arthritis phenotype quite a few years prior to cancer diagnosis. Primary peritoneal cancer is uncommon, often presenting as in this case as an incidental finding. It is usually treated like ovarian cancer Whilst methotrexate has been implicated in lung cancer, melanoma and non-Hodgkin lymphoma, overall safety data suggest any risk is quite low (e.g., EBV-associated lymphoproliferative disorders usually resolve with drug discontinuation). It is also a known chemotherapeutic agent. Anti-TNF treatment algorithms generally exclude patients with recent cancer. Rituximab, originally developed as a cancer drug, is not thought to affect risk of cancer development or progression. Treatment with disease-modifying therapy (conventional and biologics) is often withheld in patients with active malignancy undergoing chemotherapy due to a theo etical risk of potentiated immunosuppression and toxicity, particularly cytopaenias. However, maintaining arthritis control with glucocorticoids also has short- and long-term risks. Combining chemotherapy agents like carboplatin with methotrexate has been used for urothelial carcinoma and can be well tolerated with close monitoring of haematological parameters. Thus, it could be argued this patient is at risk of infections whichever treatment approach is taken and regaining control of arthritis with recommencement of methotrexate and rituximab is much better for her quality of life. Regular multidisciplinary discussions are important to outline risks versus benefits of combined treatment. This may be difficult in practice during staffing crises. Covid risk in patients receiving rituximab and/or chemotherapy, timing and response to COVID vaccination are also important considerations. Case report - Key learning points: . Primary peritoneal cancer is uncommon and can present as an incidental finding . Whilst treatment for progressive cancer is important, withholding rheumatoid arthritis treatment can have a significant adverse impact on quality of life . Morbidity and mortality risks of stopping treatment versus combined treatment (cancer therapy and disease-modifying therapy) ideally needs to be fully discussed and agreed with the patient and all care providers - lack of "named" providers, restructuring, redeployment, multi-specialty care and a global pandemic can make coordination of this difficult.