National Referral Center Research Articles

Increasing demand for ophthalmic pathology: time trends in a laboratory with nationwide coverage

PurposeTo report the time trends in basic patient characteristics and the number of specimens received at a national referral center for ophthalmic pathology.MethodsData on patient sex, age at surgical resection and geographical location of the referring unit were obtained for all specimens received at the St. Erik Ophthalmic Pathology laboratory, Stockholm, Sweden, between January 1st, 1959, and December 31st, 2021.ResultsA total of 33 057 specimens had been received, of which 14 560 (44%) came from men and 18 477 (56%) from women (for 20 patients, the sex was not specified). The average annual percent change (AAPC) in the number specimens received was + 10.5%, whereas the Swedish population increased with 0.5% per year. Patients became older throughout the period, with an average yearly increase of patient age at surgery of 0.3 years (AAPC 0.2%). Overall, women were three years older than men at surgery (59.4 versus 56.4 years, P < 0.0001) The number of specimens increased with patient age from the first to the 8th decade, after which it decreased to zero in the 11th decade. The largest portion of patients had undergone their surgery in one of the hospitals or clinics in the capital region, with four of the five largest sources corresponding to the most populous counties in the country.ConclusionsDuring six decades, the growth in number of specimens sent to our national referral center for ophthalmic pathology has greatly outpaced the growth of the population, indicating an increasing demand for subspecialized services. Throughout the period, patients have become older, and a higher number of specimens have been submitted from female patients.

Impact of patient ancestry on heterogeneity of Sjögren’s disease

ObjectivesWe aimed to compare disease characteristics between primary Sjögren’s syndrome (pSS) patients of African ancestry (AA) and Caucasian ancestry.MethodsWe conducted a retrospective, case–control study in a French national and European...

Risk factors for upper urinary tract deterioration in adult patients with spina bifida.

Neurogenic bladder associated with spina bifida disease remains a major cause for mortality or morbidity due to kidney damages. However, we currently do not know which urodynamic findings are associated with an higher risk of upper tract damages in spina bifida patients. The objective of the present study was to evaluate urodynamic findings associated with functional kidney failure and/or with morphological kidney damages. A large single-center restrospective study was conducted in our national referral center for spina bifida patients using our patients' files. All urodynamics curves were assessed by the same examinator. Functional and/or morphological evaluation of the upper urinary tract were done at the same moment as the urodynamic exam (between 1week before and 1month after). Kidney function was assessed using creatinine serum levels or 24h urinary creatinine levels (creatinine clearance) for walking patients, or with the 24h urinary creatinine level for wheelchair-users. We included 262 spina bifida patients in this study. Fifty-five patients had a poor bladder compliance (21.4%) and 88 of them had detrusor overactivity (33.6%). Twenty patients had a stage 2 kidney failure (eGFR < 60ml/min) and 81 patients out of 254 (30.9%) had an abnormal morphological examination. There were three urodynamic findings significantly associated with UUTD: bladder compliance (OR = 0.18; p = 0.007), Pdetmax (OR = 14.7; p = 0.003) and detrusor overactivity (OR = 1.84; p = 0.03). In this large series of spina bifida patients, maximum detrusor pressure and bladder compliance are the main urodynamic findings determinants of UUTD risk.

Sex Differences in the Treatment of Uveal Melanoma in a Group of 1336 Patients

(1) Background: The aim of this study was to analyze the sex differences in the treatment of uveal melanoma (UM) in a group of 1336 patients from a national referral center during the period 2018-2021. (2) Materials and Methods: The study was designed in a retrospective manner. A total of 1336 patients who were newly diagnosed with UM at the Department of Ophthalmology and Ophthalmic Oncology, Jagiellonian University Collegium Medicum, Krakow, Poland, between 1 January 2018 and 31 December 2021, were included in the study. The demographic and clinical data were compiled, including the sex of patients and the treatment methods. (3) Results: In total, 1336 patients with ocular melanoma were identified, including 726 women (54.34%) and 610 men (45.66%). A total of 49.70% of tumors were localized in the right eye and 50.30% in the left eye. UMs were localized statistically significantly more frequently posterior to the equator of the eye globe in men than in women (79.67% vs. 74.10%, Chi^2 Pearson test p = 0.035). Tumors tended to be larger in men, but this difference was not clinically significant. Men were enucleated more often than women (23.44% vs. 18.04%, Chi^2 Pearson test p = 0.015). (4) Conclusions: Statistically significant sex differences were found in the treatment of uveal melanoma in a national referral center in Poland, with men being enucleated more often than women.

Stroke Recurrence among Stroke Patients Referred for Driving Assessment and Rehabilitation: A Cohort Study.

Returning to driving is one of the priorities for stroke survivors. However, the fear of the risk of recurrent stroke has led to concern about allowing driving post-stroke. This study was performed to study the impact of various vascular risk factors on stroke recurrence among drivers referred to our national referral center for Driving Assessment and Rehabilitation Program (DARP). Medical records of subjects who were diagnosed to have a stroke and were referred to DARP were retrospectively reviewed. Data on demographics (age and gender) and vascular risk factors (hypertension-HT, diabetes mellitus-DM, hyperlipidemia-HL, cigarette smoking-SM, previous stroke-PS, and heart disease-HD) were collected. Subjects were contacted and records scrutinized for a report of recurrent stroke. A total of 133 subjects were recruited, median 54 years (range 20-77 years), 95.5% male, 59.4% had HT, 32.3% DM, 65.4% HL, 43.6% SM, 3.8% PS, and 8.3% HD. Over a median follow-up of 30 months (range 1-78 months), the recurrence rate of stroke was 11.3%, 3.69/100 patient-years. On uni-variable analysis, the risk of stroke recurrence rose with age (HR 1.08, 95%CI 1.02-1.15, p = 0.01) and heart disease (HR 5.77, 95%CI 1.46-22.83, p = 0.01). On multivariable analysis, only age remained significant (HR 1.07, 95%CI 1.00-1.13, p = 0.045). Among those aged > 60 years, the HR was 3.88 (95%CI 1.35-11.20, p = 0.012). The risk of stroke recurrence is higher among older drivers and is not influenced by other vascular factors.

Allogeneic platelet gel therapy for refractory abdominal wound healing: A preliminary study.

Refractory abdominal wounds are commonly complicated by surgical site infections, which prolong hospital stays and increase medical costs. There is little clinical data on the use of allogeneic platelet gel (PG) therapy for refractory infected wounds. This study aimed to evaluate the efficacy and safety of allogeneic PGs in the treatment of refractory abdominal wounds. A prospective single-center study was performed in a national abdominal trauma referral center between June 2019 and June 2021. A total of 11 patients with refractory abdominal wounds were treated with allogeneic PGs after the failure of standard medical treatments. The PGs were derived from platelets collected from healthy donors using apheresis, and each PG was tested for platelet count, transfusion-related diseases, aerobic and anaerobic bacteria, and growth factor concentration. Clinical efficacy was evaluated by assessing the wound surface and observing the condition of the wound, including wound area and percentage of granulation. The median age of the patients was 37 years (1st quartile, 3rd quartile (Q1, Q3): 31-55 years), median (Q1, Q3) hemoglobin level was 95 g/L (78-120 g/L) and median (Q1, Q3) serum albumin level was 39.9 g/L (34.9-42.7 g/L). The PG platelet count was 976.5 ±174.9×109/L. Results of transfusion-associated contagion tests for aerobic and anaerobic bacteria were negative. Growth factor contents (pg/mL) were: for transforming growth factor beta 1 (TGF-β1); 2542.39 ±430.60, for platelet-derived growth factor BB (PDGF-BB); 23230.03 ±4236.14 and FOR vascular endothelial growth factor (VEGF); 91.41 ±23.31. The rate of wound healing was 100%, and the median (Q1, Q3) healing time was 30 days (18-40 days). The follow-up period was 5-27 months, during which no recurrence of the wounds was found. The present study demonstrated that allogeneic PGs are a safe and effective treatment for refractory abdominal wounds.

Associated factors with the knowledge level about primary open angle glaucoma at a national referral center in Peru

Associated factors with the knowledge level about primary open angle glaucoma at a national referral center in Peru

Prevalence of genetically confirmed skeletal muscle channelopathies in the era of next generation sequencing

We provide an up-to-date and accurate minimum point prevalence of genetically defined skeletal muscle channelopathies which is important for understanding the population impact, planning for treatment needs and future clinical trials. Skeletal muscle channelopathies include myotonia congenita (MC), sodium channel myotonia (SCM), paramyotonia congenita (PMC), hyperkalemic periodic paralysis (hyperPP), hypokalemic periodic paralysis (hypoPP) and Andersen- Tawil Syndrome (ATS). Patients referred to the UK national referral centre for skeletal muscle channelopathies and living in UK were included to calculate the minimum point prevalence using the latest data from the Office for National Statistics population estimate. We calculated a minimum point prevalence of all skeletal muscle channelopathies of 1.99/100 000 (95% CI 1.981–1.999). The minimum point prevalence of MC due to CLCN1 variants is 1.13/100 000 (95% CI 1.123–1.137), SCN4A variants which encode for PMC and SCM is 0.35/100 000 (95% CI 0.346 – 0.354) and for periodic paralysis (HyperPP and HypoPP) 0.41/100 000 (95% CI 0.406–0.414). The minimum point prevalence for ATS is 0.1/100 000 (95% CI 0.098–0.102). There has been an overall increase in point prevalence in skeletal muscle channelopathies compared to previous reports, with the biggest increase found to be in MC. This can be attributed to next generation sequencing and advances in clinical, electrophysiological and genetic characterisation of skeletal muscle channelopathies.

The synovial fluid calprotectin lateral flow test for the diagnosis of chronic prosthetic joint infection in failed primary and revision total hip and knee arthroplasty

PurposeThe diagnostic criteria of prosthetic joint infection (PJI) recommended by the most commonly used diagnostic algorithms can be obscured or distorted by other inflammatory processes or aseptic pathology. Furthermore, the most reliable diagnostic criteria are garnered during revision surgery. A robust, reliable addition to the preoperative diagnostic cascade is warranted. Calprotectin has been shown to be an excellent diagnostic marker for PJI. In this study, we aimed to evaluate a lateral flow test (LFT) in the challenging patient cohort of a national referral centre for revision arthroplasty.MethodsBeginning in March 2019, we prospectively included patients scheduled for arthroplasty exchange of a total hip (THA) or knee arthroplasty (TKA). Synovial fluid samples were collected intra-operatively.We used the International Consensus Meeting of 2018 (ICM) score as the gold standard. We then compared the pre-operative ICM score with the LFT result to calculate its diagnostic accuracy as a standalone pre-operative marker and in combination with the ICM score as part of an expanded diagnostic workup.ResultsA total of 137 patients with a mean age of 67 (± 13) years with 53 THA and 84 TKA were included. Ninety-nine patients (72.8%) were not infected, 34 (25.0) were infected, and four (2.9%) had an inconclusive final score and could not be classified after surgery.The calprotectin LFT had a sensitivity (95% confidence interval) of 0.94 (0.80–0.99) and a specificity of 0.87 (0.79–0.93). The area under the receiver operating characteristic curve (AUC) for the calprotectin LFT was 0.94 (0.89–0.99). In nine cases with an inconclusive pre-operative ICM score, the calprotectin LFT would have led to the correct diagnosis of PJI.ConclusionsThe synovial fluid calprotectin LFT shows excellent diagnostic metrics both as a rule-in and a rule-out test, even in a challenging patient cohort with cases of severe osteolysis, wear disease, numerous preceding surgeries, and poor soft tissue conditions, which can impair the common diagnostic criteria. As it is available pre-operatively, this test might prove to be a very useful addition to the diagnostic algorithm.

Hematopoietic Stem Cells and Metabolic Deterioration in Alström Syndrome, a Rare Genetic Model of the Metabolic Syndrome.

Alström syndrome (AS) is a rare genetic disease caused by ALMS1 mutations, characterized by short stature, and vision and hearing loss. Patients with AS develop the metabolic syndrome, long-term organ complications, and die prematurely. We explored the association between AS and a shortage of hematopoietic stem/progenitor cells (HSPCs), which is linked to metabolic diseases and predicts diabetic complications. We included patients with AS at a national referral center. We measured HSPCs with flow cytometry at baseline and follow-up. We followed patients up to January 2022 for metabolic worsening and end-organ damage. We evaluated HSPC levels and mobilization as well as bone marrow histology in a murine model of AS. In 23 patients with AS, we found significantly lower circulating HSPCs than in healthy blood donors (-40%; P = .002) and age/sex-matched patients (-25%; P = .022). Longitudinally, HSPCs significantly declined by a further 20% in patients with AS over a median of 36 months (interquartile range 30-44). Patients with AS who displayed metabolic deterioration over 5.3 years had lower levels of HSPCs, both at baseline and at last observation, than those who did not deteriorate. Alms1-mutated mice were obese and insulin resistant and displayed significantly reduced circulating HSPCs, despite no overt hematological abnormality. Contrary to what was observed in diabetic mice, HSPC mobilization and bone marrow structure were unaffected. We found depletion of HSPCs in patients with AS, which was recapitulated in Alms1-mutated mice. Larger and longer studies will be needed to establish HSPCs shortage as a driver of metabolic deterioration leading to end-organ damage in AS.

Developmental dysplasia of the hip is common in patients undergoing total hip arthroplasty under 50years of age.

Developmental dysplasia of the hip (DDH) refers to congenital and/or developmental hip instability that can result in hip joint subluxation or dislocation. When detected neonatally, conservative treatment with hip bracing can restore normal hip anatomy. Missed detection of DDH in the neonatal period or late development of DDH often requires surgical intervention to correct the abnormal anatomy. Furthermore, despite surgical intervention, residual sequelae may persist leading to early osteoarthritis of the hip joint requiring joint replacement surgery. This study investigates the prevalence of hip dysplasia in patients undergoing total hip arthroplasty (THA) under 50years of age. The hip arthroplasty database at a national referral centre was investigated from January 2014 to December 2020. In patients under 50years of age, those with an adequate pre-operative anteroposterior pelvic radiograph without previous hip arthroplasty were included, while those with inadequate radiographs were excluded. The following measurements were made on the contralateral non-operated hip: (1) lateral centre-edge angle (LCEA), (2) Tönnis angle, (3) acetabular version, (4) acetabular depth, (5) femoral head lateralisation, (6) femoral head extrusion index, and (7) acetabular depth-to-width ratio. In total, 451 patients were included in this study. Twenty two percent of the patients had hip dysplasia, based on a LCEA of<25° and 42.6% of patients had hip dysplasia, based on a Tönnis angle of>10°. The mean LCEA and Tönnis angle were 31.47±9.64 and 9.82±6.79°, respectively. Hip dysplasia is common in patients undergoing THA under the age of 50years with over 40% having dysplasia according to the Tönnis angle. Classification of primary and secondary osteoarthritis in the joint registries will benefit our knowledge on the prevalence of DDH in the adult population.

Risk factors associated with COVID-19 morbidity and mortality at a national tertiary referral treatment centre in Ghana: a retrospective analysis

Introduction: there is a substantial variation in COVID-19 case fatality rates across different locations, which may be due to differences in population age structure, patient factors, or health system factors. The study evaluated the clinical features and risk of COVID-19 morbidity and mortality among confirmed cases at COVID-19 referral treatment centre.

Healthcare Encounters and Outcomes of Filipino Patients with Systemic Lupus Erythematosus Managed at a Tertiary COVID-19 Referral Center.

The coronavirus disease 2019 (COVID-19) pandemic has limited healthcare delivery for patients with chronic diseases, including Systemic Lupus Erythematosus (SLE). This study aims to describe the outcomes of patients with SLE in a national COVID-19 referral center in the Philippines. A review of records of all adult patients with SLE seen in the University of the Philippines-Philippine General Hospital (UP-PGH) from March 2020 to December 2021 was done. Data about patient characteristics, health encounters, and outcomes before and after the first visit during the study period were extracted. Descriptive statistics were employed. Our population of 403 patients was predominantly young (mean age 34.53 ± 11.14 years), female, and unemployed. This consisted of 370 known cases of SLE, 92 were diagnosed in institutions outside UP-PGH, and 33 new patients. Over the 22-month study period, there were 2,093 medical encounters, most of which were teleconsultations (81.70%). During an average gap of 53.6 ± 26.7 weeks between the last consultation and the first visit within the pandemic study period, 84 patients (22.70%) discontinued at least one of their SLE control medications, 68 (18.38%) patients developed a lupus flare, and 79 (21.35%) were hospitalized for various reasons. On their return to the rheumatology clinic during the pandemic, 37.47% were in lupus flare, 28.29% needed to be hospitalized, and 20 died. However, 86.75% of flares were controlled. During subsequent health encounters, 48 patients had a new flare (43 of these were controlled) and 20 died. The most common reason for hospitalization (n=160) was lupus disease flare and the most common cause of death (n=40) was pneumonia. Sixty patients acquired COVID-19 infection from which most recovered and four died. Audio teleconsultation was the most common method used by our lupus cohort to interact with their doctors during the pandemic. There was an average of a year-long interruption in medical care for 62.70%. More than a third developed a disease flare and 15% acquired COVID-19 but outcomes were good in more than 85%. Despite the challenges posed by the pandemic, the majority of our lupus cohort who were able to continue their treatment had favorable outcomes.

Vision-Related Quality of Life among Patients Attending the Diabetes and Eye Clinics in Kenyatta National Hospital, Kenya.

Our main objective was to determine the overall vision-related quality of life (VRQoL) among patients with diabetes mellitus attending the diabetes and eye clinics in Kenyatta National Hospital, Kenya. Analytical cross-sectional study conducted in December 2020 setting: This study was performed at the Diabetes and Eye Clinics in Kenyatta National Hospital, the main national referral centre in Nairobi, Kenya. Participants. Using a purposive consecutive sampling method, we enrolled 100 participants, 50 with diabetic retinopathy and 50 without diabetic retinopathy. Main Outcomes and Measures. We compared the VRQoL of participants with diabetic retinopathy with those without diabetic retinopathy and assessed whether VRQoL worsened with increasing the severity of diabetic retinopathy. VRQoL was assessed using the World Health Organization/Prevention of Blindness and Deafness Vision Function-20 Questionnaire (VF-20). With this tool, the higher the mean score, the worse the quality of life. Diabetic retinopathy was graded using the Early Treatment of Diabetic Retinopathy Study. VRQoL trend with DR were analysed using the worse eye. Participants with diabetic retinopathy had worse overall total VRQoL mean score (33.4, SD11.5) than those without (26.9, SD 4.7) in all domains; overall self-rating, 2.6 vs. 2.2, p < 0.001; general functioning, 18.0 vs. 14.7, p=0.005; psychosocial, 6.7 vs. 5.3, p < 0.001; and visual symptoms, 6.1 vs. 4.8, p < 0.001. VRQoL was worse with increasing severity of diabetic retinopathy in all domains moving from mild NPDR to moderate NPDR, severe NPDR and PDR, overall self-rating (2.2, 2.5, 3.5, 3.3; p < 0.001); visual symptoms (5.6, 5.6, 7.5, 7.4; p=0.002); psychosocial (5.7, 6.5, 6.0 8.8; p=0.004); and general functioning (15.7, 16.9, 17.5 23.6; p=0.014). Presence of DR, distance vision impairment, and diabetic macula oedema were associated with low overall self-rating. Conclusion and Relevance. Our findings underscore the need for interventions for early detection and management of diabetic retinopathy to prevent developing more advanced DR and its associated deterioration of VRQoL.

Impact of Pregnancy on the Prognosis of COVID-19 in Women Hospitalized at the National Reference Center for Patients Infected with SARS-CoV-2 in a Resource-limited Country

Aim: The aim of the study was to reduce morbidity and mortality associated with COVID-19 in pregnant women. Background: Since the detection of the first case of COVID-19 on March 6, 2020, in Togo, pregnant women have received special attention due to their usual vulnerability to infection. Objective: The objective of this study was to evaluate the influence of pregnancy on the prognosis of COVID-19 in patients hospitalized in Lomé. Methods: This was an analytical cross-sectional study of women of childbearing age (15-49 years) admitted between March 22, 2020, and December 31, 2021, to the Lomé Commune Regional Hospital, a national referral center for COVID-19 patients. Results: We registered 438 women of childbearing age, including 31 pregnant women (7.1%). Pregnant women were younger (28.8 years vs. 34.2 years, p = 0.001). Asthenia was more common in pregnant women (38.7% vs. 20.6%, p = 0.025), and SpO2 was lower (88.6% vs. 94%, p= 0.016%). Pregnancy was not associated with the occurrence of severe forms nor with prolonged hospitalization. Independent risk factors for mortality were 3rd trimester of pregnancy, mean age &gt; 34 years, diabetes, HIV, and obesity. Conclusion: Most symptoms were similar to those observed in the general population. However, in addition to comorbidities, complications in the third trimester of pregnancy have worsened the prognosis for COVID-19. These results corroborate the observations made in the subregion. However, it is important to assess the effect of COVID-19 on pregnancy outcomes.

Quality of Life and Its Associated Factors in Patients After Esophagectomy at a Single National Referral Center

Introduction. Esophagectomy is the standard surgical treatment for resectable esophageal cancer patients. However, the success rate for this procedure was about 25–35% and was associated with a severe risk of postoperative complications. In addition, patients after esophagectomy have decreased their quality of life (QOL), but no research has been done in Indonesia. Therefore, this study was conducted to determine the quality of life after esophagectomy in Indonesia based on the patient population at Dr. Cipto Mangunkusumo General Hospital (CMGH).

965 PROGNOSTIC VALUE OF CARDIOPULMONARY EXERCISE TESTING IN PATIENTS WITH TRANSTHYRETIN CARDIAC AMYLOIDOSIS

Abstract Background ATTR-CA is associated with a progressive reduction in functional capacity. The prognostic role of CPET parameters and in particular of normalized peak VO2 (%ppVO2) remains to be thoroughly evaluated. Methods In this study 75 patients with ATTR-CA underwent cardiological evaluation and CPET in a National Referral Center for cardiac amyloidosis (Careggi University hospital, Florence). Results Fifty-seven patients (76%) had wild type ATTR. Median age was 80 (75-83) years, 68 patients (91%) were men. Peak oxygen consumption (14.1±4.1 ml/kg/min) and %ppVO2 (68.4±18.8%) were blunted. Twenty-seven (36%) patients had an abnormal pressure response to exercise. After a median follow-up of 25 (12-31) months the composite outcome of death or heart failure hospitalization was registered in 19 (25.3%) patients. At univariate analysis %ppVO2 was a stronger predictor for the composite outcome than peak VO2. %ppVO2 and NTproBNP remained associated with the composite outcome at multivariate analysis. The optimal predictive threshold for %ppVO2 was 62% (sensitivity: 71%; specificity: 68%; AUC:0.77, CI: 0.65-0.88). Patients with %ppVO2 ≤ 62%and NTproBNP &amp;gt;3000pg had the worse prognosis with 1- and 2-year survival of 69±9% and 50±10%, respectively. Conclusions CPET is a safe and useful prognostic tool in patients with ATTR-CA. CPET may help to identify patients with advanced disease that may benefit of targeted therapy.

751 CANCER TREATMENT RELATED COMPLICATIONS IN PATIENTS WITH HYPERTROPHIC CARDIOMYOPATHY

Abstract Introduction Cardiotoxicity involves direct effects of cancer related treatment on heart function, commonly presenting as left ventricular contractile dysfunction. However, limited data are available regarding cardiotoxicity on hypertrophic cardiomyopathy (HCM), because most studies and registries have not specifically analyzed the effects of oncological treatment in HCM populations. This gap in knowledge may lead to unjustified restriction of HCM patients to radical cancer treatments. Aim We aimed at describing the potential clinical cardiotoxicity of oncological treatments in a cohort of consecutive HCM patients, systematically followed up at two national referral centres for HCM. Methods We retrospectively analysed all clinical and instrumental data of all consecutive HCM patients who underwent oncological treatment between January 2000 and December 2020, collected in a centralized database. Results Of 3256 HCM patients, 121 (3.7%) had a reported diagnosis of cancer; 109 (90.1%) patients underwent oncological surgery, 45 (37.2%) chemotherapic treatment, 22 (18.2%) chest radiotherapy. After a median follow up of 5 (2-13) years from oncological diagnosis, 32 of the 121 patients (26.4%) died. The cause of death was mainly attributed to the oncological condition, while 4 (12.5%) patients died of sudden cardiac death without previous chemotherapy or chest radiotherapy. No patient interrupted or reduced the dose of oncological treatment due to cardiac dysfunction. No sustained ventricular tachyarrhythmia was induced by chemotherapy or radiotherapy. Conclusions Cancer treatment is well tolerated in HCM patients. In our oncology series, none died of cardiovascular complications induced by chemotherapy or chest radiotherapy and none required interruption or substantial tapering of treatment due to cardiovascular side effects. Although expert multidisciplinary evaluation is mandatory and regimens must be tailored individually, the diagnosis of HCM per se should not be considered a contraindication to radical cancer treatment.

Clinical efficacy of implementing a Patient Blood Management (PBM) Protocol in joint replacement surgery: a retrospective cohort study in a national referral center.

The aging of population has dramatically broadened the total number of Total Hip Arthroplasty (THA) and Total Knee Arthroplasty (TKA) performed worldwide. To optimize the number of blood transfusions performed, a multimodal and multidisciplinary approach was introduced, called Patient Blood Management (PBM). The aim of the present retrospective study is to evaluate the feasibility and clinical outcomes of a PBM protocol applied in a national referral center for joint replacement surgery. Clinical reports of 9,635 patients undergoing primary THA or TKA, from 2014 to 2019, were screened. The trends of hemoglobin value at admission and at day 4 after surgery were analyzed. Furthermore, the trend of blood bags' requests and blood transfusions was longitudinally evaluated to assess the efficacy of our PBM protocol and its potential impact in reducing the length of stay in the hospital. In 2014, mean hemoglobin (Hb) levels at postoperative day 4 were 10.3 g/dl and 10.2 g/dl for TKA (unilateral and bilateral, respectively), and in 2019 were 11.3 g/dl and 11.6 g/dl (unilateral and bilateral, respectively, p=0.001). Total requested red blood cell (RBC) transfusions by each surgery over time have decreased for THA (277 in 2014 vs. 120 in 2019, p=0.001). A correlation matrix analysis between Hb level, body mass index (BMI), age, days spent in orthopedic (OR) ward and number of requested transfusions showed that RBC bags transfusions were related to the length of the hospital stay. A timely application of a PBM protocol in the perioperative period of TKA and THA was significantly associated to the reduction of blood transfusions and total length of hospital stay, with clear benefits for both the patients and the hospital.

Impact of COVID-19 Pandemic on Disease Control Status and Quality of Life of Patients with Acromegaly.

Background and Objectives: Despite the best efforts of healthcare workers and the deployment of alternative healthcare delivery solutions through telemedicine, the pandemic has disrupted standard care for patients with chronic conditions. The long-lasting pandemic has also had a profound impact on the quality of life (QoL) of the majority of patients with chronic illnesses. The management of rare diseases has been particularly challenging. We aimed to evaluate the impacts that the long-lasting pandemic had on the disease control status and QoL in patients with acromegaly. Materials and Methods: Our prospective study included 34 patients from a national referral centre. The baseline SAGIT and AcroQoL results were obtained in October 2020 during the lockdown period of the SARS-CoV2 pandemic. The follow-up results were assessed during the summer of 2022 in a period without any public health restrictions. All the patients were additionally evaluated for their attitude towards preventative public health measures against SARS-CoV2 spread and required mask wearing during the pandemic. Results: By comparing assessments in 2020 during the lockdown period and 2022 post-lockdown, we observed some improvement in SAGIT subscores T and I, most likely reflecting treatment changes in a small number of patients. The global SAGIT score remained stable. QoL measurement by AcroQoL did not demonstrate any changes. There was a negative correlation between SAGIT subscore S and the AcroQoL results. We also noted that the group of patients with the most negative attitude toward public health measurements for preventing SARS-CoV2 spread had higher AcroQoL results than others. Conclusion: Our results showcase that the SARS-CoV2 pandemic, lasting over two years, did not impact the disease control status and QoL in patients with acromegaly. The cohort continued to be well controlled and without changes in QoL. We measured a relatively favourable attitude towards the public health measures to prevent the spread of SARS-CoV2; in particular, patients who had a lower QoL had more positive attitudes towards these measures.