To report our experience applying endovascular stent graft repair to treat ascending aortic diseases in high-risk patients and to evaluate the safety and feasibility of this approach. Patients with comorbid conditions or older age are not suitable for open procedures but may be considered suitable for ascending endovascular repair. Eleven high-risk patients received zone 0 thoracic endovascular aortic repair from September 2014 to May 2020. All patients were followed up until death or December 2021. Primary outcomes were in-hospital and long-term all-cause mortality as well as in-hospital and long-term aorta-related mortality. The mean follow-up duration of all patients was 35.78 months. The cohort comprised of three pathology subgroups: penetrating atherosclerotic ulcer (PAU) (n =6), acute dissection (AD) (n = 3), and chronic dissecting aneurysm (CDA) (n = 2). The in-hospital all-cause mortality rates were 0%, 33.33% and 0% for PAU, AD, and CDA groups, respectively. Long-term all-cause mortality were 33.33%, 33.33%, and 50% for PAU, AD, and CDA groups, respectively. There was only one in-hospital death related to acute aortic dissection, and no long-term aorta-related deaths occurred during the study period. During the follow-up time, the majority of patients had good remodeling of ascending aorta, slow progression in cases with endoleak, and no aorta-related mortality. Ascending endovascular aortic repair appears to be a safe and feasible procedure for emergent aortic repair in carefully selected patients with prohibitive surgical risk who are not candidates for open procedures.

BACKGROUND: Circadian dysregulation has long been thought to be a key component in the pathophysiology of bipolar disorder (BD). However, it remains unclear whether this dysregulation constitutes a risk factor, manifestation, or consequence of BD. This study aimed to compare dim light melatonin secretion patterns between unaffected offspring of parents with BD (OBD) and offspring of control parents (OCP).
METHODS: This case-control study included unaffected OBD (mean age 14.0 years; male 50.0 %) and age- and sex-matched OCP (mean age 13.0 years; male: 43.5 %). Seventeen saliva samples were collected in dim light conditions. Dim light melatonin onset (DLMO), phase angles, and area under the curve (AUC) were calculated.
RESULTS: 185 saliva samples from 12 OBD (n = 12) and 741 from OCP (n = 46) were collected. Unaffected OBD had a significant lower nocturnal melatonin level (14.8 ± 4.6 vs. 20.3 ± 11.7 pg/mL) and a smaller melatonin AUC within two hours after DLMO (35.5 ± 11.3 vs. 44.6 ± 18.1 pg/mL) but a significant larger phase angle between DLMO and sleep onset (2.2 ± 1.0 vs. 1.4 ± 1.2 h) than OCP. There was no significant between-group difference in DLMO. The graphic illustrations showed a considerably flattened melatonin secretion in unaffected OBD.
CONCLUSIONS: The main limitations include lack of 24-h dim melatonin secretion measurement, large age range of participants, and small sample size.
CONCLUSIONS: These findings suggest that unaffected OBD already presented with circadian rhythm dysregulations. Future investigations are needed to clarify the role of abnormal melatonin secretion in the onset of BD.

OBJECTIVE: Multiple myeloma (MM) is an incurable condition with variable clinical course. The study included a group of patients with especially poor-prognosis, individuals with relapsed/refractory multiple myeloma (RRMM) and specific cytogenetic disorders. Among the currently used therapies the ixazomib-lenalidomid-dexamethasone (IRd) is considered as a candidate to improve outcomes. The aim of the study was to evaluate the safety and efficacy of IRd regimen in the treatment of patients with RMMM.
METHODS: Nine patients aged 52-82 years who received ixazomib in the early access programme, were included in the study. All patients met the criteria for recurrent/relapsed MM and had high (t(4:14), t(14:16), del17p or +1q21) risk aberrations. Previous chemotherapy regimens included thalidomide and bortezomib. Median duration of exposure to ixazomib was 12 months.
RESULTS: One patient with multiple cytogenetic aberrations and extramedullary plasmocytoma died because of progression after two months of treatment. In the remaining patients, the objective response to treatment was reached, and in four cases it was qualified as a very good partial response (VGPR). Observed adverse effects included neutropenia, infections, and oedema (in three cases Grade 3). Eight patients continue treatment, in two cases the decision was made to reduce lenalidomide doses.
CONCLUSIONS: Preliminary results suggest potentially high efficacy and good safety profile of IRd therapy in patients with RRMM and unfavourable cytogenetics.

Gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal tract accounting for less than 1% of all gut tumors. GISTs occurring in the rectum are extremely rare and these usually present at an advanced stage compared with other sites. We report a case of a middle-aged female who presented with features of anemia and subacute obstruction due to a large rectal tumor and underwent abdominoperineal resection. The histopathological examination confirmed the diagnosis of high-grade malignant GIST with multiple lymph nodal metastasis. She was started on adjuvant imatinib therapy and is on follow-up without any evidence of recurrence. The authors conclude that GIST must be included in the differential diagnosis of a rectal tumor. Diagnosis is established by biopsy and immunohistochemistry studies. Surgical resection with histological negative margins is the standard curative treatment. Adjuvant targeted therapy can reduce long-term recurrence in high-risk cases.

The low incidence rates of prostatic extra-gastrointestinal stromal tumors (EGIST), combined with the lack of published guidelines on its treatment, often results in its misdiagnosis and challenges in the treatment of patients, even in cases with high-risk factors. The present case study reported a 65-years-old Chinese male patient, who presented with intermittent hematuria and lower urinary tract symptoms for three months. The colonoscopy results revealed no gastrointestinal lesions; however, a core biopsy diagnosed an EGIST, which subsequently underwent radical prostatocystotomy, standard pelvic lymph node resection, and bricker ileal conduit diversion. The postoperative pathological results suggested a high-risk primary prostatic EGIST, according to the aggressive behavior of the GIST. The immunohistochemistry results revealed the positive expression of CD117, DOG1, CD34, androgen receptor AR, prostate-specific antigen (PSA), a 2% Ki-67 index and a positive surgical margin. The whole exome sequencing (WES) results revealed that the patient harbored a single nucleotide mutation in 121 genes and copy number variations in 601 genes, including a defect in c-Kit (in-frame deletion in p.Q556-V560; fold, 17.5%). By compiling the data obtained from the ConsensusPathDB and the drug-gene interaction databases and expert opinions, the patient was prescribed with the personalized drugs (400 mg per day imatinib mesylate and 50 mg per day bicalutamide, which were stopped when the PSA levels remained stable below 0.01 ng/ml) for 18 months follow-up and there were no signs of recurrence. In conclusion, WES identified multiple genomic alterations and the underlying genetic defect in the rare case enabled the evaluation of the prognosis and the decision of potential drug candidates. The underlying mechanism of the substantial genetic variations in the primary prostatic EGIST, as well as the malignant behaviors of the tumor, remain to be investigated.

BACKGROUND: Coronavirus disease 2019 (COVID-19) is caused by the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), a novel coronavirus that was first discovered in December 2019 in Wuhan, China. With the growing numbers of community spread cases worldwide, the World Health Organization (WHO) declared the COVID-19 outbreak as a pandemic on March 11, 2020. Like influenza viruses, SARS-CoV-2 is thought to be mainly transmitted by droplets and direct contact, and COVID-19 has a similar disease presentation to influenza. Here we present a case of influenza A and COVID-19 co-infection in a 60-year-old man with end-stage renal disease (ESRD) on hemodialysis.
METHODS: A 60-year-old man with ESRD on hemodialysis presented for worsening cough, shortness of breath, and diarrhea. The patient first developed a mild fever (37.8 °C) during hemodialysis 3 days prior to presentation and has been experiencing worsening flu-like symptoms, including fever of up to 38.6 °C, non-productive cough, generalized abdominal pain, nausea, vomiting, and liquid green diarrhea. He lives alone at home with no known sick contacts and denies any recent travel or visits to healthcare facilities other than the local dialysis center. Rapid flu test was positive for influenza A. Procalcitonin was elevated at 5.21 ng/mL with a normal white blood cell (WBC) count. Computed tomography (CT) chest demonstrated multifocal areas of consolidation and extensive mediastinal and hilar adenopathy concerning for pneumonia. He was admitted to the biocontainment unit of Nebraska Medicine for concerns of possible COVID-19 and was started on oseltamivir for influenza and vancomycin/cefepime for the probable bacterial cause of his pneumonia and diarrhea. Gastrointestinal (GI) pathogen panel and Clostridioides difficile toxin assay were negative. On the second day of admission, initial nasopharyngeal swab came back positive for SARS-CoV-2 by real-time reverse-transcriptase polymerase chain reaction (RT-PCR). The patient received supportive care and resumed bedside hemodialysis in strict isolation, and eventually fully recovered from COVID-19.
CONCLUSIONS: We presented a case of co-infection of influenza and SARS-CoV-2 in a hemodialysis patient. The possibility of SARS-CoV-2 co-infection should not be overlooked even when other viruses including influenza can explain the clinical symptoms, especially in high-risk patients.

Thrombotic events are the most frequent causes of death in patients with antiphospholipid syndrome (APS). Previous studies have reported infection to be the most important trigger of thrombosis in APS, with molecular mimicry considered to be a major mechanism. Although timely management of infections has been recommended in patients with high suspicion of infection, anti-infective therapy would not take effect in a short time due to the dilemma in determining the origins of infection, especially in patients undergoing immunosuppressive therapy. Here, we describe a 26-year-old patient with systemic lupus erythematosus with triple antiphospholipid antibody positivity who had a stroke involving her dorsolateral medulla, despite timely anti-infective treatment within the context of skin infection caused by Stenotrophomonas maltophilia. To the best of our knowledge, it is the first report about the association between Stenotrophomonas maltophilia infection and thrombotic complications in APS. Thus, solely focusing on anti-infective therapy by the current recommendation for the management of APS may be insufficient within the context of infection; early initiation of effective anticoagulation should also be suggested until the anti-infective therapy becomes effective, especially in patients with high-risk antiphospholipid antibody profiles, in whom the potential benefit would outweigh the risk of bleeding.

Gestational trophoblastic neoplasia (GTN) is an uncommon group of pregnancy-related malignancies. Delayed diagnosis is a prognostic factor for worse outcome. GTN is even harder to diagnose if the site of metastasis is uncommon. The reported case is a 27-year-old G2P1A1 woman who presented to our center with acute transient generalized tonic-clonic seizure. She had developed hemoptysis for the 2 preceding weeks, which had been treated as pneumonia. She had then noticed multiple gum lesions and vaginal spotting 1 week before her presentation. Her serum β-human chorionic gonadotropin level was 77,474 IU/L without evidence of pregnancy. She was diagnosed with GTN with lung, brain, and gum metastases. The patient was staged as IV with a World Health Organization prognostic score of 14. Etoposide, methotrexate, actinomycin D alternating with cyclophosphamide, and vincristine weekly (EMACO) were given. The gum lesions disappeared after 2 cycles of the multiagent chemotherapy, and complete remission was achieved after 8 cycles. This case study will increase awareness of uncommon metastatic sites of GTN. Any associated vaginal bleeding should be considered a clue to metastatic GTN.

Anesthetic management of patients with pericardial tamponade is challenging. A 65-year-old man diagnosed with small-cell lung carcinoma and bilateral malignant pleural effusion in the lungs and pericardial effusion was scheduled for pericardial-window-opening surgery. The severely compromised lung function of the patient led to an anesthetic plan of ultrasound-guided serratus anterior plane block combined with an intercostal block. Although serratus plane block was initially developed for postoperative analgesia, we have shown here that it can be used under deep sedation in combination with an intercostal block for anesthesia for surgeries involving the hemithorax; the block may be promising in high-risk cases.

Venous thromboembolism (VTE), including deep vein thrombosis (DVT) and pulmonary embolism (PE), is a preventable cause of in-hospital death, and one of the most prevalent vascular diseases. There is a lack of knowledge with regards to contemporary presentation, management, and outcomes of patients with VTE. Many clinically important subgroups (including the elderly, those with recent bleeding, renal insufficiency, disseminated malignancy or pregnant patients) have been under-represented in randomized clinical trials. We still need information from real life data (as example RIETE). The paper presents case series with VTE in special conditions, including cancer associated thrombosis, malignant homeopathies, as well in high risk population.