bluuebunny/crossref_metadata_2025 huggingface dataset:
Radiation-Induced Myopathy Developing in a Hodgkin Lymphoma Patient: An Autopsy Case with Systemic Muscle Sampling
Authors: Ando Jun , Ueno Yuji , Yasuda Hajime , Ando Miki , Edahiro Yoko , Honda Tadahiro , Takanashi Masashi , Taniguchi Daisuke , Hattori Nobutaka , Komatsu Norio
Published: March 2021

Radiation-induced myopathy (RIM) is a rare complication occurring years after radiotherapy. RIM basically occurs within the irradiation field, but some cases have been reported to be accommodated by myopathy outside the irradiation field, and the actual extent of RIM is obscure. The presented case also showed decreased MMT scores and abnormal needle electromyography results in the muscles outside the irradiated field, and the patient was initially thought to have RIM both within and outside the irradiated field. However, while systemic postmortem muscle sampling revealed prominent myopathy in line with RIM in the irradiated muscles, only mild myogenic changes that could be explained by other causes such as age-related sarcopenia, radiculopathy, and disuse atrophy were observed in the non-irradiated muscles. The number of biopsy sites in live patients is limited due to the invasive nature of the procedure, but we were privileged to systemically evaluate the extent of myopathy through multiple muscle sampling including muscles both inside and outside of the irradiation field because this was an autopsy case. Through the presented case, we conclude that RIM is a phenomenon most probably limited to the muscles within the irradiated field, and myopathy outside the irradiation is due to other causes.
Radiotherapy as Salvage Treatment in Intraocular Lymphoma: A Case Report
Authors: Colaço Raul , Portela Mariana , Costa Ilda , Guedes Marta , Mota António
Published: March 2021

A 67-year-old previously healthy woman presented with progressive visual impairment including bitemporal hemianopsia. A brain magnetic resonance imaging revealed a contrast-enhancing mass in the optic chiasm, spreading along the left optic tract. The patient underwent a transcranial biopsy of the left optical tract that yielded a diagnosis of diffuse large B-cell lymphoma. CT scans of the chest, abdomen, and pelvis, PET-CT, and bone marrow biopsy revealed no evidence of systemic lymphoma. Thus, the final diagnosis was of primary central nervous system lymphoma of the optic chiasm. Systemic treatment was initiated with full response. Six months after the end of the treatment, recurrence at cerebellum parenchyma and left tentorium was recorded. A new systemic treatment achieved full response. A second recurrence was noted in an optical coherence tomography of the right eye, 2 years after the initial diagnosis. The patient was treated with intravitreal methotrexate with initial success, but eventual failure after 10 months. Intravitreal rituximab was used with no effect. The patient was then referred to radiotherapy and underwent external beam radiotherapy with VMAT. There were no acute toxicities to report. After the radiotherapy treatment, at 1-year follow-up, the patient has no evidence of disease. Long-term toxicities were recorded and are considered manageable. The present case emphasizes the role of ocular irradiation as an option in the management of intraocular lymphoma patients, including in the salvage setting, with an acceptable ocular toxicity profile.
Isolated 17, 20 Lyase Deficiency Secondary to a Novel CYB5A Variant: Comparison of Steroid Metabolomic Findings with Published Cases Provides Diagnostic Guidelines and Greater Insight into Its Biological Role
Authors: Shaunak Meera , Taylor Norman F. , Hunt David , Davies Justin H.
Published: March 2021

Objective: The objective of this study was to report CYB5A deficiency, to discuss the contribution of steroid metabolomics to diagnosis and interpretation, and to highlight the presence of testicular microlithiasis. Methods: Two siblings with ambiguous genitalia at birth were later found to carry novel CYB5A variants, with resulting isolated 17, 20 lyase deficiency. We compared urine steroid data obtained between birth and adulthood with that from other cases. Results: Neonatal urine steroid profiles show a relative increase of 16-hydroxylated pregnenolone metabolites. Thereafter, there are no distinguishing features until puberty, when sex steroid deficiency drives gonadotrophin production, resulting in marked increases of 17-hydroxyprogesterone metabolites derived from the gonads. This excess may be revealed pre-pubertally by gonadotrophin stimulation testing. Novel findings are first, a considerable capacity for DHEA synthesis in the neonatal period compared to childhood and adulthood, suggesting that DHEAS production is much less dependent on CYB5A at birth; second, no consistent change in “backdoor pathway” intermediates; third, side chain cleavage of cortisol is largely unaffected, supporting the existence of a different lyase not dependent on CYB5A; fourth, increased 17-hydroxyprogesterone metabolites and very low androgen metabolites are diagnostic post-pubertally. Conclusion: This is the fourth disease-causing variant in CYB5A in isolated 17, 20 lyase deficiency and the first associated with testicular microlithiasis. Establishing a biochemical diagnosis pre-pubertally should now be possible using urine steroid profiling, supported by synacthen and gonadotrophin stimulation testing. We recommend liquid chromatography-mass spectrometry/mass spectrometry rather than immunoassay for serum steroid analysis, early methaemoglobin measurement and surveillance should testicular microlithiasis be detected.
Endoscopic Closure of an Acute Duodenal Perforation Occurring during Endoscopic Ultrasound Using Endoclips and Polyglycolic Acid Sheets with Fibrin Glue
Authors: Matsuoka Mana , Kobayashi Katsumasa , Okura Yukito , Mochida Tomohiro , Nozaka Takahito , Sato Ayako , Yauchi Masato , Matsumoto Taichi , Furumoto Yohei , Horiuchi Takao , Asano Toru
Published: March 2021

Acute duodenal perforation during endoscopic ultrasound (EUS) is a serious complication. The conventional endoscopic treatment for duodenal perforations such as endoscopic clipping is unsatisfactory; recently, the effectiveness of over-the-scope clipping (OTSC) has been reported. A 91-year-old woman was referred to our hospital with the chief complaint of jaundice. Contrast-enhanced computed tomography showed a 2-cm mass in the pancreatic head; we planned EUS-guided fine-needle aspiration. During exploration for a puncture route from the duodenal bulb using a linear echoendoscope under carbon dioxide insufflation, the duodenal lumen was suddenly filled with blood. A perforation <15 mm was identified in the superior duodenal horn. We attempted an endoscopic closure with multiple endoclips but could not completely close the perforation site. Strips of bioabsorbable polyglycolic acid (PGA) sheets were placed over the gaps between the endoclips with biopsy forceps and fixed in place with fibrin glue, completely covering the perforation site. Two days after the procedure, the perforation site had closed. Nine days later, endoscopic biliary stenting was performed. The patient was diagnosed with pancreatic cancer through bile cytology, and the optimal supportive care for her age was selected. Endoscopic tissue shielding with PGA sheets and fibrin glue is increasingly being reported for use during gastrointestinal endoscopic procedures. In this case, surgery was avoided due to successful endoscopic treatment using endoclips and PGA sheets with fibrin glue without OTSC. This method may be useful for repairing acute duodenal perforations during EUS and should therefore be known to pancreatobiliary endoscopists.
Doege-Potter Syndrome with a Benign Solitary Fibrous Tumor: A Case Report and Literature Review
Authors: Mohammed Turab , Ozcan Gonca , Siddique Ayesha S. , Araneta III Ronald N. , Slater Dennis E. , Khan Aziz
Published: March 2021

Doege-Potter syndrome is a rare paraneoplastic syndrome that is often diagnosed incidentally during the workup of hypoglycemia of unclear etiology. It is characterized by a non-islet cell tumor hypoglycemia secondary to excessive production of partially processed IGF-II hormone from a solitary fibrous tumor (SFT). Often these tumors are intrathoracic, benign, and asymptomatic. Occasionally they present as a paraneoplastic event; hypertrophic osteoarthropathy in Pierre-Marie-Bamberger syndrome and hypoglycemia in Doege-Potter syndrome. The NAB2-STAT6 gene fusion is the hallmark of the SFT. Complete surgical resection of the tumor often results in resolution of symptoms and cure in most cases. Here we present the case of an 83-year-old non-diabetic female with recurrent syncopal events who was diagnosed with the Doege-Potter syndrome secondary to a SFT of pleura. Her tumor was positive for NAB2-STAT6 gene fusion on RT-PCR. Following the resection of the giant tumor mass, she became symptom-free within 24 h, and has remained asymptomatic at 4 months follow-up.
Solitary Lung Metastasis of Prostate Cancer with a Long Disease-Free Interval and Normal Prostate-Specific Antigen Level
Authors: Yoshitake Hiroyuki , Oura Shoji , Yamaguchi Tomoyuki , Makimoto Shinichiro
Published: March 2021

An 83-year-old man with core needle biopsy-proven Gleason score 5 prostate cancer had received radiotherapy including 18 Gy brachytherapy to the prostate cancer, leading to no locoregional and distant recurrence for more than 5 years with the normalization of elevated prostate-specific antigen (PSA) level before the radiotherapy. Due to the enlargement of coexisting ground glass nodule (GGN) in the left lung from 1 to 2.1 cm, the patient underwent wide resection of the GGN 7 years later. Under the diagnosis of adenocarcinoma in situ of the lung, follow-up computed tomography 6 months after the wide resection showed a rapid enlargement of a solid nodule having been judged as a presumed inflammatory nodule in the middle lobe, highly suggesting a malignant neoplasm of the lung. Due to both the tall columnar atypical cells with trabecular pattern on frozen section and no elevation of serum PSA level, we judged the nodule as a primary adenocarcinoma of the lung and further resected the middle lobe with lymph node dissection. Immunostaining of the tumor showed all the CK7, CK20, TTF-1, napsin A, synaptophysin, chromogranin, CD56, CDX2, p53, beta-catenin, and MUC2 negative, and PSA highly positive, clearly showing the solid nodule as a solitary lung metastasis of the prostate cancer. Physicians should note the possible solitary lung metastasis of prostate cancer, especially bearing indolent biology, with no elevation of the PSA level even after the completion of standard 5-year follow-up.
Myasthenia Gravis in Poland: National Healthcare Database Epidemiological Study
Authors: Sobieszczuk Ewa , Napiórkowski Łukasz , Szczudlik Piotr , Kostera-Pruszczyk Anna
Published: March 2021

Introduction: Myasthenia gravis (MG) is a rare autoimmune disorder of the neuromuscular junction. MG epidemiology has not been studied in Poland in a nationwide study before. Methods: Our epidemiological data were drawn from the National Health Fund (Narodowy Fundusz Zdrowia, NFZ) database; an MG patient was defined as a person who received at least once medical service coded in ICD-10 as MG (G70) and at least 2 reimbursed prescriptions for pyridostigmine bromide (Mestinon®) or ambenonium chloride (Mytelase®) in 2 consecutive years. Results: On 1st of January 2019, 8,702 patients with MG were receiving symptomatic treatment (female:male ratio: 1.65:1). MG incidence was 2.36/100,000. The mean age of incident cases in 2018 was 61.37 years, 59.17 years for women and 64.12 years for men. Incidence of early-onset MG (<50 years) was 0.80/100,000 and 4.98/100,000 for late-onset MG (LOMG), with male predominance in LOMG. Prevalence was 22.65/100,000. In women, there was a constant increase in prevalence of symptomatic MG from the first decade of life up to 80–89 years. In men, an increase in prevalence appeared in the 6th decade. The highest prevalence was observed in the age group of 80–89 years: 59.65/100,000 in women and 96.25/100,000 in men. Conclusions: Our findings provide information on epidemiology of MG in Poland and can serve as a tool to evaluate healthcare resources needed for MG patients.
Cardiotoxicity after Additional Administration of Pertuzumab following Long-Term Trastuzumab: Report of 2 Cases
Authors: Ito Mayuko , Horimoto Yoshiya , Sasaki Ritsuko , Miyazaki Sakiko , Orihata Gotaro , Saito Mitsue
Published: March 2021

Pertuzumab, a humanized antibody drug, has improved outcomes of patients with human epidermal growth factor receptor 2 (HER2)-positive breast cancer, when administered in combination with trastuzumab and other chemotherapies. Cardiotoxicity due to trastuzumab is widely recognized, while data on pertuzumab-based treatments in daily clinical practice are lacking. We herein report 2 Japanese patients, aged 72 and 49 years, who developed left ventricular dysfunction after pertuzumab administration, following long-term trastuzumab treatments. Both patients underwent curative surgery for their HER2-positive breast cancer and received anthracycline-based treatments. After developing metastatic disease, trastuzumab-based treatments were administered without cardiac toxicity, but both patients developed left ventricular dysfunction after pertuzumab administration (6 and 13 cycles, respectively). Although several large randomized trials have shown no additive effect of pertuzumab on cardiac dysfunction, careful monitoring of cardiac function appears to be necessary in daily practice, particularly for patients with prior long-term trastuzumab treatments.
Bedside Percutaneous Approach in a Critically Ill ICU Patient with Complex Pancreatobiliary Disorder Followed by Endoscopic Approach: Lessons Learnt from a Tertiary Referral Center
Authors: Lesmana Cosmas Rinaldi Adithya , Herjuningtyas Caecilia , Inggriani Sri , Pratiwi Yulia Estu , Lesmana Laurentius A.
Published: March 2021

Pancreatobiliary disorder is a challenging clinical condition, especially when this condition is causing severe infection or biliary sepsis, and sometimes it requires intensive care unit (ICU) treatment. Biliary drainage is the mainstay of therapy; however, the choice of the drainage method is dependent on the patient’s clinical condition and the disease itself. A 79-year-old female was transferred on a ventilator to our ICU from another hospital due to biliary sepsis, a large common bile duct stone, and an infected pancreatic pseudocyst. The patient also has other comorbidities such as heart problems, hypothyroidism, and diabetes mellitus. Bedside percutaneous transhepatic biliary drainage without fluoroscopy and percutaneous cyst aspiration was successfully performed, which improved the patient’s condition; this was followed by an endoscopic approach, i.e., endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound-guided pancreatic pseudocyst drainage. The clinical improvement showed itself in the change of the patient’s respiratory status and ventilator mode. In conclusion, the percutaneous approach has a big role in managing critically ill patients in the ICU setting. However, expertise, training experience, and a multidisciplinary team approach are very important for successful management and patient outcome.
Clinical and Intestinal Histopathological Findings in SARS-CoV-2/COVID-19 Patients with Hematochezia
Authors: Cho Margaret , Liu Weiguo , Balzora Sophie , Suarez Yvelisse , Hoskoppal Deepthi , Theise Neil D. , Cao Wenqing , Sarkar Suparna A.
Published: March 2021

Gastrointestinal (GI) symptoms of SARS-CoV-2/COVID-19 in the form of anorexia, nausea, vomiting, abdominal pain and diarrhea are usually preceded by respiratory manifestations and are associated with a poor prognosis. Hematochezia is an uncommon clinical presentation of COVID-19, and we hypothesize that older patients with significant comorbidities (obesity and cardiovascular) and prolonged hospitalization are susceptible to ischemic injury to the bowel. We reviewed the clinical course, key laboratory data including acute-phase reactants, and drug/medication history in 2 elderly male patients admitted for COVID-19 respiratory failure. Both patients had a complicated clinical course and suffered from hematochezia, acute blood loss, and anemia which led to hemodynamic instability requiring blood transfusion around day 40 of their hospitalization. Colonoscopic impressions were correlated with the histopathological findings in the colonic biopsies that included changes compatible with ischemia and nonspecific acute inflammation, edema, and increased eosinophils in the lamina propria. Both patients were hemodynamically stable, on prophylactic anticoagulants, multiple antibiotics, and antifungal agents due to respiratory infections at the time of lower GI bleeding. Hematochezia resolved spontaneously with supportive care. Both patients eventually recovered and were discharged. Elderly patients with significant comorbid conditions are uniquely at risk for ischemic injury to the bowel. This case report highlights hematochezia as an uncommon GI manifestation of spectrum of COVID-19 complications. The causes of bleeding in these COVID-19 associated cases are likely multifactorial and can be attributed to concomitant etiologies based on their age, multiple comorbid conditions, prolonged hospitalization compounded by lung injury, and hypoxia precipitated by the virus. We hypothesize that rather than a direct viral cytopathic effect, ischemia and hypoperfusion may be unleashed due to the cytokine storm orchestrated by the virus that leads to abnormal coagulation profile. Additional factors that may contribute to ischemic injury are prophylactic use of anticoagulants and polypharmacy. There were no other causes to explain the brisk lower GI bleeding. Presentation of hematochezia was followed by hemodynamic instability that may further increase the mortality and morbidity of COVID-19 patients, and prompt consultation and management by gastroenterology is therefore warranted.
Don’t Forget about the Sump! An Uncommon Complication Many Years after a Choledochoduodenostomy
Authors: Chimal María Fernanda , Morales Carlos Ernesto , Saucedo Eric Misael
Published: March 2021

Bilioenteric anastomoses were common interventions before the rise of minimal invasion procedures, specifically, before endoscopic retrograde cholangiopancreatography. During a choledochoduodenostomy (CDS) the distal part of the common bile duct is excluded from the bile drainage and behaves as a “sump,” a poorly drained part that works as a reservoir which is responsible of the development of complications of the bilio-pancreatic tract. The consequent sump syndrome is a rare medical complication that presents a diversity of symptoms, for which there is no well-defined diagnostic algorithm. We present the case of a 72-year-old male patient with multiple comorbidities. He presented to the ER because of recurrent episodes of cholangitis; after having obtained the patient’s medical records, lab and image studies, the latter showed pneumobilia. After considering all the results plus the pneumobilia we suspect the presence of this uncommon complication of CDS. The patient was subjected to an unsuccessful endoscopic treatment followed by surgery, after which he showed signs of improvement and adequate evolution till hospital discharge.
Rapid Progressive Glioblastoma despite Radiation in a Patient with Myelodysplastic Syndrome
Authors: Wong Eric T. , Moore Justin , Hertan Lauren , Uhlmann Erik J.
Published: March 2021

The rapidity of glioblastoma progression can be exacerbated by impaired systemic immune surveillance. We describe an elderly woman with advanced 5q– myelodysplastic syndrome (MDS) associated with trilineage dysfunction in hematopoiesis. She also developed multiple solid tumor malignancies including ER/PR-positive and HER2-negative breast cancer, probable lung cancer without histologic confirmation, and primary glioblastoma with a high proliferation index of 80%. Because of low platelet counts of 20,000–30,000/µL that required periodic transfusion and a reduced white cell count of 600–900/µL, she was deemed unsafe to take concomitant daily temozolomide during radiation and her glioblastoma was treated with a shortened course of radiotherapy alone. Her baseline absolute neutrophil count was 110–390/µL, and CD4+ and CD8+ lymphocyte counts were 235/µL and 113/µL, respectively. During the last week of radiation, the patient developed a nonfluent aphasia, increased fatigue, and aspiration pneumonia. A gadolinium-enhanced head MRI, obtained 2 days after completion of radiation and 39 days after biopsy, demonstrated near tripling of the size of the left frontal tumor with a significant amount of adjacent cerebral edema. This case raises the possibility that advanced MDS is a negative immunomodulatory condition that can accelerate glioblastoma progression.
Management of Cardiovascular Disease in Kidney Disease Study: Rationale and Design
Authors: Shukla Ashutosh M. , Segal Mark S. , Pepine Carl J. , Cheung Alfred K. , Shuster Jonathan , Mohandas Rajesh , Martinez Wanda M. , Flint Jeremy J. , Shah Sudhir V.
Published: March 2021

Introduction: Atherosclerosis, inflammation, and vascular stiffness are prominent interrelated risk factors contributing to the high incidence of cardiovascular disease (CVD) in patients with CKD. Conventional CVD management strategies in CKD largely target atherosclerotic CVD and have had a limited impact on the cardiovascular mortality in this population. Multiple in vivo and in vitro studies and epidemiological evidence from the rheumatologic cohorts have shown that low-dose hydroxychloroquine has beneficial effects on inflammation, endothelial function, insulin sensitivity, and metabolic syndrome. Our recent proof-of-concept animal study showed that hydroxychloroquine has marked protection against atherosclerosis and vascular stiffness. We hypothesize that hydroxychloroquine has the potential to provide significant cardiovascular benefits in patients with CKD. Methods: The Management of Cardiovascular disease in Kidney disease study (NCT 03636152) is a phase 2B, randomized, double-blind, placebo-controlled trial evaluating the effects of low-dose hydroxychloroquine therapy on the parameters of atherosclerosis, inflammation, and vascular stiffness in patients with CKD. The study plans to enroll 100 CKD patients estimated to be at high cardiovascular risk by a combination of low estimated glomerular filtration rate and albuminuria and treat them for 18 months with hydroxychloroquine or placebo in 1:1 allocation. Results: The study will assess the change in the total carotid plaque volume as measured by serial noncontrast carotid MRI as the primary outcome and the serial changes in plasma inflammation markers, vascular stiffness, renal function, and the composition characteristics of the carotid plaque as secondary outcome measures. Discussion/Conclusion: The results of this trial will provide the proof-of-applicability for hydroxychloroquine in the CVD in CKD. If positive, this trial should lead to phase-3 trials with clinical end points for this potentially transformative, novel, and inexpensive therapy for CVD in CKD.
Rescue Pericardial Drainage and Return Connected to ECMO for Aortic Rupture into the Pericardial Sac with Acute Type A Aortic Dissection
Authors: Ijuin Shinichi , Takeuchi Mariko , Nakai Chikashi , Inoue Akihiko , Izumi So , Igarashi Nobuaki , Matsuyama Shigenari , Ishihara Satoshi , Doi Tomofumi , Nakayama Shinichi , Tsukube Takuro
Published: March 2021

We present the first documented case of emergent pericardial drainage and return (PD-R) under extracorporeal membrane oxygenation (ECMO) for the management of aortic rupture into the pericardial sac caused by acute type A aortic dissection (AADA). An 83-year-old woman collapsed during an elective coronary intervention. ECMO was eventually required. Acute accumulation of pericardial effusion with aortic dissection was revealed by echocardiography. Percutaneous pericardial drainage was performed using a drainage line connected to the venous line of the ECMO system to maintain blood flow and blood pressure. After stabilization of the patient’s hemodynamics, immediate aortic repair was successfully performed and the patient was discharged with no neurological deficit. In cases of massive amounts of pericardial drainage and persistent hemorrhagic shock due to aortic rupture with AADA, PD-R connected to ECMO is useful while waiting for aortic repair.
Sarcoma of the Heart Treated with Stereotactic MR-Guided Online Adaptive Radiation Therapy
Authors: Pomp Jacquelien , van Asselen Bram , Tersteeg Robbert H.A. , Vink Aryan , Hassink Rutger J. , van der Kaaij Niels P. , van Aarnhem Guido E.E.H.L. , Verhoeff Joost J.C.
Published: March 2021

We present the first case in the literature of a patient with a histology-proven intimal sarcoma of the heart, recurrent after surgery, treated with stereotactic MR-guided online adaptive radiation therapy on an MR-Linac machine. The treatment was feasible and well tolerated. The CT scan 6 months after the last treatment showed stable disease.
Isolated Dysphagia in a Patient with Medial Medullary Infarction – Effects of Evidence-Based Dysphagia Therapy: A Case Report
Authors: Hamzic Samra , Schramm Patrick , Khilan Hassan , Gerriets Tibo , Juenemann Martin
Published: March 2021

Medial medullary infarction (MMI) is a vascular occlusion in the medulla oblongata leading to certain constellations of neurological symptoms and seriously affecting the patient. Effective evidence-based treatment of severe dysphagia as sole symptom of MMI has not yet been reported. This case study aims to report successful effects of evidence-based therapy based on findings of dysphagia symptoms and pathophysiology of swallowing by flexible endoscopic evaluation of swallowing (FEES) in severe isolated dysphagia after MMI. FEES was performed to evaluate swallowing pathophysiology and dysphagia symptoms in a 57-year-old male with severe dysphagia after MMI. On the basis of FEES findings, simple and high-frequent evidence-based exercises for improvement of swallowing were implemented: thermal stimulation of faucial arches, Jaw Opening Exercise, and Jaw Opening Against Resistance. After 7 weeks of high-frequent evidence-based therapy and regular FEES evaluation the patient was set on full oral diet with no evidence of aspiration risk. In a first case report of isolated dysphagia in MMI our case illustrates that high-frequent evidence-based dysphagia therapy in combination with FEES as the method to evaluate and monitor swallowing pathophysiology can lead to successful and quick rehabilitation of severely affected dysphagic patients.
Molecular Phylogenetics of Hepatitis D Virus in New Zealand and the Implications for Pacific Island Countries
Authors: Jackson Kathy , Littlejohn Margaret , Gane Ed , Locarnini Stephen
Published: March 2021

Hepatitis delta virus (HDV) is considered a satellite virus that requires hepatitis B virus surface antigen for infectivity. HDV is endemic in some Pacific Island (PI) countries, including Kiribati and Nauru, with a unique genotype 1, “Pacific clade.” The aims of this study were to determine the HDV genotypes in New Zealand and investigate the link of strains to other PI countries and the rest of the world through phylogenetics. Sequencing and phylogenetic analyses were performed on 16 HDV-positive serum samples from 14 individuals collected between 2009 and 2014 at Auckland Hospital. Thirteen of 14 strains were confirmed as genotype 1 and 1 was genotype 5. Eleven of the 13 genotype 1 strains clustered with the Pacific clade. These were isolated from subjects born in Samoa, Kiribati, Tuvalu, and Niue. Another genotype 1 strain isolated from a Maori health-care worker clustered most closely with a European strain. There was an African genotype 1 and genotype 5 from African-born subjects with HIV coinfection. This study supports the probable transmission of HDV Pacific clade around the PI from Micronesia to Polynesia. The data also confirm the need to screen hepatitis B surface antigen-positive individuals for HDV.
An Update for Mesenchymal Stem Cell Therapy in Lupus Nephritis
Authors: Li Wenchao , Chen Weiwei , Sun Lingyun
Published: March 2021

Background: Lupus nephritis (LN) is the most severe organ manifestations of systemic lupus erythematosus (SLE). Although increased knowledge of the disease pathogenesis has improved treatment options, outcomes have plateaued as current immunosuppressive therapies have failed to prevent disease relapse in more than half of treated patients. Thus, there is still an urgent need for novel therapy. Mesenchymal stem cells (MSCs) possess a potently immunosuppressive regulation on immune responses, and intravenous transplantation of MSCs ameliorates disease symptoms and has emerged as a potential beneficial therapy for LN. The objective of this review is to discuss the defective functions of MSCs in LN patients and the application of MSCs in the treatment of both LN animal models and patients. Summary: Bone marrow MSCs from SLE patients exhibit impaired capabilities of migration, differentiation, and immune regulation and display senescent phenotype. Allogeneic MSCs suppress autoimmunity and restore renal function in mouse models and patients with LN by inducing regulatory immune cells and suppressing Th1, Th17, T follicular helper cell, and B-cell responses. In addition, MSCs can home to the kidney and integrate into tubular cells and differentiate into mesangial cells. Key Messages: The efficacy of MSCs in the LN treatment remains to be confirmed, and future advances from stem cell science can be expected to pinpoint significant MSC subpopulations, as well as specific mechanisms of action, leading the way to the use of more potent stimulated or primed pretreated MSCs to treat LN.
Double Pylorus in Upper Gastrointestinal Bleeding
Authors: Oktaricha Heasty , Miftahussurur Muhammad
Published: March 2021

Double pylorus, also known as acquired double pylorus, is a rare condition defined as a gastrointestinal fistula connecting stomach antrum and duodenal bulb. The prevalence of double pylorus ranges from 0.001 to 0.4% by esophagogastroduodenoscopy (EGD). Although the etiology is unknown, the formation of double pylorus is related to Helicobacter pylori infection and the use of non-steroidal anti-inflammatory drugs (NSAID). The development of the occurrence of double pylorus is still unknown, but many systemic diseases play a role. We present the case of a 59-year-old man who was admitted to Dr. Soetomo General Hospital with hematemesis and melena. The patient had a history of diabetes mellitus since 3 years and consumption of medicinal herbs for myalgia, which was suspected of NSAIDs for the past 5 months. The patient had anemia with hemoglobin at 8.3 g/dL, enterogenous azotemia with blood urea nitrogen 28 mg/dL and serum creatinine 1.14 mg/dL. At EGD, double pylorus was found and accompanied by gastric ulcer, a giant white base ulcer, part of it covered by clotting without any sign of active bleeding. Biopsy revealed chronic inactive gastritis, and no H. pylori was found. Treatment mainly depends on gastrointestinal acid suppression through a proton pump inhibitor (PPI). The patient was given a high-dose PPI and a mucosal protective agent. He was treated for 1 week and had improved complaints.
Primary Intimal Sarcoma of the Pulmonary Artery in a 25-Year-Old Woman with Dyspnea and Palpitation: A Case Report
Authors: Onagi Hiroko , Horimoto Yoshiya , Arai Takashi , Terukina Hiroyuki , Asai Tohru , Arakawa Atsushi , Saito Tsuyoshi
Published: March 2021

Intimal sarcoma arising from the tunica intima of both systemic and pulmonary circulations is a rare disorder, whereas intimal sarcoma with chondroblastic osteosarcomatous differentiation (ISCOS) is even rarer. We present the case of a 25-year-old woman with ISCOS of the pulmonary artery (PA) where the patient went through surgical treatment after careful imaging assessment under a rather emergent situation. A 25-year-old Japanese female presented to our hospital with the chief complaints of dyspnea and palpitations on exertion. Upon arrival, she had systolic murmur, moderate tricuspid regurgitation, and possible pulmonary hypertension. A contrast-enhanced chest computed tomography (CT) showed dilatation of the main PA, filled with a hypodense area with calcification adjacent to the right and left PA. The calcified lesions within the tumor were the key findings suggesting osteoid-forming sarcoma, differentiating it from pulmonary embolism. Due to presence of critical symptomatic obliteration of the pulmonary circulation, an emergency surgery was performed. A whitish shiny mass filled the lumens from the main PA to the bilateral main PAs. The tumor was not attached to the surrounding intima, except for a slight attachment to the left interlobar PA, and could be completely removed from the vessel lumen. Based on the pathological findings, it was diagnosed as a primary ISCOS of the PA, which correlated with the findings of the CT, namely intratumoral calcification. Although the diagnosis-making is quite challenging, multidisciplinary collaboration between clinicians, radiologists, and pathologists is crucial for reaching the correct diagnosis.
Between Temperament and Psychopathology: Examples from Neuropharmacological Challenge Tests in Healthy Humans
Authors: Netter Petra
Published: March 2021

Background: This paper tries to demonstrate that the questionnaire-based continuum between temperament traits and psychopathology can also be shown on the biochemical level. A common feature is the incapacity to adapt to external demands, as demonstrated by examples of disturbed hormone cycles as well as neurotransmitter (TM) responses related to affective and impulse control disorders. Methods: Pharmacological challenge tests performed in placebo-controlled balanced crossover experiments with consecutive challenges by serotonin (5-HT), noradrenaline (NA), and dopamine (DA) agonistic drugs were applied to healthy subjects, and individual responsivities of each TM system assessed by respective cortisol and prolactin responses were related to questionnaire-based facets of depressiveness and impulsivity, respectively. Results: The depression-related traits “Fatigue” and “Physical Anhedonia” were characterized by low and late responses to DA stimulation as opposed to “Social Anhedonia,” which rather mirrored the pattern of schizophrenia. Reward-related and premature responding-related impulsivity represented by high scores on “Disinhibition” and “Motor Impulsivity,” respectively, as well as the questionnaire-based components of attention deficit hyperactivity disorder, “Cognitive” and “Motor Impulsivity,” could be discriminated by their patterns of DA/NA responses. 5-HT responses suggested that instead of the expected low availability of 5-HT claimed to be associated with impulse control disorders, low NA responses indicated lack of inhibition in impulsivity and high NA responses in depression-related “Anhedonia” indicated suppression of approach motivation. Conclusions: In spite of the flaws of pharmacological challenge tests, they may be suitable for demonstrating similarities in TM affinities between psychopathological disturbances and respective temperament traits and for separating sub-entities of larger disease spectra.
A Case Report of Familial Extramammary Paget's Disease in Female Siblings
Authors: Rutnumnoi Thanachat , Leeyaphan Charussri
Published: March 2021

Extramammary Paget’s disease (EMPD) is a rare intraepithelial neoplasm that occurs in apocrine-bearing areas of skin. Most EMPD patients initially present with chronic pruritic eczematous lesions involving genitalia, perineum and perianal area. Familial form of EMPD is extremely rare. Several genetic mutations have been proposed but specific modes of inheritance are still unknown. This article reports two cases of familial extramammary Paget’s disease in female siblings.
Risk Factors for Falls in Patients with Alzheimer Disease: A Retrospective Study of Balance, Cognition, and Visuospatial Ability
Authors: Oki Mayuka , Matsumoto Miyuki , Yoshikawa Yukiko , Fukushima Mitsuko , Nagasawa Akira , Takakura Tomokazu , Suzuki Yukiko
Published: March 2021

Aim: This study aimed to determine the main risk factors for falls in patients with Alzheimer disease (AD) by comparing balance, cognition, and visuospatial ability between those who have experienced a fall and those who have not. Methods: Forty-seven AD patients were admitted to a ward for patients with dementia (22 men and 25 women). The balance of patients was evaluated using the Functional Reach Test (FRT), the one-leg standing duration, and the Timed Up and Go (TUG) test. The Mini-Mental State Examination-Japanese (MMSE-J) was used to evaluate cognition. For visuospatial ability assessment, the Clock-Drawing Test (CDT) as well as overlapping figure identification and shape discrimination in the Visual Perception Test for Agnosia (VPTA) were used. The patients were allocated to either the fall group or the nonfall group based on their history of falls in the past year. The relationships between patients’ characteristics and evaluation outcomes were compared and examined. Logistic regression analysis was performed using a fall as the objective variable. The area under the curve (AUC) and the cutoff value were calculated. Results: Of the 47 participants, 22 had experienced falls within the past year (46.8%). The results of the FRT, one-leg standing duration, the TUG, the CDT, and the VPTA were significantly lower in the fall group. No significant difference between the MMSE-J scores of the fall group and those of the nonfall group was observed. The results of the logistic regression analysis indicated that falls in AD patients were significantly associated with the FRT. It was found that a shorter FRT distance (cm) had a significant impact on falls. For the FRT, the fall-related AUC was 0.755. At a cutoff value of 24.5 cm, the level of sensitivity was 68.0%, and the level of specificity was 77.3%. Conclusions: The findings of this study indicate that balance and visuospatial abilities are risks factors for falls in AD patients. In contrast, cognitive impairment was not a risk factor for falls. It was demonstrated that the FRT could be an appropriate risk predictor for falls in AD patients. In particular, falls in AD patients were strongly affected by a reduced dynamic balance.
Concurrent Validity of Inertially Sensed Measures during Voluntary Body Sway in Silence and while Exposed to a Rhythmic Acoustic Stimulus: A Pilot Study
Authors: Emmanouil Analina , Rousanoglou Elissavet , Georgaki Anastasia , Boudolos Konstantinos
Published: March 2021

Introduction: The effect of rhythmic acoustic stimuli on body sway is of increasing interest due to their positive contribution when training or restoring the control of movement. Inertial sensors show promise as a portable, easier, and more affordable method compared to the force plate “gold standard” concerning the evaluation of postural sway. This study examined the concurrent validity of inertially sensed measures of voluntary body sway against those obtained with a force plate, in silence and while exposed to a rhythmic acoustic stimulus. Methods: Temporal (sway duration and variability) and spatial (trajectory length, variability, range, velocity, and area) body sway variables were extracted using an inertial sensor (at L5) in synchronization with a force plate, during anteroposterior body sway in silence and while exposed to a rhythmic acoustic stimulus (n = 18 young women; two 70-s trials in each condition). Statistics included bivariate correlations between the inertially sensed and the force plate measures, separately, in silence and with a rhythmic acoustic stimulus, as well as for the effect of the rhythmic acoustic stimulus (percentage difference from silence) (p ≤ 0.05, SPSS v25.0). Results: The inertially sensed measures demonstrated good-to-excellent concurrent validity for all temporal and almost all spatial variables, both in silence and with rhythmic acoustic stimulus (r > 0.75, p = 0.000), as well as for the rhythmic acoustic-stimulus effect (r > 0.75, p ≤ 0.05). Conclusion: The inertially sensed measures of the voluntary anteroposterior body sway demonstrated an overall good-to-excellent concurrent validity against those obtained with the force plate “gold standard,” both in the silence and the rhythmic acoustic stimulus conditions, as well as for the rhythmic acoustic-stimulus effect. The findings of this pilot study allow the recommendation of inertial sensing for the evaluation of postural control alterations when exposed to rhythmic acoustic stimuli, a condition of increasing interest due to the positive contribution of such stimuli when training or restoring the control of movement.
The Multi-Course Approach of Photodynamic Therapy to Treat Invasive Cervical Cancer IB2: A Case Report
Authors: Afanasiev Maxim , Dushkin Alexander , Grishacheva Tatyana , Afanasiev Stanislav , Nesvizhsky Yuri , Karaulov Alexander V. , Pylev Andrey
Published: March 2021

Cervical cancer is an important problem in women’s health and a worldwide oncological disease. In 2018, the WHO registered 569,847 new cases in the world, and 3.4% were in the Russian Federation. We describe here a case of invasive cervical cancer stage IB2 associated with human papilloma virus in a woman who was treated by multicourse photodynamic therapy (PDT). A 38-year-old woman presented with abdominal pain and genital tract spotting in October 2015. Colposcopy revealed a neoplasm in cauliflower form. PAP smear result was cancer in situ (Tis). The biopsy result from the cervical canal and neoplasm was invasive squamous cell carcinoma. The patient underwent full preoperative examination (blood test, biochemical blood test, coagulation test, urinalysis, X-ray of chest organs, ECG, ultrasound investigation of pelvic organs, and PAP smear). Magnetic resonance imaging investigation showed a heterogeneous tumor, uneven contours, and intensity accumulating contrast. The patient was not pregnant, and a fertility-preserving treatment method was used. Three PDT sessions allowed to avoid vaginal radical trachelectomy. Pregnancy occurred 3 years and 8 months after the first PDT session. The patient had testing after treatment 4 times (3rd, 12th, 24th, and 60th months). She had a pregnancy without complications and had operative delivery by Cesarean section in April 2020. There was a 5-year remission period without episodes of relapse. The patient has an 8-month-old baby.
Transient Vision Loss – A Rare Oxaliplatin-Induced Ophthalmologic Side Effect: A Report of Two Cases
Authors: Ah-Thiane Loic , Raoul Jean-Luc , Hiret Sandrine , Senellart Hélène , Dumont Frédéric , Raimbourg Judith
Published: March 2021

Oxaliplatin, a platinum-based chemotherapeutic agent, is responsible for induced peripheral sensory neuropathy. Only a few cases of ophthalmologic toxicity have been reported. We report here two cases of sudden transient vision loss after oxaliplatin administration, in one case intraperitoneally. These symptoms likely reflect optic neuritis that could be included in the spectrum of oxaliplatin-induced neuropathy.
Ambulatory Intrapleural Fibrinolytic Therapy in Highly Viscous Recurrent Malignant Pleural Effusion
Authors: Foo Chuan Tai , Herre Jurgen
Published: March 2021

Malignant pleural effusion (MPE) is common in lung cancer. We report a case of highly viscous recurrent MPE associated with lung cancer. The viscosity prohibited gravity-dependent drainage initially with a 6-Fr aspiration catheter and subsequently with a 12-Fr intercostal drain. The effusion was eventually evacuated after a single dose of intrapleural fibrinolytic therapy. This process was repeated a total of 13 times over a 12-month period in an ambulatory setting. No bleeding complications were observed. This case highlights the feasibility and safety of repeated intrapleural fibrinolysis in the management of highly viscous recurrent MPE in an ambulatory setting.
Obesity and Serial NT‐proBNP Levels in Guided Medical Therapy for Heart Failure With Reduced Ejection Fraction: Insights From the GUIDE‐IT Trial
Authors: Parcha Vibhu , Patel Nirav , Kalra Rajat , Suri Sarabjeet S. , Arora Garima , Wang Thomas J. , Arora Pankaj
Published: March 2021

Background

Obese patients have lower NT‐proBNP (N‐terminal pro‐B‐type natriuretic peptide) levels. The prognostic implications of achieving NT‐proBNP levels ≤1000 pg/mL in obese patients with heart failure (HF) receiving biomarker‐guided therapy are not completely known. We evaluated the prognostic implications of obesity and having NT‐proBNP levels (≤1000 pg/mL) in the GUIDE‐IT (Guiding Evidence‐Based Therapy Using Biomarker‐Intensified Treatment in HF) trial participants.

Methods and Results

The risk of adverse cardiovascular events (HF hospitalization or cardiovascular mortality) was assessed using multivariable‐adjusted Cox proportional hazard models based on having NT‐proBNP ≤1000 pg/mL (taken as a time‐varying covariate), stratified by obesity status. The study outcome was also assessed on the basis of the body mass index at baseline. The predictive ability of NT‐proBNP for adverse cardiovascular events was assessed using the likelihood ratio test. Compared with nonobese patients, obese patients were mostly younger, Black race, and more likely to be women. NT‐proBNP levels were 59.0% (95% CI, 39.5%–83.5%) lower among obese individuals. The risk of adverse cardiovascular events was lower in obese (hazard ratio [HR], 0.48; 95% CI, 0.29–0.59) and nonobese (HR, 0.32; 95% CI, 0.19–0.57) patients with HF who had NT‐proBNP levels ≤1000 pg/mL, compared with those who did not. There was no interaction between obesity and having NT‐proBNP ≤1000 pg/mL on the study outcome (
P
>0.10). Obese patients had a greater risk of developing adverse cardiovascular events (HR, 1.39; 95% CI, 1.01–1.90) compared with nonobese patients. NT‐proBNP was the strongest predictor of adverse cardiovascular event risk in both obese and nonobese patients.

Conclusions

On‐treatment NT‐proBNP level ≤1000 pg/mL has favorable prognostic implications, irrespective of obesity status. NT‐proBNP levels were the strongest predictor of cardiovascular events in both obese and nonobese individuals in this trial.

Registration

URL:
https://www.clinicaltrials.gov
; Unique identifier: NCT01685840.