bluuebunny/crossref_metadata_2025_split huggingface dataset:
Case Report. Cryoglobulin Hyaline-thrombi Associated Acute Jejunitis in A Patient with Type 2 Cryoglobulinemic Glomerulonephritis
Authors: Al-Othman Y , Qu Z , Zhang P 
Published: October 2021

Abstract

Introduction/Objective
Only one prior case report indicates that mixed positive cryoglobulin in serum can be associated with intestinal vasculitis (Annals of Internal Medicine, 1974).

Methods/Case Report
We report a 63-year old man with history of positive serum cryoglobulin and hepatitis-C 4 years ago and membranoproliferative pattern of glomerulonephritis with possible cryoglobulin type of deposits by electron microscopy on renal biopsy. After treatment, his hepatitis C became negative. But he was recently found to have monoclonal IgM-kappa and positive cryoglobulin in his serum, and the concurrent renal biopsy showed membranoproliferative pattern of glomerulopathy with many hyaline-thrombi (eosinophilic vascular occlusions with no lamination, inflammatory cells or nuclear debris) in the glomerular capillary loops (Figure, left panel). Both immunofluorescent and electron microscopy confirmed a mixed IgG polyclonal and IgM monoclonal type 2 cryglobulinemic glomerulonephritis. The patient also developed abdominal pain and underwent intestinal endoscopy with biopsy. His jejunal biopsy revealed neutrophil infiltration into glands and surface epithelium, with superficial sloughed epithelial cells, consistent with acute jejunitis with features of ischemic etiology. In addition, hyaline-thrombi were identified in the submucosal vessels with surrounding vasculitis (Figure, right panel); the central part of thrombi was morphologically similar to that found in glomerular capillary loops. Therefore, we conclude that cryoglobulin associated hyaline-thrombi were the most likely etiology to cause the acute ischemic jejunitis in this patient.

Results (if a Case Study enter NA)
NA

Conclusion
NA
Recurrent Focal Segmental Glomeruloscleosis Progressing to Collapsing Glomerulopathy in Renal Graft of an Autopsy study
Authors: Thorburn C , Jabbar K J , Li W , Kanaan H , Zhang P 
Published: October 2021

Abstract

Introduction/Objective
Collapsing glomerulopathy (CGN) mainly occurs in patients of African descent because a majority of these patients have APOL-1 gene mutations that results in damage of terminally differentiated podocytes, diffuse fusion of foot processes, and podocyte hyperplasia. Idiopathic FSGS is associated with high rates of recurrent FSGS in renal transplants and can be seen in patients with APOL-1 gene mutations as well, but recurrent FSGS progressing to CGN is not reported. Here we report an autopsy case with renal transplant showing recurrent FSGS progressing to CGN.

Methods/Case Report
Our patient was a 32 year old African American man who had a native renal biopsy which showed primary FSGS (with no infectious history) 8 years ago. Last year he received a renal transplantation (complex donor kidney from a deceased 25 year old man with pre-mortem serum creatinine (sCr) at 0.7 mg/dl). His initial post- transplant sCr level was as low as 1.17 mg/dl. However, in 4 months his sCr went up and he began to have higher levels of proteinuria. Sequential biopsies indicated that the patient developed a recurrent FSGS that progressed to show features of CGN. In his autopsy kidney graft, approximately 50% of glomeruli show collapsed loops with various degrees of hyperplasic podocytes, confirmed by positive CD133 staining (a progenitor cell marker). In addition, the hyperplastic podocytes lost WT-1 expression and were positive for Ki-67 staining. Distal tubules showed obvious cystic dilation. Overall findings were consistent with a severe form of CGN.

Results (if a Case Study enter NA)
NA

Conclusion
The clinical presentation of recurrent FSGS progressing to collapsing FSGS in our patient suggests that CGN and idiopathic FSGS may share a common pathophysiologic mechanism of disease.
Papillary Renal Cell Carcinoma with Extensive Spindle Cell Foci: Mimicker of Mucinous Tubular and Spindle Cell Carcinoma
Authors: Rajack F , Naab T J 
Published: October 2021

Abstract

Introduction/Objective
Papillary Renal Cell Carcinoma (PRCC), the 2nd most common RCC, accounts for 10-15% of cases and is usually composed of tubules and papillae with foamy histiocytes in papillary cores. Mucinous tubular and spindle cell carcinoma (MTSC) is composed of tightly packed, elongated, curvilinear tubules with smooth luminal surfaces, separated by mucinous stroma. MTSC is associated with a more favorable prognosis than PRCC. PRCC and MTSC have significant histologic and histochemical overlap including elongated tubules and stromal Alcian blue positive mucin deposits.

Methods/Case Report
We report a case of a 75 year old female who underwent a robotic assisted partial nephrectomy for resection of a 5.7 x 5.2 x 5.0 cm left upper pole solid renal mass. Spindle cell change with elongated tubules reminiscent of MTSC was present in several blocks; however, the luminal surface was shaggy favoring PRCC. Patchy prominent extracellular Alcian blue positive mucin deposits were also present. PRCC and MTSC both express CK7, AMACR, and EMA. However, absent expression of E-cadherin and strong CD10 expression favored PRCC. Multiple foci of solid spindle cells in a whorled pattern with clear cell change, necrosis, and high grade nuclei bordering on sarcomatoid RCC were present in other blocks. Multiple papillations and psammoma bodies also supported PRCC. A spectrum of spindle cell change was present, ranging from elongated tubules reminiscent of MTSC to whorled foci with high grade nuclei approaching sarcomatoid RCC.

Results (if a Case Study enter NA)
NA

Conclusion
Submission of multiple sections and awareness of the protean morphologic features of PRCC are essential in making the correct diagnosis.
Single institution retrospective review of CD34+ apheresis collection based on mobilization regimen and primary diagnosis
Authors: Puzo C , Li P , Siddon A , Tormey C 
Published: October 2021

Abstract

Introduction/Objective
Plerixafor has been increasingly used in conjunction with G-CSF to increase mobilization of CD34+ stem cells in patients undergoing leukapheresis collection for autologous stem cell transplantation. While G- CSF and plerixafor have been shown to increase CD34+ counts, studies examining patients’ ability to meet collection goals for patients with multiple myeloma (MM), diffuse large B-cell lymphoma (DLBCL), Hodgkin lymphoma (HL), and non-Hodgkin lymphoma (NHL) have not been well documented.

Methods/Case Report
All autologous stem cell collection patients between 2017-2021 were retrospectively reviewed and categorized by primary diagnosis. Patients received a mobilization regimen of G-CSF alone, G-CSF and plerixafor, G-CSF and chemotherapy, or G-CSF, plerixafor, and chemotherapy. CD34+ stem cell collection results were recorded. All units of CD34+ stem cells were processed according to protocol and viability was assessed by Trypan blue method on a thawed aliquot 2 weeks after processing.

Results (if a Case Study enter NA)
385 patients (271 MM, 41 DLBCL, 30 HL, and 43 NHL patients; male: 242; female: 143; age range: 16-78) were identified. Binomial logistic regression demonstrated that use of plerixafor with G- CSF was negatively associated with meeting CD34+ goal collection (OR= -1.57, p= 0.003) compared to G-CSF alone. This result was particularly true for MM patients (OR= -1.62, p= 0.014). A pairwise t-test indicated that patients receiving G-CSF and plerixafor had lower CD34+ cell viabilities (t= 2.21, p = 0.028); after Bonferroni correction for multiple comparisons, MM samples also had significantly lower percentage viability than DLBCL (p= 0.003) or HL (p= 0.022) samples.

Conclusion
A higher percentage of patients mobilized on G-CSF alone were able to meet collection goal versus patients who were mobilized on G-CSF and plerixafor. Patients who received G-CSF and plerixafor had significantly lower viability than those who received G-CSF alone. We hypothesize these findings to be due to lower baseline mobilization for patients on G-CSF and plerixafor.
Two Individuals with Rare Blocked Antigen Phenomenon and Coinciding Warm Autoantibody Mimicking Alloanti-Jk3 Resolved with JK Analysis
Authors: Zinni J G , Mullins D , DeChristopher P J , Ramsey G , Vission B , Stef M 
Published: October 2021

Abstract

Introduction/Objective
Kidd antigens can bind complement (C3) as well as Kidd specific warm autoantibodies (WAAb). An 838G&gt;A single nucleotide variant (SNV) defines JK*01 and JK*02 which codes the antithetical Jka and Jk b, respectively. Both alleles translate the high prevalence (&gt;99%) Jk3 (JK3). The 130G&gt;A is associated with weak Jka and weak Jkb expression. In vivo binding of non-agglutinating globulins can cause false-negative phenotypes by means of the blocked antigen phenomenon (BAP).

Methods/Case Report
Transfusions were requested for a 74-year-old Caucasian (CA) female with Evan’s Syndrome, and an 85-year-old African American (AA) female with metastatic uterine cancer. Both had a history of nonspecific WAAb. Direct antiglobulin testing (DAT) detected moderate in vivo sensitization of IgG and C3. They phenotyped Jk(a- b-) with untreated and EDTA glycine-acid (EGA) treated IgG DAT-negative cells. Their serum contained anti-Jk3 reactivity, while a panreactive WAAb in the eluate reacted with Jk3- donor and EGA treated DAT-negative autologous cells. Weak anti-Jka and anti-Jkb reactivity remained in the alloadsorbed serum of the antithetical adsorbing cells.
Genetic testing of the CA revealed JK*01W.01(130A)/02 alleles, while cDNA confirmed the alleles would be transcribed into mRNA. Sequencing of the AA detected 130G/A, and 838G/A as well as other silent mutations predicting either a Jk(a+wb+) or Jk(a+b+w) phenotype. The CA received one compatible JK:-3 transfusion, and both individuals benefited from multiple least incompatible transfusions of Jk a+ and/or Jk b+ donors with expected hemoglobin increases (1 g/dL per transfusion). The CA serologically phenotyped Jk(a-b+) 132 days later following prolonged immunosuppressive therapy while a normocytic normochromic anemia and the WAAb persisted. No follow up evaluations of the AA are available.

Results (if a Case Study enter NA)
NA

Conclusion
Unexpected BAP can confound immunohematology testing and lead WAAbs mimicking alloanti-Jk3 to be mischaracterized as allogeneic. By predicting phenotypes, genetic analysis can aid serological techniques in antibody characterization and help circumvent complications searching for rare JK:-3 donors.
Allo-Anti-D vs. Auto-Anti-D in Females of Child Bearing Years: The Critical Role of Rh Genotyping
Authors: Keith J T , Hastings H 
Published: October 2021

Abstract

Introduction/Objective
In females of child-bearing years, establishing accurate D-antigen identification is critical; this can be further complicated when a D+ patient seemingly develops an allo-anti-D. We present a unique case series highlighting the critical role of genotyping in distinguishing allo-anti-D vs. auto-anti-D.

Methods/Case Report
Case 1: An African American, 21-year-old, nulliparous female with a history of sickle cell disease presented for transfusion. The patient’s blood type was O, Rh Positive on multiple testing platforms (ORTHO VISION® Analyzer, 1001 US Highway 202, Raritan, NJ 08869, and Immucor, Inc., 3130 Gateway Drive, Norcross, GA 30071). On type and screen performed by manual tube testing, anti-D was identified. DAT IgG was weakly positive. The patient’s phenotype had been performed previously, and she was negative for both the C and E antigens. Genotyping results: RhD homozygote. RHD*DAU0 and RHD*DIVa type 3.
The patient was determined to have auto-anti-D formation.

Case 2
A 42-year-old female presented for routine prenatal care. At a previous facility, the patient tested as D+ and typed as O, Rh Positive (ORTHO VISION® Analyzer). An anti-D pattern was identified. Due to the patient’s Rh+ history and early pregnancy status, no Rh immune globulin was administered. A sample was sent to the reference laboratory which confirmed D+ status and a pattern of anti-D. The current titer was 8.

Genotyping results
RhD hemizyote. Positive for hybrid Rhesus box associated with deletion of RhD and RHD*DIVa, Go(a+).
The patient was determined to have an allo-anti-D.

Results (if a Case Study enter NA)
NA

Conclusion
These two cases highlight the importance of RhD genotyping for resolutions of anti-D. In case 1, the patient had two altered alleles. While DIVa, type 3, is associated with anti-D formation, she also expressed RhD*DAU0 which is not considered to lack D proteins. We report the rare association between DAU0 expression and auto-anti-D formation.
Within-Visit Blood Pressure Variability in an Unselected Adult Population
Authors: Chen Xin , Xu Shao-kun , Li Yan , Hu Zhe , Wang Hong-yu , Yu Wei , Wang Sheng-huang , Chen Xiao-ping , Chu Shao-li , Wang Ji-guang 
Published: October 2021

Abstract

Background
To investigate blood pressure variability among 3 successive blood pressure measurements in an unselected nationwide population in China.

Methods
A total of 77,549 participants were included from measurements in May 2017 in China. Blood pressure was measured 3 times consecutively with a half minute interval. Blood pressure variability was estimated with the standard deviation and coefficient of variation of the systolic and diastolic blood pressure.

Results
Not all participants showed a decreasing trend with increasing number of measurements. In fact, 14% of the participants showed at least 5 mm Hg increase in systolic blood pressure. The coefficient of variation of systolic and diastolic blood pressure in women was higher than in men [(4.2 ± 3.3)% vs. (4.1 ± 3.3)%, (4.7 ± 4.0)% vs. (4.6 ± 4.1)%; P &lt; 0.05]. The differences were significant (P &lt; 0.01) among different groups of age and blood pressure levels. Multiple linear regression analysis showed that the systolic blood pressure variability indexes were inversely associated with age but positively associated with the level of the first systolic blood pressure reading (P &lt; 0.01). The systolic blood pressure standard deviation and coefficient of variation in females were higher than in males (P &lt; 0.01).

Conclusions
Not all subjects demonstrate a decreasing trend with increasing number of blood pressure measurements. Within-visit blood pressure variability varies with age, gender, and blood pressure.
Conditions, Fictions and the Basic Norm
Authors: Albrecht Kristin Y 
Published: November 2021

Abstract:
This paper reflects on Christoph Kletzer’s absolute positivism and puts a focus on a view of the basic norm that grounds the validity of law in the law itself. I will try to explain the significance of this idea against the backdrop of Hans Kelsen’s transformation of the basic norm from a “hypothesis” to a “fiction.” I shall argue that the goal of an ultimate foundation of the objective validity of a legal order can only be accomplished by a fictional basic norm. In my eyes, contrary to what Kelsen claimed in his later work, such a norm cannot amount to a genuine epistemological fiction (echte Fiktion) in the sense of Hans Vaihinger’s “The Philosophy of ‘As If,’” but has to be regarded, rather, as a practical legal fiction.
EP.WE.48Emergency appendicectomy during COVID-19 pandemic – A single UK centre experience
Authors: Aladeojebi Adebimpe , Lo Ning , GD Sam , Salgaonkar Hrishikesh , Tsiamis Achilleas , Cheruvu Chandra 
Published: October 2021

Abstract

Background
In December 2019 following an outbreak of Novel coronavirus infection (COVID-19) in Wuhan, China, it spread rapidly overwhelming the healthcare systems globally. With little knowledge of COVID-19 virus, very few published reports on surgical outcomes; hospitals stopped elective surgery, whilst emergency surgery was offered only after exhausting all conservative treatment modalities. This study presents our experience of outcomes of emergency appendectomies performed during the pandemic.

Methods
Prospectively we collected data on 132 patients in peak pandemic period from 1st March to 5th June 2020 and data compared with 206 patients operated in similar period in2019. Patient demographics, presenting symptoms, pre-operative events, investigations, surgical management, postoperative outcomes and complications were analysed.

Results
Demographics and ASA grades of both cohorts were comparable. In study cohort 84.4% and 96.7% in control cohort had laparoscopic appendicectomy. Whilst the study cohort had 13.6% primary open operations, control cohort had 5.3%. Mean length of stay and early post-operative complications (&lt;30 days) were similar in both cohorts apart from surgical site infections (p = 0.02) and one mortality in study cohort.

Conclusion
In these overwhelming pandemic times, although conservative treatment of acute appendicitis is an option, a proportion of patients will need surgery. Our study shows that with careful planning and strict theatre protocols, emergency appendicectomy can be safely offered with minimal risk of spreading COVID-19 infection. These observations warrant further prospective randomised studies.
EP.WE.120Improving consenting for Emergency General Surgery during the COVID-19 pandemic
Authors: Elzaafarany Ahmed , Oyewole Bankole , Tawfik Ahmed , Ma Jennifer , Sandhya Anu 
Published: October 2021

Abstract

Aims
Patients undergoing surgery during the Covid pandemic are exposed to increased risks of pulmonary complications and mortality. These novel risks need to be documented on the consent form. We carried out various interventions to ensure appropriate consenting and documentation following an initial audit that revealed poor compliance with published guidelines.

Methods
The initial audit reviewed consent forms of patients undergoing emergency surgery over two-weeks in May 2020 while the re-audit was over a two-week period in June 2020 following implementation of interventions.
Inclusion Criteria: Age &gt;18-years, urgent or emergency laparoscopic surgery
Exclusion criteria: Age &lt;18-years, Open surgery, ‘Covid-light’ areas, NELA.

Results
57 consent forms were assessed during the audit loop; 22 laparoscopic appendicectomies and diagnostic laparoscopies, 14 incision and drainage, 8 laparoscopic cholecystectomies, 4 hernia repairs, and 9 other procedures.
Consenting for covid pneumonia increased from 70% to 89%, potential ITU admission 56% from 25% and the risk of death 63% from 21%

Conclusions
The covid pandemic changed our surgical practice. There are many unknowns regarding the risks to surgical patients, however, evidence shows increased risks of covid pneumonia, ITU admission and death in the perioperative period. Our consenting and the documentation of such conversations with patients must reflect our new reality.
EP.WE.135Analysis of National Bariatric Surgery Related Clinical Incidents
Authors: Omar Islam , Madhok Brijesh , Parmar Chetan , Khan Omar , Wilson Michael , Mahawar Kamal 
Published: October 2021

Abstract

Background
Hundreds of thousands of patient-safety clinical incidents are reported to the National Reporting and Learning System (NRLS) database in the United Kingdom (UK) every year. The purpose of this study was to identify bariatric surgery-related learning points from these incidents.

Methods
We analysed bariatric surgery-related clinical incidents reported to the UK NRLS database between 01 April 2005 and 31st October 2020.

Results
We identified 541 bariatric surgery-related clinical incidents in 58 different themes. Preoperative incidents represented 30.31% (N = 164), intraoperative 38.09% (N = 206), and postoperative accounted for 31.61% (N = 171). In terms of severity of incidents, (150;27.7%) were of high severity, whereas medium and low severity incidents were (244;45.1%) and (147;27.2%) respectively. The most common high severity theme was failure of thromboprophylaxis (50;9.2%). Intraoperative high severity incidents included 17 incidents of stapling of orogastric/nasogastric tubes or temperature probes, 8 missed needles, 8 broken graspers, and 6 incidents of band parts left behind. Postoperatively, the most common high severity theme was improper management of diabetes mellitus (DM) (35;6.5%). Medications errors represented a significant proportion of the medium severity incidents and included (26;4.8%) incidents of improper or missed prescription of routine medications and anticoagulants preoperatively and (45;8.3%) wrong prescriptions, dosage or prescribing of contraindicated medications postoperatively. Among the low severity themes identified there were (23;4.3%) incidents of postoperative diet problems, and (19;3.5%) patients listed for wrong procedures.

Conclusion
We identified 58 specific themes of bariatric surgery-related clinical incidents. Awareness of these themes should help to improve the safety of bariatric surgery worldwide.
EP.WE.173A Ticking Time Bomb – incidental finding of a giant thoracoabdominal aneurysm
Authors: Kam Cheuk Tung , Rait Jaideep 
Published: October 2021

Abstract

Background
Giant aortic aneurysm is a rare clinical entity. They may present with typical features of chest pain, or most feared complications with dissection and rupture. However, an asymptomatic and unruptured giant thoracic aneurysm is extremely rare with only two case reports in the literature.

Case presentation
An 80-year-old lady admitted to a local district general hospital with a 5-day history of productive cough with shivers. She tested positive for COVID-19 on admission.
Diagnostic workup demonstrated an incidental finding of a giant TAAA. Her case was referred to a tertiary hospital for vascular Multidisciplinary Team (MDT) discussion and planning.
It was decided that for her to have pre-operative assessment and MDT discussion after her recovery from infection to have definitive management of the TAAA. The patient is currently being managed supportively in hospital.

Discussion
According to National Institute for Health and Care Excellence (NICE), asymptomatic and 5.5 cm or larger aneurysm should be considered for repair. The case should be discussed in terms of the overall balance of benefits and risks with repair and conservative management, based on the current status of health and the expected future health. In this case, it was deemed that the risk of proceeding with repair at present outweighed the benefits.

Conclusion
This case describes a rare incidental finding of a giant TAAA. It emphasises the importance of MDT approach to direct and achieve appropriate management for a complex TAAA. Care treatment prioritisation, surgical planning and full complement expertise are required in complex cases such as this.
EP.WE.202Atypical presentation of Acute Aortic Dissection Standford type A dissection
Authors: Lim Su Kwan , Ekpete Neville 
Published: October 2021

Abstract

Introduction
Acute aortic dissection type A (AADA), a tear in the intima lining of the aorta, is a surgical emergency and contributes to high mortality rate if not managed promptly with surgical intervention.

Case presentation
We describe a case of a 63-year-old female with a history of hypertension presented with presumed seizure and hypotension to the emergency department. She did not have Computed Tomography (CT) chest despite having hemopericardium on her CT abdomen and pelvis. Her condition deteriorated to pericardial effusion, cardiac tamponade, multi-organ failure and shock. A diagnosis of AADA was only found on the stage of post-mortem.

Conclusion
AADA may not present with classical symptoms of tearing chest pain. The combination of hypotension and neurological deficit should trigger hospital team to consider aortic dissection higher up in the differential diagnosis for shock. If there is unclear diagnosis for an acutely unwell patient, hospital team should review the case and radiological imaging again. Hemopericardium on CT abdomen, pelvis should trigger hospital team to request for a CT chest to look out for the cause of hemopericardium. AADA is fatal without prompt surgical intervention. Immediate diagnosis can significantly reduce the mortality rate.