A rare case of non-lupus full house nephropathy in a transplanted kidney, case report.

Key Clinical Message: Non-lupus full house nephropathy is a rare entity that is still poorly understood. It can complicate post-transplant kidneys and result in a de novo process. Treatment is difficult but can be possibly achieved with optimization of immune suppression. Abstract: Non-lupus full house nephropathy is a rare entity with an unclear incidence. It describes the kidney biopsy findings of positive deposits for IgG, IgA, IgM, C3, and C1q on immunofluorescence in the absence of the classical diagnostic features of systemic lupus nephritis. This disease entity is becoming more recognized but further studies are still needed to evaluate the incidence, etiologies, and management of this condition. Transplant glomerulopathy is a major cause for renal graft loss. It can present with a wide variety of manifestations; it can cause AKI, CKD, or glomerular inflammations through an immune complex or autoimmune-mediated damage.

A young man with Guillain-Barré syndrome, with a stroke-like presentation and physical findings suggestive of cervical myelopathy.

We describe the case of a 44-year-old gentleman with hypertension and asthma presenting to the emergency department after noticing right upper-extremity weakness upon awakening. Brain imaging did not reveal a stroke. Initial neurological examination pointed to cervical myelopathy with radiculopathy as well as possible underlying length-dependent peripheral neuropathy as there was right arm strength of 4/5 and there were brisker (3+) reflexes all over except at the right biceps reflex and both ankle reflexes. Cervical spine magnetic resonance imaging (MRI) showed myelomalacia at the C2 level and an os odontoideum (OO). Os odontoideum is a chronic condition that occurs due to the failure of the center of ossification of the dens to fuse with the body of C2. By the next day after a few hours of sustaining a fall, weakness progressed to quadriparesis, without a sensory level on examination, followed by urinary retention. This situation was attributed to a possible cervical cord contusion due to the fall in the presence of OO, with other possibilities being spinal cord hemorrhage, infarct and transverse myelitis. However, repeat scanning of the cervical spine (MRI) did not reveal any acute cord changes. The initial examination for common causes of peripheral neuropathy did not reveal any findings. Finally, the diagnosis of Guillain-Barré syndrome (GBS) was considered, and treatment was initiated with intravenous immunoglobulin. Cerebrospinal fluid analysis was normal. The diagnosis was confirmed using electromyography. Our patient's initial presentation of monoparesis and progression in an asymmetric descending manner was unusual for GBS. His initial presentation mimicked a stroke, and the later progression masqueraded as cervical myelopathy secondary to a chronic cervical cord lesion. The presence of a cervical cord lesion (upper motor neuron) concealed the expected areflexia in GBS. The presence of OO on spine imaging, absence of expected areflexia in GBS, and progression to paraparesis after the fall sidetracked the direction of the initial investigation and led to a relative delay in diagnosis. Nonetheless, appraising the diagnostic data in the clinical context led to an appropriate diagnosis. We emphasize the importance of reconciling the available clinical and diagnostic information to reach the correct diagnosis.

Genotypic and Phenotypic Composition of Sickle Cell Disease in the Arab Population - A Systematic Review.

Sickle cell disease (SCD) is a genetic disease influenced by ethnicity and regional differences in its clinical course. Recent advances in the management of SCD with newer therapies are being introduced to the Western population. However, many of these treatments are yet to be used in the Arabic SCD population. Understanding the genetic variations of SCD regionally is essential to anticipate the utilization of new treatments. This systematic review's main objective is to pool the available data on the genetic composition of SCD in the Arabic population. Data for 44,034 patients was extracted from 184 studies (11 case reports, 8 case series, 56 retrospectives, 107 prospective observational studies, and 2 clinical trials) using PubMed, Scopus, and Google Scholar. Male (49%) and female (51%) patients were equally reported wherever gender was available (N=13105). Various SCD genotypes were reported in a total of 14,257 patients, including Hb SS (77%) Hb Sß0 (9.9%), and Hb Sß+ (7.2%), while the rest of the genotypes, including HbSC, HbSD, HbSE, HbSO Arab, Hb S/α-Thal, Hb Sß0 + α-Thal, and HBS Oman were individually reported in <4% of the cases. Major SCD complications in the Arab population included pain crises (48.25%) followed by neurological complications (33.46%), hepatobiliary complications (25.53%), musculoskeletal complications (24.73%), and hemolytic anemia (23.57%). The treatments reported for SCD included hydroxyurea (20%), blood transfusion (14.32%), and Deferasirox (3.03%). We did not find the use of stem cell transplantation or newer treatments such as L-Glutamine, Voxelotor, Crizanlizumab, or gene therapy reported in any of the studies included in our review. This review highlights the genetic makeup of SCD in Arab countries and its common phenotypic manifestations and will help direct further research on SCD in this region, especially concerning genetic therapy. Systematic Review Registration: The protocol has been registered in the International Prospective Register of Systematic Reviews(PROSPERO):CRD42020218,666. https://www.crd.york.ac.uk/PROSPERO/display_record.php?RecordID=218666.

Asymptomatic Severe Aortic Stenosis: Contemporary Evaluation and Management.

Aortic stenosis (AS) is the most prevalent valvular heart disease in developed countries and most prevalent in the elderly. According to the current guidelines, intervention is recommended in symptomatic severe AS; however, in asymptomatic patients, aortic valve replacement (AVR) is considered when symptoms appear or the left ventricular dysfunction occurs, but the evidence supports these indications are poor. The optimal timing and modality of intervention in asymptomatic severe AS (ASAS) remain controversial. Earlier AVR in certain scenarios has been increasingly supported by some groups before subclinical irreversible myocardial damage occurs. In addition, the continuous advancement of percutaneous and surgical approaches where associated with a substantial decrease in mortality and perioperative complications which made many authors advocate for early intervention in those patients. Our review highlights the contemporary evaluation and management of ASAS and summarizes the current scientific evidence regarding optimal timing for intervention and indications for early AVR in such patients.

Protocol for "Genetic composition of sickle cell disease in the Arab population: A systematic review".

Background: Sickle Cell Disease (SCD) is a global health issue in hematology with a progressively increasing prevalence. There are recent advances in the management of SCD, with new drugs being introduced. It is essential to analyze the genetic makeup of SCD regionally to anticipate the effectiveness of management modalities. This systematic review's main objectives are (a) to combine the existing knowledge of the genetic composition of SCD in the Arab population and (b) to analyze the various phenotypes of SCD prevalent in the Arab population. Methods: We will perform a systematic review and search multiple electronic databases predefined search terms to identify eligible articles. Eligible studies should report findings on the genetic testing of Sickle Cell disease in the 22 Arab countries. Case reports, case series, observational studies with cross-sectional or prospective research design, case-control studies, and experimental studies will be included. Study quality will be independently evaluated by two reviewers using the statistical methodology and categories guided by the Cochrane Collaboration Handbook and PRISMA guidelines. Discussion: This review will explore and integrate the evidence available on the various genotypes and phenotypes of SCD in the Arab population. By acquiring and summarizing data about the genetic and phenotypic variants of the SCD patient population, this study will add to the knowledge and help find more precise treatments. Systematic review registration: The protocol is registered at the International Prospective Register of Systematic Reviews (PROSPERO; registration number: CRD42020218666).

Priapism in Lymphoproliferative Disorders: A Systematic Review.

Priapism is defined as a persistent penile erection lasting more than 4 h. We searched the literature for reviews, case reports, and series for patients with lymphoproliferative disorders who developed priapism. The search involved all the lymphoproliferative disorders included in the revised 2016 World Health Organization classification of lymphoid neoplasms including chronic lymphocytic leukemia, multiple myeloma, Waldenström macroglobulinemia, and lymphomas. A total of 16 articles were found. The search included cases up to 4th January 2021. Priapism was seen most commonly as the first manifestation of lymphoproliferative disorders, rarely seen after treatment or after diagnosis.

Clinicopathological Variables and Outcome in Chronic Myeloid Leukemia Associated With BCR-ABL1 Transcript Type and Body Weight: An Outcome of European LeukemiaNet Project.

OBJECTIVE: It is debatable whether BCR-ABL1 transcript type has an impact on outcome of treatment of patients with CML, and it is not widely studied whether body weight influences response to treatment. In this study, we tried to find out if any of these factors has an impact on response to treatment and outcome. METHODOLOGY: We conducted a retrospective analysis of the files of 79 patients being treated in our center for CML with known BCR-ABL1 breakpoints, and patients' management and response assessment was done based on ELN 2013 guidelines. The analysis was performed based on two main groups, obese vs. normal BMI, and then based on BCR-ABL1 transcripts: e13a2 vs. e14a2. Cumulative incidence of MMR, CCyR, and DMR were estimated using the Kaplan-Meier survival curve method, and comparisons between groups were performed by the Log-rank/Gray test methods. RESULTS/CONCLUSION: In the patient-cohort studied, there was no statistically significant difference in molecular response between patients with CML based on body weight or transcript type although patients in the obesity group achieved higher and faster MMR with no statistical significance.

Eslicarbazepine-induced severe hyponatremia resulted in generalized tonic-clonic seizure.

Although eslicarbazepine is an anti-seizure medication, it may result in seizure worsening if its use is complicated by severe hyponatremia, even long time after commencing this medication. The treatment is a replacement for another ASM.

Time for change in practice of in-patient oxygen therapy: a period-limited, multidimensional approach to improve oxygen prescription compliance: quality improvement project at Hamad General Hospital, Qatar.

Prescription of oxygen therapy has traditionally poor compliance across the globe and mostly given to patients on verbal orders leading to under or overuse. The British Thoracic Society (BTS) guidelines (2017) recommend that oxygen therapy must be prescribed. Our study aimed to assess the prescription practice of oxygen therapy for patients admitted to acute medical assessment unit and general medical wards at Hamad General Hospital, Qatar and to achieve 80% compliance of valid oxygen therapy prescription implementing the quality improvement model against the BTS guidelines.The prescription practice of oxygen therapy was audited between April 2019 and August 2019. Using a Plan, Do, Study, Act (PDSA) model of improvement and multiple interventions was performed in the eight PDSA cycles, including (1) educational sessions for residents/fellows/nurses, (2) introduction of electronic prescription, (3) emails, posters/flyers, (4) nurse-led reminders and (5) re-enforced teaching for new residents. Data were then collected using a questionnaire assessing electronic prescriptions and documentation. Our baseline study regarding oxygen therapy showed limited awareness of BTS guidelines regarding the documentation of initiation and further adjustment of oxygen therapy. There was a lack of compliance with oxygen prescription; none of the patients had a valid prescription on our computer-based prescription (Cerner). The duration, target range and indications of Oxygen therapy were documented in 25% (18/72), 45.8% (33/72) and 42% (30/72) patients, respectively. Oxygen was initiated by communication order only. In a total of 16 weeks period, the repeated PDSA cycles showed significant improvement in safe oxygen prescription practices. Following intervention, oxygen electronic prescription, documentation of indications for oxygen therapy, target oxygen saturation and wean-off plan improved to 93%, 85%, 86 % and 80 %, respectively.We concluded that poor compliance to oxygen therapy Orders is a universal issue, which can be successfully managed using small-scale PDSA cycles to ensure sustained improvement through multidimensional interventions, continuous reinforcement and frequent reassessments.

Acute Myocardial Infarction Attributed to Coronary Artery Embolism in a Patient with Atrial Fibrillation Secondary to Thyrotoxicosis - An Underrecognized Entity: A Case Report and Literature Review.

Nonatherosclerotic causes of acute myocardial infarction (MI) are infrequent, with atrial fibrillation as the most common etiology in cases of embolic MI. This entity, however, along with other causes of coronary embolus remains underappreciated as a probable cause of acute coronary syndromes.[1] Our case delineates a rare presentation of STEMI due to cardioembolic origin secondary to atrial fibrillation in a patient with thyrotoxicosis, previously undiagnosed to have an abnormal cardiac rhythm.

Cardiac Amyloidosis Presenting with Pre-Excitation Syndrome, Heart Failure, and Severe Factor X Deficiency as Part of Systemic Amyloid Light-Chain (AL) Amyloidosis - A Fatal Combination.

BACKGROUND Amyloid light-chain (AL) amyloidosis is a disease that results in systemic amyloid deposition, which may present with multi-organ dysfunction. It carries a poor prognosis at the time of diagnosis. CASE REPORT A 37-year-old patient with a history of Wolff-Parkinson-White syndrome and thyroiditis presented with syncope and hypovolemia. ECG showed non-specific T wave inversions in the lateral leads with no signs of ischemia. Laboratory investigations revealed deranged coagulation parameters with prolonged prothrombin time (PT) and activated partial thromboplastin time (aPTT) and follow-up factor assays revealed severe factor X deficiency. A transthoracic echocardiogram and subsequent cardiac MRI showed signs of cardiac amyloidosis. Bone marrow biopsy was consistent with AL amyloidosis, demonstrating period acid-Schiff (PAS)-positive adipose deposits and interstitial infiltration by clusters of lambda restricted plasma cells with aberrant expression of CD 56 and CD 117.The patient was treated with bortezomib, cyclophosphamide, and dexamethasone, but died early during his treatment due to cardiac arrest, suspected to be secondary to conduction abnormalities caused by cardiac infiltration. CONCLUSIONS This case represents a novel pattern of disease in AL amyloidosis with cardiac, thyroid, and hematological involvement as a result of systemic amyloid deposition.Our report highlights the need for physicians to be aware of cardiac amyloidosis-related complications and the morbidity and mortality associated with concurrent hematological involvement in these cases.

Artesunate-induced hemolysis in severe complicated malaria - A diagnostic challenge: A case report and literature review of anemia in malaria.

Malaria infection, which results due to the parasitic protozoan Plasmodium, has several known etiologies of hemolytic anemia as a possible complication in cases such as concurrent G6PD deficiency, severe parasitemia, or use of parenteral antimalarials. Although artemisinin-based antimalarial therapies are generally well-tolerated, several cases of severe post-artemisinin delayed hemolysis (PADH) have been recently reported, which present a diagnostic challenge, and affect morbidity and mortality in patients with malarial infection. We highlight the case of a young lady with Plasmodium falciparum severe parasitemia who developed hemolytic anemia after parenteral artesunate therapy.

Isolated facial diplegia: A rare presentation of Guillain-Barre syndrome.

The paper presents a case of bilateral facial nerve palsy and its unique presentation. It discusses the etiologies of bilateral facial nerve palsy. We aim to provide awareness to its presentation, diagnosis, and management.

The diagnostic dilemma of bilateral optic neuritis and idiopathic intracranial hypertension coexistence in a patient with recent COVID-19 infection.

Owing to autoantibody production and thrombophilic disorders in COVID-19, physicians must have low threshold to investigate secondary IIH and demyelinating disorders in patients with headache and decreased vision following recent COVID-19 infection.

Impact of Chronic Kidney Disease on Severity and Mortality in COVID-19 Patients: A Systematic Review and Meta-analysis.

Coronavirus disease 19 (COVID-19) has affected over 180 countries, resulting in global mass death. It has been reported that patients with underlying disease are more likely to contract the disease and become critically ill. The impact of chronic kidney disease (CKD) on the severity of COVID-19 has been underlined in the literature. In this analysis, we have provided evidence of an association between CKD and COVID-19. We followed the PRISMA protocol and conducted a literature search using Google Scholar, EMBASE, PubMed, and Clinical trail.gov. The initial search yielded 2102 articles. We included 20 cohorts based on inclusion criteria reporting an association between CKD and COVID-19 after excluding irrelevant articles, including review articles and duplicates. We conducted pooled prevalence of CKD and meta-analysis to estimate the odds ratio (OR), 95% confidence interval (CI) using Cochrane RevMan (version 5.4, Copenhagen: The Nordic Cochrane Centre, The Cochrane Collaboration), and R programming language version 4.16-2 (University of Auckland, New Zealand). Our study involved 4350 patients from different countries, and 212 (4.9%) patients had CKD. Among 20 cohorts, 57.27% were male with a median age of 55.5 years. Eight hundred sixty-six patients developed severe COVID-19, and out of which, 39 (4.5%) were CKD patients. CKD patients had a significantly increased risk of severe disease as compared to non-CKD patients with a pooled OR of 2.15 (95% CI 1.16-4.01) (I2=41; p=0.02). Out of 443 COIVD-19 patients who died, 85 patients had CKD, with a prevalence of 19.18%. CKD patients had an increased risk of death as compared to non-CKD patients with a pooled OR of 5.58 (95% CI 3.27-9.54) (I2=0; p<0.00001). CKD is manifested as a common underlying disease in COVID-19 patients who had a worse prognosis, including mortality.

Thalassemia and Priapism: A Literature Review of a Rare Association.

Thalassemia is a hematologic disorder caused by genetic mutation resulting in impaired hemoglobin chain production. Patients with thalassemia commonly experience complications such as anemia, blood transfusion-related issues, hepatic or cardiac involvement, and psychosocial impacts. Rarely, priapism has been associated with thalassemia as an initial presentation or subsequently occurring at any time in the disease course. Our literature review summarizes the reported cases of thalassemia-associated priapism and delves into underlying mechanisms of its pathophysiology and appropriate management.

Non-pregnancy-related ovarian vein thrombosis: A rare cause of chronic abdominal pain.

BACKGROUND: Ovarian vein thrombosis (OVT) commonly occurs during the peripartum and postpartum period. However, few cases of idiopathic OVT unrelated to pregnancy have been described. CASE REPORT: We report a case of a previously healthy, 32-year-old female who presented with chronic right-sided abdominal pain. Abdominal and pelvic gadolinium-enhanced MRI showed a right OVT. The patient was not in the peripartum or postpartum period. Thrombophilia test results were negative, and no risk factors for thrombosis were noted. The patient received warfarin for 6 months, with resolution of her symptoms. CONCLUSION: The presented case emphasizes the significance of considering OVT as a cause of unexplained abdominal pain in a young female. We describe a rare case of idiopathic OVT with a unique presentation.

The Association of Acute Kidney Injury With Disease Severity and Mortality in COVID-19: A Systematic Review and Meta-Analysis.

Background and objective The coronavirus disease 2019 (COVID-19) pandemic has become a global healthcare emergency. The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), the causative agent of COVID-19, has a wide range of clinical manifestations ranging from subclinical infection to multi-organ failure. In addition to the respiratory system, COVID-19 also adversely affects the kidneys. In this study, we aimed to measure the prevalence of acute kidney injury (AKI) in COVID-19 and its association with the disease severity and mortality in COVID-19 patients. Materials and methods We conducted our study by following the Preferred Reporting Items for Systematic Review and Meta-analyses (PRISMA) guidelines. A comprehensive literature search using four databases (PubMed, EMBASE, Google Scholar, and clinicaltrial.gov) was performed. Our initial search returned 2,771 articles. After excluding review articles, duplicates, and non-relevant studies, we included 20 articles that reported an association between COVID-19 and AKI. We subsequently performed a random effect analysis to find the pooled prevalence, pooled odds ratio (OR) estimates, and 95% confidence intervals for severe COVID-19 and mortality outcomes in AKI using Cochrane RevMan (version 5.4) and R programming language (version 4.16-2). Results A total of 14,415 patients from various countries were included. Among the 20 cohorts, the median age was 55.8 ±8.39 years (range: 43-72 years), and 43.78% of the subjects were female. Out of a total of 14,415 patients, 3,820 developed AKI with a pooled prevalence of 11% (95% CI: 0.07-0.15; p<0.01; I2=98%). AKI was found to have a significant association with severe COVID-19 disease, with a pooled OR of 8.45 (95% CI: 5.56-12.56; p<0.00001; I2=0%). AKI was associated with significantly higher mortality in patients with COVID-19 with an OR of 13.52 (95% CI: 5.43-33.67; p<0.00001; I2=88%). Conclusion AKI manifests as a common COVID-19 complication, and COVID-19 patients with AKI generally have poor outcomes in terms of disease severity and mortality.

Hepatosplenic Bartonellosis in an Immunocompetent Teenager: An Atypical Presentation of Cat-Scratch Disease.

Infection with Bartonella henselae,a gram-negative coccobacillus, most frequently presents as cat-scratch disease (CSD) and often accompanies a recent history of cat bite or scratch. As compared to adults, teenagers and children or immunocompromised patients are predominantly affected by CSD. In immunocompetent individuals, CSD is typically a self-limiting clinical syndrome with complete resolution of febrile illness in two to four weeks with or without antimicrobial therapy. While most cases present with fever of unknown origin (FUO), previous reports have also documented atypical clinical presentation or systemic symptoms in few cases, including reports of hepatosplenic involvement. We present a case of visceral bartonellosis in an immunocompetent 15-year-old female, who presented with a six-week history of fever and abdominal pain with hepatosplenomegaly. She recovered completely after prolonged antibiotic treatment for six weeks with doxycycline and amikacin. We emphasize that in the workup of FUO, it may be pertinent to include bartonellosis as a differential especially in cases exhibiting hepatosplenomegaly on examination along with hepatosplenic lesions on imaging.

Acute Myopericarditis Induced by Subacute Thyroiditis: A Very Rare Association.

We present the case of a 38-year-old male with a history of acute chest pain associated with electrocardiographic ST-segment elevation and levels of myocardial damage markers. Few studies have evaluated chest pain and elevated troponin T during subacute thyroiditis. To the best of our knowledge, this is the reported case of myopericarditis associated with increased thyroid hormones in the bloodstream and accompanied by a significant increase in troponin T and cardiac magnetic resonance imaging findings of myopericarditis during the acute phase of subacute thyroiditis.