Cardiac amyloidosis and aortic stenosis: a state-of-the-art review.

Cardiac amyloidosis is caused by the extracellular deposition of amyloid fibrils in the heart, involving not only the myocardium but also any cardiovascular structure. Indeed, this progressive infiltrative disease also involves the cardiac valves and, specifically, shows a high prevalence with aortic stenosis. Misfolded protein infiltration in the aortic valve leads to tissue damage resulting in the onset or worsening of valve stenosis. Transthyretin cardiac amyloidosis and aortic stenosis coexist in patients > 65 years in about 4-16% of cases, especially in those undergoing transcatheter aortic valve replacement. Diagnostic workup for cardiac amyloidosis in patients with aortic stenosis is based on a multi-parametric approach considering clinical assessment, electrocardiogram, haematologic tests, basic and advanced echocardiography, cardiac magnetic resonance, and technetium labelled cardiac scintigraphy like technetium-99 m (99mTc)-pyrophosphate, 99mTc-3,3-diphosphono-1,2-propanodicarboxylic acid, and 99mTc-hydroxymethylene diphosphonate. However, a biopsy is the traditional gold standard for diagnosis. The prognosis of patients with coexisting cardiac amyloidosis and aortic stenosis is still under evaluation. The combination of these two pathologies worsens the prognosis. Regarding treatment, mortality is reduced in patients with cardiac amyloidosis and severe aortic stenosis after undergoing transcatheter aortic valve replacement. Further studies are needed to confirm these findings and to understand whether the diagnosis of cardiac amyloidosis could affect therapeutic strategies. The aim of this review is to critically expose the current state-of-art regarding the association of cardiac amyloidosis with aortic stenosis, from pathophysiology to treatment.

Nephrotic syndrome following COVID-19 vaccination: a systematic review.

BACKGROUND: Severe acute respiratory syndrome coronavirus 2 infection has caused significant morbidity and mortality. Vaccines produced against this virus have proven highly effective. However, adverse events following vaccination have also been reported. One of them is nephrotic syndrome, that can be associated with different pathologic pictures. This review aims to provide a wider understanding of incidence, etiopathogenesis, and management of nephrotic syndrome following vaccination against SARS-CoV-2. METHODS AND RESULTS: A literature search was undertaken using appropriate keywords in various databases like PubMed, Google Scholar, Europe PMC, and Science Direct. Twenty-one articles were included following qualitative assessment. Data of 74 patients from these articles were included. DISCUSSION: The pathogenesis of nephrotic syndrome following COVID vaccination has been widely attributed to the activation of angiotensin-converting enzyme-2 receptors, leading to podocyte effacement. Relapses have also been reported in patients with prior history of nephrotic syndrome following COVID-19 vaccination. A renal biopsy is necessary to identify the histopathological picture. Management of COVID-19 vaccine-induced nephrotic syndrome was mainly reported as successfully attainable with corticosteroids and supportive management. CONCLUSION: Further investigations will help in establishing an early diagnosis and salvaging kidney function.

Neuroprotective Role of Klotho on Dementia.

Klotho, a gene found on chromosome 13q12, is involved in a variety of processes and signaling pathways in the human body related to vitamin D metabolism; cardiovascular, renal, musculoskeletal, and skin diseases; and cancer biology. However, more importantly, it has been linked to beneficial effects related to anti-aging. The levels of soluble Klotho in the blood have been found to decline with age, increasing the risk of age-related diseases. When the Klotho gene was silenced or defective, it caused a shorter lifespan. However, when the gene was overexpressed, it resulted in a longer lifespan. Klotho has positive benefits on the neurological system by causing a higher representation of useful longevity genes, preventing further neuronal damage, and offering neuroprotection. Thus, it has the potential to become a new treatment for many age-related diseases that cause dementia, including multiple sclerosis, Alzheimer's disease, and Parkinson's disease. In this review, we discuss the mechanisms of Klotho's benefits and roles on various organ systems, specifically on nervous system disorders that lead to dementia.

Ondansetron-induced pseudoallergy with non-ischemic myocardial injury: A rare case report of Kounis syndrome.

Ondansetron is an FDA-approved selective serotonin 5-HT3 receptor commonly indicated as an anti-emetic agent for nausea and vomiting. It is rare to observe fatal reactions from ondansetron despite having no allergies or previous exposure. We report a case of anaphylactoid reaction with spontaneous coronary vasospasms in response to intravenous ondansetron.

Secondary Infertility due to Fetal Bone Retention A systematic literature review.

Prolonged intrauterine retention of fetal bones during an abortion procedure can lead to secondary infertility. This review aimed to raise awareness among obstetric/gynaecologists about the possibility of this condition. A total of 17 case reports, seven case series and one retrospective study were included in this review, with 75 patients in total. Overall, 60% had a pregnancy termination in the second trimester, while 20% had a termination during the first trimester. Hysteroscopic resection was used to remove the intrauterine fetal bones in 69% of patients. In total, 59% of patients conceived following the procedure, 1% conceived despite the presence of intrauterine bones, 24% could not conceive at the time of the study and 16% had an unknown outcome. Transvaginal ultrasound was used for diagnosis in 41 (55%) patients, while pelvic ultrasound was used in 21 (28%) patients. In conclusion, secondary infertility is a common occurrence after a dilation and curettage procedure partially due to fetal bone retention. The gold standard for an accurate diagnosis and treatment is hysteroscopy.

Bitter bottle gourd poisoning: A case report and review of literature.

Bottle gourd juice is considered a panacea in traditional Indian medicine and used in various chronic diseases. Increased levels of Cucurbitacin can turn it bitter causing toxic effects in gastrointestinal system. We report the case of an elderly female who presented with shock in few hours after consumption of bitter bottle gourd juice. After proper evaluation we considered the possibility of bottle gourd poisoning as a probable diagnosis due to the Naranjo adverse drug reactions probability scale. Since there is no specific antidote available, management is mostly supportive with intravenous fluids, control of bleeding and management of shock.

Guillain-Barre syndrome following subclinical COVID-19 infection in a child.

The entire world is coping up with the challenges imposed by COVID-19 pandemic caused by a novel coronavirus, which started from a single case in Wuhan city of China in November 2019. Its outcomes range from asymptomatic cases to most severe diseases like severe acute respiratory syndrome. Neurological manifestations have also been reported as an outcome of coronavirus infection and Guillain-Barre Syndrome (GBS) is one of them. In our present case, we describe the correlation of GBS with subclinical SARS-CoV-2 in a pediatric patient. The patient was successfully managed with intravenous immunoglobulin and physiotherapy. In the current pandemic, any case of GBS should be evaluated for recent or remote SARS-CoV-2 infection.

Hemopericardium in the Setting of Direct Oral Anticoagulant Use: An Updated Systematic Review.

BACKGROUND: Spontaneous hemopericardium, associated with direct oral anticoagulant (DOAC) use, is one of the uncommon complications with high morbidity that has not been extensively studied We aimed to determine demographic characteristics, clinical features, lab evaluation, management, and outcomes of the studies focusing on hemopericardium as a DOAC use. METHODS: PubMed, Web of Science, Google Scholar, and CINAHL databases were searched for relevant articles using MeSH key-words and imported into referencing/review software. The data regarding demographics, clinical characteristics, cardiac investigations, and management were analyzed in IBM Statistics SPSS 21. t-Test and Chi-square test were used. A P score of <0.05 was considered statistically significant. RESULTS: After literature search, a total of 41 articles were selected for analysis. The mean age of the patients was 70.09 ± 11.06 years (p < 0.05); the majority of them were males (58.5%). Most of the patients presented with shortness of breath (75.2%) and had more than 3 co-morbid conditions (43.9%). The most frequently used anticoagulant was rivaroxaban (15/41; 36.6%); the common indication being arrhythmia (78.0%). CYP4503A4/P-Gp inhibitors (22.2%) were commonly used by the patients. Majority of the cases had a favorable outcome (95.1%). Pericardial tamponade was noted in 31/41 cases. Pericardiocentesis was performed in 37/41 cases. CONCLUSIONS: Hemopericardium from DOAC use has a favorable outcome but requires urgent pericardiocentesis. However, long term mortality, monitoring of DOAC activity, and drug-drug interactions have not been widely studied.

Bowel ischemia in COVID-19: A systematic review.

BACKGROUND: Gastrointestinal complications of COVID-19 have been reported over the last year. One such manifestation is bowel ischaemia. This study thus aims to provide a more holistic review of our current understanding of COVID-19-induced bowel ischaemia. METHOD AND RESULTS: A meticulous search was performed using different keywords in PubMed and Google Scholar. Fifty-two articles were included in our study after applying inclusion and exclusion criteria and performing the qualitative assessment of the studies. A total of 25 702 patients were included in our study after the completion of the qualitative assessment. DISCUSSION: The common symptoms of GIT in COVID-19 patients are as diarrhoea, vomiting, nausea and abdominal pain. The mechanism of bowel ischaemia is associated with the formation of emboli which is related to COVID-19's high affinity for angiotensin-converting enzyme-2 on enterocytes, affecting the superior mesenteric vessels. Clinically, patients present with abdominal pain and vomiting. CT angiography of the abdomen and pelvis showed acute intestinal ischaemia (mesenteric). Management is usually initiated with gastric decompression, fluid resuscitation, and haemodynamic support. Surgical intervention is also sought. CONCLUSION: Intestinal ischaemia presenting in patients with COVID-19 has to be considered when symptoms of severe abdominal pain are present. More research and guidelines are required to triage patients with COVID-19 to suspect intestinal ischaemia and to help in diagnosis and management.

Glutaric Aciduria Type II With Ketosis in a Male Infant.

Glutaric aciduria type II (GA II) also known as multiple acyl-CoA dehydrogenase deficiency is an inborn metabolic disorder belonging to the family of organic acidurias. It is a disorder that interferes with the body's ability to break down proteins and fats to produce energy. Tandem mass spectrometry (TMS) acts as a screening tool, while the diagnosis of GA-II with ketosis is confirmed by a combination of tests like organic acids, quantitative random urine, and a full urine panel. Early diagnosis, compliance to specialized diet, affordability, and regular follow-ups are required to tackle this potentially life-threatening condition. Herein, we report a case of glutaric aciduria type-II with ketosis in a 4.5 months old male infant who was managed with a low-protein diet, which was free of tryptophan, lysine, and other specific dietary supplements.

Rare manifestation of common disease: An adolescent girl with transient ischemic attack (TIA) like presentation in multiple tuberculoma.

Central nervous system (CNS) tuberculosis is a life-threatening infection and has a wide variety of presentations in children. We report a case of intracranial tuberculomas with tuberculous cervical lymphadenitis in an adolescent girl with a recurrent transient ischemic attack (TIA) like unusual presentation managed with antitubercular therapy and anti-convulsant.