Does Caplacizumab for the management of thrombotic thrombocytopenic purpura increase the risk of relapse, exacerbation, and bleeding? An updated systematic review and meta-analysis based on revised criteria by the International Working Group for thrombotic thrombocytopenic purpura.

Thrombotic thrombocytopenic purpura (TTP) is a rare and life-threatening condition marked by abnormal blood clotting and organ damage. Caplacizumab is a potential treatment for the TTP management. This systematic review and meta-analysis aimed to assess Caplacizumab's effectiveness and safety in the TTP management. A comprehensive database search identified nine studies, including randomized controlled trials and observational studies. Primary outcomes included TTP exacerbation, relapse, and major bleeding. Major bleeding risk was evaluated using updated definitions recommended by the International TTP Working Group in 2021. Revised criteria proposed by the IWG for TTP recurrence were employed for a comprehensive assessment of Caplacizumab's impact on relapse and exacerbation. Analysis revealed Caplacizumab significantly reduced all-cause mortality in TTP patients. Some studies raised concerns about bleeding risk, but overall, it did not significantly differ from standard treatment. Likewise, there was no significant difference in TTP relapse rates between Caplacizumab and standard care. This study supports Caplacizumab as a potential adjunct therapy for TTP. However, careful consideration of its advantages and risks is crucial in clinical practice. Further research is needed to address concerns related to adverse effects like bleeding risk and relapse rates associated with Caplacizumab in the TTP management. The findings emphasize the importance of weighing potential benefits and risks when considering Caplacizumab as an adjunct therapy for TTP.

Differences in treatment and outcomes among patients with ST-segment elevation myocardial infarction with and without standard modifiable risk factors: a systematic review and meta-analysis.

There are limited data available on outcomes and pathophysiology behind ST-segment elevation myocardial infarction (STEMI) in populations without standard modifiable risk factors (SMuRFs). The authors carried out this meta-analysis to understand the differences in treatment and outcomes of STEMI patients with and without SMuRFs. Methods: A systematic database search was performed for relevant studies. Studies reporting desired outcomes among STEMI patients with and without SMuRFs were selected based on predefined criteria in the study protocol (PROSPERO: CRD42022341389). Two reviewers independently screened titles and abstracts using Covidence. Full texts of the selected studies were independently reviewed to confirm eligibility. Data were extracted from all eligible studies via a full-text review of the primary article for qualitative and quantitative analysis. In-hospital mortality following the first episode of STEMI was the primary outcome, with major adverse cardiovascular events (MACE), repeat myocardial infarction (MI), cardiogenic shock, heart failure, and stroke as secondary outcomes of interest. Odds ratio (OR) with a 95% CI was used to estimate the effect. Results: A total of 2135 studies were identified from database search, six studies with 521 150 patients with the first STEMI episode were included in the analysis. The authors found higher in-hospital mortality (OR: 1.43; CI: 1.40-1.47) and cardiogenic shock (OR: 1.59; 95% CI: 1.55-1.63) in the SMuRF-less group with no differences in MACE, recurrent MI, major bleeding, heart failure, and stroke. There were lower prescriptions of statin (OR: 0.62; CI: 0.42-0.91) and Angiotensin converting enzyme inhibitor /Angiotensin II receptor blocker (OR: 0.49; CI: 0.28-0.87) at discharge in SMuRF-less patients. There was no difference in procedures like coronary artery bypass graft, percutaneous coronary intervention, and thrombolysis. Conclusion: In the SMuRF-less STEMI patients, higher in-hospital mortality and treatment discrepancies were noted at discharge.

An Unusual Diagnosis of Sporadic Type III Osteogenesis Imperfecta in the First Day of Life.

Osteogenesis imperfecta (OI) is a group of rare, permanent genetic bone disorders resulting from the mutations in genes encoding type 1 collagen. It usually is inherited by an autosomal dominant pattern, but it can sometimes occur sporadically. Among the four main types, type III is the most severe type which presents with multiple bone fractures, skeletal deformities, blue sclera, hearing, and dental abnormalities. It is estimated that only 1 in 20,000 cases of OI are detected during infancy, and the diagnosis carries a poor prognosis. This case is reported for the rarity of sporadic OI diagnosis in neonates. We present a case of a 1-day-old neonate following a normal vaginal delivery referred to our center in the view of low birth weight and multiple bony deformities. Physical examination revealed an ill-looking child with poor suckling, gross bony deformities in upper and lower limbs, and blue sclera. X-ray showed thin gracile bones with multiple bone fractures. Echocardiography revealed a 4 mm patent ductus arteriosus. The patient was diagnosed with type III OI with patent ductus arteriosus. Though OI is rare in neonates and infants, it should be considered in the differentials in a newborn presenting with multiple bony deformities regardless of family history, history of trauma, or physical abuse. OI is also associated with cardiac anomalies such as the atrial septal defect and patent ductus arteriosus for which echocardiography is recommended routinely.

A rare occurrence of psoas abscess with uterine didelphys and renal agenesis: A case report.

INTRODUCTION: Mullerian duct anomaly such as uterine didelphys is a rare congenital anomaly of female genitourinary tract presenting with dysmenorrhea, hematocolpus, and subfertility in adolescent females. It is commonly associated with wolffian duct anomaly like renal agenesis. Here we present a rare association of psoas abscess with uterine didelphys and renal agenesis in a 21-year-old unmarried female. CASE PRESENTATION: We report a case of 21-year-old female presenting with right hip pain, recurrent genital infections and dysmenorrhea. Her labs suggested infectious etiology whereas radiological investigation revealed right psoas abscess. In addition, she was found to have uterine didelphys with pyometra, right adnexal mass, and right renal agenesis. CLINICAL DISCUSSION: Uterine didelphys commonly present with dysmenorrhea and hematocolpos along with various non-specific symptoms. Patients can develop psoas abscess secondary to uterine didelphys, but uterine didelphys presenting with psoas abscess is fairly rare. Psoas abscess on itself is a difficult condition to diagnose, more so when associated with rare uterine anomalies. CONCLUSION: This case highlights the possibility of psoas abscess as a primary presentation of Mullerian duct anomaly. Further, a differential of uterine didelphys should be considered in every reproductive age female presenting with recurrent pelvic infection.

Pericardial effusion in hypothyroidism: A case report.

INTRODUCTION: and importance: Hypothyroidism is an endocrine disorder with multiorgan involvement and various complications. One of the significant but less often seen complications is pericardial effusion. Since it can progress to life-threatening conditions like cardiac tamponade and hemodynamic instability, early diagnosis, and management of the pericardial effusion in hypothyroidism is a must. CASE PRESENTATION: We present a case of a 35-year-old male who presented with bilateral lower limb swelling, facial puffiness, cold intolerance, fatigue, and hoarseness of voice for one week. Laboratory investigation showed high thyroid-stimulating hormone (TSH), low triiodothyronine (T3), and raised serum anti-thyroid peroxidase (anti-TPO). The lipid profile demonstrated hypertriglyceridemia. Ultrasonography of the neck revealed normal thyroid size with decreased echo texture and increased vascularity. An electrocardiogram showed low voltage complexes with sinus bradycardia. 2D echocardiography revealed minimal pericardial effusion with normal ventricular function. The patient was managed with thyroxine therapy which gradually resolved his symptoms and pericardial effusion. CLINICAL DISCUSSION: Pericardial effusion in hypothyroidism is due to the increased capillary permeability and albumin distribution volume and reduced lymph drainage in the pericardial cavity. Its presence in mild cases of hypothyroidism is uncommon although it can be seen in severe, long-standing hypothyroidism. Pericardial effusion in hypothyroidism, though rare, can present in mild cases and if overlooked can be fatal due to conditions like cardiac tamponade. CONCLUSION: With early cardiac assessment and adequate thyroid replacement therapy, pericardial effusion in hypothyroidism can be reversible at an early stage. So, pericardial effusion which can be overlooked in mild cases of hypothyroidism needs to be identified and managed early.

Vitamin C in Critically Ill Patients: An Updated Systematic Review and Meta-Analysis.

BACKGROUND: Vitamin C is a water-soluble antioxidant vitamin. Oxidative stress and its markers, along with inflammatory markers, are high during critical illness. Due to conflicting results of the published literature regarding the efficacy of vitamin C in critically ill patients, and especially the concerns for nephrotoxicity raised by some case reports, this meta-analysis was carried out to appraise the evidence and affirmation regarding the role of vitamin C in critically ill patients. METHODS: We searched the database thoroughly to collect relevant studies that assessed intravenous vitamin C use in critically ill patients published until 25 February 2021. We included randomized controlled trials and observational studies with 20 or more critically ill patients who have received intravenous ascorbic acid (vitamin C). After screening 18,312 studies from different databases, 53 were included in our narrative synthesis, and 48 were included in the meta-analysis. We used the Covidence software for screening of the retrieved literature. Review Manager (RevMan) 5.4 was used for the pooling of data and Odds Ratios (OR) and Mean difference (MD) as measures of effects with a 95% confidence interval to assess for explanatory variables. RESULTS: Pooling data from 33 studies for overall hospital mortality outcomes using a random-effect model showed a 19% reduction in odds of mortality among the vitamin C group (OR, 0.81; 95% CI, 0.66-0.98). Length of hospital stay (LOS), mortality at 28/30 days, ICU mortality, new-onset AKI and Renal Replacement Therapy (RRT) for AKI did not differ significantly across the two groups. Analysis of data from 30 studies reporting ICU stay disclosed 0.76 fewer ICU days in the vitamin C group than the placebo/standard of care (SOC) group (95% CI, -1.34 to -0.19). This significance for shortening ICU stay persisted even when considering RCTs only in the analysis (MD, -0.70; 95% CI, -1.39 to -0.02). CONCLUSION: Treatment of critically ill patients with intravenous vitamin C was relatively safe with no significant difference in adverse renal events and decreased in-hospital mortality. The use of vitamin C showed a significant reduction in the length of ICU stays in critically ill patients.