Mortality-associated risk factors for transfusion-associated circulatory overload.

BACKGROUND AND OBJECTIVES: Respiratory transfusion reactions associate strongly with morbidity and mortality, and transfusion-associated circulatory overload (TACO) is the leading cause of reaction-related deaths. Risk factors for TACO include transfusion speed and volume and cardiorenal comorbidities. MATERIALS AND METHODS: An academic health network haemovigilance database was interrogated to assess variables associating with 371 cases of TACO and involved-visit outcomes, using univariate and multivariate regression analysis. RESULTS: TACO reactions over 11 years were reported in 179 males and 192 females, median age (interquartile range) 65 (53-75) years. In-hospital and 28-day mortality were 17.5% and 12.9%, respectively. In univariate regression modelling, male sex, injury severity grade, product volume administered, the use of platelets and intensive care admissions were each associated with in-hospital and 28-day mortality (p < 0.05). However, after multivariate regression analysis, only male sex in transfusion recipients independently associated with mortality (p < 0.05). CONCLUSION: In this cohort, male recipient sex and platelet administration were associated with TACO-involving admissions not ending in survival.

Outcome of Initial 3 Years of Cranial Endoscopy at DG Khan Medical College and Teaching Hospital, Dera Ghazi Khan.

OBJECTIVE: With the evolution of surgical approaches, endoscopic skull base surgery has emerged as a suitable alternative to many other invasive methods. The aim of this study was to investigate the efficacy and outcome of cranial endoscopy in treating various neurosurgical intracranial pathologies in terms of procedural success and complications. METHODS: This observational, prospective case series was conducted at the Department of Neurosurgery of DG Khan Medical College from November 2017 to October 2020. The study enrolled 74 patients with indications for cranial neuroendoscopy. Clinical examination was performed, and a detailed history of the disease was obtained. Follow-up was conducted in the outpatient department. The collected data were analyzed using statistical analysis software. RESULTS: Of 77 procedures performed, endoscopic third ventriculostomy, arachnoid cyst fenestration, septostomy, colloid cyst excision, endoscopic assisted ventricular catheter placement, and intraventricular tumor biopsy were performed in 53.3%, 18.2%, 10.4%, 7.8%, 6.5%, and 3.9% of patients. Aqueductal stenosis was identified as the most common cause of obstructive hydrocephalus. Seizures and cerebrospinal fluid leaks were the most commonly reported complications (12% and 8.1%, respectively). The observed mortality rate was 2.7%. CONCLUSIONS: Neuroendoscopic surgery has become safe and effective, as surgeon experience and learning have lowered the risk of complications, and offers a low-cost alternative intervention.

A Cross-Sectional Study of Nemaline Myopathy.

OBJECTIVE: Nemaline myopathy (NM) is a rare neuromuscular condition with clinical and genetic heterogeneity. To establish disease natural history, we performed a cross-sectional study of NM, complemented by longitudinal assessment and exploration of pilot outcome measures. METHODS: Fifty-seven individuals with NM were recruited at 2 family workshops, including 16 examined at both time points. Participants were evaluated by clinical history and physical examination. Functional outcome measures included the Motor Function Measure (MFM), pulmonary function tests (PFTs), myometry, goniometry, and bulbar assessments. RESULTS: The most common clinical classification was typical congenital (54%), whereas 42% had more severe presentations. Fifty-eight percent of individuals needed mechanical support, with 26% requiring wheelchair, tracheostomy, and feeding tube. The MFM scale was performed in 44 of 57 participants and showed reduced scores in most with little floor/ceiling effect. Of the 27 individuals completing PFTs, abnormal values were observed in 65%. Last, bulbar function was abnormal in all patients examined, as determined with a novel outcome measure. Genotypes included mutations in ACTA1 (18), NEB (20), and TPM2 (2). Seventeen individuals were genetically unresolved. Patients with pathogenic ACTA1 and NEB variants were largely similar in clinical phenotype. Patients without genetic resolution had more severe disease. CONCLUSION: We present a comprehensive cross-sectional study of NM. Our data identify significant disabilities and support a relatively stable disease course. We identify a need for further diagnostic investigation for the genetically unresolved group. MFM, PFTs, and the slurp test were identified as promising outcome measures for future clinical trials.