Perioperative anesthetic concerns in postcovid mucormycosis patients posted for surgical intervention: An observational Study.

Background: COVID 19 pandemic caused by severe acute respiratory syndrome coronavirus -2 has proven to be the deadliest pandemic till date. Multiple covid waves have hit people hard on each part of the continent throughout the world. The second wave in India turned out to be highly infectious and virulent. Sudden surge in cases of mucormycosis after recovery of COVID surprised many clinician. Mucormycosis being a rapidly progressive and fulminant fungal infection required surgical debridement of necrotic tissue on emergency basis. The fatal combination of immunocompromised status, multisystemic involvement, and difficult airway in these patients pose numerous new challenges regarding anesthetic management. The present study was conducted to outline major concerns and the anesthetic management of patients undergoing surgical resection for rhinoorbital mucormycosis (ROM). Materials and Methods: A retrospective observational study was conducted in our institute for a duration of 2 months (June and July 2021). The data of all the cases posted for ROM was collected from the ENT and Anesthesia record register. Total 70 patients presented with mucormycosis, for surgical debridement out of which 25 patients were posted for surgery under general anesthesia or monitored anesthesia care (MAC). Demographic characteristics, comorbidities, duration of COVID illness, treatment taken during COVID (oxygen therapy/steroid intake), hemodynamic parameters, monitoring methods, and surgical procedures were recorded for each patient. Statistical Evaluation: SPSS version 21.0 was used for data analysis. Mean and SD were used to analyze the difference in mean values, and independent Student's t-test were utilized to compare the quantitative variables. Frequency distribution and percentage were used for qualitative parameters. Significant difference was accepted at P ≤ 0.05 with 95% CI (confidence interval) in the study. Results: Demographic data were comparable with respect to age, gender distribution, and ASA status. Mean duration of Covid illness was (12.18 ± 3.68) days. The mean HbA1C measured was (10.8 ± 1.42). Strong correlation was found between steroid intake and raised HbA1c in all patients (r = 0.77). Regarding the comorbidities, 24 (96%) patients had associated type 2 diabetes mellitus, 16 patients (64%) had pneumonitis, and 1 patient had pulmonary TB and hepatitis. Conclusion: Considering the perioperative risk associated with high HbA1C and pneumonitis, MAC was preferred in majority of cases. Strict hemodynamic monitoring, perioperative glucose control, difficult airway cart, metabolic and electrolyte balance and vigilant peri-operative monitoring are cornerstone for better outcome and short length of hospital stay.

Comparison of women with possible endocervical and non-cervical glandular neoplasms detected in liquid-based cervical cytology- incidence, clinical characteristics and outcomes: A cohort study.

OBJECTIVE: To compare the incidence, demographics and clinical outcomes of women presenting with possible non-cervical (NC) and endocervical (EC) glandular neoplasms in their cervical smears. STUDY DESIGN: Retrospective analysis of a prospective cohort within the NHS Greater Glasgow and Clyde- the largest health organisation in Scotland, UK. METHODS: Cases identified from the Scottish Cervical Call Recall System between January 2013 and December 2017. Incidence and clinical trajectories of NC and EC were reviewed. RESULTS: Two-hundred-and-thirty cases (NC = 41; EC = 189) from 486,240 smears were evaluated. The incidence was 8.4 and 38.9 per 100,000 smear-year for NC and EC, respectively. Compared to women with EC, women with NC were significantly older (p < 0.0001), had higher body mass index (p < 0.0001), more likely to present with symptoms (58.5 % vs 10.5 %; p < 0.0001), had cancers (48.8 % vs 13.8 %; p < 0.0001) and died from their diseases (9.8 % vs 0.5 %; p < 0.0001). Even in the asymptomatic screen-detected NC group, almost a quarter (23.5 %) had endometrial cancer. Age was not associated with high-risk histology (p = 0.289). High-risk colposcopic appearance had good positive predictive value (90.0 %; 95 %CI: 81.2-95.6 %) for high-risk histology, but poor negative predictive value (41.3 %; 95 %CI: 29-54 %). Negative excision margin was associated with favourable outcomes. CONCLUSIONS: NC and EC are rare, but they are distinct and should be reported separately in future studies. The risks of malignancies are high, particularly in women with NC, even if they are asymptomatic. Thus, prompt and thorough investigations and treatments are required to prevent and treat malignancies.

Defining the clinical, molecular and imaging spectrum of adaptor protein complex 4-associated hereditary spastic paraplegia.

Bi-allelic loss-of-function variants in genes that encode subunits of the adaptor protein complex 4 (AP-4) lead to prototypical yet poorly understood forms of childhood-onset and complex hereditary spastic paraplegia: SPG47 (AP4B1), SPG50 (AP4M1), SPG51 (AP4E1) and SPG52 (AP4S1). Here, we report a detailed cross-sectional analysis of clinical, imaging and molecular data of 156 patients from 101 families. Enrolled patients were of diverse ethnic backgrounds and covered a wide age range (1.0-49.3 years). While the mean age at symptom onset was 0.8 ± 0.6 years [standard deviation (SD), range 0.2-5.0], the mean age at diagnosis was 10.2 ± 8.5 years (SD, range 0.1-46.3). We define a set of core features: early-onset developmental delay with delayed motor milestones and significant speech delay (50% non-verbal); intellectual disability in the moderate to severe range; mild hypotonia in infancy followed by spastic diplegia (mean age: 8.4 ± 5.1 years, SD) and later tetraplegia (mean age: 16.1 ± 9.8 years, SD); postnatal microcephaly (83%); foot deformities (69%); and epilepsy (66%) that is intractable in a subset. At last follow-up, 36% ambulated with assistance (mean age: 8.9 ± 6.4 years, SD) and 54% were wheelchair-dependent (mean age: 13.4 ± 9.8 years, SD). Episodes of stereotypic laughing, possibly consistent with a pseudobulbar affect, were found in 56% of patients. Key features on neuroimaging include a thin corpus callosum (90%), ventriculomegaly (65%) often with colpocephaly, and periventricular white-matter signal abnormalities (68%). Iron deposition and polymicrogyria were found in a subset of patients. AP4B1-associated SPG47 and AP4M1-associated SPG50 accounted for the majority of cases. About two-thirds of patients were born to consanguineous parents, and 82% carried homozygous variants. Over 70 unique variants were present, the majority of which are frameshift or nonsense mutations. To track disease progression across the age spectrum, we defined the relationship between disease severity as measured by several rating scales and disease duration. We found that the presence of epilepsy, which manifested before the age of 3 years in the majority of patients, was associated with worse motor outcomes. Exploring genotype-phenotype correlations, we found that disease severity and major phenotypes were equally distributed among the four subtypes, establishing that SPG47, SPG50, SPG51 and SPG52 share a common phenotype, an 'AP-4 deficiency syndrome'. By delineating the core clinical, imaging, and molecular features of AP-4-associated hereditary spastic paraplegia across the age spectrum our results will facilitate early diagnosis, enable counselling and anticipatory guidance of affected families and help define endpoints for future interventional trials.