Wilms tumor with Mulibrey Nanism: A case report and review of literature.

BACKGROUND: Mulibrey-Nanism (Muscle-liver-brain-eye Nanism = dwarfism; MUL) is a rare genetic syndrome. The underlying TRIM37 mutation predisposes these children to develop tumors frequently. In the largest published series of MUL, 8% patients were reported to develop Wilms tumor (WT). The published literature lacks data regarding the best treatment protocol and outcome of this cohort of children with WT and MUL. We report here a 2-year-old boy with WT and MUL and present a review of literature on WT in MUL. CASE: Our patient had associated cardiac problems of atrial septal defect, atrial flutter and an episode of sudden cardiac arrest. We managed him successfully with chemotherapy, surgery and multi-speciality care. He is alive and in remission at follow-up of 6 months. CONCLUSION: A total of 14 cases (including present case) of WT have been reported in MUL and treatment details were available for six cases. They were managed primarily with surgery, chemotherapy with/without radiotherapy, and all achieved remission. The outcome data is available only for two cases, one has been followed up till 15 years post treatment for WT and other is our patient.

COVID-19 in children with severe aplastic anemia

Introduction Coronavirus disease 2019 (COVID-19) affects children but mostly has mild course. There is meagre published data on the impact of COVID-19 illness in children with Severe Aplastic anemia (SAA). We describe our experience of managing COVID-19 in children with SAA. Method Three children of SAA who developed SARS-CoV-2 infection are included in this study. Results Patient 1 was post Immunosuppressive therapy (IST) for SAA and had an asymptomatic course and uneventful recovery. Patient 2 was several months post IST with no response and had an asymptomatic COVID-19 illness but had delayed viral clearance, however he succumbed to bacterial sepsis soon after. Patient 3 was awaiting IST and while he contracted severe acute respiratory syndrome-Coronavirus-2 (SARS-CoV-2) infection, he had symptomatic COVID-19 illness followed by bacterial and fungal sepsis to which he succumbed. Conclusion: COVID-19 in children with SAA can be mild to fatal course and virus may have delayed clearance. It can lead to delay in therapy of SAA.

Clinico-pathological features in fatal COVID-19 infection: a preliminary experience of a tertiary care center in North India using postmortem minimally invasive tissue sampling.

OBJECTIVES: To study the histopathology of patients dying of COVID-19 using post-mortem minimally invasive sampling techniques. METHODS: This was a single-center observational study conducted at JPNATC, AIIMS. Thirty-seven patients who died of COVID-19 were enrolled. Post-mortem percutaneous biopsies were taken from lung, heart, liver, kidney and stained with hematoxylin and eosin. Immunohistochemistry was performed using CD61 and CD163. SARS-CoV-2 virus was detected using IHC with primary antibodies. RESULTS: The mean age was 48.7 years and 59.5% were males. Lung histopathology showed diffuse alveolar damage in 78% patients. Associated bronchopneumonia was seen in 37.5% and scattered microthrombi in 21% patients. Immunopositivity for SARS-CoV-2 was observed in Type II pneumocytes. Acute tubular injury with epithelial vacuolization was seen in 46% of renal biopsies. Seventy-one percent of liver biopsies showed Kupffer cell hyperplasia and 27.5% showed submassive hepatic necrosis. CONCLUSIONS: Predominant finding was diffuse alveolar damage with demonstration of SARS-CoV-2 protein in the acute phase. Microvascular thrombi were rarely identified in any organ. Substantial hepatocyte necrosis, Kupffer cell hypertrophy, microvesicular, and macrovesicular steatosis unrelated to microvascular thrombi suggested that liver might be a primary target of COVID-19.

Assessment of healthcare worker's hand hygiene and infection prevention practices of their personal belongings in a healthcare setting: a survey in pre COVID-19 era and literature review on standard disinfection practices.

BACKGROUND: Healthcare workers' (HCW) hands and personnel belongings are vehicles of transmission of nosocomial infections. Knowledge, attitude, and practice of hand hygiene have been extensively studied suggesting adequate knowledge but poor compliance. Similar data on aprons, mobile phone and stethoscope disinfection practices are lacking. This becomes an extensively important topic of discussion in current COVID-19 pandemic where inadequacy in hygiene practices is devastating. AIM: To study the knowledge, attitude, and infection prevention practices of HCWs aprons, electronic devices, stethoscopes, and hands. METHODS: A cross sectional questionnaire-based survey was conducted among HCWs of Medicine ward and ICU. RESULTS: Sixty-six HCWs responded to the survey. Awareness that hands, aprons, mobile phones, stethoscopes could cause cross transmission and knowledge of correct practices was present in majority of the respondents. Hand hygiene was performed by 65.2% of the respondents before touching a patient and 54.5% after touching the patient surroundings while 13.6% performed only when it was visibly soiled. Mobile phones and stethoscopes were disinfected by 13.6 and 30.3% of the respondents after each patient encounter, respectively. Aprons were washed after using them at a stretch for a median duration of 5 days (1-30 days). Forgetfulness, lack of reinforcement, lack of time, inadequate awareness on standard disinfection practices and fear of damaging electronic devices from disinfectants use were reasons for poor compliance. CONCLUSIONS: There is an urgent need to spread awareness and formulate standard guidelines on disinfection practices especially for mobile phones, stethoscopes, and aprons in addition to reinforcing hand hygiene practices.

Profile of co-infections & secondary infections in COVID-19 patients at a dedicated COVID-19 facility of a tertiary care Indian hospital: Implication on antimicrobial resistance.

BACKGROUND: The COVID-19 pandemic has raised concerns over secondary infections because it has limited treatment options and empiric antimicrobial treatment poses serious risks of aggravating antimicrobial resistance (AMR). Studies have shown that COVID-19 patients are predisposed to develop secondary infections. This study was conducted to ascertain the prevalence and profiles of co- & secondary infections in patients at the COVID-19 facility in North India. METHODS: We studied the profile of pathogens isolated from 290 clinical samples. Bacterial and fungal pathogens were identified, and antimicrobial susceptibility was determined by the Vitek2® system. Additionally, respiratory samples were tested for any viral/atypical bacterial co-infections and the presence of AMR genes by FilmArray test. The clinical and outcome data of these patients were also recorded for demographic and outcome measures analyses. RESULTS: A total of 151 (13%) patients had secondary infections, and most got infected within the first 14 days of hospital admission. Patients aged >50 years developed severe symptoms (p = 0.0004) and/or had a fatal outcome (p = 0.0005). In-hospital mortality was 33%.K.pneumoniae (33.3%) was the predominant pathogen, followed by A. baumannii (27.1%). The overall resistance was up to 84%.Majority of the organisms were multidrug-resistant (MDR) harbouring MDR genes. CONCLUSION: A high rate of secondary infections with resistant pathogens in COVID-19 patients highlights the importance of antimicrobial stewardship programs focussing on supporting the optimal selection of empiric treatment and rapid-de-escalation, based on culture reports.

Convalescent plasma therapy for severe Covid-19 in patients with hematological malignancies.

BACKGROUND: Data on convalescent plasma therapy (CPT) in patients of hematological malignancies with severe Covid-19 is scarce. OBJECTIVE: To study 14-day mortality in patients who received CPT. PATIENTS & METHODS: Retrospective multicentre observational study conducted in 4 centres treating haematological malignancies across Delhi-national capital region. Total 33 haematological malignancies patients with severe Covid-19 who received CPT were analysed. RESULTS: The median age of the study cohort was 62 years (18-80 years). Twenty one percent patients had 1 comorbidity, 18 % had 2 comorbidities and 6% patients had 3 and 5 comorbidities each. Twenty four patients were on active therapy. Sixty nine percent of patients required ICU stay. Twenty five patients received plasma therapy within 7 days (early) of diagnosis of Covid-19 infection. Median day of plasma infusion from date of diagnosis of Covid-19 infection was 4 days (range: 2-25 days). Patient who had early initiation of plasma therapy had shorter duration of hospitalisation (12.7 vs 24.3 days, p = 0.000). Overall mortality in the cohort was 45.5%. There was no effect of disease status, active therapy, presence of comorbidity on mortality. There was no difference in the mortality in patients receiving early vs late initiation of plasma therapy or in patients receiving one versus two plasma therapy. CONCLUSIONS: We provide a large series of patients with hematological malignancies and role of CPT in this group.