Spectrum of Primary Immunodeficiency Disorders in Hospitalized Children: Multicentric Data From Kolkata.

OBJECTIVE: To evaluate the incidence and types of primary immunodeficiency diseases (PIDs) in hospitalized children with infection. METHODS: This prospective study was conducted in five tertiary-care facilities in Kolkata over two consecutive years between November 1, 2018 and October 31, 2020. We included all children aged upto 12years who were hospitalized and screened them for PID. Children were screened for suspected IPD using Jeffrey Modell Foundation (JMF) Criteria; any child who satisfied at least 2 out of 10 warning signs was further evaluated for PIDs. RESULTS: Out of 33,204 hospital admissions, 50 children satisfied JMF criteria. Out of 50 children screened during the study period, 27 were finally diagnosed with an underlying PID, with a prevalence of 1 in 1000 hospitalized children. Majority (37.03%) of them had antibody deficiency followed by phagocytic defect (33.3%). Chronic granulomatous disease was the commonest PID followed by common variable immunodeficiency. Around 62.97% children presented with respiratory infections and overall Acinetobacter baumannii was the commonest isolated organism. CONCLUSION: Our study presents the first cohort of PID from eastern India. A methodical step-wise clinical and diagnostic approach can facilitate early diagnosis and timely therapeutic interventions.

Mortality indicators with clinical profile of multisystem inflammatory syndrome in children during SARS-CoV-2 second wave in India: A tertiary referral center experience.

Background: Post-COVID-19 cases are being reported with features of hyperinflammatory state causing multiple system dysfunctions in previously healthy children. Objectives: To describe clinical characteristics, laboratory, and radiological profile of children affected with COVID-19-related multisystem inflammatory syndrome postsecond wave in India and compare them with respect to adverse outcome. Materials and Methods: This prospective, observational study was conducted in the department of pediatrics of a tertiary care center in Eastern India over a period of 3 months. Demographic data, clinical details, biochemical parameters, and treatment with clinical outcome were recorded. Children who survived the clinical course were compared with those died during hospital stay. Results: Thirty-five children with a median age of 4.8 (3.9) years were included who were admitted between June 16 and September 15, 2021. Only 17.14% had reverse transcription-polymerase chain reaction positivity previously with 77.14% had positive COVID-19 serology. Most common features were fever (100%), edema (68.6%), gastrointestinal (71.4%), mucocuteneous (65.7%), cardiovascular (57.1%), and neurological symptoms (45.7%). Twenty (57.1%) children had shock at presentation. Decreased ejection fraction (<55%) was the most common echocardiographic feature (37.14%) followed by coronary dilatation (20%). Majority (77.14%) of the patients required intensive care with inotrope requirement in 62.86% cases. Forty percent patients were intubated with mean duration of 9.94 (±10.5) days. All patients received methylprednisolone and 76% were given intravenous immunoglobulin. Tocilizumab was used in three patients. Nine patients died (25.7%) with overall median pediatric intensive care unit stay of 13 (14) days. Conclusion: Of the parameters described, we have found shock, heart failure, neurological involvement at presentation, infancy, and laboratory parameters such as C-reactive protein, CPK, D-Dimer, and lactate dehydrogenase were the predictors of mortality.

An extremely rare case of endophthalmitis in an infant with congenital tuberculosis.

Congenital tuberculosis (CTB) is the transmission of Mycobacterium tuberculosis infection from mother to infant during the intrauterine period or delivery. An 82-day-old infant presented to Nilratan Sircar Medical College and Hospital, Kolkata, with a history of persistent fever from Day 15 of age. Over the course of more than 2 months, there were pneumonia, hepatosplenomegaly and endophthalmitis which were unresponsive to a range of antibiotics, and there had been several admissions to local hospitals. On this admission, his chest radiograph and contrast-enhanced computed tomography (CECT) of the thorax demonstrated bilateral nodules and enlarged mediastinal lymph nodes. Ultrasound and CECT of the abdomen demonstrated hepatosplenomegaly with multiple hypodense enhancing lesions. GeneXpert of a gastric lavage on Day 2 of this admission detected M. tuberculosis without rifampicin resistance. The infant was diagnosed with tuberculosis (TB) complicated by caseating hepatic granulomas, which fulfilled Cantwell's diagnostic criteria for CTB. Magnetic resonance imaging of the orbit demonstrated focal heterogeneous lesions involving the anterior portion of the left ocular bulb and vitreous, suggesting panophthalmitis, which was unresponsive to intravitreal antibiotics. Following commencement of standard anti-TB therapy from Day 90 of life, there was clinical and radiological recovery of endophthalmitis. The mother had a cachectic appearance owing to weight loss, and she had attended only one antenatal appointment. She had a positive acid-fast bacilli sputum stain but was unwilling to allow a genital tract smear. In the spectrum of CTB, TB panophthalmitis is an extremely rare presentation, and, as far as we are aware, it has not been reported in a child.Abbreviations: AFB: acid-fast bacilli; ATT: anti-tuberculous therapy; CTB: congenital tuberculosis; CECT: contrast-enhanced computed tomography; CSF: cerebrospinal fluid; ESR: erythrocyte sedimentation rate; HIV: human immunodeficiency virus; TB: tuberculosis; MRI: magnetic resonance imaging; USG: ultrasonogram.

Self-passivating (Re,Al)B2 coatings synthesized by magnetron sputtering.

(Re0.67Al0.10)B2 and (Re0.74Al0.11)B2 solid solution as well as Re0.85B2 thin films were deposited by hybrid RF-DC magnetron sputtering. X-ray diffraction (XRD) showed that all films exhibit the ReB2 (P63/mmc) crystal structure. X-ray photoelectron spectroscopy (XPS) analyses performed on atmosphere exposed thin film surfaces suggest that ReB2 corrodes, consistent with literature, by forming perrhenic acid (HReO4) already after two days, while (Re0.74Al0.11)B2 forms a self-passivating Al-oxide layer preventing corrosion in a time period ≥ 60 days. Hence, it is evident that Al additions to ReB2 significantly increase the chemical stability during atmosphere exposure.