Endemic pediatric fungal infections in India: clues to diagnosis.

This review is intended to familiarize readers with an emerging group of fungal infections that mostly manifest in immunocompetent individuals. This group was initially considered endemic to the tropics, but increasing worldwide prevalence has been reported. The organisms have been divided into dominant non-invasive forms and dominant invasive forms for ease of understanding. The non-invasive organisms include the group Entomophthoromycota, under which two genera Basidiobolus and Conidiobolus, have been identified as human pathogens. They present with plaques in the extremities and rhinofacial region, respectively. The invasive organisms are dematiaceous fungi (phaeohypomycosis), which includes Cladophialophora and Exophiala among others. They cause invasion of deep tissues, with the central nervous system being the most common target. The mycology, epidemiology, diagnosis, and treatment options have been summarized in brief. The clinical presentation, imaging manifestations, differentiation from other common infections and malignancies that show similar features have been detailed.

Pediatric Allogeneic Hematopoietic Stem Cell Transplantation Including Matched Sibling and Alternate Donor Transplants - Adaptations and Outcomes From a Tertiary Care Government Institute in India.

OBJECTIVE: To report the outcomes of pediatric patients who underwent allogeneic hematopoietic stem cell transplant (HSCT) in single rooms without high-efficiency particulate air (HEPA) filters, laminar air flow or positive pressure at our centre and discuss the adaptations of a high-volume government centre. METHODS: Data of the first 20 children who underwent allogeneic HSCT between May 2019 and July 2023 in adaptive settings were reviewed retrospectively. All patients were managed in in single rooms without HEPA filters, positive pressure or laminar air flow. Supportive care in the form of antimicrobial prophylaxis, veno-occlusive disease prophylaxis, anti-epileptics (with busulfan) and irradiated blood products were provided. Trained manpower including multi-specialty consultations were readily available. All complications including infections were managed as per standard guidelines. RESULTS: The median (range) of children included was 6 (1-20) years. For eight patients we used alternate donors. The mean (SD) time to neutrophil and platelet engraftment were 17.0 (8.07) days and 18.8 (10.1) days, respectively. The mean (SD) time to discharge from the hospital was 30.9 (10.04) days. There were no deaths within 30 days. Six children each developed acute and chronic graft-versus-host disease (GVHD). The overall survival at a median follow-up of 292 days was 70% (n = 14). CONCLUSION: With certain adaptations in the existing infrastructure in resource-limited settings, allogeneic HSCT can be performed with good outcomes, provided experienced, dedicated and adequate personnel, comprehensive supportive care, multidisciplinary consultative support and isolation are provided.

Evaluation of Infantile Brachial Plexopathy Using 3T MRI and High-Resolution Ultrasound: Experience From a Tertiary Care Centre.

BACKGROUND: Currently, clinical assessment is the main tool for the evaluation of brachial plexus injury, complemented by electrophysiologic studies (EPS), and imaging studies whenever available. Imaging plays an important role as it enables the differentiation of pre-ganglionic and postganglionic injuries, and adds objectivity to presurgical evaluation. OBJECTIVES: The primary objective was to evaluate the utility of magnetic resonance imaging (MRI) and high-resolution ultrasonography (USG) in the localization and characterization of brachial plexus injury in infants. MATERIALS AND METHODS: In this prospective study, 34 infants with signs and symptoms of brachial plexus injury were evaluated by clinical examination, EPS, MRI, and USG. Imaging findings were correlated with intraoperative findings in infants who underwent surgical management. The association between EPS and MRI findings, and USG and MRI findings were assessed using Fisher's exact test. Semi-quantitative subjective analysis of various MRI sequences was done as well. RESULTS: The most common findings of preganglionic injury and postganglionic injury, in our study, were pseudomeningocele and nerve thickening, respectively. MRI detection of injuries had a significant association with EPS findings. All MRI-detected injuries had a muscle power of grade 3 or less. muscle. Three-dimensional (3D) short tau inversion recovery (STIR) sequence was found to be superior for detecting postganglionic injuries (P < 0.05). CONCLUSION: Imaging studies enable localization of the site of injury, determining the extent, and nature/morphology of injury. The gamut of findings obtained from MRI is far wider compared to that from USG. USG can be used as the first-line screening investigation.

68 Ga-DOTANOC PET/CT in 2 Siblings With Von Hippel-Lindau Disease.

ABSTRACT: Von Hippel-Lindau disease is a rare multisystem disorder that shows autosomal dominant inheritance. It is a cancer syndrome that is characterized by the development of a variety of benign and malignant tumors-CNS hemangioblastomas, retinal angiomas, endolymphatic sac tumors, renal cysts and tumors, pancreatic cysts and tumors, adrenal pheochromocytomas, and epididymal cystadenomas. Here we present the 68 Ga-labeled DOTANOC scans of 2 siblings who show an interesting spectrum of findings consistent with Von Hippel-Lindau disease.

Autologous hematopoietic stem cell transplant at a tertiary care centre in India: achieving comparable outcomes with adaptations.

Autologous stem cell transplantation (ASCT) is the standard treatment for many high-risk solid tumors. Patients undergoing ASCT should be managed in a dedicated hematopoietic stem cell transplantation (HSCT) unit with isolation rooms, high-efficiency particulate air (HEPA) filters, and positive pressure. We report the outcomes of the first 20 pediatric patients who underwent ASCT in isolation rooms with no HEPA filters or positive pressure. Moreover, the isolation rooms were not part of a dedicated HSCT unit. Data from 20 patients were analyzed. All patients included in the study underwent ASCT after harvest and cryopreservation of the hematopoietic stem cells (HSC). Furthermore, all patients also underwent myeloablative conditioning. The most common indications for ASCT included high-risk neuroblastoma (HR-NB) (n=9) and refractory/relapsed Hodgkin's lymphoma (HL) (n=6). The median CD-34 positive HSC administered was 4.5 (0.8-21.9) million per kg. The median time to neutrophil and platelet engraftment was 16.5 (10-35) and 19 (10-87) days, respectively. Additionally, only one transplant-related mortality was observed and the mean time to discharge from the hospital was 27.6+8.3 days. The overall survival for all our patients was 75% at a median follow-up of 33.2 months (15 out of 20 patients survived), and the disease-free survival was 60% (median follow-up, 28.4 months). The overall survival for the patients with HL was 85.7% at a median of 45.3 months and for the HR-NB was 66.7% at a median of 34.9 months. This study provides evidence that ASCT can be safely performed in isolation rooms without HEPA filters and positive pressure if expertise and supportive care are available. In settings with limited resources, such a model could help establish low-cost HSCT units.

Ultrasound in Causes of Stridor in Children.

Airway ultrasound (US) is an easily available, portable, radiation-free imaging modality for quick, non-invasive, dynamic evaluation of the airway without sedation. This is useful in children with stridor, which is an emergency due to upper airway obstruction requiring immediate management. Several causes of stridor including laryngomalacia, laryngeal cyst, subglottic hemangioma, vocal cord palsy, and lymphatic malformations can be evaluated accurately. Thin musculature and unossified cartilages in children provide a good acoustic window. Thus, airway US is valuable, but underutilized for the evaluation of children with stridor. In this case-based review, we describe the technique, indications, anatomy, and pathologies on airway US.

MDCT and MRI in Bronchiectasis in Older Children and Young Adults - A Non-Inferiority Trial.

OBJECTIVES: To compare and evaluate the usefulness of magnetic resonance imaging (MRI) with computed tomography (CT) in bronchiectasis; to compare MRI and CT scores with pulmonary function tests (PFT) and to evaluate the role of Diffusion-weighted imaging (DWI) in bronchiectasis. METHODS: In this prospective study, 25 patients between 7-21 y of age with a clinical/radiological diagnosis of bronchiectasis underwent MDCT and MRI chest. MRI and CT scoring was performed using modified Bhalla-Helbich's score by two independent radiologists for all parameters. A final consensus score was recorded. The overall image quality of different MRI sequences to identify pathologies was also assessed. Appropriate statistical tests were used for inter-observer agreements, and correlation amongst CT and MRI; as well as CT, MRI and PFT. RESULTS: Strong agreement (ICC 0.80-0.95) between CT and MRI was seen for extent and severity of bronchiectasis, number of bullae, sacculation/abscess, emphysema, collapse/ consolidation, mucus plugging, and mosaic perfusion. Overall CT and MRI scores had perfect concordance (ICC 0.978). Statistically significant (p-value <0.01) intra-observer and inter-observer agreement for all CT and MRI score parameters were seen. A strong negative correlation was seen between total CT and MRI severity scores and forced expiratory volume in 1 s (FEV1), forced vital capacity (FVC), forced expiratory flow (FEF) 25-75%. DWI MR, with an apparent diffusion coefficient (ADC) cut-off of 1.62 × 10-3 mm3/s had a sensitivity of 70% and specificity of 75% in detecting true mucus plugs. CONCLUSIONS: MRI with DWI can be considered as a radiation-free alternative in the diagnostic algorithm for assessment of lung changes in bronchiectasis, especially in follow-up.

Kawasaki disease or polyarteritis nodosa: coronary involvement, a diagnostic conundrum.

Polyarteritis nodosa (PAN) is a medium-vessel vasculitis presenting with cutaneous and multisystem involvement with considerable morbidity. The necrotizing vasculitis in PAN typically involves renal, celiac, and mesenteric vascular beds. Coronary artery involvement is a characteristic feature of Kawasaki disease, another medium-vessel vasculitis; however, it has been rarely reported with PAN. Here, we present 2 cases with PAN involving coronaries mimicking Kawasaki disease. A 3.5-year-old boy with classical features of Kawasaki disease with giant coronary aneurysm refractory to IVIg, methylprednisolone, infliximab presented with persistent rise in inflammatory markers and gastrointestinal bleeding. Digital subtraction angiography (DSA) revealed celiac artery branches stenosis and beading suggestive of PAN. Another 2-year-old girl presented with persistent fever, abdominal pain, and distension. She had hypertension, hepatomegaly, and splenomegaly on examination. Echocardiography revealed multiple coronary aneurysms and DSA revealed numerous renal artery aneurysms. Coronary aneurysm although is a rare presentation of childhood PAN, and can mimic Kawasaki disease. Although both are medium-vessel vasculitis differentiation between these two entities is pivotal, as there are differences in treatment modalities, duration of immunomodulatory therapy, and the outcome. This manuscript describes the salient differences which can help differentiate PAN masquerading as Kawasaki disease at initial presentation.

Society of Chest Imaging and Interventions Consensus Guidelines for the Interventional Radiology Management of Hemoptysis.

The recommendations from the Society of Chest Imaging and Interventions expert group comprehensively cover all the aspects of management of hemoptysis, highlighting the role of diagnostic and interventional radiology. The diversity existing in etiopathology, imaging findings, and management of hemoptysis has been addressed. The management algorithm recommends the options for effective treatment while minimizing the chances of recurrence, based on the best evidence available and opinion from the experts.

Decoding the Guidelines of Invasive Pulmonary Aspergillosis in Critical Care Setting: Imaging Perspective.

Invasive pulmonary aspergillosis (IPA) is a common, life-threatening opportunistic fungal infection seen in susceptible individuals especially those admitted in critical care units. Multiple guidelines have been promulgated for the diagnosis of IPA, some of which are all inclusive, while others cater to specific patient groups. Microbiology forms the crux of the majority of the diagnostic tests/criteria; however, results take a considerable amount of time. Radiology can play an important role by bridging the gap to reach at an early diagnosis. Thus, the role of a radiologist cannot be overemphasized to recognize the typical and atypical imaging manifestations of invasive aspergillosis and aid in the swift management of these cases. This review decodes the terminology and various diagnostic criteria for IPA relevant to imaging studies. Further, the differences in imaging manifestations of IPA in neutropenic and non-neutropenic patients are also discussed.

Technical Report.

Diaphragmatic structure and function assessment can be performed using grayscale as well as M-mode ultrasound. This article discusses the application of M-mode ultrasound in the assessment of diaphragmatic dysfunction.

Fibrosing Interstitial Lung Disease in Children: An HRCT-Based Analysis.

OBJECTIVE: To determine high resolution CT (HRCT) patterns of pulmonary fibrosis (PF) in children; and their etiological correlates. METHODS: This was a retrospective study involving 149 children with diffuse lung disease (DLD). Patterns of involvement were classified based on dominant lung finding as ground glass opacity (GGO) dominant, nodule dominant, cystic lung disease, or PF. Patterns of PF were classified based on distribution and morphology into airway centric fibrosis (ACF), subpleural fibrosis (SPF), progressive massive fibrosis (PMF) and fibrocavitary. A comparison was made between the two dominant groups for apicobasal distribution, associated findings (GGO, nodules, cysts), and pulmonary artery hypertension (PAH). RESULTS: Nineteen patients showed PF on HRCT. ACF was commonest (52.6%), followed by SPF (42.1%). The common etiology was sarcoidosis (30%) in ACF, and connective tissue disorders (CTD) (50%) in SPF. Significant difference was found between ACF and SPF in apicobasal distribution (p = 0.04), presence of nodules (p = 0.03), and cysts (p = 0.02). CONCLUSION: PF may present as an end stage of several childhood lung diseases. PF on imaging has discernible morphological patterns that correlate with underlying etiology.

Hypersensitivity Reactions and the Respiratory System: Imaging Based Review.

Hypersensitivity reactions are characterized by inappropriate response of the immune system to an inciting antigen, which results in damage to various body tissues. Respiratory system can be involved as a part of hypersensitivity reaction by a myriad of conditions ranging from infective pathologies like tuberculosis to non-infective processes such as asthma, graft- versus host disease, sarcoidosis and vasculitic disorders. Recognition of specific imaging features in appropriate clinical setting helps in diagnosing these conditions. We present a review of mechanism of different types of hypersensitivity reactions; and imaging features of various such pathological conditions affecting the respiratory system.