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.

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.

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.

Imaging Findings in Chronic Granulomatous Disease (CGD).

OBJECTIVES: To describe prevalence of various imaging findings in chronic granulomatous disease (CGD) patients; and find imaging biomarkers for differentiating chest infections caused by different micro-organisms. METHODS: A retrospective study was conducted on 15 patients (49 scans) with proven CGD. Scans which had a correlative microbiological diagnosis for organisms were included in the analysis. The scans were reviewed by 3 radiologists on a predefined proforma, under the lung parenchymal, airway, pleural, mediastinal, and extrathoracic abnormalities. Analysis of various imaging parameters on a semiquantitative scale was performed, followed by a correlation of each imaging findings with causative organisms. RESULT: The mean age of presentation was nearly 7 y, with a male preponderance. Definitive proof of causative organisms was obtained in 22 scans. Bacterial infection was found in 7, fungal in 12, tubercular in 2, and viral in 1 scan. Most prevalent thoracic imaging manifestations included lymphadenopathy (commonest), consolidation, nodules, air trapping, and bronchiectasis. Fungal infections showed necrotic conglomerate lymphadenopathy, cavitating nodules, and multilobar consolidation more frequently than bacterial infections (though not statistically significant). Abscesses and lymphadenopathy were the most common extrathoracic manifestations. CONCLUSION: In patients with CGD, multifocal or multilobar consolidation, mass-like consolidation, cavitating nodules, and conglomerate necrotic lymphadenopathy should alert the radiologist to a possible fungal cause.

Role of computed tomography angiography in the evaluation of haemoptysis in children: Decoding the abnormal vessels.

Background & objectives: Haemoptysis in children is potentially life-threatening. In most cases, the bleeding arises from the systemic circulation, and in 5-10 per cent of cases, it arises from the pulmonary circulation. The role of computed tomography angiography (CTA) in this setting is important. This study was undertaken (i) to study the role of single-phase split-bolus dual energy contrast-enhanced multidetector row CTA (DECTA) in the evaluation of haemoptysis in children; (ii) to analyze the patterns of abnormal vascular supply in the various aetiologies encountered. Methods: A retrospective study of 86 patients who underwent split bolus DECTA for the evaluation of haemoptysis was performed. Final diagnoses were categorized as normal computed tomography, active tuberculosis (TB), post-infectious sequelae, non-TB active infection, cystic fibrosis (CF), non-CF bronchiectasis, congenital heart disease (CHD), interstitial lung disease, vasculitis, pulmonary thromboembolism and idiopathic pulmonary haemosiderosis. Abnormal bronchial arteries (BAs) and non-bronchial systemic collateral arteries (NBSCs) were assessed for number and site and their correlation with underlying aetiologies. Results: A total of 86 patients (45 males, age from 0.3 to 18 yr, mean 13.88 yr) were included in the study; among these only two patients were less than five years of age. The most common cause of haemoptysis was active infection (n=30), followed by bronchiectasis (n=18), post-infectious sequelae (n=17) and CHD (n=7). One hundred and sixty five abnormal arteries were identified (108 BA and 57 NBSC), and were more marked in bronchiectasis group. Interpretation & conclusions: Active infections and bronchiectasis are the most common causes of haemoptysis in children. While post-infectious sequelae are less common, in patients with haemoptysis, the presence of any abnormal arteries correlates with a more frequent diagnosis of bronchiectasis. NBSCs are more common in post-infectious sequelae and CHD.

Comparison of Cisplatin Monotherapy and PLADO in the Management in Children with Standard-Risk Hepatoblastoma in a Resource-Challenged Nation.

Background: Recent SIOPEL studies have shown cisplatin monotherapy to be equally effective in management of Standard risk Hepatoblastoma (SRHB)as compared to PLADO. Aims and Objectives: To study the chemotherapy, response and outcomes in children with SRHB. Material and Methods: A retrospective study was conducted and all children with SRHB who presented to us from June 2007 to December 2017 were included. All patients with standard risk hepatoblastoma who had received at least 2 cycles of chemotherapy were included. Data regarding the demographics, PRETEXT stage, chemotherapy, response to chemotherapy and outcomes were recorded. Kaplan Meier survival analysis was performed to calculate 5 year overall survival (OS) and event free survival (EFS). Results: Thirty two children were included in the study. The disease was PRETEXT I in 5 (15.6%), II in 9 (28.1%) and 18 (56.2%). Nineteen children (59.4%) received Cisplatin monotherapy and of these 6 patients (all PREXT III) had poor response and the chemotherapy was upgraded to PLADO. The remaining 13 (40.6%) received upfront PLADO chemotherapy. Only 31 patients could be operated. Tumor recurred in 5 patients, 2 who had upfront PLADO and 3 patients had been upgraded to PLADO. The 5 year OS and EFS was 100% in the monotherapy group (n=13), 92% and 69% in the upfront PLADO group (n=13), and 62% and 22% in the upgraded to PLADO group (n=6). Patients with PRETEXT III disease in whom chemotherapy was upgraded to PLADO had significantly lower survival (p=0.036) compared to those who received upfront PLADO chemotherapy. Conclusion: Two thirds of patients with PRETEXT stage III who received cisplatin monotherapy showed poor response and were upgraded to PLADO chemotherapy. These patients had a significantly poorer outcome compared to the rest of the cohort. PRETEXT stage III standard-risk hepatoblastoma may benefit from PLADO chemotherapy instead of cisplatin monotherapy.

Ultrasound-guided Percutaneous Embolization of Post Renal Biopsy Gonadal Artery Pseudoaneurysm.

Hemorrhage is the most frequently encountered post renal biopsy complication; reported in 12% to 14% of patients. Although the vast majority of these are due to renal artery injury, involvement of gonadal arteries is also rarely seen. These may be managed by the endovascular route, which has several limitations in this subset of patients. We report a case of a 69-year-old male with rapidly progressive glomerulonephritis, who underwent renal biopsy and developed a testicular artery pseudoaneurysm (PA). Successful embolization of this PA was performed under ultrasound guidance using a direct percutaneous approach. This is the first such case reported in the literature.

Outcome and Determinants of Neutropenic Enterocolitis in Pediatric Cancer Patients.

BACKGROUND: Neutropenic enterocolitis (NEC) is a dreaded complication of chemotherapy. There is scant literature regarding incidence, clinical features, and determinants. The understanding of gut dysbiosis in NEC and pediatric cancer is evolving. METHODS: Pediatric cancer patients with neutropenia and gastrointestinal symptoms were evaluated for NEC with contrast-enhanced computed tomography abdomen. Clinical, imaging, and laboratory features were analyzed. Fecal samples were analyzed for fecal calprotectin by sandwich enzyme-linked immunoassay and gut microbiota by conventional culture and compared with healthy controls and children without NEC. RESULTS: NEC was diagnosed in 44 children based on clinical and imaging features with incidence of 7.4% (4 had recurrent episodes). Common manifestations included fever (98%), pain abdomen (88%), and diarrhea (83%). Hypoalbuminemia was observed in 78% of patients. Large bowel involvement (94%) with diffuse bowel involvement (63%) and pancolitis (64%) were common. Fecal calprotectin was significantly elevated in NEC group than non-NEC group and healthy controls (median: 87, 53, and 42 µg/g, respectively). A higher degree of gut dysbiosis was observed in children with NEC with higher isolation of Bacteroides and infrequent isolation of Lactobacilli. Mortality rate of 23% was observed. Only the presence of free fluid predicted higher mortality. Though levels of fecal calprotectin and gut dysbiosis were higher in NEC, they did not increase mortality. Isolation of Bacteroides and absence of Lactobacilli predicted a longer duration of intravenous alimentation. CONCLUSIONS: NEC caused significant morbidity and mortality in pediatric cancer patients. Gut dysbiosis was significantly higher in NEC group suggesting a role in pathogenesis and influencing outcome. This highlights the role of targeted interventions towards gut dysbiosis like prebiotics and probiotics.

Ingestion poisoning related lung injury- a pictorial review.

Poison ingestion is a medical emergency requiring immediate care in the emergency department. Respiratory symptoms with ingested poisons can occur due to aspiration, cardiopulmonary effects, or direct lung toxicity due to injury of the alveolar epithelium. Chest imaging (chest radiographs/CT) is usually performed in the emergency setting to evaluate such symptoms. It is often impossible to elicit the nature of the poison ingested by the patients due to their unconscious state. Identification of the culprit poison can expedite the patient's management towards a specific antidote or help understand the underlying mechanism causing the pulmonary symptoms. The imaging manifestations depend on the underlying mechanisms, varying for each ingested poison, forming an imaging signature which has not been adequately discussed in existing literature. Poisons like paraquat and organophosphate are important to differentiate as indiscriminate use of oxygen therapy in the former can exacerbate the lung injury caused by redox cycling. In this pictorial assay, we present the chest imaging spectrum of commonly ingested poisons, and further suggest algorithmic approach towards identification of common poisons based on their chest imaging.

Ultrasonography in the Evaluation of Pediatric Chest "Masses": When to Consider?

Thoracic ultrasound is radiation-free, easily available, portable modality with added advantage of real-time assessment. It is useful in mediastinal lesions and peripheral lung, pleural and chest wall masses. Not only is it a valuable modality in differentiating solid from cystic lesion, it can also depict internal architecture without the use of contrast material. The added advantages of its use in children are the lack of ionizing radiation, and no need for sedation or general anesthesia in most cases. Although it has its limitations with a longer learning curve, it can act as a second-line modality to chest radiograph and adjunctive modality to CT in cases of a thoracic mass in a child.

Predicting Histology of Tracheobronchial Neoplasms: A CT Based Differentiation Model.

BACKGROUND: Tracheobronchial (TB) tumors follow same pathological classification as lung neoplasms; however, some entities are known to favor airways. Distinction of pathological types is necessary for suggesting appropriate management strategy. PURPOSE: To evaluate utility of multidetector CT (MDCT) in differentiation of primary TB tumors; and assess validity of a scoring system based on imaging biomarkers to differentiate tumor types. METHODS: MDCT features of 45 patients were analyzed for location, shape, calcification, attenuation, parenchymal changes, bronchoceles, extraluminal extension, lymphadenopathy, metastases. The two largest groups were compared with each other and remaining entities using Chi square tests. Six-point scoring system combining the differentiating features was devised and receiver operating characteristic curve analysis performed. RESULTS: The most frequent type was neuroendocrine tumors (NET) (51.1%), followed by salivary gland tumors (SGT) (20%); including adenoid cystic carcinoma (ACC) (13.3%) and mucoepidermoid carcinoma (MEC) (6.7%). Comparing NETs with other entities as a whole, and independently with SGTs, significant difference was found among location (p = 0.05 and 0.001 respectively), shape (p < 0.001), calcification (p = 0.038 and 0.041 respectively), attenuation (p = 0.001 and 0.019 respectively), bronchoceles (p = 0.013 and 0.010 respectively). Significant difference was found among ACC and MEC in location (p = 0.01) and morphology (p < 0.001). On receiver operating characteristic (ROC) curve analysis of the score, areas under curve for NET, SGT and ACC were 0.913, 0.872 and 0.962 respectively. Suggested cut-off values were >3.5 for carcinoid (sensitivity 70%, specificity 91%), <2.5 for SGT (sensitivity 78%, specificity 75%), <1.5 for ACC (sensitivity 83%, specificity 92%). CONCLUSION: Use of a scoring system enables maximum diagnostic accuracy in MDCT differentiation of TB tumors.

Transcutaneous Mediastinal Ultrasonography for Lymphadenopathy in Children: A Pictorial Essay of Technique and Imaging Findings.

Ultrasonography (US) forms the mainstay of imaging in children; however, in the chest, its use has traditionally been limited to evaluation of pleural pathology. US techniques such as endobronchial and endoscopic ultrasound, which are commonly used for detection of mediastinal lymphadenopathy are invasive, aerosol generating, and often require sedation. Transcutaneous mediastinal sonography (TMUS) offers a useful alternative, which is easier to perform and overcomes these limitations. In this review, we summarize the technique, as well as imaging appearances of lymph nodes on TMUS. We also list common problems faced by operators and suggest troubleshooting methods for these.

Myriad Faces of Active Tuberculosis: Intrapulmonary Bronchial Artery Pseudoaneurysm.

Bronchial artery (BA) pseudoaneurysm is an uncommon vascular complication of tuberculosis (TB), and early diagnosis is crucial due to risk of rupture and life-threatening hemorrhage. Immediate intervention is warranted in massive hemoptysis due to high mortality. Various causes of massive hemoptysis are TB, bronchiectasis, aspergilloma, lung abscess, lung cancer, necrotizing pneumonia, and cystic fibrosis. Active pulmonary TB as well as chronic pulmonary TB can manifest with massive hemoptysis. Hemoptysis in active TB occurs due to ulceration in bronchiolar wall, eroding the wall of the adjacent BA or pulmonary artery, and in chronic TB due to hypertrophied bronchial arteries, or bronchiectasis, or aspergilloma. Herein, we report a case of pulmonary TB causing intrapulmonary BA pseudoaneurysm in a young male patient who presented with acute massive hemoptysis. The BA pseudoaneurysm as well as other hypertrophied bronchial arteries were embolized using polyvinyl alcohol (PVA) particles.

Evaluation of Non-infectious Pulmonary Complications in Hematological Malignancies on MDCT: Decoding Imaging Markers.

Patients with hematological malignancies are at risk of developing of various infectious and non-infectious pulmonary complications. Common non-infectious pulmonary complications include pulmonary edema, leukostasis, diffuse alveolar haemorrhage (DAH) and differentiation syndrome. The overlapping imaging features pose diagnostic dilemma. We retrospectively analysed the CT findings in identifying differentiating imaging markers and developing an algorithm. 46 diagnosed patients of non-infectious pulmonary complications who underwent CT chest between February 2017 to March 2020 were included. The CT findings were recorded as parenchymal (GGO, consolidation, septal thickening, peribronchovascular interstitial thickening, and nodules), pleural effusion, and mediastinal lymphadenopathy. We categorized non-infectious pulmonary complications as: differentiation syndrome (Group1, n = 6), DAH (Group 2, n = 8), leukostasis (Group 3, n = 14),leukemic infiltrate (Group 4, n = 5), and pulmonary edema(Group 5, n = 13). Chi-square or Fisher exact test were used with p value < 0.05 as statistically significant.Absence of diffuse GGO in Group 4, interlobular septal thickening in Group 2 and Group 3, nodules in Group 5, and peribronchovascular interstitial thickening in Group 2 were statistically significant. Presence of interlobular septal thickening in Group 5, nodules in Group 4, and peribronchovascular interstitial thickening in Group 5 were statistically significant. Based on the results, an algorithm was developed which may suggest a possible diagnosis in an appropriate clinical scenario.