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.

Infant Pulmonary Function Tests in Children with Airway Anomalies and Correlation with Bronchoscopy Findings.

OBJECTIVES: To evaluate the role infant pulmonary function tests (Tidal Breathing Flow Volume Loops, TBFVL) in children with airway anomalies and to correlate the TBFVL so obtained with bronchoscopy findings. METHODS: In this prospective cohort study, we enrolled children aged 0-2 years with airway anomalies and performed TBFVL and bronchoscopy. The primary outcome measure was graphic pattern of TBFVL in laryngomalacia. Secondary outcome measures were types of TBFVL results in various airway anomalies and controls. RESULTS: Out of 53 children enrolled, 28 (52.3%) had laryngomalacia. Pattern 3 (fluttering of inspiratory limb) was commonest TBFVL pattern in laryngomalacia. Among TBFVL parameters, the ratio of inspiratory time to expiratory time (Ti/Te) and tPTEF/tE was significantly high in children with isolated laryngomalacia compared to controls. At six months of follow-up, TBFVL pattern 1 (normal) became the commonest pattern. CONCLUSION: A particular type of airway anomaly may have a characteristic graphic pattern in TBFVL and TBFVL pattern may indicate improvement in airway anomalies in follow-up.

A "Cool" extraction technique for difficult pediatric airway foreign bodies: Report of two cases.

Foreign body (FB) aspiration is a potentially life-threatening accident in children. Traditionally, rigid bronchoscopy has been the procedure of choice for FB removal, however it may miss distally lodged FBs. We report two pediatric cases with distal impacted FBs that could not be retrieved by rigid bronchoscopy (RB) and were mobilised using Fogarty balloon followed by flexible bronchoscopic cryoextraction. The advantage of a cryoprobe is lower risk of fragmentation of FB that may occur with forceps. Cryoextraction is particularly advantageous for removing water-containing FBs. In both patients, FB was removed more than 2 weeks following aspiration, leading to the formation of granulation tissue around the FB, which considerably hampered the process. Using a laryngeal mask airway to secure the airway, FB removal by flexible bronchoscopy may be a safe and effective technique in skilled hands, especially for FBs impacted in distal airways with granulation tissue where RB fails.

Transition Care in Cystic Fibrosis.

Cystic fibrosis (CF) is a chronic childhood illness with gradually improving survival and significant burden of disease during adult life. Transition of CF care from pediatric to an adult based multidisciplinary team is a complex process and careful coordination with a transition key worker is necessary for successful transition without adverse outcome. Transition is associated with a key change in CF management with shift from family-centred care to self-reliance and independence on part of the patient. Readiness and skills of self-managed care play central part in successful transition. Resource materials for transition are available online for different countries for improved readiness and smooth transition. Situation is worse in resource-limited settings as facilities of fully functional adult multidisciplinary care for CF is not readily available.

Oral Propranolol Therapy for Infantile Hemangioma: Long-term Follow-up.

ß-blocker therapy is currently the treatment of choice for infantile hemangiomas (IH), albeit with limited data on long-term treatment outcomes. Herein, authors treated 67 IH lesions in 47 patients with oral propranolol at 2 mg/kg/d for a median of 9 mo and followed them up for a median of 48 mo. While no maintenance therapy was required for 18 lesions (26.9%), the rest needed maintenance therapy. Both treatment regimens had comparable efficacy (83.3±23.9% and 92.0±13.8%) but chances of IH recurrence was higher in lesions requiring maintenance therapy. Also, patients treated at ≤5 mo of age had a significantly better response and a lower recurrence rate than patients treated at >5 mo of age (95.0±7.9% vs. 87.0±17.5%, p = 0.05). Authors' experience suggests that longer durations of maintenance therapy offered no added advantage to the overall improvement of IH while treatment initiation at an earlier age showed better improvement and lower recurrence rates.

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.

Development of algorithm for diagnosis of cystic fibrosis in absence of sweat chloride testing in resource-limited setting.

OBJECTIVE: To develop a diagnostic algorithm for cystic fibrosis (CF) in the setting of unavailability of sweat chloride, based on clinical features and basic laboratory investigations. METHODS: In a prospective observational study, we enrolled children with recurrent/persistent pneumonia with either malabsorption or poor growth, undergoing a sweat chloride test, between January 2019 and December 2020. They were simultaneously evaluated for aquagenic wrinkling of hands, stool fat globules, sputum for bacterial culture, blood gas, and serum electrolytes. Sensitivity and specificity were calculated for parameters having a significant difference between CF and non-CF groups. Scoring systems and algorithms for the diagnosis of CF were developed. RESULTS: Of 134 children enrolled, 46 (34%) had CF. The sensitivity and specificity of various parameters to diagnose CF was: sibling death due to respiratory illness (30.43%, 96.59%), aquagenic wrinkling (76.74%, 47.67%), metabolic alkalosis (17.78%, 94.12%), hyponatremia (28.89%, 89.41%), stool fat globules (38.46%, 81.18%), and presence of Pseudomonas in sputum culture (23.68%, 98.80%). Using coefficients of significant parameters on stepwise logistic regression, the composite score for diagnosis of CF was calculated as: 3X sibling death due to respiratory illness + 1.5X hyponatremia + 1.5X metabolic alkalosis + 1.5X aquagenic wrinkling + 1X stool fat globules + 2.5X presence of Pseudomonas in sputum culture (each of the variables scores 0 or 1 for absence and presence, respectively). The cut-off of ≥2.5 had sensitivity and specificity of 81.82% and 76.83%, respectively. CONCLUSIONS: In resource-limited settings, the proposed diagnostic algorithm can be used for the diagnosis of presumptive CF with fair sensitivity and specificity.

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.

Allergic Bronchopulmonary Aspergillosis in Children Presenting as Lung Masses.

Allergic bronchopulmonary aspergillosis (ABPA) is an IgE-mediated hypersensitivity reaction predominantly occurring in patients of asthma and cystic fibrosis. The typical radiological findings in ABPA include central bronchiectasis and fleeting opacities. In this retrospective study, the aim was to describe cases of ABPA in children who had a mass-like lesion in the lung. There were 5 cases of ABPA in children, who presented as mass-like lesions that responded very well to treatment for ABPA. All cases, except 1, had asthma as the underlying disease. There was a delay in the diagnosis of ABPA in all 5 cases. There had been unnecessary invasive investigations in some of these cases before the diagnosis of ABPA was made. To conclude, children with ABPA may present with a mass-like lesion in the lung and high index of suspicion is required to diagnose ABPA timely to prevent its consequences.

Poor Respiratory Health Following Relapsing SARS-CoV-2 Infection in Children with Cystic Fibrosis.

Children with cystic fibrosis (CF) constitute a high-risk group for COVID-19 with underlying chronic lung disease. COVID-19 severity varying from mild infection to need of intensive care has been described in children with CF. Two children with significant underlying pulmonary morbidity are described here, who developed severe disease following SARS-CoV-2 infection. Case 1 (a 9-y-old boy) had pneumonia with respiratory failure requiring noninvasive ventilation support. He had delayed clearance of SARS-CoV-2, with recurrence of symptomatic disease with short asymptomatic period in between. He was also diagnosed with CF-related diabetes and allergic bronchopulmonary aspergillosis during the second episode. Case 2 (an 18-mo-old boy) had two episodes of SARS-CoV-2-related severe lower respiratory infection within a period of 2 mo, requiring high-flow nasal oxygen support. Both children had 3rd pulmonary exacerbation but SARS-CoV-2 was not detected in respiratory secretions. To conclude, children with CF with underlying pulmonary morbidity, can develop severe COVID-19 and prolonged SARS-CoV-2 shedding.

A Novel Homozygous HAX1 Mutation in a Child With Cyclic Neutropenia: A Case Report and Review.

BACKGROUND: Cyclic neutropenia is a rare genetic disorder causing the arrest of neutrophil function and is characterized by periodic neutropenia and recurrent infections. Patients with cyclic neutropenia with autosomal dominant, sporadic, and X-linked may have mutations in the ELANE gene, and autosomal recessive cases have homozygous/compound heterozygous variants in the HAX1 gene primarily. OBSERVATION: The authors describe a novel variant in the HAX1 gene, which was detected by next-generation sequencing in an 8-year-old male child who presented with recurrent infections and neutropenia. CONCLUSION: The patient extends the clinical variability associated with HAX1 variants and highlights the importance of genetic investigations in patients with suspected cyclic neutropenia.

Efficacy and safety of EBUS-TBNA and EUS-B-FNA in children: A systematic review and meta-analysis.

BACKGROUND: Endobronchial ultrasound-guided transbronchial needle aspiration (EBUS-TBNA) and transesophageal bronchoscopic ultrasound-guided fine-needle aspiration (EUS-B-FNA) are established modalities for evaluation of mediastinal/hilar lymphadenopathy in adults. Limited literature is available on the utility of these modalities in the pediatric population. Herein, we perform a systematic review and meta-analysis on the yield and safety of EBUS-TBNA and EUS-B-FNA in children. METHODS: We performed a systematic search of the PubMed and EMBASE databases to extract the studies reporting the utilization of EBUS-TBNA/EUS-B-FNA in children (<18 years of age). The pooled diagnostic yield and sampling adequacy (proportions with 95% confidence intervals [CIs]) were calculated using meta-analysis of proportions using the random effects model. Details of any procedure-related complications were noted. RESULTS: The search yielded 12 relevant studies (5 case series and 7 case reports on EBUS-TBNA/EUS-B-FNA, 173 patients). Data from five case series (164 patients) were summarized for the calculation of the sampling adequacy and diagnostic yield. Safety outcomes were extracted from all publications. The pooled sampling adequacy and combined diagnostic yield of EBUS TBNA/EUS-B-FNA were 98% (95% CI, 92%-100%) and 61% (95% CI, 43%-77%), respectively. A procedure-related major complication was reported in one patient (1/173, a major complication rate of 0.6%), and minor complications occurred in six patients (6/173, a minor complication rate of 3.5%). CONCLUSIONS: EBUS-TBNA and EUS-B-FNA are safe modalities for evaluation of mediastinal lymphadenopathy in the pediatric population. EBUS-TBNA/EUS-B-FNA may be considered as the first-line diagnostic modalities for this indication, as they have a good diagnostic yield and can avoid the need for invasive diagnostic procedures.

Cystic fibrosis in low and middle-income countries (LMIC): A view from four different regions of the world.

Cystic fibrosis (CF) has been shown to affect people all over the world. While life expectancy for people with CF has increased substantially, CF is still associated with death in infants and young children in many regions, particularly in low and middle-income countries (LMIC). These countries face significant challenges to promote CF diagnosis and improvements to CF care due to financial constraints and a significant burden of other diseases. In this review, we describe the status of CF diagnosis and care in different LMIC settings, from four different parts of the world (Brazil, South Africa, Israel and India). We highlight challenges and opportunities for CF practitioners in LMIC to improve CF care and outcomes. While early CF diagnosis is the key to optimising outcomes, newborn screening may not be feasible for countries with lower CF incidence and higher birth rates, such as India or South Africa. CF therapies and care in LMIC need to be adapted to available resources of these countries. Collaboration initiatives of the global CF community with LMIC may improve CF care in these countries. Most individuals with CF in LMIC are not benefiting from CFTR modulator treatments due to the prohibitive cost of these drugs.

Wall-mounted Versus Handheld Syringe Suction for Pediatric Bronchoalveolar Lavage: A Randomized Controlled Trial.

BACKGROUND: Bronchoalveolar lavage (BAL) via flexible bronchoscopy is a valuable diagnostic technique in children. The quality of BAL is directly related to the volume of the fluid recovered. Continuous wall suctioning and handheld syringe suctioning are the 2 commonly used methods, but they are rarely compared in children. We aimed to compare the above 2 suctioning techniques for BAL in the pediatric age group. METHODS: This randomized controlled study enrolled children from 1 month to 18 years of age undergoing flexible bronchoscopy and BAL. We compared continuous wall suctioning and the handheld syringe suctioning technique. The primary outcome was the percentage of BAL fluid recovery in 2 different suctioning techniques. Secondary outcomes included technical acceptable BAL and yield of various diagnostic tests in BAL. RESULTS: The study included 73 children (48 boys) with a median (interquartile range) age of 30 (8, 108) months. There were 37 children in the wall mount group and 36 children in the syringe suction group. Baseline characteristics of the groups were similar. The wall mount suction had more recovery of BAL fluid compared with the syringe method (43.6±8.4% vs. 37.8±8.5%, P=0.004). The proportion of BAL having a fluid recovery of ≥40% was also high in the wall mount suction [31 (83.8%) vs. 17 (47.2%); P=0.001]. There was no difference in diagnostic yield between the groups. CONCLUSION: Wall mount suction had better BAL fluid recovery compared with handheld syringe suction in children undergoing flexible bronchoscopy. The diagnostic yield was similar in both groups.

Entomophthoromycosis in a child: Delayed diagnosis and extensive involvement.

Entomophthoromycosis is a rare fungal infection of the skin and subcutaneous tissue occurring predominantly in tropical and subtropical regions. In children, it mostly affects the lower half of the body. With this, we report a case of Entomophthoromycosis in a 6-year-old girl who presented late with extensive involvement of the upper half of the body. She responded well to treatment with potassium iodide and itraconazole. We also reviewed cases of Entomophthoromycosis reported in children.