Approach to clinically significant vascular anomalies in children

Vascular anomalies consist of tumours or malformations made up of abnormal growth or collections of blood vessels that can result in functional or cosmetic problems. While many vascular anomalies are present at birth, some do not appear until later in life, making diagnosis more challenging. Although many vascular anomalies are benign, some are associated with serious complications and may involve multiple organ systems. This article highlights the important features of clinically significant vascular anomalies to help physicians promptly identify and refer these cases to a specialised multidisciplinary team for evaluation and management. The discussion includes the various presenting complaints of vascular anomalies in children, namely rapidly growing birthmarks, painful lesions, seizures/neurological manifestations, bleeding diathesis, cardiac/airway abnormalities or part of an overgrowth syndrome.

Clinical analysis of the “small plateau” sign on the flow-volume curve followed by deep learning automated recognition

Background Small plateau (SP) on the flow-volume curve was found in parts of patients with suspected asthma or upper airway abnormalities, but it lacks clear scientific proof. Therefore, we aimed to characterize its clinical features. Methods We involved patients by reviewing the bronchoprovocation test (BPT) and bronchodilator test (BDT) completed between October 2017 and October 2020 to assess the characteristics of the sign. Patients who underwent laryngoscopy were assigned to perform spirometry to analyze the relationship of the sign and upper airway abnormalities. SP-Network was developed to recognition of the sign using flow-volume curves. Results Of 13,661 BPTs and 8,168 BDTs completed, we labeled 2,123 (15.5%) and 219 (2.7%) patients with the sign, respectively. Among them, there were 1,782 (83.9%) with the negative-BPT and 194 (88.6%) with the negative-BDT. Patients with SP sign had higher median FVC and FEV1% predicted (both P < .0001). Of 48 patients (16 with and 32 without the sign) who performed laryngoscopy and spirometry, the rate of laryngoscopy-diagnosis upper airway abnormalities in patients with the sign (63%) was higher than those without the sign (31%) (P = 0.038). SP-Network achieved an accuracy of 95.2% in the task of automatic recognition of the sign. Conclusions SP sign is featured on the flow-volume curve and recognized by the SP-Network model. Patients with the sign are less likely to have airway hyperresponsiveness, automatic visualizing of this sign is helpful for primary care centers where BPT cannot available.

Pediatric anterior larynx: presentations and associations

Objective: The literature on the entity of the anterior larynx (AL) is restricted to little evidence on the difficulty encountered in exposing the larynx for intubation, perioperative morbidity and mortality, and scant reports on its prevalence in general adults and children. We here describe the prevalence of AL in a series of children presenting with aerodigestive symptoms and explore its association with functional abnormalities, congenital and structural anomalies or conditions. Setting: Tertiary pediatric center Methods: we conducted a retrospective case-control study. Using a prospectively collected surgical database we identified patients diagnosed with AL (grades IIb-III-IV Modified Cormack-Lehane scale) and sex and age-matched controls who had undergone full airway endoscopy for aerodigestive symptoms. We collected the demographics, presentations, associated diagnoses, and investigations. We compared the proportion of patients with large airway abnormalities and dysmorphism and estimated the prevalence of AL. Results: over a 5.5-year period, 58 children (28 females) were matched with 58 controls. Although both groups presented with permutations of stridor, respiratory failure, cyanotic spells, swallowing and feeding difficulties and SDB, AL presented much more with swallowing and feeding problems (33 vs 20, p<0.05). There were significantly more children with dysmorphism in the AL group (29 vs 9, P < 0.05). The prevalence of AL was 4.9%. Conclusions: This is the first study to report the prevalence of AL amongst a cohort of children with aerodigestive symptoms. Its significant association with dysmorphic features warrants genetic consultation. It is associated with swallowing abnormalities that justifies a systematic instrumental evaluation

Airway Abnormalities in Adult Mucopolysaccharidosis and Development of Salford Mucopolysaccharidosis Airway Score

(1) Background: Mucopolysaccharidoses (MPS) are a heterogeneous group of lysosomal storage disorders caused by the absence of enzymes required for degradation of glycosaminoglycans (GAGs). GAGs deposition in tissues leads to progressive airway narrowing and/or tortuosity. Increased longevity of patients has posed newer problems, especially the airway. This study aims to characterise various airway abnormalities in adult MPS from a regional centre and proposes a method to quantify the severity of the airway disease. (2) Methods: Retrospective analysis by case notes review, clinical examination, endoscopy, cross-sectional imaging, 3-dimensional reconstruction, and physiological investigations were used to assess the airway abnormalities. Quantitative assessment of the airway severity was performed a validated questionnaire of 15 parameters to derive Salford Mucopolysaccharidosis Airway Score (SMAS). (3) Results: Thirty-one adult MPS patients (21M/ 9F; median 26.7 years; range 19–42 years) were reviewed. There were 9 MPS I, 12 MPS II, 2 MPS III, 5 MPS IV, 2 MPS VI, and 1 MPS VII. Airway abnormalities in each MPS type are described. Patients scoring more than 35 on SMAS had some form of airway intervention. The area under curve of 0.9 was noted at a score of 25, so SMAS more than 25 may predict a difficult airway and potential to have complications. Pearson’s correlation between SMAS and height, weight, BMI were poor (p < 0.05). (4) Conclusions: Airway abnormalities in adult MPS are varied and complex. Assessment of the airway should be holistic and include multiple parameters. An objective multidimensional score such as SMAS may help to predict and manage difficult airways warranting further investigation and validation.

High eosinophil counts predict decline in FEV1: Results from the CanCOLD study

IntroductionThe aim of this study was to examine the association between blood eosinophil (EOS) levels and the decline in lung function in individuals over the age of 40 from the general population.MethodsThe study evaluated the EOS counts from thawed blood in 1120 participants (mean age 65 years) from the prospective population-based Canadian Cohort of Obstructive Lung Disease (CanCOLD) study. Participants answered interviewer-administered respiratory questionnaires and performed pre-/post-bronchodilator spirometric tests at 18-month intervals; computed tomography (CT) imaging was performed at baseline. Statistical analyses to describe the relationship between EOS levels and decline in forced expiratory volume in 1 s (FEV1) were performed using random mixed effects regression models with adjustments for demographics, smoking, baseline FEV1, ever-asthma and history of exacerbations in the previous 12 months. CT measurements were compared between EOS subgroups using an analysis of variance.ResultsParticipants who had a peripheral EOS count of ≥300 cells·µL−1 (n=273) had a greater decline in FEV1 compared with those with EOS counts of <150 cells·µL−1 (n=430) [p=0.003] (reference group) and 150 to <300 cells·µL−1 (n=417) [p=0.003]. The absolute change in FEV1 was −32.99 mL·year−1 for participants with EOS counts <150 cells·µL−1; −38.78 mL·year−1 for those with 150 to <300 cells·µL−1; and −67.30 mL·year−1 for participants with ≥300 cells·µL−1. In COPD, higher EOS count was associated with quantitative CT measurements reflecting both small and large airway abnormalities.ConclusionA blood EOS count of ≥300 cells·µL−1 is an independent risk factor for accelerated lung function decline in older adults and is related to undetected structural airway abnormalities.

The Effect of Thymoquinone on Interleukin-8 Levels, %FEV1 and CAT Scores in Chronic Obstructive Pulmonary Disease

Background: Chronic obstructive pulmonary disease (COPD) is a characterized by respiratory symptoms and persistent airway obstruction due to alveoli and/or airway abnormalities caused by continuous exposure to harmful particles or gases. Airway inflammation plays an important role in the pathogenesis of COPD. Nigella sativa, known as habbatusauda, contains thymoquinone, which has an anti-inflammatory effect. Airway inflamation improvement was measured based on decreased plasma IL-8 level, maintain the optimization of %FEV1 value and clinical improvement based on the decreased CAT score. Methods: The pretest and posttest experimental clinical trial was carried out in 40 patients with stable COPD in the pulmonology outpatient clinics of Dr. Moewardi Surakarta and dr. Soehadi Prijonegoro Sragen Hospital from 29 June to 3 August 2019. Subjects were grouped into treatment group (n=20) receiving standard therapy along with thymoquinone oil capsules 500 mg/day and placebo group (n=20) receiving standard therapy only for 30 days. Results: The study revealed statistically insignificant decreases of IL-8 level (P=0.052) and %FEV1 value (P=0.943) in the treatment group, while CAT score showed significant decreases in the treatment group (P=0.0005). Conclusion: Thymoquinone administration reduce inflammation as it can lower IL-8 level and improves the clinical condition of patients with stable COPD based on a decrease in CAT score.