Optical coherence tomography (OCT) and OCT angiography allow early identification of sickle cell maculopathy in children and correlate it with systemic risk factors.

PURPOSE: To determine the presence of sickle cell retinopathy and maculopathy and to identify associations between markers of hemolysis and systemic and ocular manifestations in children affected by sickle cell disease. METHODS: Eighteen children with sickle cell disease, aged 5-16 years, underwent complete eye examination including best-corrected visual acuity, slit-lamp biomicroscopy, ophthalmoscopy after pharmacological mydriasis, spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCTA). Blood test results and clinical history information were collected for each child, including fetal hemoglobin (HbF), hemoglobin (Hb), hematocrit (Htc), mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), reticulocytes percentage (%ret), lactic dehydrogenase (LDH), total and direct bilirubin, glomerular filtration rate, number of painful crises, acute chest syndromes, and splenic sequestration. Therapeutic regimen and transfusion therapy were also evaluated. RESULTS: Sixteen of 36 eyes (44.4%) had non-proliferative sickle cell retinopathy on ophthalmoscopic evaluation. No patients had proliferative sickle cell retinopathy. In 13 of 36 eyes (36.1%), SD-OCT and OCTA detected signs of sickle cell maculopathy. Nine eyes (25%) presented sickle cell retinopathy and maculopathy, 7 eyes (19.4%) sickle cell retinopathy alone, and 4 eyes (11.1%) sickle cell maculopathy alone. A statistically significant association was found between sickle cell retinopathy; lower levels of HbF, Hb, and Htc; and higher MCV and percentage of reticulocytes. Sickle cell maculopathy was associated with lower values of H and Htc and higher levels of reticulocytes and total bilirubin. CONCLUSIONS: We identified early signs of sickle cell retinopathy and maculopathy in a pediatric population with SD-OCT and OCTA. These two retinal complications were more frequent in children with higher hemolytic rates.

An urgent need for African spirometry reference equations: the Paediatric and Adult African Spirometry study.

BACKGROUND: The GLI2012 (Global Lung Initiative 2012) has provided the largest data set to date for multi-ethnic spirometry reference equations; however, data on African populations are limited. In pulmonary function testing, diagnosis of lung disorder is based on comparing the individual's lung function to a reference appropriate for sex and ethnicity.METHODS: We conducted a systematic review of studies reporting spirometry results in healthy children and adults in Africa. Data from these studies were collated for Z-scores of forced expiratory volume in 1 sec (zFEV1), forced vital capacity (zFVC) and zFEV1/FVC compared to GLI reference equations.RESULTS: Nine studies, covering a total of 4750 individuals from North, South, East, West and Central Africa (52% were female), were reviewed. Marked differences were noted between individuals from North Africa and sub-Saharan Africa. The Southern zFEV1 (-0.12 ± 0.98), zFVC (-0.15 ± 0.98) and zFEV1/FVC (0.05 ± 0.89), Central zFEV1 (-0.16 ± 0.79), zFVC (-0.09 ± 0.83) and zFEV1/FVC (-0.17 ± 0.71) and East African zFEV1 (0.10 ± 0.88), zFVC (0.16 ± 0.85) and zFEV1/FVC (-0.10 ± 0.95) cohorts had an excellent fit with the GLI-African American. The West African showed a poor fit to all reference equations. The North African group showed the best fit for the GLI Caucasian zFEV1 (-0.12 ± 1.37), zFVC (-0.26 ± 1.36) and zFEV1/FVC (0.25 ± 1.11). The zFEV1/FVC ratios were stable across all the populations.CONCLUSION: Current evidence seems to support the use of GLI2012 reference values in North African and sub-Saharan African populations after taking into account ethnic correction factors.

Obstructive sleep apnea syndrome in the pediatric age: the role of the pneumologist.

OBJECTIVE: This review paper aims to summarize the current state of knowledge on the role of the pneumologist in the diagnosis and respiratory treatment of children affected by obstructive Sleep Disordered Breathing (SDB). MATERIALS AND METHODS: A literature review has been performed on the following topics: obstructive SDB and its clinical entities, indications for respiratory treatment of pediatric SDB, and Continuous Positive Airway Pressure (CPAP) and Noninvasive Positive Pressure Ventilation (NIPPV) treatment approach to obstructive SDB. RESULTS: OSDB is related to obesity, craniofacial pathologies, neuromuscular disorders and, most commonly, oadenotonsillar hypertrophy. Adenotonsillectomy is the first-choice treatment in children with obstructive apnea secondary to adenotonsillar hypertrophy. CPAP and NIPPV are recommended in cases where Obstructive Sleep Apnea (OSA) persists after surgery or when surgery is contraindicated. Treatment interventions are usually implemented gradually by separately addressing each abnormality that would predispose to obstructive SDB, then reevaluating after each intervention to detect any residual disease and to assess the need for additional treatment. CONCLUSIONS: Many pediatric patients continue to experience problems and symptoms such as hypersomnia and apnea after adenotonsillectomy and need CPAP/NIPPV treatment. Current knowledge is still incomplete, especially with regard to the mechanisms of pathogenesis of pediatric OSA, the factors affecting pediatric OSA, and the phenotypic variability of the disease. A better understanding of these aspects would contribute to the development of new therapies.

Obstructive sleep apnea syndrome in the pediatric age: the role of the otorhinolaryngologist.

OBJECTIVE: Obstructive sleep apnea (OSA) is the primary indication for tonsillectomy, one of the most common pediatric surgical procedures, commonly performed in association with adenoidectomy. The objective of this review article is to evaluate the role of the otorhinolaryngologist in pediatric OSA. MATERIALS AND METHODS: A literature review has been performed on the following topics: peculiarities of sleep-disordered breathing in pediatric age; discrimination of sleep disorders; adenotonsillar hypertrophy; surgical techniques; adjuvant surgical procedures. RESULTS: The role of the otorhinolaryngologist in pediatric OSA is important for the evaluation of the upper airways and of essential biometric and polysomnographic data and for indication and execution of appropriate surgical treatment. In the majority of healthy children, adenotonsillectomy for OSA results in a dramatic improvement in respiratory parameters as measured by polysomnography. When post-surgical residual OSA occurs, it is essential to monitor patients by means of drug-induced sleep endoscopy (DISE). CONCLUSIONS: Otolaryngologic assessment is of paramount importance to correctly classify a child with OSA. Correct inspection of the upper airway and quantification of the quality of sleep through polysomnography lead to the right therapeutic choice. Knowledge of different surgical techniques helps to deal with residual OSA after studying the obstruction sites by drug-induced sedation endoscopy.

Prospective multicentre study on barbed reposition pharyngoplasty standing alone or as a part of multilevel surgery for sleep apnoea.

OBJECTIVES: The aim of this study was to demonstrate in a prospective multicentre study that Barbed Reposition Pharyngoplasty (BRP) procedure is safe and effective in management of obstructive sleep apnoea/hypopnea syndrome (OSAHS) patients. DESIGN: Prospective study. SETTING: Multicentre study. PARTICIPANTS: Patients suffering from obstructive sleep apnoea. MAIN OUTCOMES MEASURES: Values of postoperative apnoea-hypopnea index (AHI), oxygen desaturation index (ODI), epworth sleepiness scale (ESS). RESULTS: 111 Barbed Reposition Pharyngoplasty procedures standing alone or as a part of multilevel surgery for OSAHS, performed between January and September 2016, were analysed in 15 different centres. The average hospitalisation period was 2.5 ± 0.5 days. The mean patient age was 46.3 ± 10.5 years. The average body mass index at the time of the procedure was 27.9 ± 3.2, and the majority of the patients were men (83%). The mean preoperative and postoperative apnoea/hypopnea index was 33.4 ± 19.5 and 13.5 ± 10.3, respectively (P < .001). The mean preoperative and postoperative ESS score was 10.2 ± 4.5 and 6.1 ± 3.6, respectively (P < .001). The mean preoperative and postoperative ODI were 29.6 ± 20.7 and 12.7 ± 10.8, respectively (P < .001). CONCLUSIONS: Patients undergoing BRP standing alone or as part of a multilevel approach for the treatment of OSAHS have a reasonable expectation for success with minimal morbidity.