Search

ObjectivesTo investigate the agreement between pulse oximetry (SpO2) and oxygen saturation (SaO2) measured by CO-oximetry on arterialised earlobe blood gas (EBG) in children and adolescents with sickle cell disease (SCD).Design and settingWe retrospectively reviewed 39 simultaneous and paired SaO2 EBG and SpO2 measurements from 33 ambulatory patients with SCD (32 subjects with Haemoglobin SS and one with Haemoglobin Sß+, 52% male, mean±SD age 11.0±3.6, age range 5–18). Measurements were performed between 2012 and 2015 when participants were asymptomatic. Hypoxaemia was defined as SaO2 ≤93%. A Bland-Altman analysis was performed to assess the accuracy of SpO2 as compared with EBG SaO2.ResultsThe mean±SD SpO2 and SaO2 values in the same patients were, respectively, 93.6%±3.7% and 94.3%±2.9%. The bias SpO2–SaO2 was −0.7% (95% limits of agreement from −5.4% to 4.1%) and precision was 2.5%. In 9/39 (23%) cases, the difference in SpO2–SaO2 was greater than the expected error range ±2%, with SaO2 more often underestimated by SpO2 (6/9), especially at SpO2values ≤93%. Thirteen participants (33%) were hypoxaemic. The sensitivity of SpO2 for hypoxaemia was 100%, specificity 85% and positive predictive value 76%.ConclusionsPulse oximetry was inaccurate in almost a quarter of measurements in ambulatory paediatric patients with SCD, especially at SpO2values ≤93%. In these cases, oxygen saturation can be confirmed through EBG CO-oximetry, which is easier to perform and less painful than traditional arterial blood sampling.

Newborn babies positively screened for cystic fibrosis (CF) (high serum immunoreactive trypsin (IRT) with DNA analysis) are referred for a diagnostic sweat test, which may be normal (sweat chloride Of 511 babies referred, 95 (19%) had a normal sweat test. Five (5%) had CF diagnosed genetically, two of them on extended genome sequencing after clinical suspicion. Eleven (12%) were designated as CF screen positive inconclusive diagnosis (CFSPID); one of the five CF children was originally designated as CFSPID. Seventy-nine (83%) were assumed to be false-positive cases and discharged; follow-up data were available for 51/79 (65%); 32/51 (63%) had no health issues, 19/51 (37%) had other significant non-CF pathology.These results are reassuring in that within the limitations of those lost to follow-up, CF symptoms have not emerged in the discharged children. The high non-CF morbidity in these children may relate to known causes of high IRT at birth. Clinicians need to be aware that a child can have CF despite a normal sweat test following NBS, and if symptoms suggest the diagnosis, further testing, including extended genome sequencing, is required.

Coughing is a primary pulmonary defence mechanism that enhances clearance of secretions and particles from the airways and protects against aspiration of foreign materials. Coughing may affect 30% of children at any given time (1). Many are healthy children but some may have serious underlying disease. Childhood cough accounts for a large number of consultations and 80% of families who are referred to a paediatric respiratory clinic for chronic cough have sought medical advice five times or more (2). The majority of childhood coughs are secondary to an acute respiratory tract infection and will improve once the infection resolves, usually within 1 to 3 weeks. With pre-school children who may experience between 6 and 10 respiratory infections a year differentiating acute recurrent cough from chronic cough is key (Table 1). Chronic cough can significantly impact a family’s quality of life, as it affects the child’s sleep, school attendance and play. Parents experience distress and anxiety, worrying that the cough may lead to long-term chest damage or even death (3). This article aims to guide clinicians through the assessment of the child with a chronic cough. It will discuss identifying causes, use of first line investigations, initiating appropriate management and addressing parental anxiety and exacerbating factors (4,5).

Pediatric pneumonia can be complicated by necrotizing pneumonia or a parapneumonic effusion either in the form of an empyema or a clear effusion. Ultrasonography (US) and computed tomography represent well-established modalities for evaluation of complicated pediatric pneumonia. Contrast-enhanced ultrasound (CEUS) was recently introduced and is gaining increasing acceptance in pediatric imaging. In this case series, we present our initial experience with both intravenous and intracavitary use of CEUS in children with complicated pneumonia. Intravenous CEUS accurately and confidently showed necrotizing pneumonia and delineated pleural effusions, whereas intracavitary CEUS accurately identified the chest catheter location and patency and showed the presence of loculations, suggesting the use of fibrinolytics.

IntroductionSickle cell disease (SCD) is one of the most common inherited diseases worldwide. It is associated with lifelong morbidity and reduced life expectancy. Hydroxyurea (HU) has been shown to reduce the frequency and severity of vaso-occlusive episodes in SCD. Hypoxaemia and intermittent nocturnal oxygen desaturations occur frequently in children with SCD and contribute to the associated morbidity, including risk of cerebrovascular disease.ObjectiveTo evaluate the effect of HU on oxygen saturation (SpO2) overnight and on daytime SpO2 spot checks in children with SCD.MethodsA retrospective review of children with SCD and respiratory problems who attended two UK tertiary sickle respiratory clinics and were treated with HU. Longitudinal data were collected from 2 years prior and up to 3 years after the commencement of HU.ResultsForty-three children, 23 males (53%) with a median age of 9 (range 1.8–18) years were included. In the 21 children who had comparable sleep studies before and after starting HU, mean SpO2 was higher (95.2% from 93.5%, p=0.01) and nadir SpO2 was higher (87.2% from 84.3%, p=0.009) when taking HU. In 32 of the children, spot daytime oxygen saturations were also higher (96.3% from 93.5%, p=0.001).ConclusionChildren with SCD had higher oxygen saturation overnight and on daytime spot checks after starting HU. These data suggest HU may be helpful for treating persistent hypoxaemia in children with SCD pending more evidence from a randomised clinical trial.

A 6-year-old previously healthy boy presented with a 5-day history of fever, malaise and pharyngitis. His parents brought him to the emergency department due to a new onset of cough and difficulty in breathing. He was fully immunised with no recent travel history or infectious contacts. At

Introduction Many factors might influence length of chest drainage (LOCD) when managing parapneumonic effusions/empyema including effusion size, septations, extent of pneumonia, presence of air leaks, pathogen virulence, fibrinolytic therapy with urokinase and local policies. UK guidelines recommend using the same fixed volumes for infants and for all children older than a year regardless of size. This preliminary retrospective survey was undertaken to determine whether there may be a signal to suggest an optimal urokinase volume based on weight which might warrant a controlled study. Objectives To investigate clinical factors that affect length of chest drain insertion (LOCD) in children with empyema and to examine if there is an optimal urokinase dosing based on patient’s weight would affect the LOCD. Methods We conducted a retrospective review of clinical data from 52 children with empyema admitted to our centre between January 2015-December 2016. Chest drains were in place for a range of 2 to 12 days with a median of 5 days. We conducted a comparison of these data between the group of patients who required chest drain insertion for ≤5 days and those ≥5 days. We also grouped the patients into two levels of urokinase dose based on patients weight to create a frequency table and eventually to look if a certain dose is associated with a shorter LOCD. Results The median LOCD insertion in our group was 5 days. Patients with a longer LOCD showed a trend to be younger and had a higher WCC, but this was not statistically significant. There were no statistically significant difference in the dose/kg of urokinase and LOCD. Conclusion Our study did not show weather a certain urokinase dose based on weight would affect the LOCD. There were no other clinical indicators among our population that can predict the LOCD.

There is conflicting evidence of the effectiveness of montelukast in preschool wheeze. A recent Cochrane review focused on its use in viral-induced wheeze; however, such subgroups are unlikely to exist in real life and change with time, recently highlighted in an international consensus report. We have therefore sought to investigate the effectiveness of montelukast in all children with preschool wheeze (viral-induced and multiple-trigger wheeze). The PubMed, Cochrane Library, Ovid Medline and Ovid EMBASE were screened for randomised controlled trials (RCTs), examining the efficacy of montelukast compared with placebo in children with the recurrent preschool wheeze. The primary endpoint examined was frequency of wheezing episodes. Five trials containing 3960 patients with a preschool wheezing disorder were analysed. Meta-analyses of studies of intermittent montelukast showed no benefit in preventing episodes of wheeze (mean difference (MD) 0.07, 95% confidence interval (CI) −0.14 to 0.29; mean for montelukast 2.68 vs placebo 2.54 (p = 0.5)), reducing unscheduled medical attendances (MD −0.13, 95% CI −0.33 to 0.07; mean for montelukast 1.62 vs placebo 1.78 (p = 0.21)) and reducing oral corticosteroids (MD −0.06, 95% CI −0.16 to 0.02; mean for montelukast 0.35 vs placebo 0.36 (p = 0.25)). The pooled results of the continuous regimen showed no significant difference in the number of wheezing episodes between the montelukast and placebo groups (MD −0.40, 95% CI −1.00 to 0.19; mean for montelukast 2.05 vs placebo 2.37 (p = 0.18)). Conclusions: This review highlights that the currently available evidence does not support the use of montelukast in preschool children with recurrent wheeze. We recommend further studies to investigate if a ‘montelukast responder’ phenotype exists, and how these can be easily identified in the clinical setting.What is Known:• Current guidelines recommend montelukast use in preschool children with recurrent wheeze.• A recent Cochrane review has found montelukast to be ineffective at reducing courses of oral corticosteroids for viral-induced wheeze.What is New:• This meta-analysis has examined all children with preschool wheeze and found that montelukast was not effective at preventing wheezing episodes or reducing unscheduled medical attendances.• A specific montelukast responder phenotype may exist, but such patients should be sought in larger multicentre RCTs.

Pediatric pneumonia can be complicated by necrotizing pneumonia or a parapneumonic effusion either in the form of an empyema or a clear effusion. Ultrasonography (US) and computed tomography represent well-established modalities for evaluation of complicated pediatric pneumonia. Contrast-enhanced ultrasound (CEUS) was recently introduced and is gaining increasing acceptance in pediatric imaging. In this case series, we present our initial experience with both intravenous and intracavitary use of CEUS in children with complicated pneumonia. Intravenous CEUS accurately and confidently showed necrotizing pneumonia and delineated pleural effusions, whereas intracavitary CEUS accurately identified the chest catheter location and patency and showed the presence of loculations, suggesting the use of fibrinolytics.

Introduction: Pseudomonas aeruginosa (PA) is associated with worsening morbidity and mortality in children with long term O2, long term ventilation or tracheostomy. There are currently no guidelines for management of PA in these patients. Aim: To report on incidence and management of PA infection in children with long term O2, long term ventilation or tracheostomy. Methods: All children with long term O2 therapy, ventilation or tracheostomy and initial PA infection, at King9s College Hospitals from January 2010 to August 2015 were included. PA infection was defined as isolation of PA from a respiratory sample. Patients with cystic fibrosis or bronchiectasis were excluded. Cases were identified using diagnostic codes. Electronic medical records were reviewed for demographics, presentation, comorbidities and management. Results: There were 30 children with long term O2 (10% grew PA), 27 on long term ventilation (30% grew PA) and 7 with tracheostomy (85% grew PA). There was huge variability in respiratory sampling practices (range 0 to 5 samples per month), with more frequent sampling in tracheostomy patients. Treatment was initiated in 13 (76%) and eradication was achieved in 50% of treated patients. Conclusions: Many patients on long term O2 therapy, ventilation or tracheostomy grew PA with huge variation in management practices. There is need for further research and consensus for management of these patients.

Background Wheeze in the preschool age has a high prevalence and healthcare burden. There is conflicting evidence of the effectiveness of montelukast, a leukotriene receptor antagonist, in preschool wheeze. Recent meta-analyses have focused on its use in viral induced or multiple-trigger wheeze, however such groups are unlikely to exist in real-life and change with time, recently highlighted in an international consensus report. We have therefore sought to investigate the effectiveness of montelukast in all children with preschool wheeze. Methods The PubMed, Cochrane Library, Ovid Medline and Ovid EMBASE were screened for randomised controlled trials (RCTs), examining the efficacy of montelukast compared with placebo in children with the preschool wheeze. Results Five trials containing 3960 patients (6 months to 5 years) with a preschool wheezing disorder were analysed. Meta-analyses of studies of intermittent montelukast, showed no benefit in preventing episodes of wheeze (Mean Difference (MD) 0.07, 95% CI -0.14 to 0.29), reducing unscheduled medical attendances (MD -0.13, 95% CI -0.33 to 0.07) and reducing oral corticosteroid use (MD -0.06, 95% CI -0.16 to 0.02). The pooled results of the continuous regimen showed no significant difference in the number of wheezing episodes between the montelukast and placebo groups (MD -0.40, 95% CI -1.00 to 0.19). Discussion This review highlights that the currently available evidence do not support the routine use of montelukast in children with preschool wheeze. We recommend further studies to investigate if the specific 9montelukast responder9 phenotype exists, and how these can be easily identified in the clinical setting.

Background Obesity is an increasing problem, 31% of children being obese or overweight. Obesity increases the risk of obstructive sleep apnoea (OSA), is linked to lower lung volumes and restrictive lung disease. Children who are morbidly obese are at such high risk of co-morbidities that bariatric surgery is performed at King9s College Hospital, with excellent success. Aims We aim to perform respiratory assessments prior to bariatric surgery, to ensure that children are safe for the procedure, and to check for treatable respiratory co-morbidities. Methods We have performed respiratory assessments prior to bariatric surgery in 11 children (5 male) under 19 years. The mean BMI was 50 (31.8-62.6). The assessment includes a sleep history, sleep study, and spirometry. Results Three patients had moderate-severe sleep disordered breathing prior to surgery, and were commenced on CPAP. A mean BMI reduction of -9.2 and mean weight loss of -19 Kg, 8 months following surgery was seen. One patient was able to stop CPAP post operatively due to significant weight loss. Conclusions Morbidly obese paediatric patients listed for bariatric surgery have a high incidence of OSA, a respiratory assessment is essential pre-operatively, so that treatment can be instituted prior to surgery.

Introduction: Children with Sickle cell disease (SCD) have a higher prevalence of Obstructive sleep apnea (OSA) and OSA has been identified as an important comorbidity in children with SCD. There evidence linking nocturnal desaturations with an increased rate of both vaso-occlusive episodes (VOE) and acute chest syndrome (ACS). Methods: In our centre, all children and adolescents with SCD and OSA are evaluated for adenotonsillar hypertrophy, as adenotonsillectomy may be curative in some. Retrospective review of children with SCD who underwent adenotonsillectomyfor OSA between 2004 to 2015. Sleep study data and admission rates due to VOE & ACS were compared, before & after adenotonsillectomy. Results: 37 children with SCD underwent adenotonsillectomy during this time frame. 22 children (13 male) with a median age of 7 years (range 3-12 years) had sleep study before and after adenotonsillectomy. Discussion: Children with SCD and OSA, who had adenotonsillectomy, appeared to have a significant increase in overnight nadir oxygen saturations, as well as a reduction in the overnight ODI; but no change in the rates of severe ACS or VOE. Further testing by a randomised controlled trial or prospective longitudinal study is needed.

Incidence of pulmonary infection with nontuberculous mycobacteria (NTM) is increasing among persons with cystic fibrosis (CF). We assessed prevalence and management in CF centers in the United Kingdom and found 5.0% of 3,805 adults and 3.3% of 3,317 children had recently been diagnosed with NTM. Of those, 44% of adults and 47% of children received treatment.

Aim Accurate measurement of Hb saturation is essential in the care of children with sickle cell disease (SCD). The gold standard of Hb saturation measurement is by ABG with co-oximetry. Minute-by-minute changes are detected with pulse oximetry (SpO2). SpO2 shows good correlation to ABG co-oximetry in well patients with normal Hb. SCD patients represent a different population: previous small studies have shown discrepancies in calculated ABG and SpO2 Hb saturations. SpO2 may be falsely low due to high carboxyhaemoglobin and metahaemaglobin levels following heme catabolism, or high due to hyperbilirubinaemia. Both occur in acute and chronic haemolysis. With treatment decisions in acute and ambulatory settings driven by bedside SpO2, inaccuracies can result in mismanagement. Earlobe blood gas (EBG) co-oximetry is an alternative better tolerated method of measuring Hb O2 saturation in arterialised capillary blood (SaO2), with good correlation to ABG measurements. We sought to investigate the correlation between SpO2 and SaO2 measured by EBG co-oximetry in ambulatory SCD children. Methods We retrospectively reviewed paediatric SCD patients attending a UK tertiary sickle-respiratory clinic from February 2012–August 2015, and who had simultaneous EBG and SpO2 measurements. Positive difference results from SpO2 value greater than EBG SaO2. Results We identified 39 simultaneous paired SaO2 and SpO2 readings from 33 patients (52% male, median age 10years, range: 5–17). Mean difference between readings was –0.7% (Figure 1 and 2). In 23% of cases, difference was >±2%. SaO2 was overestimated in one-third and underestimated in two-thirds. Of 17 hypoxaemic measurements (SpO2 Conclusion Pulse oximetry as a measure of Hb oxygen saturation was inaccurate in a significant proportion of ambulatory SCD children. SpO2 tended to be lower than actual SaO2 as measured with EBG, with SpO2≥94% more likely to predict true result than SpO2

A 3 yr old boy presented with abdominal pain, fever and red jelly stools. Intussusception was diagnosed and effectively reduced with air insufflation. However, despite an improvement in his clinical condition, the child remained febrile and miserable; 5 days later he developed characteristic signs of Kawasaki disease and was treated with intravenous immunoglobulin and high dose aspirin with good results. Intussusception prior to the typical features of Kawasaki disease has not been described previously in the English literature. This case illustrates a novel presentation of Kawasaki disease.

Introduction Sickle cell disease (SCD) causes lifelong morbidity and reduced life expectancy. Resting hypoxaemia and intermittent nocturnal oxygen desaturation are often seen in children with SCD, which may contribute to morbidity associated with vaso-occlusive episodes. Treatment with hydroxyurea reduces the frequency and severity of vaso-occlusive episodes 1 but the impact of hydroxyurea on oxygen saturation and sleep apnoea is unknown. Objective To look for any difference in baseline oxygen saturation asleep and awake and the frequency of intermittent nocturnal desaturation after starting hydroxyurea in children with SCD. Methods A retrospective review of children who were commenced on hydroxyurea between March 2006 and July 2014 attending two UK sickle-respiratory clinics. Data was collected from overnight sleep studies and averaged pulse oximeter spot check recordings in clinic notes when awake from a) 6 months before starting hydroxyurea and b) up to 2 years after. Lung function and haemoglobin changes were also noted over the same time periods. Results Forty six children (25 male) with a median age of 10 years (range 5–19 years) were started on hydroxyurea. Haemoglobin and HbF rose significantly on hydroxyurea as expected (Table 1). After starting hydroxyurea the average overnight oxygen saturation increased from median of 93.5% to 95.2% (p = 0.01) and the median daytime spot oxygen saturation rose from 93.5% to 96.3% (p = 0.001). There was no significant change in the median intermittent nocturnal 3% oxygen desaturation index (ODI), nocturnal PCO 2 or spirometry. Conclusion In children with SCD, the use of hydroxyurea was associated with a significant increase in awake and nocturnal baseline oxygen saturation, but no change in intermittent nocturnal desaturation indices or lung function. This preliminary data suggests that improving oxygen saturation may be an important outcome of hydroxyurea therapy with potential benefits in reducing not only vaso-occlusive crises but future respiratory morbidities. This hypothesis would need to be tested by a prospective multicenter trial. Reference 1 Charache S, Terrin ML, Moore RD, et al . Effect of hydroxyurea on the frequency of painful crises in sickle cell anemia. Investigators of the Multicenter Study of Hydroxyurea in Sickle Cell Anemia. N Engl J Med 1995; 332 :1317–1322

To study the incidence, clinical presentation, management, complications and outcome of tuberculosis in pediatric liver transplant recipients.A retrospective review of the medical records of children who underwent liver transplantation between 1991 and 1998.Mycobacterium tuberculosis infection occurred in 6 of 254 (2.4%) children undergoing liver transplantation between 1991 and 1998. Cough, pyrexia and poor appetite were common presentations; one-half had normal chest radiographs. The median time to confirmation of diagnosis was 8 months (range, 1 to 17 months). Tests contributing to diagnosis included: Ziehl-Neelsen (ZN) stain (2 patients), M. tuberculosis polymerase chain reaction (1 patient), Mantoux test (1 patient) and histopathology (4 patients). Family health screening was productive for 4 patients. Duration of treatment varied from 9 to 18 months. Isoniazid-induced hepatitis was observed in 2 patients but resolved with dose reduction. Two patients died while receiving treatment, one of Klebsiella spp. septicemia and the other of pulmonary hemorrhage.Tuberculosis after liver transplantation has a significant morbidity and mortality. Pretransplantation a personal and family history of tuberculosis must be sought, and screening of patients and their families should be considered. Standard regimens incorporating isoniazid and rifampin are effective, but regular monitoring of liver function is essential to detect drug-induced hepatotoxicity.

A prematurely born infant had three episodes of feeding intolerance in the first three weeks after birth. In the post-partum period, his mother, who was from the Ukraine, had a respiratory arrest; unusually, a high-resolution computerised tomograph demonstrated miliary tuberculosis (TB). As a consequence, and due to the continuing ill health of the infant, gastric aspirates were sent from the infant. Acid fast bacilli were seen on microscopy. Variable nucleotide tandem repeat analysis of acid fast bacilli facilitated the rapid diagnosis of connatal TB. We conclude that connatal TB should be considered in a prematurely born infant poorly responsive to standard management and whose mother falls into a high-risk group.

Rituximab (chimeric anti-CD20 monoclonal antibody) has been reported to be effective in various autoimmune conditions, especially in autoimmune cytopenias, but there are few reports of its use in paediatric population with underlying immunodeficiency states. We report three cases of autoimmune cytopenias (Evan’s syndrome in DiGeorge syndrome, autoimmune thrombocytopenia in a HIV positive patient and lastly autoimmune thrombocytopenia in a post liver transplant patient) where rituximab was effective in reversing the cytopenias. First patient (11yrs) had DiGeorge syndrome which was complicated by Evan’s syndrome. It was treated with multiple courses of steroids and immunosuppressive agents, with resultant recurrent systemic bacterial and fungal infections. She had rituximab as a last resort, which resulted in sustained improvements in her Haemoglobin and platelet count. Second patient (12yrs) was diagnosed with HIV in 1993. She had sudden onset Intra-cranial bleed as a result of immune thrombocytopenia. She was treated with Intravenous immunoglobulin (IVIg) and subsequently with prednisolone with no response. She however responded to rituximab and then went on to have rituximab for a further 2 courses, each 12 months apart. The third patient (14yrs) had auxiliary liver transplant in 2002. He developed immune thrombocytopenia and neutropenia in which remission was sustained only for a maximum of 6 weeks following IVIg. As he was already on immunosuppression, he was given rituximab to which he has had a sustained remission. Side effects: The patient with DiGeorge syndrome had recurrent chest infection which was thought to be due to aspiration as a result of her hiatus hernia. The other patients did not have major side-effects. Immunoglobulin levels in all three patients were in normal range. Thus, in our experience rituximab is a useful adjunct in management of autoimmune cytopenias with an underlying immunodeficiency state and should be considered early in steroid refractory cases, as it leads to sustained remissions in the blood counts. It is well tolerated without major infections in patients who are already immunosuppressed due to their immunodeficiency state and the fear of immuneparesis is not seen.