Sustained pharyngeal inflation in infant airway-Flexible bronchoscopy measurements.

Sustained pharyngeal inflation (SPI) with pharyngeal oxygen flow and nasal closure (PhO2-NC) technique create positive inflation pressure in the airway. This study measured the peak inflation pressure (PIP) levels and image changes with SPI-assisted flexible bronchoscopy (SPI-FB) and compared the effects in the pharyngeal space and mid-tracheal lumen. This prospective study enrolled 20 participants aged 6 months to 3 years. Each participant underwent sequential SPI-FB of four different durations (0, 1s, 3s, and 5s) for three cycles. We used a 3.8 mm OD flexible bronchoscope to measure and analyze PIP levels, images, and lumen dimension scores. A total of 480 data were collected. The mean (SD) age and body weight were 12.0 (11.5) months and 7.8 (7.5) kg, respectively. The mean (IQR) PIPs were 4.2 (2.0), 18.5 (6.1), 30.6 (13.5), and 46.1 (25.0) cmH2O in the pharynx and 5.0 (1.6), 17.5 (6.5), 28.0 (12.3), 46.0 (28.5) cmH2O in the mid-trachea at SPI durations of 0, 1s, 3s, and 5s, respectively. The PIP levels had a positive correlation (p <0.001) with different SPI durations in both pharynx and trachea, and were nearly identical (p = 0.695, 0.787, and 0.725 at 1s, 3s, and 5s, respectively) at the same duration except the 0 s (p = 0.015). Lumen dimension scores also significantly increased with increasing SPI durations (p <0.05) in both locations. The identified lesions significantly increased as PIP levels increased (p <0.001). Conclusion: SPI-FB using PhO2-NC with durations up to 3s is safe and informative technique that provides controllable PIP, dilates airway lumens, and benefits lesion detection in the pharyngeal space and mid-tracheal lumen.

A Single Center Observational Study of Spirometry Assessments in Children with Congenital Heart Disease after Surgery.

Background: Children with congenital heart disease (CHD) have impaired pulmonary function both before and after surgery; therefore, pulmonary function assessments are important and should be performed both before and after open-heart surgery. This study aimed to compare pulmonary function between variant pediatric CHD types after open-heart surgery via spirometry. Methods: In this retrospective study, the data for forced vital capacity (FVC), forced expiratory volume in the first second (FEV1), and the ratio between FEV1 and FVC (FEV1/FVC) were collected from patients with CHD who underwent conventional spirometry between 2015 and 2017. Results: A total of 86 patients (55 males and 31 females, with a mean age of 13.24 ± 3.32 years) were enrolled in our study. The diagnosis of CHD included 27.9% with atrial septal defects, 19.8% with ventricular septal defects, 26.7% with tetralogy of Fallot, 7.0% with transposition of the great arteries, and 46.5% with other diagnoses. Abnormal lung function was identified by spirometry assessments after surgery. Spirometry was abnormal in 54.70% of patients: obstructive type in 29.06% of patients, restrictive type in 19.76% of patients, and mixed type in 5.81% of patients. More abnormal findings were found in patients who received the Fontan procedure (80.00% vs. 35.80%, p = 0.048). Conclusions: Developing novel therapies to optimize pulmonary function will be critical for improving clinical outcomes.

Flexible Endoscopy With Non-invasive Ventilation Enables Clinicians to Assess and Manage Infants With Severe Bronchopulmonary Dysplasia.

Objectives: The objectives of the study were to determine the efficacy of flexible endoscopy (FE) to assess the approachable aeroesophageal tract (AET) and subsequent changes in clinical management in infants with severe bronchopulmonary dysplasia (sBPD). Methods: This retrospective study investigated sBPD infants who received FE measurement from 2011 to 2020. FE was supported with non-invasive ventilation (FE-NIV) of pharyngeal oxygen with nose closure and abdominal compression without any mask or laryngeal mask airway. Data on AET lesions, changes in subsequent management, and FE therapeutic interventions were collected and analyzed. Results: Forty-two infants were enrolled in the study. Two thin scopes (1.8- and 2.6-mm outer diameter) were used. FE analysis revealed 129 AET lesions in 38 (90.5%) infants. Twenty-eight infants (66.7%) had more than one lesion. Thirty-five (83.3%) infants had 111 airway lesions where bronchial granulations (28, 25.2%), tracheomalacia (18, 16.2%), and bronchomalacia (15, 13.5%) were the main complications. Eighteen esophageal lesions were found in 15 (35.7%) infants. No significant FE-NIV complications were observed. The FE findings resulted in changes in management in all 38 infants. Thirty-six (85.7%) infants underwent altered respiratory care with pressure titrations (29, 45.3%), shortened suction depth (17, 26.6%), immediate extubation (8, 12.5%), changed insertion depth of endotracheal tube (7, 10.9%) and tracheostomy tube (3, 4.7%). Twenty-one (50%) infants had 50 pharmacotherapy changes, including added steroids, anti-reflux medicine, antibiotics, and stopped antibiotics. Eighteen (42.8%) infants received 37 therapeutic FE-NIV procedures, including 14 balloon dilatations, 13 laser-plasty, and 10 stent implantations. Seven (16.7%) infants underwent surgeries for four tracheostomies and three fundoplications. Conclusion: Flexible endoscopy with this non-invasive ventilation could be a safe and valuable technique for direct and dynamic visual measurement of AET, which is essential for subsequent medical decision making and management in infants with sBPD.

Air Pollution Exposure and Risk of Spontaneous Pneumothorax in Children: A Longitudinal, Nationwide Study.

Spontaneous pneumothorax (SP) involves the spontaneous appearance of air in the pleural space. Atmospheric pressure, temperature change, and seasonal factors may precipitate SP, but its association with air pollution remains unclear. Therefore, we conducted this nationwide, retrospective population-based study to evaluate the risk of SP in Taiwanese children exposed to air pollution. We collected data on SP incidence from the Longitudinal Health Insurance Database; the Taiwan Air Quality-Monitoring Database provided daily concentrations of nitric oxide (NO), nitrogen dioxide (NO2), and hydrocarbons in 2000-2012. SP risk was evaluated for four quartiles (Q1, Q2, Q3, Q4). The NO adjusted hazard ratios (aHRs) for Q2, Q3, and Q4 compared to Q1 were 1.11 (95% confidence interval (CI): 0.77-1.61), 1.24 (95% CI: 0.88-1.76), and 1.66 (95% CI: 1.17-2.34), respectively. The NO2 aHRs for Q2, Q3, and Q4 were 1.12 (95% CI: 0.77-1.64), 1.31 (95% CI: 0.0.90-1.90), and 1.51 (95% CI: 1.04-2.19), respectively. Hydrocarbons aHRs for Q2, Q3, and Q4 were 0.87 (95% CI: 0.64-1.18), 1.16 (95% CI: 0.90-1.49), and 1.40 (95% CI: 1.06-1.85), respectively. Increased exposure to NO, NO2, and hydrocarbons is associated with increased SP risk in Taiwanese children.

Polysomnography Is an Important Method for Diagnosing Pediatric Sleep Problems: Experience of One Children's Hospital.

In this study, we collected and analyzed polysomnography (PSG) data to investigate the value of PSG in diagnosing sleep problems in children. The results of PSG studies of children (<18 years old) with sleep problems conducted from April 2015 to May 2017 at a children's hospital in Taiwan were collected and analyzed retrospectively. Data for 310 patients (209 males and 101 females) who underwent PSG were collected. The final diagnoses were as follows: obstructive sleep apnea in 159 (51.3%), snoring in 81 (26.4%), limb movement sleep disorder in 25 (8.1%), hypersomnias in 12 (3.9%), central apnea in 8 (2.9%), enuresis in 7 (2.3%), bruxism in 5 (1.6%), sleep terrors in 5 (1.6%), narcolepsy in 3 (1.0%), sleep seizures in 3 (1.0%), sleep walking in 1 (0.3%), and insomnia in 1 (0.3%). PSG may help detect significant sleep-related problems in children and is useful for making therapeutic decisions regarding children. Obstructive sleep apnea syndrome (OSAS) was the primary sleep problem for most of the children (51.3%); however, only 7.4% of them underwent surgery for OSAS. We therefore suggest that children with sleep problems should undergo PSG.

Lamotrigine Induced DRESS Syndrome in a Child: A Case Report and Literature Review.

Lamotrigine is an important anticonvulsant drug. Its use, however, has been limited by the risk of potentially life-threatening dermatological reactions, such as a drug reaction with eosinophilia and systemic symptoms (DRESS). Here, we report the case of a 7-year-6-month-old girl with a history of epilepsy who developed a skin rash with dyspnoea after 2 weeks of lamotrigine treatment, with DRESS ultimately being diagnosed. After discontinuation of the offending drug and the initiation of systemic glucocorticosteroids, the DRESS symptoms were relieved and the patient was discharged in a stable condition. Anticonvulsant drugs such as lamotrigine are among the factors that induce DRESS in children. When a patient displays skin rash and systemic organ involvement following the initiation of an anticonvulsant drug, DRESS should not be overlooked as a diagnosis, and immunosuppressant drugs should be considered as an option for treating DRESS patients.

NAXE gene mutation-related progressive encephalopathy: A case report and literature review.

RATIONALE: Progressive encephalopathy with brain edema and/or leukoencephalopathy-1 is an infantile, lethal neurometabolic disorder caused by a NAD(P)HX epimerase (NAXE) gene mutation. It is characterized by a fluctuating disease course with repeated episodes of improvement and regression. In this report, we present a rare case of NAXE gene mutation-related encephalopathy with unexpected neurological recovery and long survival time. PATIENT CONCERNS: A 20-month-old girl presented with progressively unsteady gait and bilateral hand tremors after a trivial febrile illness. Her disease rapidly progressed to consciousness disturbance, 4-limb weakness (muscle power: 1/5 on the Medical Research Council scale), and respiratory failure. The patient gradually recovered 2 months later. However, another episode of severe fever-induced encephalopathy developed 2 years after the initial presentation. DIAGNOSES: Results of laboratory investigations, including complete blood count, blood chemistry, inflammatory markers, and cerebral spinal fluid analysis were unremarkable. Electroencephalography and nerve conduction velocity studies yielded normal results. Brain magnetic resonance imaging on diffusion-weighted imaging revealed abnormal sysmmetric hyperintensity in the bilateral middle cerebellar peduncles. A genetic study using whole exome sequencing confirmed the diagnosis of NAXE gene mutation-related encephalopathy. INTERVENTIONS: Pulse therapy with methylprednisolone, intravenous immunoglobulin, coenzyme Q10, and carnitine were initially introduced. After a NAXE gene defect was detected, the vitamin B complex and coenzyme Q10 were administered. A continuous rehabilitation program was also implemented. OUTCOMES: NAXE gene mutation-related encephalopathy is usually regarded as a lethal neurometabolic disorder. However, the outcome in this case is better than that in the previous cases. She showed progressive neurological recovery and a longer survival time. The muscle power of the 4 limbs recovered to grade 4. At present (age of 5.5 years old), she can walk with an unsteady gait and go to school. LESSONS: Although NAXE gene mutation-related encephalopathy is rare, it should be considered as a differential diagnosis of early onset progressive encephalopathy.

Sustained pharyngeal inflation on upper airway effects in children-Flexible bronchoscopy measurement.

OBJECTIVE: Sustained pharyngeal inflation (SPI) with pharyngeal oxygen and nose-closure (PhO2 -NC) can create positive peak inflation pressure (PIP) inside the pharyngolaryngeal space (PLS). This study measured and compared the effects of four different SPI durations in the PLS. METHODS: A prospective study, 20 consecutive children aged between 6 months and 3 years old, scheduled for elective flexible bronchoscopy (FB) suspected positive PLS findings were enrolled. SPI was performed twice in four different durations (0, 1, 3, and 5 s) sequentially in each infant. PIP was measured for each SPI in the pharynx, while simultaneously record images at two locations of the oropharynx and supra-larynx. Patient demographic details, PIP levels, lumen expansion scores, and images of PLS were measured and analyzed. RESULTS: Twenty patients with 40 measurements were collected. The mean (SD) age and weight were 11.6 (9.1) months and 6.8 (2.4) kg, respectively. The measured mean (SD) pharyngeal PIPs were 4.1 (3.3), 21.9 (7.0), 42.2 (12.3), and 65.5 (18.5) cmH2 O at SPI duration of 0, 1, 3, and 5 s, respectively, indicating significant (p<.001) positive correlation. At assigned locations, corresponding PLS images also displayed a significant increase in lumen expansion scores and a number of detected lesions with an increase in SPI duration (p < .004). The mean (SD) procedural time was 5.7 (1.2) min. No study-related complication was noted. CONCLUSIONS: FB utilizing PhO2 -NC as SPI of 1-3 s is a simple, less invasive, and valuable ventilation modality. It provides an adequate PIP level to expand the PLS and improve FB performance in children.

Comparison of severe pediatric complicated influenza patients with and without neurological involvement.

ABSTRACT: Although influenza is generally an acute, self-limited, and uncomplicated disease in healthy children, it can result in severe morbidity and mortality. The objectives of this study were to analyze and compare the clinical features and outcome of severe pediatric influenza with and without central nervous system (CNS) involvement.We conducted a retrospective observational study of children admitted to the pediatric intensive care unit (PICU) of China Medical University Children's Hospital in Taiwan with a confirmed diagnosis of influenza. The demographic data, clinical and laboratory presentations, therapeutic strategies, and neurodevelopmental outcomes for these patients were analyzed. Furthermore, comparison of patients with and without CNS involvement was conducted.A total of 32 children with severe influenza were admitted during the study periods. Sixteen children were categorized as the non-CNS (nCNS) group and 16 children were categorized as the CNS group. Nine of them had underlying disease. The most common complication in the nCNS group was acute respiratory distress syndrome, (n = 8/16), followed by pneumonia (n = 7/16, 44%). In the CNS group, the most lethal complication was acute necrotizing encephalopathy (n = 3/16) which led to 3 deaths. The overall mortality rate was higher in the CNS group (n = 6) than in the nCNS group (n = 1) (37.5% vs 6.25%, P = .03).The mortality rate of severe complicated influenza was significantly higher with CNS involvement. Children with primary cardiopulmonary abnormalities were at high risk of developing severe complicated influenza, while previously healthy children exhibited risk for influenza-associated encephalitis/encephalopathy.

End-of-Life Care in Taiwan: Single-Center Retrospective Study of Modes of Death.

OBJECTIVES: Medical advances and the National Health Insurance coverage in Taiwan mean that mortality in the PICU is low. This study describes change in modes of death and end-of-life care in a single center, 2011-2017. SETTING: Multidisciplinary PICU in a tertiary referral Children's Hospital in Taiwan. PATIENTS: There were 316 deaths in PICU patients. INTERVENTIONS: Palliative care consultation in the PICU service occurred after the 2013 "Hospice Palliative Care Act" revision. MEASUREMENTS AND MAIN RESULTS: In the whole cohort, 22 of 316 patients (7%) were determined as "death by neurologic criteria". There were 94 of 316 patients (30%) who had an event needing cardiopulmonary resuscitation within 24 hours of death: 17 of these patients (17/94; 18%) died after failed cardiopulmonary resuscitation without a do-not-resuscitate order, and the other 77 of 94 patients (82%) had a do-not-resuscitate order after cardiopulmonary resuscitation. Overall, there were 200 of 316 patients (63%) who had a do-not-resuscitate order and were entered into the palliative program: 169 of 200 (85%) died after life-sustaining treatment was limited, and the other 31 of 200 (15%) died after life-sustaining treatment was withdrawn. From 2011 to 2017, the time-trend in end-of-life care showed the following associations: 1) a decrease in PICU mortality utilization rate, from 22% to 7% (p < 0.001); 2) a decrease in use of catecholamine infusions after do-not-resuscitate consent, from 87% to 47% (p = 0.001), in patients having limitation in life-sustaining treatment; and 3) an increase in withdrawal of life-sustaining treatment, from 4% to 31% (p < 0.001). CONCLUSIONS: In our practice in a single PICU-center in Taiwan, we have seen that the integration of a palliative care consultation service, developed after the revision of a national "Palliative Care Act," was associated with increased willingness to accept withdrawal of life-sustaining treatment and a lowered PICU care intensity at the end-of-life.

Vallecular cyst with coexisting laryngomalacia: Successful diagnosis and laser therapy by flexible endoscopy with a novel noninvasive ventilation support in infants.

OBJECTIVE: Vallecular cyst coexisting with laryngomalacia (VC-LM) can cause significant pharyngolaryngeal obstruction. Traditionally, it is diagnosed with flexible endoscopy (FE) and treated by rigid endoscopy. This study evaluates the effectiveness of solely using FE with novel noninvasive ventilation (NIV) of sustained pharyngeal inflation (SPI) support for both diagnosis and treatment in such infants. METHODS: A retrospective review of consecutive infants who were diagnosed and treated for VC-LM in the 12-year period, 2007 to 2018, was conducted. Clinical variables, techniques, and outcomes were analyzed and reported. RESULTS: Eighteen infants (10 males) were included. The mean age was 3.0 ± 0.6 months and the mean body weight was 4.6 ± 1.3 kg. Before FE, 14 infants were supported with bi-nasal prongs NIV (BN-NIV) and four infants with tracheal intubation. During diagnostic and therapeutic FE, all infants supported with a nasopharyngeal NIV (NP-NIV) only. All diagnoses were made in the first FE inspection of 3.5 ± 1.2 minutes. Thirteen lesions were immediately treated with FE laser therapy in 18.1 ± 1.7 minutes in the same FE course. Total FE time was 24.6 ± 2.8 minutes. Three infants needed revision laser therapy 4 days later. There was no desaturation (<90%), bradycardia (<100/min), or pneumothorax. After FE therapy, all infants were supported with BN-NIV only with significantly (<0.01) lower pressure and completely weaned off before being discharged 8.4 ± 1.5 days later. All infants, followed up for a 6-month period, showed many clinical improvements. CONCLUSIONS: FE, with this NP-NIV and SPI supports, could offer accurate diagnosis and successful laser therapy of the VC-LM with procedural sedation in the same session in infants.

Clinical manifestation of pediatric mediastinal tumors, a single center experience.

Medical decisions should be well-planned to improve prognosis and reduce complications of mediastinal tumors. In this study, we analyzed the clinical presentations of pediatric mediastinal tumors and their correlation with long-term clinical outcome.Forty patients under 18 years of age diagnosed with mediastinal tumors at China Medical University Children's Hospital between 2001 and 2016 were enrolled. The patients' sex, age of onset, initial clinical symptoms, and treatment outcomes were analyzed.75% of the patients with mediastinal tumors in this study were men, and the median age of onset was 13 years old (age range: 0-17 years). The overall mortality rate was 40%. The most common tumors were lymphoma (47.5%), followed by germ cell tumors (12.5%), neuroblastoma (12.5%), and thymoma (7.5%). Neuroblastoma was more prevalent in girls younger than 5 years old. The initial presentations of these patients included breathing difficulty (65%), productive cough (47.5%), pleural effusion (54.5%), superior vena cava (SVC) syndrome (35%), neck mass (35%), airway compression (32.5%), fever (30%), chest pain (27.5%), and pericardial effusion (25%). Lymphomas were more likely to be accompanied by neck mass (52.6% vs19.0%, P = .04) and SVC syndrome (52.6% vs 19.0%, P = .026), yet also had a better 1-year-survival rate (68.4% vs 52.4%, P = .02).Overall, lymphoma should be suspected when children present with neck mass and SVC syndrome. Neuroblastoma with a posterior mediastinal origin should be suspected among children younger than 5 years old. Tumor-related airway obstruction, pleural effusion, and pericardial effusion were leading cause of cardiopulmonary instability during sedation for invasive procedures, which should be managed cautiously.

Esophageal duplication cyst presenting with stridor in a child with congenital pulmonary airway malformation: A case report and literature review.

RATIONALE: Esophageal duplication cyst (EDC) is a rare developmental aberration originated from the embryonic foregut. It may remain asymptomatic but produce local mass effect on surrounding organs if rapid enlarges. EDC may sometimes accompany with other congenital malformations. Congenital pulmonary airway malformation (CPAM) is a congenital lung malformation with an unknown chance of developing symptoms. Here we report a rare case of esophageal duplication cyst with type 2 congenital pulmonary airway malformation (CPAM). PATIENT CONCERNS: A 16-month old boy with a prenatal diagnosis of type 2 CPAM presented progressive stridor and respiratory distress and was admitted to our hospital under the diagnosis of pneumonia. The patient responded poorly to antibiotics. A chest Xray (CXR) showed consolidation over the left upper lobe with trachea deviated to right side. A chest computed tomography (CT) revealed a cystic lesion sized 3.3 × 3.3 cm in the superior mediastinum. DIAGNOSES: Post-operative pathological report confirmed the diagnosis of esophageal duplication cyst. INTERVENTIONS: We pre-medicated the patient with steroids and inhaled bronchodilators for airway maintenance. Then the patient received tumor resection via median sternotomy. OUTCOMES: The patient recovered without complication and discharged smoothly 4 days after the surgery. LESSONS: EDC is a rare but potentially life-threatening disease owning to compression of large airways. Chest CT scan could detect the lesion non-invasively and should be considered in patients with persistent stridor, as well as CXR findings of the trachea deviated by a mass lesion in mediastinum, especially for those with CPAM.

Chromosomal Microarray Analysis in Taiwanese Patients with Williams-Beuren Syndrome.

Williams-Beuren Syndrome (WBS; OMIM #194050) is a rare neurodevelopmental disorder that results from a deletion at 7q11.23 spanning 25-27 genes. We performed chromosomal microarray analysis (CMA) in 9 Taiwanese patients with WBS to confirm the diagnosis. These samples had already been examined by FISH and diagnosed as WBS. Pathogenic copy number variations (CNVs) were identified in all patients, including 24 genes (spanning from FKBP6 to GTF2I) with typical 7q11.23 microdeletion. A deletion in TRIM50 was common in Taiwanese patients with WBS (8/9). Furthermore, 1 patient had 2 additional gene deletions in NCF1 and GTF2IRD2. We also found 4 patients with duplications of 4p16.1, 16p13.11, 10q26.3, and 21q22.3. All 9 WBS patients exhibited distinctive facial dysmorphisms, including a wide mouth, thick prominent lips, short nose with anteverted nares, and periorbital puffiness. However, cardiac defects were not frequent in our patients (3/9). In conclusion, we detected CNVs associated with WBS in a Taiwanese population using CMA. Although CMA is expensive and labor-intensive, it is useful for identifying typical/atypical CNVs, delineating distal break points, and detecting other CNVs.