As the population of ventilator-dependent children (VDC) with tracheostomies due to underlying severe BPD grows, there is an increasing need to shift the care of these children from hospital to home. Transitioning the ventilator-dependent child from the hospital to home is a complex process that requires coordination between the medical team and the family. One crucial step in the process is transitioning from an Intensive care unit (ICU) ventilator to a portable home ventilator (PHV). The Clinical team needs to understand the nuances in transitioning to PHV, including assessing readiness to transition and choosing the optimum settings on an available home ventilator. In recent years, various ventilator modes have been available in PHV that can help achieve synchronous breathing to allow for adequate gas exchange for the infant. This review details some approaches to asses readiness to transition and the process of Transition along with commonly used modes of support available in PHV, as well as the primary and secondary settings in which we should be mindful in supporting a child with chronic respiratory failure in the home setting.

The ancient Greeks took the entertainment of their crowds very seriously and used drama as a way of investigating the world they lived in. The theatrical re-enactment of suffering and grief in front of a large audience was to foster compassion, as well as a cathartic release of emotion that was restorative through a deeply felt interconnection between actors and audience. When our patients encounter tragedy, we are also drawn into their ordeal and experience it first hand. Such it was with Rose.

The diagnosis of primary ciliary dyskinesia (PCD) is made through a combination of clinical features supported by a panel of diagnostic tests. Our cases highlight the similarities in the clinical presentation of patients with the specific immunodeficiency activated phosphatidylinositol 3-kinase delta syndrome 1 (APDS1 or PIK3CD) and PCD. We highlight the importance of repeating nasal nitric oxide testing (nNO) when PCD has not been confirmed by genetic or ciliary electron micrograph (EM) analysis in the setting of an expanded suppurative lung disease differential that includes considerations for immunodeficiency as well as PCD.

Pediatric Pulmonology , LetterElevated serum TARC/CCL17 levels are associated with childhood interstitial lung disease in patients with SFTPC gene mutationYuto Otsubo MD1; Yuji Fujita MD1; Yusuke Ando MD, PhD1; George Imataka MD, PhD1; Shigemi Yoshihara MD, PhD11Department of Pediatrics, Dokkyo Medical University, 880, Kitakobayashi, Mibu, Shimotsuga, Tochigi, 321-0207, JapanCorresponding author:Yuto OtsuboDepartment of Pediatrics, Dokkyo Medical University, 880, Kitakobayashi, Mibu, Shimotsuga, Tochigi, 321-0293, JapanTel: +81-0282-86-1111Fax: +81-0282-86-7152E-mail: [email protected]:Childhood interstitial lung disease; TARC/CCL17; SFTPCRunning head:Childhood ILD and increased TARC/CCL17 levelTo the Editor,Childhood interstitial lung disease (ILD) is a serious and often life-threatening disease that causes interstitial lung lesion formation during childhood. Several causative genes of childhood ILD, such asSFTPC , SFTPB , and ABCA3 , have been identified in some children. The pathogenesis of ILD caused by SFTPC mutations may include the accumulation of surfactant protein C (SP-C) in vesicles, inhibition of pulmonary surfactant reuptake, and decreased secretion of SP-C, but this remains to be confirmed.1Thymus and activation-regulated chemokine/C-C motif chemokine ligand 17 (TARC/CCL17) is a known disease marker of atopic dermatitis. Recently, an association between idiopathic pulmonary fibrosis, a representative disease with pulmonary interstitial lesions, and TARC/CCL17 has been reported,2 but none between childhood ILD and TARC/CCL17.Here we report our experience of a case of childhood ILD in which the patient had an SFTPC mutation and an elevated TARC/CCL17 level at disease onset that decreased as the patient improved with treatment. TARC/CCL17 may be involved in the pathogenesis of ILD in children withSFTPC mutation, which is different from the pathogenesis of atopic dermatitis.An otherwise healthy 15-month-old girl was admitted to our hospital with fever, difficulty breathing, and poor oral intake. Nine days prior to the visit, nasal discharge and cough appeared and gradually worsened; subsequently, poor oral intake appeared. No fine crackles were noted. The patient required supplemental oxygen and was admitted to the hospital. She had no history of respiratory impairment at birth, but she had a family history of ILD in her maternal grandmother. Informed consent was obtained from the patient’s guardians for the publication of this case report.Laboratory tests showed a white blood cell count of 941 × 109/L, neutrophil count of 58%, C-reactive protein level of 0.01 mg/dL, lactate dehydrogenase level of 929 IU/L, Krebs von den Lungen-6 (KL-6) level of 909 U/mL (normal range < 500 U/mL), surfactant protein A level of 2770 ng/mL (normal <43.8 ng/mL), and surfactant protein D level of 319 ng/mL (normal <43 ng/mL), which were suspicious findings for ILD (Table 1). ß-D-glucan level was 7.9 pg/mL (normal <20 pg/mL) and cytomegalovirus antibodies were negative for both IgG and IgM. Chest radiography showed bilateral frosted shadows (Fig. 1), and a chest computed tomography scan showed bilateral diffuse frosted shadows (Fig. 2), leading to ILD diagnosis.Although prednisolone was started on the 2nd day of admission, the patient’s respiratory status did not sufficiently improve, and oxygen supplementation was required. Therefore, methylprednisolone (30 mg/kg/day) was administered twice for 3 consecutive days on days 13-15 and 19-21; however, the respiratory status remained poor. Hydroxychloroquine (10 mg/kg/day) was then started on day 21, and azithromycin (10 mg/kg/day) three times a week was started on day 56, following which the respiratory status gradually improved. On drinking cold water, the patient often coughed and sometimes vomited. When the water was warmed from 4°C to approximately 20°C, coughing and cough-induced vomiting drastically decreased. On day 66 of hospitalization, the patient was discharged with home-based oxygen. Respiratory status, oxygenation, and laboratory data for ILD markers such as KL-6 gradually improved (Table 1).Genetic analysis of SFTPB , SFTPC , ABCA3 ,CSF2RA , and CSF2RB showed SFTPC mutation and p.I73T (c.218T>C), and the ILD was determined to be caused by theSFTPC mutation.Additional examination revealed elevated TARC/CCL17 level at 10,270 pg/mL (normal <998 pg/mL), but IgE (24.2 IU/mL; normal <173 IU/mL) and IL-4 (2.7 pg/mL; normal <6 pg/mL) levels were not elevated in the early stages of treatment. TARC/CCL17 level decreased to 2,122 pg/mL on day 131 after the admission. Granulocyte-macrophage colony-stimulating factor (GM-CSF) level was measured twice on days 11 and 83, and on both, the GM-CSF level was under 5 pg/mL without significant elevation. Anti-GM-CSF antibody (0.3 U/mL; normal <1.7 U/mL) was negative.It has been hypothesized that many of these effector cell populations are recruited by TARC/CCL17 and act profibrogenically, but the details remain largely unknown.3) SFTPC mutations increase the number of abnormal alveolar type 2 epithelial cells (AT2) due to impaired metabolism of SP-C. A knock-in mouse model capable of regulating the expression of an isoleucine-to-threonine substitution at codon 73 (p.I73T) in SFTPC, at the same site as in the present case, showed persistently elevated TARC/CCL17 level in the bronchoalveolar lavage fluid (BALF). Furthermore, the same study also reported that TARC/CCL17 is specifically released by AT2.3 In ILD caused by SFTPC mutations, its pathogenesis involves AT2 hyperplasia. The decrease in TARC/CCL17 level in our patient suggests that either AT2 itself or TARC/CCL17 production from AT2 itself decreased with treatment. In this case, we report, for the first time, elevated serum TARC/CCL17 level in a patient with SFTPC mutation, which decreased with treatment.Our patient showed no symptoms of atopic dermatitis, and she had no skin condition, and no elevation of IgE and IL-4 levels. In the SFTPC p.I73T mouse model mentioned above, no significant level of IL-4 or IL-13 was detected in BALF, and no involvement of the Th2 response was observed.This high TARC/CCL17 level was not considered to be a result of the GM-CSF cascade. TARC/CCL17 is released from macrophages as a product of the GM-CSF cascade.4) GM-CSF is also known to be produced by AT2.5) However, in this case, serum GM-CSF levels were normal and not elevated, both at the beginning of treatment and after improvement.The limitation of this case is that bronchoalveolar lavage was not performed; therefore, the evaluation was based on serum level rather than local lung findings.This case suggests that TARC/CCL17 is involved in ILD pathogenesis. Further elucidation of the chemokine and receptor signaling cascade may lead to the targeting of some stages for therapy, which may be an important issue for future medical treatment. Therefore, elucidation of this pathogenesis is desirable.References1) Beers MF, Mulugeta S. Surfactant protein C biosynthesis and its emerging role in conformational lung disease. Annu Rev Physiol 2005;67:663-696.2) Sivakumar P, Ammar R, Thompson JR, Luo Y, Streltsov D, Porteous M, McCoubrey C, Ill EC, Beers MF, Jarai G, et al. Integrated plasma proteomics and lung transcriptomics reveal novel biomarkers in idiopathic pulmonary fibrosis. Respir Res 2021;22;273:1-13.3) Nureki SI, Tomer Y, Venosa A, Katzen J, Russo SJ, Jamil S, Barrett M, Nguyen V, Kopp M, Mulugeta S, et al. Expression of mutant Sftpc in murine alveolar epithelia drives spontaneous lung fibrosis. J Clin Invest 2018;128:4008-4024.4) Hamilton JA. GM-CSF-dependent inflammatory pathways. Front Immunol 2019;10;2055:1-8.5) Woo YD, Jeong D, Chung DH. Development and functions of alveolar macrophages. Mol Cells 2021;44:292-300.Conflicts of interest:The authors disclose no conflicts.Contributors:YO cared for the patient, conceived the concept of the case report, and drafted the initial manuscript. YF, YA, GI, and SY critically reviewed the manuscript for intellectual content. All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work. All authors have read and approved the final manuscript.Acknowledgment:We would like to thank Editage (www.editage.com) for English language editing.We thank Dr. Goro Koinuma, Division of Pulmonology, National Center for Child Health and Development, Tokyo, Japan, for his invaluable expert opinion regarding the diagnosis and treatment of the patients.Sources of funding:No funding was obtained for this study.

Background: There is considerable variation in recommendations from clinical practice guidelines on when and how to screen for glucose intolerance in people with cystic fibrosis (CF). In terms of glucose tolerance, the Australian Standards of Care state that blood glucose monitoring should be performed to exclude hyperglycaemia on a regular basis. Annual oral glucose tolerance test (OGTT) in patients 10 years or older should be considered and performed in patients with symptoms suggestive of glucose impairment or unexplained weight loss, growth failure or worsening respiratory disease.   In 2018, 13 (14%) of the eligible 99 patients with CF ≥10 years old had an OGTT.  Aims: To evaluate the added benefit of enhanced screening to children and adolescents with CF by improving screening to 50% by the end of 2020.  Methods:  A prospective cross-sectional quality improvement cycles was performed on patients in the CF clinic, ≥11 years for OGTT. Patients were excluded from OGTTs if they were prescribed insulin or prolonged oral/parenteral steroids.  Multiple quality improvement initiatives were tried. Each intervention was done as a plan-do-study-act cycles with refinement between each.  Results:  Screening for CF related diabetes (CFRD) increased in 2020 to 60% of >10 year olds. Four patients had new results suggestive of CFRD and ten had new impaired glucose tolerance.  The most successful strategy was sending letters to families.  Conclusion:  The Australian Standards of Care may require revision, as there may be a benefit of routine screening of all children at certain ages as opposed to waiting for symptoms.

Huiyong Hu1#, Xiaoping Jing2#, Xiuhua Duan3, Leiping Zhou4, Yunfeng Xu1*1 Department of the Ultrasonography, Shanghai Children’s Hospital, Shanghai Jiao Tong University, school of medicine, Shanghai 200040, China;2 Department of Traditional Chinese Medicine, Shanghai Children’s Hospital, Shanghai Jiao Tong University, school of medicine, Shanghai 200040, China;3 Department of Radiology, Shanghai Children’s Hospital, Shanghai Jiao Tong University, school of medicine, Shanghai 200040, China;4 Department of Radiology, International Peace Maternity & Child Health Hospital of China welfare institute, Shanghai Jiao Tong University, school of medicine, Shanghai 200030, China;# These authors contributed equally to this work.* Corresponding author: Yunfeng Xu, Department of the Ultrasonography, Shanghai Children’s Hospital, Shanghai Jiao Tong University, school of medicine;Address: 1400 West Beijing Road, Shanghai, China Lane 24 Zip Code 200040;Phone: 18917128478E-mail: xuyunfeng65@163. com (F X).During a prenatal ultrasonography examination late in the second trimester, a fetus was found to have a right diaphragmatic hernia (Figure S1). Multidepartment dynamic monitoring was instituted, and the fetus was later successfully delivered by cesarean section after fetal distress became evident. After intubation, the infant was stabilized and transferred to the Department of Neonatology at our hospital.The enhanced computed tomography of the chest and stomach displayed multiple air-filled intestinal shadows in the right chest cavity, the widest being about 20.0 mm. The right lung, mediastinum, and heart were compressed and displaced, and most of the lung tissue in the right lung was consolidated. Atelectasis is evident in the irregular enhancement shadow at the right upper abdomen, about 43.5 × 32.0 mm in size. The boundary between some sections and the posterior margin of the right lobe of the liver was unclear, but the blood supply (hepatic artery and portal vein branches) was visible (Figure). Blood gases, routine bloodwork, liver and kidney function, and myocardial enzymes were essentially normal.At 40 + 4 weeks, with the infant under total anesthesia, hernia repair was performed. The liver and intestines in the thoracic cavity were brought back into the abdominal cavity; the tissues around the hernia ring in the diaphragm were carefully dissociated; and patch repair and suturing were performed (Figures S2–S4). After the operation, the infant’s vital signs were stable and their condition remained good during follow-up.Congenital diaphragmatic hernia (CDH) is a potentially fatal birth defect[1-3]. In China today, all pregnant women undergo ultrasonography to uncover pregnancy- related conditions[4]. A “green channel” – that is, a multidepartment collaborative for the emergency treatment of perioperative pulmonary hypertension, pulmonary dysplasia, and other complications in newborns with CDH – has been established, helping to assure the best prognosis for those infants.

Objective: This retrospective observational cohort study aimed to assess the real-life application of bronchial challenge test (BCT) in the management of preschool children presenting with atypical recurrent respiratory symptoms (ARRS). Methods: We included children, aged 0.5-6 years referred to a pediatric-pulmonology clinic, who underwent BCT using methacholine or adenosine between 2012-2018 due to ARRS including uncertain severity of airway hyperactivity. BCT was considered positive based on spirometry results and/or wheezing, desaturation, and tachypnea reactions. We collected data on demographics, BCT results, pre-BCT treatment, post-BCT treatment change and post-BCT symptoms control. The primary outcome measure was the change in treatment post-BCT (step-up or step-down). Secondary outcome included clinical improvement observed 3-6 months after BCT. Results: A total of 228 children (55% males) with a mean age of 4.2±0.6 years underwent BCT (52% adenosine-BCT, 48% methacholine-BCT). Children referred for methacholine were significantly younger compared to adenosine (3.6±1.2 vs. 4.2±1.2 years, P<0.01). Methacholine and adenosine BCTs were positive in 95% and 61%, respectively. Overall, changes in management were observed in 122(53.5%) children following BCT, with 83(36.4%) being stepped up and 37(17%) being stepped down. Significantly more children in the methacholine group were stepped up compared to the adenosine (46% vs 28%, p=0.004). During the follow-up assessment, we observed a clinical improvement in 119/162 (73.4%) of the children. Conclusion: This study demonstrates the importance of BCT in the management of preschool children presenting to pediatric pulmonary-units with ARRS. The change in treatment and subsequent clinical improvement observed, highlight the added-value of BCT in this populations.

Editorial: Long-term respiratory complications of OA-TOF: The need for better data and a more specialised multidisciplinary approach

A dynamic case report in which the authors present a multidisciplinary collaboration in a dyspnoic adolescent girl who coughs up blood, has a high temperature and rapidly progresses to respiratory failure. Laboratory findings, imaging results, and their interpretation are shown. Current recommended management is discussed.

Background: Less invasive forms of ventilation have evolved aiming to decrease bronchopulmonary Dysplasia (BPD) morbidity. It is unclear whether changes in ventilation practices have been associated with improvements in respiratory outcomes. Objective: To examine trends of ventilation modes in preterm neonates over the last decade and their impact on BPD. Methods: A retrospective chart review of very low birth weight infants (VLBW) and those born at less than 32 weeks gestation hospitalized during two periods: the years 2012-2013 and 2018-2019. The primary outcome was the prevalence of BPD. Study variables included the mode and duration of ventilation, duration of oxygen need, and perinatal clinical parameters. Results: Four hundred eighty-one infants were enrolled. Between the two study periods, a significant increase was observed in invasive (33% to 47%, p=0.002), and non-invasive ventilation rates (44% to 72%, p<0.001). The average duration of non-invasive ventilation increased significantly (from 9.24 to 14.08 days, p=0.016). The total duration of respiratory support remained unchanged. The overall prevalence of moderate and severe BPD at 36 weeks corrected age remained approximately 40% in preterm infants born at less than 28 weeks gestation. Conclusion: The increasing use of non-invasive ventilation was not accompanied by a reduction in the use of invasive ventilation, nor by a reduced prevalence of BPD. The high prevalence of BPD remains a significant problem in preterm infants born < 28 weeks of age. Other interventions, in addition to less aggressive ventilation, need to be explored.

We submit this manuscript without an abstract because it is a Letter to the Editor.

Background: Inappropriate humidification of inspired gas during mechanical ventilation can impair lung development in extremely low birthweight (ELBW) infants. Humidification depends on multiple factors, such as the heater-humidifier device used, type of ventilation, and environmental factors. Few studies have examined inspired gas humidification in these infants, especially during high-frequency oscillatory ventilation (HFOV). Our objective was to compare humidity during HFOV and intermittent positive pressure ventilation (IPPV), in vitro and in vivo. Methods: In-vitro and in-vivo studies used the same ventilator during both HFOV and IPPV. The bench study used a neonatal test lung and 2 heater-humidifiers with their specific circuits; the in-vivo study prospectively included preterm infants born before 28 weeks of gestation.  Results: On bench testing, mean absolute (AH) and relative (RH) humidity values were significantly lower during HFOV than IPPV (RH = 79.4% ± 8.1% vs 89.0% ± 6.2%, P<0.001). Regardless of the ventilatory mode, mean relative humidity significantly differed between the 2 heater-humidifiers (89.6% ± 6.7% vs 78.7% ± 6.8%, P=0.003). The in-vivo study included 10 neonates (mean ± SD gestational age: 25.7 ± 0.9 weeks and birth weight: 624.4 ± 96.1 g). Mean relative humidity during HFOV was significantly lower than during IPPV (74.6% ± 5.7% vs 83.0 ± 6.7%, P=0.004).  Conclusion: Relative humidity was significantly lower during HFOV than IPPV, both in vitro and in vivo. The type of heater-humidifier also influenced humidification. More systematic measurements of humidity of inspired gas, especially during HFOV, should be considered to optimize humidification and consequently lung protection in ELBW infants.

As our understanding of the uncommon group of disorders called plastic bronchitis has evolved, so too has the classification of these disorders.

Infants born before 32 weeks post-menstrual age (PMA) and receiving respiratory support at 36 weeks PMA are diagnosed with bronchopulmonary dysplasia. This label suggests that their need for supplemental oxygen is primarily due to acquired dysplasia of airways and airspaces, and that the supplemental oxygen (O2) is treating residual parenchymal lung disease. However, current approaches to ventilatory support in the first days of life, including artificial surfactant use and lower ventilating pressures have changed the pathology of chronic lung disease, and emerging evidence suggests that immature ventilatory control may also contribute to the need for supplemental oxygen at 36 weeks PMA. In all newborns, maturation of ventilatory control continues ex utero and is a plastic process. Supplemental O2 mitigates the hypoxemic effects of delayed maturation of ventilatory control, as well as reduces the duration and frequency of periodic breathing events. Prematurity is associated with altered and occasionally aberrant maturation of ventilatory control. Infants born prematurely, with or without a diagnosis of BPD, are more prone to long-lasting effects of dysfunctional ventilatory control. Awareness of the interaction between parenchymal lung disease and delayed maturation of ventilatory control is essential to understanding why a given premature infant requires and is benefitting from supplemental O2 at 36 weeks PMA.

A 16-Year old with Lemierre’s Syndrome and Multiple Septic Pulmonary EmboliChristopher M Oermann, MDDepartment of Pediatrics, Children’s Mercy Kansas City, Kansas City MOCorresponding Author:Christopher M Oermann, MDChildren’s Mercy Kansas City2401 Gillham RoadKansas City, MO 64108816-302-3354 (telephone)816-302-9736 (fax)[email protected] Head: Pulmonary Septic Emboli in Lemierre SnKey Words: Lemierre Syndrome, Septic EmboliWord Count: 1035To the Editor,Lemierre’s syndrome (LS) is a rare disease resulting from infective thrombophlebitis of the internal jugular vein (JV) following oropharyngeal infection. The incidence of LS has increased after decades of decline. Septic pulmonary emboli (PE) are common, but all organ systems can be involved. Although LS is most often caused byFusobacterium necrophorum , other pathogens have been implicated. Persistent high fever and organ-specific signs and symptoms result from the septic emboli (SE). The diagnosis is often suspected following growth of Fusobacterium from blood cultures and is confirmed by head/neck imaging. Therapy includes antimicrobials, drainage of abscesses, and anticoagulation. Significant morbidity/mortality occur if diagnosis and treatment are delayed. Pediatric pulmonologists must be familiar with the diagnosis and management of LS.A previously healthy 16-year old male was admitted to hospital following two weeks of fever, myalgia, nausea/vomiting/diarrhea with 10-pound weight loss, and dry cough. He had been evaluated by his primary care provider (PCP) ten days prior to admission for pharyngitis and fever of 40oC. Rapid streptococcal antigen and SARS-CoV-2 testing were negative, and he was treated with antipyretics. Continued symptoms led to PCP reevaluation after 7 days. Chest imaging demonstrated a single round pneumonia in the right lower lobe, and he received 3 days of azithromycin. He failed to improve and was referred to the emergency department 3 days later. Computed tomography (CT) of the chest/abdomen/pelvis demonstrated bilateral pulmonary nodules. He was admitted for evaluation and treatment.Considerations from the admitting team included atypical infection (fungal or mycobacterial) versus e-cigarette or vaping product use associated lung injury (EVALI), so Pulmonary Medicine service was consulted. EVALI was thought unlikely based on the radiographic appearance of the lesions (Figure 1). Additional considerations included septic PE, paragonimiasis, autoimmune disease with small vessel vasculitis (polyangiitis with granulomatosis), and inflammatory bowel disease. Evaluation for these potential diagnoses was initiated and empiric therapy with vancomycin and ceftriaxone was commenced. Echocardiogram, infectious diseases testing, and autoimmune evaluation were all normal. Painful swelling in the right, anterior neck and a tender spot on his upper right back developed the day after admission. His blood culture grew gram-negative anaerobic rods, raising concern for LS. Doppler ultrasound of the neck demonstrated occlusive thrombus within the right external JV with extension into the internal JV. Metronidazole was added to his intravenous antimicrobial regimen and anticoagulation therapy was initiated.CT Imaging demonstrated normal CNS with confirmation of thrombosis of the JVs and inflammation over the surrounding soft tissues of the anterior right neck and a large abscess involving the posterior spinal musculature of the upper thorax. A drain was placed, and 25 cc of pus removed. Magnetic resonance imaging of the spine demonstrated extension of the abscess into the T1 spinous process suggesting osteomyelitis. The blood culture grew Fusobacterium necrophorum and antibiotic coverage was changed to ampicillin/sulbactam to treat potential polymicrobial infection. Fevers resolved, pain and other symptoms improved, and the drain was removed after 3 days. He was discharged to continue 6 weeks of ampicillin/sulbactam and anticoagulation therapy.LS is a rare complication of infections of the head/neck, with a reported incidence of one per million people per year.1 Common during the “pre-antibiotic era”, LS had decreased in incidence for decades but has increased since the 1990s. This is possibly due to better antimicrobial stewardship and decreased use of antimicrobials for pharyngitis.1Disease is attributed to infections of the tonsils and peritonsillar tissue in 87% of cases and infections of the pharynx, parotid glands, sinuses, mastoids, middle ears, and teeth/gums in 13%.2 Males and females are equally affected.3 LS has been reported in individuals aged 2 months to 78 years, although it is most commonly reported among previously healthy adolescents and young adults.2,3LS originates with primary infection of the head/neck.4 This is followed by spread of the infection through the soft tissues of the neck resulting in thrombophlebitis of the internal JV. Dissemination of infection via SE occurs, with lung involvement in up to 80% and bones/joints in up to 27%.1-4 Additional sites of infection include cardiovascular, skin/muscles, central nervous system, abdominal organs (liver and spleen), and kidneys. Pulmonary involvement has included septic PE, abscess formation, necrotizing pneumonia, empyema, pneumothorax, pulmonary embolism, and acute respiratory distress syndrome. LS is most often caused by Fusobacterium necrophorum , part of the normal flora of the pharynx, accounting for up to 85%. Additional causes include other Fusobacterium species, anaerobes (Bacteroides , Peptococcus , and Peptostreptococcusspecies), and aerobes (Streptococcus , and Staphylococcusspecies), among others. Polymicrobial infections are reported in up to 30%.2 Significant morbidity results from delayed diagnosis. Mortality has been reported as 4-18%; more recent reviews suggest a lower rate.3,5LS often presents 4-12 days after the initiating oropharyngeal infection, which may have resolved by presentation. Symptoms include high fever (up to 80%), gastrointestinal (50%), pharyngitis with cervical adenopathy or neck pain/swelling, myalgia/arthralgia, rigors, and respiratory symptoms (cough or dyspnea).1,2,5Additional symptoms may be secondary to organ dysfunction caused by SE. Diagnosis is often made via Doppler ultrasound or CT imaging of the head/neck or the growth of Fusobacterium from blood/abscess cultures.Therapy for LS involves long term, intravenous antibiotics, surgical drainage of abscesses, and anticoagulation.1-5Metronidazole is often reported as standard therapy for LS. Other antibiotic considerations include carbapenems or penicillin/beta-lactamase inhibitor combinations, which provide broader coverage for polymicrobial infections. Duration of therapy is 4-6 weeks, allowing for adequate penetration of thrombi and treatment of secondary problems such as osteomyelitis. Anticoagulation therapy is more controversial; some data suggest that anticoagulation hastens overall response while others indicate adequate clinical response without additional therapy.LS is a rare disorder of the head/neck that may be associated with significant morbidity/mortality if diagnosis and treatment are delayed. Pediatric pulmonologists will typically encounter LS in patients hospitalized with prolonged fever and multiple septic PE. LS must be considered in every child with multiple cavitary nodules identified on chest imaging. This is particularly true if there is a history of preceding oropharyngeal infection or signs/symptoms suggesting pathology in the head/neck. The growth of Fusobacterium species from blood culture or abscesses should also suggest LS. Doppler ultrasound examination of the neck and CT imaging of the head/neck should be obtained. Therapy is long-term, intravenous antibiotics. Adjuvant therapy includes surgical incision and drainage of abscesses and anticoagulation therapy.

Unperceived button battery ingestion in infants may be associated with significant morbidity, especially when medical assistance is restricted.

A document by Daniel Penela-Sánchez. Click on the document to view its contents.

Asthma deaths were rare in children but attention should be paid to those with a severe exacerbation (requiring systemic steroids, an ED visit or hospitalization) in the past year, more than three beta-agonist refills in a year and poor adherence to controller medication.