Neonatal Manifestations of Chronic Granulomatous Disease: MAS/HLH and Necrotizing Pneumonia as Unusual Phenotypes and Review of the Literature.

Chronic granulomatous disease (CGD) is a rare inborn error of immunity (IEI), characterized by a deficient phagocyte killing due to the inability of NADPH oxidase to produce reactive oxygen species in the phagosome. Patients with CGD suffer from severe and recurrent infections and chronic inflammatory disorders. Onset of CGD has been rarely reported in neonates and only as single case reports or small case series. We report here the cases of three newborns from two different kindreds, presenting with novel infectious and inflammatory phenotypes associated with CGD. A girl with CYBA deficiency presented with necrotizing pneumonia, requiring a prolonged antibiotic treatment and resulting in fibrotic pulmonary changes. From the second kindred, the first of two brothers developed a fatal Burkholderia multivorans sepsis and died at 24 days of life. His younger brother had a diagnosis of CYBB deficiency and presented with Macrophage Activation Syndrome/Hemophagocytic Lympho-Histiocytosis (MAS/HLH) without any infection, that could be controlled with steroids. We further report the findings of a review of the literature and show that the spectrum of microorganisms causing infections in neonates with CGD is similar to that of older patients, but the clinical manifestations are more diverse, especially those related to the inflammatory syndromes. Our findings extend the spectrum of the clinical presentation of CGD to include unusual neonatal phenotypes. The recognition of the very early, potentially life-threatening manifestations of CGD is crucial for a prompt diagnosis, improvement of survival and reduction of the risk of long-term sequelae.

Septicaemia with deep venous thrombosis and necrotising pneumonia caused by acute community-acquired methicillin-resistant Staphylococcus aureus in an infant with a three-year follow-up: a case report.

BACKGROUND: Community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) is a common pathogen that usually causes bacteraemia, osteomyelitis, as well as skin and soft tissue infections. However, deep venous thrombosis (DVT) and necrotising pneumonia are rare in infants. CASE PRESENTATION: We report the case of a one-month-five-day-old girl who was hospitalised for DVT and necrotising pneumonia due to septicaemia associated with Staphylococcus aureus. She recovered after treatment with intravenous antibiotics and multiple anticoagulant therapy, but DVT persisted at the three-year follow-up. Collateral circulation around the DVT was well-formed. Post thrombotic syndrome was not observed. CONCLUSIONS: Staphylococcus aureus complicated by DVT and necrotising pneumonia is rare and can be successfully treated.

Retrospective analysis of necrotizing pneumonia in children between 2015-2019.

INTRODUCTION: An increased incidence rate of cases of complicated pneumonia, reaching up to the stage of necrotizing pneumonia was observed at University Hospital Brno in the past period. The aim of this study was to perform a single-center retrospective analysis of patients with acquired inflammatory lung disease requiring surgical treatment, comprising a long-term follow-up group. METHODS: Patients hospitalized for community-acquired pneumonia and surgically treated in the years 2015-2019 were analyzed. The rates of necessary chest drainages, decortications and lung resections in relation to the whole group and individual years were monitored. Clinical and X-ray examinations were performed one year after hospitalization and the prognosis was determined for individual types of required treatments. The age, gender and etiological agents were also monitored. RESULTS: A total of 688 patients were included in the study with the incidence rising until 2018 and decreasing slightly in 2019. A statistically significantly higher number of community-acquired pneumonias and complications was recorded between 2017 and 2018 (p.

Getting the wind knocked out of him - Report of a broncho-cutaneous fistula caused by staphylococcal pneumonia, and review of literature.

A broncho-cutaneous fistula (BCF) refers to the formation of an abnormal fistulous connection between the tracheobronchial tree and the cutaneous surface of skin. A rare occurrence in and of itself, the disease entity may have varied etiologies, and may or may not be associated with a broncho-pleural fistula. We describe a case of a young patient who developed a BCF as a complication of a necrotizing pneumonic process, and his subsequent clinical course. In so doing, we review the clinical features of this peculiar disease entity, analyzing the available medical literature similarities in etiology and variations in management strategies described in the literature thus far.

Prolonged extracorporeal membrane oxygenation for pediatric necrotizing pneumonia due to Streptococcus pneumonia and influenza H1N1 co-infection: how long should we wait for native lung recovery?

Most children with severe respiratory failure require extracorporeal membrane oxygenation (ECMO) for 7-10 days. However, some may need prolonged duration ECMO (> 14 days). To date, no consensus exists on how long to wait for native lung recovery. Here we report the case of a 3-year-old boy who developed severe necrotizing pneumonia requiring venovenous (VV) ECMO after 19 days of mechanical ventilation. In the first 4 weeks of his ECMO run, he showed no lung aeration, requiring total extracorporeal support. However, after we started strategies for promoting lung recovery such as daily prone positioning and regular use of toilet bronchoscopy and inhalative DNAse to clear secretions, by week five his tidal volumes gradually increased and he was successfully decannulated after 43 days. Moreover, we decided not to proceed to a surgical removal of the necrotic lung area. At present, he is 1-year post discharge and has fully recovered. This report shows that unexpected native lung recovery is possible even after prolonged loss of lung function and that a previous healthy lung can recover from apparent irreversible lung injury.

Successful rescue combination of extracorporeal membrane oxygenation, high-frequency oscillatory ventilation and prone positioning for the management of severe methicillin-resistant Staphylococcus aureus pneumonia complicated by pneumothorax: a case report and literature review.

BACKGROUND: To describe the experience of combination therapy with extracorporeal membrane oxygenation(ECMO), high-frequency oscillatory ventilation(HFOV) and prone positioning in treating severe respiratory failure caused by community acquired methicillin resistant Staphylococcus aureus(CA-MRSA). CASE PRESENTATION: A 30-year-old female presented with fever and dyspnea for 3 days. She was diagnosed CA-MRSA pneumonia complicated by severe respiratory failure, pneumothorax and neutropenia. Venovenous ECMO was applied within 8 h of the pneumothorax diagnosis. For amelioration of ventilator-induced lung injury, HFOV and prone positioning were combined with ECMO. The patient's condition improved considerably. ECMO was weaned on day 19, and she was discharged on day 48 with good lung recovery. CONCLUSIONS: To the best of our knowledge, this was the first case in which ECMO was combined with HFOV and prone positioning to treat severe necrotic CA-MRSA pneumonia complicated with pneumothorax. This combination therapy may provide safe respiratory support, may minimize the risk of barotrauma, and provide better drainage of secretions in patients with necrotizing pneumonia.

Non-dysbaric arterial gas embolism associated with chronic necrotizing pneumonia, bullae and coughing: a case report.

Arterial gas embolism (AGE) can be clinically devastating, and is most often associated with exposure to changes in ambient pressure, medical procedure or congenital malformation. Here we report a case of AGE in a 78-year-old male without these traditional risk factors. Rather, the patient's history included chronic obstructive pulmonary disease, necrotizing pneumonia, bullous disease and coughing. He was safely treated with hyperbaric oxygen (HBO2) therapy for AGE, with initial clinical improvement, but ultimately died from his underlying condition. Pathophysiology is discussed. This case illustrates the possibility that AGE can occur due to rupture of lung tissue in the absence of traditional risk factors. HBO2 therapy should be considered in the management of such patients.

Group B streptococcal necrotizing pneumonia in a diabetic adult patient.

The aim of this report is to describe a rare case of necrotizing pneumonia due to group B Streptococcus serotype III in a relatively young male adult (48 years old) suffering from diabetes. The organism was isolated from his pleural fluid and was only resistant to tetracycline. The patient first received ceftazidime (2g/8h i.v.)+clindamycin (300mg/8h) for 18 days and then he was discharged home and orally treated with amoxicillin clavulanic acid (1g/12h) for 23 days with an uneventful evolution. As in the cases of invasive infection by Streptococcus pyogenes, clindamycin could prevent streptococcal toxic shock syndrome.

Community Acquired Staphylococcus Aureus Necrotizing Pneumonia and Guillain Barre Syndrome: An Unusual Presentation in An Adolescent Patient.

Community-acquired Staphylococcus aureus (SA) pneumonia can present with multiple complications but has not been reported earlier to present as or lead to Guillain Barre syndrome (GBS). However, there are few case reports of GBS following SA infective endocarditis, polymyositis, and meningitis. We report an unusual presentation of GBS most probably secondary to community-acquired SA necrotizing pneumonia in a young immunocompetent adult. The clinical course, challenges in the management, and unfortunate death of the patient due to an unforeseen complication have been discussed. This report adds to the clinical knowledge of rare association of community-acquired SA necrotizing pneumonia and GBS.

Surfactant for a Patient with Refractory Pyopneumothorax and Acute Respiratory Distress Syndrome Due to Pneumococcal Necrotizing Pneumonia Complicated by a Bronchopleural Fistula.

Background: Necrotizing pneumonia rarely occurs in children, but when it does it can be complicated by bronchopleural fistula, empyema, pneumothorax, sepsis, and acute respiratory distress syndrome (ARDS). Antimicrobial therapy is the cornerstone of its management; however, surgery is necessary in some cases. Ideally, surgical interventions are kept to a minimum, but this is not always possible if there is a mass effect from air and fluid in the pleural space, pulmonary necrosis leading to massive hemoptysis, uncontrolled sepsis, or difficulties with assisted ventilation. Case Presentation: Herein we present a patient with refractory pyopneumothorax and ARDS due to pneumococcal necrotizing pneumonia complicated by a bronchopleural fistula. The patient's clinical condition deteriorated despite antibiotics, surgical drainage, and assisted ventilation. Owing to pneumothorax with a high percentage of air leakage, bilateral diffuse collapse of the lungs, and insufficient oxygenation, surgical treatment was considered, but because of the patient's lack of tolerance for surgery due to hemodynamic reasons and the complications associated with surgery, medical treatment was determined to be more appropriate. Surfactant treatment was administered to the patient, resulting in significant clinical improvement. Conclusion: To the best of our knowledge, this is the first report of the use of surfactant to treat ARDS due to necrotizing pneumonia. Based on the presented case, we think surfactant can be considered as a salvage treatment for such patients.

A 21-Year-Old Immunocompetent Man With Hemoptysis and Rash.

CASE PRESENTATION: A 21-year-old man presented to the ED of The George Washington University Hospital complaining of chills, shortness of breath, hemoptysis, and a generalized rash. Three days before admission, he noticed a productive cough, severe sore throat, and subjective fever. He also experienced extreme fatigue, generalized sweating, and chest pain with coughing. On the day before admission, he experienced a nonpruritic rash on his neck, palms, and dorsal surfaces of his feet and sputum with streaks of blood. The patient had no significant medical or family history. He had no sick contacts, and his only recent travel was to an outdoor concert in a woody area of the northeastern United States about a month earlier. He did not report recent contact with birds or visits to caves. He is single, lives alone in an apartment, and consumes about 4 alcoholic beverages a week. Occasionally, he smokes cannabis and e-cigarettes. He is sexually active with men, and his last unprotected sexual encounter was a month earlier. He denied photophobia, rhinorrhea, ear pain, nasal congestion, abdominal pain, nausea, vomiting, diarrhea, or dysuria.

Dual RVAD-ECMO Circuits to Treat Cardiogenic Shock and Hypoxemia Due to Necrotizing Lung Infection: A Case Report.

Utilization of venoarterial extracorporeal membrane oxygenation (VA-ECMO) is expanding, but dual VA-ECMO circuits to treat cardiogenic shock with refractory hypoxemia is unreported. We describe the case of combined cardiogenic and distributive shock due to necrotizing pulmonary blastomycosis. After initial central VA-ECMO cannulation, acute respiratory distress syndrome (ARDS) with increasing shunt resulted in significant central hypoxemia due to progressive ventilation-perfusion mismatch. An additional circuit provided complete oxygenation of the high circulating volume. After 4 months on support, he underwent successful heart-lung-kidney transplantation. Dual ECMO circuits are technically feasible and may be advantageous in specific circumstances of high pulmonary shunting resulting in excessive hypoxemia unbalanced with appropriate oxygen delivery.

Successful Right Atrium-Pulmonary Artery ECMO in an Infant With Severe Necrotizing Pneumonia and Bilateral Bronchopleural Fistula.

We report an infant with necrotizing pnuemonia and bilateral broncho pleural fistula, who failed on conventional and high frequency ventilation and was managed successfully on Veno-venous Extra Corporeal Membrane Oxygenator (V-V ECMO) with a unique configuration for 12 days, and weaned off successfully.

Influenza virus infection complicated by bacterial necrotising pneumonia: two case reports.

Necrotising pneumonia (NP) is a potentially severe complication of community-acquired pneumonia characterised by necrosis of consolidated lung tissue. A 7-year-old boy and a 6-year-old boy are presented, both of whom had a complicated influenza infection which evolved into severe NP caused by Streptococcus pneumoniae. Both needed intensive care for invasive respiratory support. Despite extensive pleural involvement in both cases, only one required thoracic surgery. Case 1 also developed anaemia, hyponatraemia and hypo-albuminaemia, resulting in generalised oedema. Despite the severe morbidity, both boys made a full recovery. The diagnosis of NP should always be considered in a child with pneumonia who remains unwell despite 72 hours of appropriate antibiotics, particularly if there is evidence of pleural disease. Although S. pneumoniae is the main agent for NP, the influenza virus may be a precipitating factor.

Giant lung cysts following necrotizing pneumonia: Resolution with conservative treatment.

RATIONALE: Necrotizing pneumonia is characterized by destruction and liquefaction of the lung tissue and loss of the normal pulmonary parenchymal architecture. During the course of resolution areas of hyperlucency are formed, sometimes with the development of giant lung cysts that can be a field with fluid resembling lung abscess. There is no consensus on the management of these abnormalities. OBJECTIVE: To assess the prevalence of giant lung cysts as a complication of necrotizing pneumonia and to report our experience with conservative treatment that achieved complete resolution. METHODS: Medical chart reviews of all children aged 0 to 18 years hospitalized with necrotizing pneumonia in a single tertiary center from 2015 to 2017, demographic data, and clinical course during and after hospitalization as well as serial chest imaging were collected. RESULTS: During the study period, 761 children were diagnosed with community-acquired pneumonia, 16 of 761 (2.3%) had necrotizing pneumonia and 6 of 16 (37.5%) with necrotizing pneumonia complicated by a giant lung cyst or lung abscess. All were closely observed and showed complete clinical and radiographic resolution with antibiotic treatment. CONCLUSIONS: Treatment of giant lung cyst formation following necrotizing pneumonia by a conservative approach with prolonged antibiotics results in complete recovery with no need for invasive procedures.